- Low Birth Weight Baby
- Neonatal Convulsions
- Neonatal Respiratory Distress
- Neonatal Jaundice
- Neonatal Sepsis or Septicemia
- Neonatal Vomiting
- Neonatal Bleeding
- Neonatal Anemia
- Neonatal Crying
- Neonatal Cutaneous Lesions
Quick examination to detect critical conditions
Special examination for peculiar neonatal problems
Apgar score is a simple and practical method to assess the condition of the newborn immediately after birth. The score assesses 5 variables and each one takes a score of zero, one or two (total score is 10). The score is made initially at 1 minute to discover newborns who are depressed and are in need of resuscitation, then it is repeated at 5, 10, 15 and 20 minutes to evaluate how resuscitation was successful. Low score at 20 minutes is associated with high mortality and serious neurological sequelae.
Response to catheter in nostril
Body pink, limbs blue
• High score (10-8): Good condition. Routine care.
• Moderate score (7-4): Mild to moderate depression. Simple resuscitative measures as suction, oxygen and manual ventilation with bag and mask.
• Low score (3-0): Severe depression. Vigorous resuscitative measures including endotracheal intubation, assisted ventilation and drug therapy.
Beyond the immediate postnatal period, quick examination can be made by assessing the level of consciousness and activity, color (appearance) and vital signs.
- Level of consciousness and activity: Normal newborn is conscious and active and he responds to stimulation by crying or active movements. He has a good suckling power.
- Disturbed consciousness as lethargy or coma indicates a serious cerebral lesion or severe infection. Convulsions and/or increased intracranial pressure (bulging fontanel) may be also present (see neonatal convulsions).
- Poor or absent suckling also denotes a serious illness as intracranial hemorrhage or severe infection. In fact, evaluation of suckling power is the most important single test in examination of the newborn. Good suckling indicates a good general condition, while poor or absent suckling denotes a serious illness.
- Color (appearance): Normal newborn is pinkish in color. Abnormal findings include:
- Pallor: It indicates hypoxia, shock or anemia.
- Central cyanosis: It indicates a serious brain lesion, severe pulmonary disease or congenital cyanotic heart disease. Hematological conditions (as polycythemia) and metabolic conditions (as hypoglycemia) may be also responsible. Clinical differentiation between different causes depends mainly on the level of consciousness and respiratory pattern:
|Diagnostic clues of neonatal cyanosis|
Disturbed consciousness, ± convulsions and increased ICP.
Respiratory depression (slow irregular respiration).
Severe pulmonary disease
Respiratory distress (tachypnea, retractions, grunting).
(with heart failure)
Triad of tachycardia, tachypnea and enlarged tender liver.
Normal respiration and normal consciousness.
(without heart failure)
± Heart murmurs.
Plethora, ± respiratory distress.
Hematocrit value above 65%.
± Disturbed consciousness, heart failure.
Blood sugar below 30 mg/dl.
Responds to I.V. glucose.
CHD: Cyanotic heart disease. ICP: Intracranialpressure
- Central cyanosis with normal consciousness and normal respiration is mostly due to congenital cyanotic heart disease. Cyanosis becomes more intense with crying and it is not relieved with l00% oxygen.
- Central cyanosis with disturbed consciousness and respiratory depression (slow irregular shallow respiration) usually indicates a serious brain lesion as hypoxic ischemic encephalopathy or intracranial hemorrhage.
- Central cyanosis with respiratory distress (rapid respiration, retractions and grunting) is mostly caused by severe pulmonary disease. Congenital cyanotic heart disease with congestive heart failure should be also considered.
- Measurement of hematocrit value and blood sugar level should be a routine to exclude polycythemia and hypoglycemia.
- Plethora: It usually indicates polycythemia, which may lead to respiratory distress and cyanosis. Diagnosis is confirmed by presence of hematocrit value above 65.
- Jaundice: It is a common problem, which may be physiological or pathological. Diagnosis of the cause of jaundice depends on the onset and type of hyperbilirubinemia.
- Vital signs: Normal newborn has a heart rate of about 120-140/minute, respiratory rate of around 40/minute and a temperature of 36.5 – 37.5°C. Abnormalities in vital signs include:
- Tachycardia (heart rate above 180/minute in a quiet baby) may indicate hypoxia or heart failure. Bradycardia (heart rate below 80/minute) usually indicates hypoxia.
- Tachypnea (respiratory rate above 60/minute) is the first sign of respiratory distress and may be caused by pulmonary, cardiac or metabolic conditions (see neonatal respiratory distress). Slow, shallow and irregular breathing (respiratory depression) is usually associated with serious brain lesions as hypoxic ischemic encephalopathy or intracranial hemorrhage. Recurrent apnea (cessation of respiration for more than 20 seconds and is significant when it is associated with bradycardia and/or cyanosis) is common in very low birth weight infants (below 1500 gm) and in several other conditions.
- Fever (rectal temperature above 38.0°C) is caused by high environmental temperature (overheated infant) or true fever (febrile infant). Overheated infant looks healthy, pink with warm hands and feet while febrile infant, on the other hand, looks unwell, pale with cool hands and feet. Serious infections (septicemia, pneumonia and meningitis) and cerebral abnormalities (birth asphyxia, holoprocencephaly and encephalocele) are the main causes of true fever. Hyperpyrexia (rectal temperature above 41.0°C) is invariably caused by overheating. Hypothermia (rectal temperature below 35.5°C) is mainly caused by low environmental temperature (cold exposure). Other causes include severe prematurity, bacterial sepsis, severe heart failure and drugs given to the mother especially sedatives as diazepam. Hypothermia may be mild (35.5° −34.0°C), moderate (34.0 – 30.0°C) or severe (below 30.0°C). Moderate to severe hypothermia is associated lethargy, poor feeding, poor activity and weak cry.
|Causes of neonatal recurrent apnea|
Hypoxic ischemic encephalopathy, intracranial hemorrhage, kernicterus. Drugs.
Hyaline membrane disease, pneumonia, aspiration, pneumothorax. Airway obstruction.
Septicemia, meningitis, necrotizing enterocolitis. Hypoglycemia, hypomagnesemia, hyponatremia, hypernatremia, acidosis, hypothermia, hyperthermia.
Hypovolemia, hypotension, anemia, heart failure.
Oral feeding, esophagitis, intestinal perforation.
Apnea of prematurity
Diagnosis is by exclusion of above conditions, mainly in very low birth weight infants and in those with gestational age below 32 weeks.
Persistent severe hypothermia is serious and it leads to facial edema, sclerema (skin hardening), ileus and even death. Rewarming over few hours using a radiant warmer or an incubator should be carried out.
Thorough and complete examination includes measurements, regional examination and systemic examination.
- Measurements: A normal newborn has approximately a weight of 3 kg, length of 50 cm and head circumference of 35cm. Abnormal measurements include:
- Low birth weight (below 2500 gm) is caused by prematurity, intrauterine growth retardation (small for gestational age) or both conditions. High birth weight (weight above 4000 gm) is usually seen in infants of diabetic mothers.
- Small head is caused by congenital microcephaly, chromosomal anomalies and congenital infections. Large head mostly indicates congenital hydrocephalus.
- Regional examination: The same items mentioned in general history and examination are applied. Relevant abnormal findings include:
- Head and neck: Look for birth trauma (as caput succedaneum or caphalohematoma), bulging anterior fontanel, abnormal features (as Mongolism), eyes (cataract or subconjunctival hemorrhage), ears (malformed or low-set ears), nose (choanal atresia) and mouth (cleft lip or palate and oral moniliasis).
- Limbs: Look for birth trauma (as Erb's palsy or fractures) and for congenital anomalies as talipes equinovarus, polydactyly or syndactyly. Special examination for congenital dislocation of the hip is important (see below).
- Skin: Meconium staining of the skin and cord is an important sign of fetal distress. Skin mottling may indicate poor peripheral perfusion and shock. Generalized edema at birth (hydrops fetalis) is mostly caused by a severe hemolytic disease especially when associated with intense pallor. Sclerema (skin hardening) is an ominous sign, which is associated with very high mortality rate especially in very low birth weight babies who were subjected to cold injury. Skin examination should include a search for lacerations and rashes especially in the napkin area as napkin dermatitis is quite common.
- Back and spine: It is important to look for vertebral defects or swellings as meningocele (covered with skin) or meningomyelocele (covered with thin membrane).
- Genitalia: Examination of genitalia should be a routine step in neonatal examination. Ambiguous genitalia (genitalia in which the sex cannot be determined) is a medical emergency. It may represent an underdeveloped male genitalia or overdeveloped female genitalia. In males, hypospadius and undescended testes should be excluded. Scrotal swellings as congenital hydrocele are common.
- Systemic examination: It includes examination of the main 4 systems (neurological, cardiac, chest and abdominal).
- Neurological examination: After evaluation of the level of consciousness and activity, two other points are left: muscle tone and neonatal reflexes.
- Muscle tone (posture): A normal newborn has a posture of complete flexion (flexion of four limbs). Frog-leg position indicates severe hypotonia.
- Neonatal reflexes: There are 2 types of reflexes: tendon reflexes and primitive reflexes. Tendon reflexes as knee and ankle jerks are normally present. Primitive neonatal reflexes are a group of reflexes peculiar to neonatal period and early infancy. Eliciting these reflexes is important for several reasons:
- Evaluation of the general condition: Normal response indicates a good neurological condition while poor or absent response is associated with general neurological depression as in hypoxic ischemic encephalopathy and intracranial hemorrhage. Suckling reflex and Moro reflex are the most useful reflexes in evaluation of the general condition.
- Evaluation of vision and hearing: Optic blink and acoustic blink are useful in evaluation of vision and hearing of the newborn.
- Detection of focal lesions: Asymmetric response is usually associated with a focal lesion. In Erb's palsy, fracture clavicle or septic arthritis, asymmetric Moro reflex, grasp reflex or withdrawal reflex are usually evident. Also in patients presenting with convulsions and disturbed consciousness, asymmetric response, especially in pupillary reaction to light, is very suggestive of intracranial hemorrhage.
- Assessment of gestational age: The time of appearance of some reflexes can be useful in assessment of gestational age. Glabellar reflex and grasp reflex are particularly important (see low birth weight baby).
- Persistence of some reflexes as Moro reflex and grasp reflex beyond the age of 4 months may indicate cerebral palsy especially when it is associated with hypotonia or spasticity.
|Primitive neonatal reflexes|
1. Cranial nerve reflexes
Sudden exposure of eyes to bright light leads to blinking
Exposure of the eyes to bright light leads to pupillary constriction.
When turning the head slowly to right or left, the eyes do not move with the head.
With light contact to the cheek, the infant turns towards the point of contact.
Tapping of the forehead leads to eye closure.
Clapping the hands near the infant's head leads to motor response (Moro).
On feeding, suckling and swallowing normally occur.
Important for evaluation of vision
Asymmetric response indicates a focal lesion
Disappears at 2 weeks and abnormally appears in comatose patients.
It represents a physiological searching for the nipple.
Important in assessment of gestational age.
Important for evaluation of hearing.
Absent reflexes indicates a serious brain lesion or serious infection.
2. Cutaneous reflexes
If you put your finger in the infant's palm, he grasps it.
On pricking the sole of the foot with a pin, rapid flexion of the hip, knee and foot occurs.
Important in assessment of gestational age and detection of focal lesions.
Important in detection of focal lesions.
3. Extensor reflexes
Sudden noise or sudden drop of the head for few centimeters results in abduction and extension of arms followed by adduction and flexion.
Rotating the neck to one side results in increased tone and partial extension of the arm and leg on the side to which the head is rotated.
Absent reflex indicates a serious brain lesion. Asymmetric response is observed in focal lesions as Erb's palsy.
Same significance of Moro reflex.
4. Progression reflexes
(walking reflex, placing reflex) are of little significance.
- Cardiac examination: After counting the heart rate in quick examination, three other points are important:
- Apex beat: N ormally, the maximal intensity of the apex beat is in the left 4th intercostal space just outside the mid-clavicular line. Apex beat to the right of the sternum indicates dextrocardia or a left sided pathology pushing the heart to right as in pneumothorax or diaphragmatic hernia.
- Cardiac murmurs: More than 90% of murmurs heard at birth are innocent transient murmurs that will disappear over few days or weeks and only less than l0% of murmurs represent a congenital heart disease. It should be noted, on the other hand, that murmurs which are not present at birth and appear later in neonatal period or early infancy are mostly representing a congenital heart disease. Significant persistent murmurs necessitate further evaluation including chest X-ray, ECG and echocardiography.
- Femoral pulsations: Palpation of femoral pulsations should be a routine step. A weak or absent pulsations should suggest coarctation of aorta.
|Clinical presentations of neonatal cardiac disease|
Congenital cyanotic heart disease (after exclusion of other causes) Chest X-ray, ECG and echocardiography are necessary.
Innocent (90%) or congenital heart disease (10%). Persistent significant murmurs necessitate further evaluation.
Congestive heart failure
Congenital cyanotic heart disease.
Congenital noncyanotic heart disease.
Myocardial ischemia. Arrhythmias.
Apex beat to the right
Dextrocardia (isolated or with situs inversus).
Absent femoral pulsations
Coarctation of aorta.
- Chest examination: After counting the respiratory rate in quick examination, three other points are important:
- Rhythm of breathing: A normal newborn has a regular breathing with no apneic spells. Apnea (cessation of respiration for more than 20 seconds with or without cyanosis and bradycardia) is present in prematurity and other several pathological conditions.
- Pattern of breathing: A normal newborn has a breathing of normal rate and adequate chest expansion. Abnormalities include respiratory distress and respiratory depression. In respiratory distress, the respiration is rapid and may be associated with intercostal and subcostal retractions and expiratory grunting. In respiratory depression, respiration is slow and shallow (hypoventilation) and is commonly associated with disturbed consciousness and may be cyanosis (see neonatal respiratory distress).
- Chest auscultation: Thorough examination of the chest is important to differentiate between different causes of respiratory distress.
- Abdominal examination: The abdomen of a normal newborn is slightly distended and lax. The liver may be normally palpated just below the costal margin. Relevant signs on examination are:
- Abdominal distention: Observed in severe septicemia or intestinal obstruction.
- Organomegaly: Hepatomegaly and/or splenomegaly should suggest congenital infections or metabolic disease.
- Retracted or scaphoid abdomen should suggest diaphragmatic hernia.
- Umbilical examination: Umbilical sepsis, umbilical granuloma or hernia, are all common problems that should not be overlooked.
Special attention should be given to peculiar neonatal problems especially prematurity, congenital anomalies and birth injuries.
- Signs of prematurity: A normal newborn has a well developed sole creases, normal genitalia (descended testes in males and prominent labia majora in females), normal breast nodule (0.5 cm in diameter), well-formed cartilaginous ear lobule and coarse silky hair. A premature baby has incomplete sole creases, underdeveloped genitalia (undescended testes in males and prominent labia minora in females), small breast nodule (less than 3 mm), shapeless and pliable ear lobule and fine woolly scalp hair. Assessment of gestational age and degree of low birth weight is important (see low birth weight baby).
- Congenital anomalies: Several anomalies can be recognized at birth or in neonatal period. Anomalies can be superficial and evident (as abnormal features, limb defects or hernias) or hidden (as cardiac or renal anomalies). Clinically, a complete “head-to-toe” examination is important taking into consideration the early detection of life-threatening anomalies. Common anomalies are:
- Head anomalies: Hydrocephalus, microcephaly, encephalocele and abnormal features as Mongolism (see genetics, odd-looking face).
- Limb anomalies: Short limbs, polydactyly, syndactyly or talipes can be easily recognized: Special attention should be given to congenital dislocation of the hip. With the hips and knees flexed at right angles, the thighs are abducted. During this maneuver, a dislocated femoral head will clunk back into the acetabulum (Ortolani maneuver). This palpable clunk can be felt by the middle finger of each hand placed over each great trochanter. Both hips should be examined independently. Limitation of abduction is an early sign of congenital dislocation. Suspicion should be confirmed by an X-ray on hip regions.
- Skin anomalies: Birth marks, naevi and pilonidal sinus.
- Back anomalies: Meningocele and meningomyeloceles.
- Genital anomalies: Undescended testes, hypospadius and hydrocele.
- Hidden anomalies: Congenital heart disease, respiratory anomalies (choanal atresia, tracheoesophageal fistula), gastrointestinal (diaphragmatic hernia, intestinal obstruction) and renal anomalies (congenital hydronephrosis) are the most common.
Life-threatening congenital anomaliesAnomalyClinical presentationBilateral choanal atresiaRespiratory distress at birth (increases with feeding and decreases with crying). Failure to pass a catheter through nostrils.Tracheoesophageal fistulaVomiting, choking and cyanosis on feeding. Failure to pass a nosogastric tube to stomach.Diaphragmatic herniaRespiratory distress and mediastinal shift. Scaphoid abdomen.Intestinal obstructionBile-stained vomiting, abdominal distention. In imperforate anus: Inability to pass a thermometer through the anal canal.MeningomyeloceleBack swelling.Congenital cyanotic heart diseaseCentral cyanosis with or without murmurs Chest X-ray, ECG and echocardiography are necessary.Gastroschisis, omphaloceleIntestinal obstruction.Renal agenesisAnuria, renal failure.
- Birth injuries: They include potentially avoidable mechanical injuries occurring during labor or delivery. Common injuries are:
- Cranial injuries: Caput succedaneum is a diffuse edematous swelling of the soft tissue of the scalp over the presenting part of the head. It may extend over the middle line and may be associated with ecchymotic patches. The edema subsides spontaneously within the first dew days. Cephalohematoma is a subperiosteal hemorrhage that presents as a firm swelling limited to the surface of one cranial bone usually the parietal. The swelling does not appear except after several hours after birth. Anemia and jaundice are the main complications and phototherapy may be needed. It usually subsides gradually over 4-6 weeks. Associated skull fractures or even intracranial hemorrhage should be excluded. The swelling usually subsides spontaneously over 2-8 weeks depending on the size. Incision and drainage is contraindicated as it may lead to serious infection. Subaponeurotic hemorrhage between the scalp aponeurosis and subperiosteum may occasionally follow vacuum deliveries and may lead to hypovolemic shock, anemia and jaundice. Whole blood transfusion may be necessary in severe cases. Erythema, abrasions and bruises are commonly seen in the face or scalp soft tissues following instrumental deliveries.
- Intracranial injuries: Hypoxic-ischemic encephalopathy and intracranial hemorrhage are common serious injuries. Disturbed consciousness, convulsions and hypoventilation are the main clinical presentations (see neonatal convulsions).
- Spinal cord injuries: Forceful longitudinal or lateral traction on the spine may lead to injury of the lower cervical and upper thoracic cord. Paralysis with flaccidity below the lesion occurs with accompanying constipation and urinary retention. Intercostal paralysis and hypoventilation may be associated. In survivors, initial flaccidity is replaced by rigidity after several weeks or months.
- Peripheral nerve injuries: Injury to brachial plexus following traction on the head and neck is common and leads to paralysis of one upper limb. In Erb's palsy (C5, C6),the affected arm is flaccid with forearm pronated and wrist flexed (waiter's tip position). Moro reflex is asymmetric but finger movements and grasp reflex are normal. In Klumpke palsy (C7, C8, T1), the forearm and hand are paralyzed (absent grasp reflex). Associated ipsilateral ptosis and myosis may be associated. In phrenic palsy (C4, C5, C6) paralysis of one upper limb similar to Erb's palsy occurs in addition to a unilateral diaphragmatic paralysis. Cyanosis, irregular labored breathing, diminished breath sounds on the affected side and absent abdominal bulge on inspiration (paradoxical or seesaw movements) are the main findings. Facial palsy (VII) is usually peripheral from forceps pressure and results in absent facial or forehead movements of the affected side during crying. Failure to close the eye in the affected side is evident and it needs protection.
- Fractures: Fracture of clavicle is the most common. The main signs are inability to move the affected limb and absent Moro reflex on the affected side. Crepitus and bony irregularity may be palpated. Callus formation appears within a week and produces visible swelling. The condition should be differentiated from other causes of inability to move one upper limb and asymmetric Moro reflex especially Erb's palsy and fracture of humerus.
- Visceral injuries: Liver injury and formation of subcapsular hematoma may occasionally occur and lead to anemia, jaundice and respiratory distress. The abdomen may appear blue and right upper quadrant mass may be palpated. Splenic injury may also occur alone or in association with liver injury.
Low Birth Weight Baby
Intrauterine growth retardation (IUGR)
Premature baby (50%)
Dysmature baby (45%)
Hypoplastic baby (5%)
Both prematurity and intrauterine growth retardation may coexist.
Low birth weight (weight of 2500 gm or less) is a common condition occurring in about 8% of all births. The incidence is higher in low socio-economic classes where maternal illnesses and nutritional deficiencies are commoner.
- Premature baby is a baby born before 37 weeks of gestations. It accounts for 50% of cases of low birth weight. The degree of prematurity and birth weight depends on the gestational age.
- Dysmature baby (asymmetric IUGR) is a full term baby who appears small for his gestational age. It accounts for about 45% of cases of low birth weight. The main cause of retardation is fetal malnutrition due to placental insufficiency. The baby has an asymmetric retardation and the head is relatively larger.
- Hypoplastic baby (symmetric IUGR) is a low birth weight baby that accounts for about 5% of all cases. The cause of retardation is a serious fetal problem mainly chromosomal abnormalities (trisomies and deletions) and congenital infections. The baby has a symmetric retardation because brain growth is also impaired (i.e. the head is not relatively larger). This type is obviously more serious than asymmetric retardation.
|Comparison between low birth weight babies|
Serious fetal problem
Features of prematurity
Failure to thrive
• What is the degree of low birth weight?
• Is it prematurity, intrauterine growth retardation or both?
• What is the estimated gestational age?
• What are the complications?
What is the degree of low birth weight?
As the mortality rate is largely dependent on birth weight and gestational age, low birth weight is classified into the following 4 grades:
- Low birth weight (LBW): It is a weight between 2500-1500 gm. The cause is either mild prematurity or intrauterine growth retardation. The mortality rate is very low in this group (not more than 5%).
- Very low birth weight (VLBW): It is a weight between 1500-1000 gm. The main cause is moderate prematurity. The mortality rate is significantly higher in this group (more than 20%).
- Extremely low birth weight (ELBW): It is aweight between 1000 – 750gm. It is mainly caused by severe prematurity. The mortality rate is very high in this group (more than 40%).
- Impossibly or incredibly low birth weight (ILBW): It is a weight below 750gm. It is caused by extreme prematurity, and the infant is actually “immature”. The mortality rate in this group is probably exceeding 80%.
Is it prematurity, intrauterine growth retardation or both?
Clinical differentiation between prematurity and intrauterine growth retardation depends on the activity, skin, muscle tone and characteristic physical features:
- Premature baby: The main diagnostic features are:
- Activity: It is proportionate to the gestational age but it is proportionately less when compared to dysmature baby of the same birth weight. Most prematures with very low birth weight (below 1500 gm) have a poor suckling power.
- Skin: The skin of premature baby is reddish in color and has a waxy appearance. It is covered with a fine hair (lanugo). The umbilical cord is normal.
- Muscle tone: Premature baby has a posture of incomplete flexion of the limbs. Frog-leg position and head lag may be evident in severe cases.
- Features of prematurity: The premature baby has incomplete sole creases, under-developed genitalia (undescended testes and small scrotum in males and prominent labia minora in females), small breast nodule (less than 3 mm), underdeveloped ear lobule (shapeless and pliable) and fine woolly scalp hair.
- Dysmature baby (Asymmetric IUGR): The head is relatively larger and:
- Activity: He is generally active and he may be even hyperalert, opening his eyes and looking around with a wizened old man look. He has a good suckling power.
- Skin: The skin of dysmature baby is pale in color. It looks loose and dry with cracking and peeling. The cord is pencil-like and dries rapidly. In severe cases, marked loss of subcutaneous fat and muscle wasting may occur.
- Muscle tone: Dysmature baby has a posture of complete flexion of the limbs.
- Features of maturity: It pure cases of dysmaturity, the physical features of prematurity are absent. Sole creases are complete with deep indentations, genitalia are developed, breast nodule is 5mm, ear lobule is cartilaginous and scalp hair is coarse and silky.
- Hypoplastic baby (Symmetric IUGR): The main difference from dysmature baby is that the head is not relatively larger (symmetric retardation). Other clinical features suggesting chromosomal abnormalities (abnormal features, congenital heart disease) or congenital infection (jaundice, hepatomegaly) may be present. The postnatal growth is slow and failure to thrive in infancy is common.
What is the estimated gestational age?
Gestational age can be estimated postnatally by different ways:
- Obstetric calculation: Gestational age can be calculated from the date of birth and the date of the first day of last menstrual period. This method is inaccurate due to the frequent occurrence of irregular menses.
- Accurate assessment: It is recently made by the “expanded new Ballard score”. The score uses 13 criteria (7 external physical and 6 neurological). The score is refined to include extremely premature infants (see text). The score has replaced the old complicated “Dubowitz score” using 21 criteria.
- Simple rough assessment: Practical and rapid assessment can be made depending on the birth weight and time of appearance of some reflexes. This method is useful in pure prematurity not associated with intrauterine growth retardation.
Time of appearance of reflexes
Pupillary reaction to light
Head turn in prone position
Grasp reflex (you can lift the baby up)
What are the complications?
Several complications are commonly seen especially in prematures with very low and extremely low birth weight. The main complications are:
- Neurological complications: Prematures are at a risk of hypoxic-ischemic encephalopathy and intracranial hemorrhage. Disturbed consciousness, convulsions and increased intracranial pressure (bulging fontanel) are the main findings.
- Respiratory complications: Hyaline membrane disease due to surfactant deficiency is the most common. The incidence and severity of illness is more in those with a shorter gestational age. Recurrent apnea is also common in those with gestational age less than 32 weeks. Bronchopulmonary dysplasia is a common complication in those exposed to high oxygen pressure and prolonged mechanical ventilation and it leads to a prolonged respiratory distress. Other complications as pneumothorax, pulmonary hypoplasia and pulmonary hemorrhage may also occur.
- Metabolic complications: Hypothermia is a common serious complication of low birth weight babies. Hypoglycemia and hypocalcemia are also common and may lead to convulsions. Exaggerated physiological jaundice is a definite risk as kernicterus commonly occurs at a lower bilirubin levels (below 20 mg/dl).
- Infections: Premature babies are immunologically deficient and are susceptible to serious fatal infections. Septicemia, pneumonia and meningitis are the most common. The invading organisms are bacterial, viral, fungal or protozoal (see neonatal infections).
- Nutritional and gastrointestinal complications: Poor suckling is a common feeding problem of very low birth weight babies that usually necessitates nasogastric tube feeding. Gastrointestinal intolerance (as vomiting and abdominal distention) is also common and may require the use of diluted formulas, special low birth weight formulas or expressed mother's milk. Necrotizing enterocolitis is a common serious problem of very low birth weight infants that leads to vomiting, abdominal distention, bleeding per rectum and may be intestinal perforation and peritonitis (see neonatal vomiting).
- Ophthalmologic complications: Oxygen toxicity to the developing retina may lead to retrolental fibroplasia and blindness. Oxygen should be only given in concentrations that relieves hypoxemia and keeps PaO2 between 60-70 mmHg.
- Cutaneous complications: Pitting edema over the hands, feet and legs is a common benign and transient finding of severe prematurity. Sclerema, on the other hand, is a serious fatal complication of severe prematurity especially those exposed to prolonged hypothermia. Other predisposing factors are hypoxia, acidosis and septicemia. It starts as a skin hardening over lower limbs and abdominal wall. It may rapidly extend to involve the chest wall leading to hypoventilation and death.
- Congenital anomalies: The incidence of congenital anomalies is higher in low birth weight infants. Chromosomal disorders and congenital heart diseases are the most common.
Brain damaged convulsions
Hypoxic ischemic encephalopathy
Hypoglycemia (below 30 mg/dl)
Hypocalcemia (below 6 mg/dl)
Hypomagnesemia (below 1.5 mg/dl)
Hyponatremia (below 120 mEq/liter)
Hypernatremia (above 150 mEq/liter)
Other cause (uncommon)
Both conditions may coexist in the same patient
The cause may remain unknown in up to 8% of cases
Convulsions in the newborn is a common potentially serious problem that occurs in about 2% of full-terms and in up to 10% of very low birth weight babies.
Hypoxic-ischemic encephalopathy is by far the commonest cause (50% of cases), followed by intracranial hemorrhage (20%) and neonatal meningitis (10%). Common metabolic causes as hypoglycemia and hypocalcemia occur in about 10% of cases. Most cases of convulsions occur during the first week after birth and particularly during the first few days.
• What are the characters of convulsions?
• Is it brain damaged or metabolic convulsions?
• What is the cause?
What are the characters of convulsions?
Description of convulsions should include the following aspects:
- Type of convulsions: Neonatal convulsions can be classified into 5 types:
- Subtle: It is the commonest type (50%of cases). It may include oral movements (sucking, chewing or yawning), eye movements (repetitive blinking, nystagmus or tonic horizontal deviation), limb movements (bicycling or pedaling) or respiratory movements (irregular breathing or apnea). Apnea due to subtle fits is usually associated with tachycardia or normal heart rate while apnea due to other causes is usually associated with bradycardia.
- Focal clonic: It is a rhythmic twitching of muscle groups especially in the face
- Multi-focal clonic: Several muscle groups are involved simultaneously.
- Tonic: It is a rigid posturing of extremities and trunk (decorticate or decerebrate), which may be focal or generalized and may be associated with clonic movements or apnea.
- Myoclonic: It is a brief focal or generalized jerks of extremities or body mainly in the distal muscle groups.Tonic and myoclonic fits have a poor prognosis as they reflect a diffuse CNS disease or intraventricular hemorrhage. Subtle and clonic fits have a better prognosis.Convulsions should not be confused with jitteriness, which is commonly seen in normal newborns or in association with hypoglycemia, hypocalcemia, hypoxia or in infants of diabetic mothers. Jitteriness is a tremor-like movements that is usually precipitated by sensory stimuli and can be stopped by holding the infant's extremity. It is not associated with abnormal eye movements or EEG abnormalities.
- Onset of convulsions: Convulsions occurring immediately after birth may be due to dilutional hyponatremia or drug-withdrawal. Convulsions during the first day are mostly due to hypoxic-ischemic encephalopathy or metabolic causes as hypoglycemia or hypocalcemia. In intracranial hemorrhage, the onset of convulsions is usually after the first day and in meningitis, it is usually not during the first few days. Convulsions that appear after 3-4 days in an infant who was normal at birth and was fed normally before the onset of convulsions should suggest errors of metabolism. After the first week, convulsions is mostly due to meningitis or late-onset hypocalcemia.
- Duration and frequency of convulsions: Fits may be very transient (lasting for seconds), transient (lasting for few minutes), short (lasting for 5-15 minutes) or prolonged (more than 15 minutes). Fits may be also infrequent (just few fits), frequent (recurring several times per day) or very frequent (recurring every hour or less). Prolonged and very frequent fits have a poor prognosis.
- Response to therapy: Fits can be easily controllable, difficult to control or intractable (not responding to combined anticonvulsant therapy). The prognosis of intractable fits and fits persisting for more than 3 days is generally poor.
Is it brain damaged or metabolic convulsions?
Clinical differentiation depends on 4 clinical criteria; type of convulsions, associated neurological findings, general condition and respiratory pattern.
- In brain damaged convulsions, convulsions are usually tonic and may be clonic also. Other neurologic signs are usually present as disturbed consciousness (lethargy or coma), increased intracranial pressure (bulging fontanel), lateralizing signs (unequal pupils or focal motor weakness) or meningeal irritation (neck retraction and/or arched back). The baby looks sick with poor activity, weak or absent suckling and vomiting. In advanced cases, there is respiratory depression with slow irregular respiration and apneic spells.
- In metabolic convulsions, convulsions are usually clonic and the baby does not look sick except in hypoglycemia and errors of metabolism. There are no other neurological findings and no respiratory depression.
- Both conditions (brain damaged and metabolic) may coexist in the same patient. Hypoglycemia and/or hypocalcemia are commonly present in patients with hypoxic-ischemicencephalopathy and intracranial hemorrhage.
What is the cause?
Although the cause can be suggested in many cases, some investigations are required to confirm the diagnosis.
|Possible investigations of neonatal convulsions|
Blood sugar, calcium, magnesium and sodium: In ALL cases.
Cranial ultrasonography or CT scan: In brain damaged convulsions.
Lumbar puncture and CSF examination: In suspected meningitis.
Sepsis screen (CBC, ESR, CRP, blood culture): In suspected septicemia or meningitis.
Metabolic screen (acid-base balance, ammonia level, aminogram): In suspected errors of
metabolism or in unexplained convulsions.
BRAIN DAMAGED CONVULSIONS
- Hypoxic-ischemic encephalopathy: It is by far the commonest cause (50% of cases). It occurs in infants exposed to perinatal hypoxia, which results in brain edema and encephalopathy. The patient usually presents with low Apgar score and cyanosis at birth. Convulsions usually start within 12 hours after birth and usually last for few days. Other neurological findings and respiratory depression are present with variable degrees according to the severity and the duration of hypoxia. Other manifestations of hypoxia may be also present (see below). Prognosis is generally good in mild to moderate cases. In severe cases characterized by prolonged fits and coma, death or severe neurological sequelae (epilepsy, motor and intellectual deficits) may occur. Cranial ultrasonography or CT scan is important to demonstrate brain edema and to exclude intracranial hemorrhage.
|Clinical staging of hypoxic-ischemic encephalopathy|
Mild (stage 1)
Moderate (stage 2)
Severe (stage 3)
|Multiple organ system effects of hypoxia|
Hypoxic-ischemic encephalopathy, intracranial hemorrhage.
Myocardial ischemia, cardiogenic shock.
Pulmonary hypertension, pulmonary hemorrhage.
Acute renal failure (acute tubular or cortical necrosis). Hypoglycemia, hypocalcemia, hyponatremia.
Disseminated intravascular coagulation (DIC).
Perforation, ulceration, hemorrhage.
- Intracranial hemorrhage: It is the second most common cause of neonatal convulsions (20% of cases). It occurs principally in 3 groups of patients: (1) those with severe prematurity, (2) severe perinatal hypoxia and (3) severe birth trauma to the head. Occasionally, bleeding may follow disseminated intravascular coagulation or congenital vascular anomalies. Bleeding may occur in one or more of the following 5sites (epidural, subdural, subarachnoid, intracerebral and intraventricular). Intraventricular hemorrhage (IVH) is the most serious and most common site especially in prematures and in those with severe hypoxia. In addition to the clinical findings of hypoxic-ischemic encephalopathy, intracranial hemorrhage should be considered in the following clinical situations: (1) onset of signs in the second or third day, (2) persistence of convulsions and other neurological signs for more than a few days, (3) presence of lateralizing signs as unequal pupils and focal paralysis, (4) progressive pallor and fall in hemoglobin level. Cranial ultrasonography or CT scan is important for diagnosis, localization and grading of severity. Prognosis is generally poor and it depends on the site and extent of hemorrhage. Mortality rate is high and neurological sequelae (epilepsy, motor and intellectual deficits) are very common in survivors.
- Neonatal meningitis: Itis the third most common cause of neonatal convulsions (10% of cases). It occurs at any time during the neonatal period but usually not during the first few days. It may accompany cases of neonatal septicemia especially those of late-onset sepsis. It may also follow infected wounds or infected meningo-myelocele. Clinical manifestations start with nonspecific features as poor suckling, vomiting, irritability and fever or hypothermia. The classic signs appear late and include convulsions, disturbed consciousness and increased intracranial pressure (bulging fontanel). Lumbar puncture and CSF examination is important for diagnosis. Other laboratory means for diagnosis of sepsis as CBC, ESR and CRP are also important. Prognosis depends on the time of diagnosis and efficacy of antibiotic therapy.
- Kernicterus: It occurs as a complication of severe neonatal hyperbilirubinemia when indirect serum bilirubin exceeds the critical level (20 mg/dl in full-terms and lower values in low birth weight babies). Untreated Rh disease is the commonest cause. Clinical manifestations usually appear between 3-6 days after birth. In addition to deep jaundice, the baby looks very sick with poor suckling, vomiting and absent Moro reflex. Twitches and convulsions then follow with characteristic spasms and rigidity (opisthotonos). Mortality rate is very high (75%) and the remainders develop cerebral palsy in late infancy and early childhood (hypotonia, choreoathetosis).
- Cerebral anomalies: Rare anomalies as lissencephaly, schizencephaly, holoprocencephaly or adrenoleukodystrophy may be the cause. A neurocutaneous syndrome as tuberus sclerosis may cause intractable convulsions. CT scan of the head or magnetic resonance imaging (MRI) is essential for diagnosis of various anomalies.
- Hypoglycemia: It occurs mainly in low birth weight babies, infants of diabetic mothers, infants of toxemic mothers and in sick neonates (hypothermia, hypoxia or sepsis). Persistent or recurrent hypoglycemia should suggest errors of metabolism as galactosemia, glycogen storage disease or maple syrup urine disease. Clinical manifestations usually appear during the first few days and include jitteriness, convulsions and disturbed consciousness. Episodes of central cyanosis, apneic spells and heart failure may be so evident to a degree suggesting congenital cyanotic heart disease. Unlike other common metabolic causes, the baby looks sick with poor activity and weak or absent suckling. Diagnosis is confirmed by the presence of low blood sugar level (below 30 mg/dl) and disappearance of symptoms with I.V. glucose administration (2-4 ml/kg of glucose l0%).
- Hypocalcemia: It may appear early (in the first few days) or late (after the first week). Early hypocalcemia occurs mainly in premature babies, infants of diabetic mothers and in association with severe perinatal hypoxia. Late hypocalcemia occurs in some infants receiving cow milk due to the high phosphate content. The baby looks generally well in spite of the muscular twitches, jitteriness and convulsions. Diagnosis is confirmed by the presence of low serum calcium level (below 6 mg/dl). Failure of response to I.V calcium gluconate should suggest an associated condition as hypoxic-ischemic encephalopathy or hypomagnesemia.
- Hypomagnesemia: It is less common than hypoglycemia and hypocalcemia. It occurs mainly in low birth weight babies, infants of diabetic mothers and in those receiving intravenous fluids without supplementation of magnesium. It should be also suspected in every case of convulsions with normal glucose and calcium level or in case of tetany not responding to I.V calcium. Diagnosis is confirmed by low serum magnesium level (below 1.5 mg/dl). It responds to I.V.magnesium (1.0 ml/kg of magnesium sulphate 10%). Primary hypomagnesemia is a rare condition characterized by defective intestinal absorption of magnesium. Tetanic spasms starting in neonatal period or early infancy and not responding to I.V. calcium is the main presentation.
- Dilutional hyponatremia: It occurs in infants born to mothers who received a large amount of intravenous hypotonic solutions shortly before birth. It may also occur in infants with renal salt losses, or those receiving hypotonic I.V. fluids. Diagnosis is confirmed by low serum sodium level (below 120 mEq/liter). It responds to I.V. sodium chloride solution 3%.
- Hypernatremia: It may occur with dehydration or iatrogenic due to repeated bicarbonate therapy or hypertonic solutions. Diagnosis is confirmed by a high serum sodium level (above 150 mEq/liter).
- Errors of metabolism: Although uncommon, errors of metabolism should be considered in any infant who is normal at birth and becomes symptomatic after a few days of milk intake. Symptoms include poor feeding, vomiting, lethargy and convulsions. The condition may progress rapidly to deep coma. Three main categories of amino acid disorders should be considered: (1) aminoacidopathies as maple syrup urine disease, phenylketonuria and nonketotic hyperglycemia, (2) organic acidemia, as propionic acidemia and methylmalonic acidemia, (3) urea cycle disorders with hyperammonemia as argininemia, citrullinemia and argininosuccinic acidemia. Laboratory approach for diagnosis should initially include plasma ammonia level and acid-base status:
- Hyperammonemia, without acidosis suggests urea cycle disorders.
- Metabolic acidosis (with or without hyperammonemia) suggests organic acidemia.
- Normal ammonia and pH suggests aminoacidopathies.(Aminogram is necessary for precise diagnosis).
- Drugs: Withdrawal convulsions may occur in infants born to mothers receiving large doses of sedatives or hypnotics. Clinical manifestations also include tremors, poor feeding, yawning and sneezing. Convulsions may also occur with some drugs given to the baby especially theophylline.
- Toxins: Convulsions may occur with any severe infection especially septicemia. Other manifestations suggesting sepsis are usually evident. Tetanus neonatorum occurs due to infection with clostridium tetani organism usually at birth during cutting the cord under poor hygienic conditions. The infection remains localized at the umbilicus producing powerful exotoxins, which are absorbed through motor end plates or lymphatics and produce the characteristic picture. Clinical manifestations appear after an incubation period of about one week and start with trismus (lock jaw) and difficult milk intake. Clonic convulsions and characteristic spasms rapidly appear with stiff extremities, neck retraction and arched back (opisthotonos). Convulsions and spasms are characterized by the following: (1) can be precipitated by the slightest stimulus (hyperexcitability), (2) intermittent early and separated by complete relaxation, (3) spasms are painful as the consciousness is not impaired, (4) may lead to intramuscular hemorrhage, fracture spine and laryngeal spasm and death. Umbilical sepsis is usually evident. Diagnosis is clinical and it depends on the characteristic picture. Lumbar puncture and CSF examination is normal.
Familial neonatal convulsions is an autosomal dominant condition characterized by frequent fits in the first 3 weeks. The prognosis is excellent as the condition rarely persists beyond the neonatal period. Diagnosis depends on exclusion of other causes and the presence of positive family history.
Neonatal Respiratory Distress
Hyaline membrane disease
Transient tachypnea of the newborn
Noncyanotic CHD (as PDA, coarctation)
Persistent fetal circulation
Congenital lobar emphysema
Congestive heart failure
Severe metabolic acidosis
• CHD (congenital heart disease), TGA (transposition of great arteries), TAPVR (total anomalous pulmonary venous return), PDA (patent ductus arteriosus), IDM (infant of diabetic mother).
Respiratory distress is the most common neonatal emergency and the main cause of admission to neonatal intensive care units. Hyaline membrane disease is by far the most common cause (more than 50% of cases) followed by neonatal pneumonia, transient tachypnea and meconium aspiration.
• What is the degree of distress?
• What is the cause of distress?
What is the degree of distress?
Clinical assessment of the degree of distress is important for determination of the severity and course of illness and for choice of the appropriate line of respiratory support.
|Grading of respiratory distress|
Grade I (mild distress): Rapid respiration (above 60/minute) and working alae nasi.
Grade II (moderate distress): Intercostal and subcostal retractions.
Grade III (severe distress): Expiratory grunting.
Grade IV (advanced distress): Central cyanosis and disturbed consciousness.
Clinical evaluation should be combined with blood gas analysis to identify those in respiratory failure (PaO2 below 50 mmHg with or without PaCO2 above 50 mmHg). Persistent hypoxemia in spite of oxygen therapy and/or CO2 retention (PaCO2 above 60 mmHg) are indications for mechanical ventilation.
Respiratory distress should not be confused with respiratory depression as both can lead to cyanosis and respiratory failure.
|Neonatal respiratory failure|
Lung failure (Type I RF) (Respiratory distress)
Pump failure (Type II RF) (Respiratory depression)
CNS narcosis: Drugs, anesthesia
Severe brain insult
Severe pulmonary disease
Tachypnea ± retractions,
Slow irregular respiration with
frequent apnea, cyanosis.
Lung failure (Type I RF)
• Arterial hypoxemia (low PaO2)
± hypoventilation (high PaCO2)
Pump failure (Type II RF)
• hypoventilation (high PaCO2)
± hypoxemia (low PaO2)
• Oxygen therapy
± Assisted ventilation
• Assisted ventilation
± Oxygen therapy
• Acute respiratory failure is always associated with acute respiratory acidosis (low pH, high bicarbonate) or combined respiratory and metabolic acidosis (very low pH, normal bicarbonate).
What is the cause of distress?
In spite of the long list of conditions presenting with respiratory distress, it is usually not difficult to distinguish between the different causes based upon the history, clinical examination and simple investigations. Clinical evaluation should include the mode of delivery (vaginal or C.S.), birth weight, gestational age, color (pallor, plethora or cyanosis) and systemic examination (CNS, chest, heart and abdomen). Initial clinical exclusion of congestive heart failure is important. Chest examination should begin with comparison of the air entry on both sides to detect conditions with unequal air entry as pneumothorax and diaphragmatic hernia.
|Possible investigations of neonatal respiratory distress|
Chest X-ray: in ALL cases. To distinguish between different pulmonary causes.
Blood gases: in ALL cases. To detect metabolic acidosis and respiratory failure.
Hemoglobin level and hematocrit value: To detect anemia and polycythemia.
Sepsis screen (CBC, ESR, CRP): With suspected pneumonia.
Echocardiography: With suspected congenital heart disease.
- Hyaline membrane disease: It is by far the most common cause. It occurs principally in premature babies due to deficient synthesis of surfactant by type II alveolar cells, which results in massive alveolar atelectasis. Other factors as cold injury, anoxia, acidosis and hypovolemia further impair surfactant synthesis. Manifestations of respiratory distress appear at birth or few hours later and increase gradually to reach its peak in the second or third day. Auscultation of the chest reveals bilateral fine consonating crepitations and air entry may be markedly diminished especially in severe cases. Chest X-ray reveals fine granular appearance (ground glass appearance) in mild to moderate cases and complete opacification of both lung fields (white lungs) in severe cases (see Basis Pediatric Radiology). Natural gradual improvement occurs in survivors after the third day over 3-5days. Complications as respiratory failure, apnea, intraventricular hemorrhage and paralytic ileus are common. Mechanical ventilation may be complicated with pneumothorax, interstitial emphysema and bronchopulmonary dysplasia and the course may be prolonged for few weeks.
- Transient tachypnea of the newborn: It mainly occurs in full term babies born by Caesarean section due to delayed clearing of lung fluids. Respiratory distress appears within few hours after birth and is usually mild (just tachypnea). Rapid improvement occurs over 24 hours but tachypnea may remain for 2-3 days. Chest X-ray reveals coarse streaking and fluid in the fissures (wet lung appearance).
- Meconium aspiration syndrome: It mainly occurs in full term and post-term babies subjected to fetal distress with passage of meconium into the amniotic fluid. Aspiration of meconium-stained amniotic fluid usually occurs at birth and results in respiratory distress within few hours after birth. The skin and umbilical cord may be stained with meconium. Pneumothorax and pneumonia are common complications of severe cases. Chest X-ray reveals hyperinflated lungs with bilateral patchy consolidation. Similar disorders may be caused by aspiration of normal amniotic fluid or milk and result in aspiration pneumonia. Predisposing conditions, as tracheoesophageal fistula and gastroesophageal reflux should be considered.
- Neonatal pneumonia: It can be congenital or acquired. Congenital pneumonia is characterized by early onset of respiratory distress within 3-6 hours after birth (early onset pneumonia). The clinical and radiological findings may be very similar to those of hyaline membrane disease or aspiration pneumonia. Helpful differentiating features include temperature instability, apneic spells and acidosis. Skin rash or hepatosplenomegaly may be also present. In acquired pneumonia, the onset of respiratory distress is usually after the first 24 hours or at any time in neonatal period (late onset pneumonia). It commonly follows aspiration, mechanical ventilation or septicemia. The pneumonia may be a bronchopneumonia or lobar pneumonia with massive unilateral consolidation. In case of bronchopneumonia, bilateral fine consonating crepitations is the main finding. With massive consolidation, unilateral dullness and diminished air entry over the involved area are evident. Simple sepsis screen (CBC, ESR, CRP) is useful in suggesting an infection. Definite diagnosis depends on isolation of the organism by blood culture and culture of tracheal aspirate.
- Persistent fetal circulation (PFC): Persistent pulmonary hypertension causes right-to-left shunting through the foramen ovale and ductus arteriosus, which results in severe hypoxemia. It may occur as an idiopathic disorder or secondary to other critical illness as hyaline membrane disease, meconium aspiration, neonatal pneumonia, diaphragmatic hernia or polycythemia. Idiopathic cases are characterized by central cyanosis unresponsive to oxygen therapy and the condition can be easily confused with congenital cyanotic heart disease. Respiratory distress is variable, chest auscultation is unrevealing and cyanosis characteristically becomes more intense with crying. In secondary cases, the manifestations of the primary disease are evident in addition to central cyanosis, which becomes more intense with crying. Diagnosis depends on the presence of arterial oxygen gradient above 20 mmHg between preductal sample (right radial artery) and postductal sample (umbilical artery). Doppler echocardiography can demonstrate the shunt and measure the elevated pulmonary blood pressure.
- Pneumothorax: It may occur spontaneously during the course of illness of a severe pulmonary disease as hyaline membrane disease, meconium aspiration, diaphragmatic hernia or congenital lobar emphysema. More commonly, it is iatrogenic and follows vigorous resuscitative measures or mechanical ventilation. Clinically, sudden deterioration of the condition with cyanosis, pallor or skin mottling should raise the possibility. Chest examination reveals diminished air entry over the involved side with mediastinal shift to the other side. Transillumination of the thorax is often helpful in emergency situations. Urgent chest X-ray is diagnostic and it reveals hypertransradiant hemithorax with absent bronchovascular markings.
- Diaphragmatic hernia: Herniation of abdominal viscera into the thoracic cavity (mostly on the left side through the foramen of Bochdalek) may result in severe respiratory distress at birth or later in neonatal period or infancy. Chest auscultation reveals diminished air entry over the involved side with mediastinal shift to the other side. Respiratory distress in conjunction with scaphoid abdomen should always raise the possibility. Chest X-ray is usually diagnostic where multiple cysts (air-filled bowel) occupying one hemithorax and pushing the mediastinum to the other side are demonstrated (see Basic Pediatric Radiology). Pneumothorax is a common complication.It is important to know that trials of resuscitation with bag and mask may result in worsening of the condition because of the more distention of the herniated viscera.
- Congenital lobar emphysema: It is caused by partial obstruction of a bronchus by external (cyst or aberrant vessel) or internal (plugs, mucosal folds or stenosis) conditions. The left upper lobe is the most commonly involved (50%of cases), followed by the right middle lobe (30%) and right upper lobe (20%). Half the cases are symptomatic in neonatal period but usually not during the first week. Clinical diagnosis is difficult but chest X-ray demonstrates the emphysematous lobe with mediastinal shift to the other side. Bronchoscopy is helpful in demonstrating the obstructed bronchus and in removing mucous plugs. Treatment is surgical.
- Bronchopulmonary dysplasia (BPD): It is a chronic lung disease of progressive alveolar collapse that occurs principally in premature babies treated with mechanical ventilation for severe hyaline membrane disease. Clinically, instead of the natural improvement on the 3rd or 4th day, the condition deteriorates and respiratory distress persists for few or several weeks and the baby becomes ventilator and oxygen dependent. Chest auscultation may reveal fine crepitations and expiratory wheezing. Corpulmonale and congestive heart failure commonly occur. Radiological findings are gradually changing over weeks.
|Radiological stages of bronchopulmonary dysplasia|
Stage I (first week): Ground glass appearance similar to hyaline membrane disease.
Stage II (second week): Generalized opacity and pulmonary plethora.
Stage III (third week): Bilateral multiple small cysts (bubbly lungs).
Stage IV (fourth week): Hyperinflation, widespread stranding, cardiomegaly.
Bronchopulmonary dysplasia should be differentiated from other causes of neonatal chronic lung disease characterized by chronic respiratory distress.
|Causes of neonatal chronic lung disease|
Bronchopulmonary dysplasia: Mainly in ventilated prematures.
Wilson-Mikity syndrome: Mainly in nonventilated very low birth weight babies.
Chronic pulmonary insufficiency of prematurity.
Chronic pneumonia: Chronic bacterial or viral interstitial pneumonia.
Recurrent aspiration: With tracheoesophageal fistula, gastroesophageal reflux.
Congenital lobar emphysema.
Heart failure due to patent ductus arteriosus.
- Other causes: Several other conditions may lead to respiratory distress in the newborn. Bilateral choanal atresia leads to marked distress and cyanosis, which is relieved by crying. Diagnosis is confirmed by failure to pass a nasogastric tube through both nostrils. Pulmonary hypoplasia occurs in association with lung compression as diaphragmatic hernia or with oligohydramnios. Pulmonary atelectasis occurs in extreme prematurity (less than 28 weeks). Massive pulmonary hemorrhage is a form of hemorrhagic pulmonary edema that complicates cases of severe hypoxia, hypothermia, hypoglycemia, pneumonia and coagulation defect as DIC. The onset is usually between the second and fourth day after birth where a red frothy fluid is aspirated from the mouth or from the endotracheal tube in ventilated babies. Chest auscultation reveals widespread crepitations. Cystic adenomatoid malformation is a form of congenital cystic disease of the lung. The multiple cysts may be large, medium-sized or small. The condition should be differentiated from other causes of multiple cysts especially diaphragmatic hernia, bronchopulmonary dysplasia and multiple pneumatoceles of staphylococcal pneumonia. Other lung cysts as bronchogenic cyst and sequestration of the lung are very rare.
- Congestive heart failure: Clinical diagnosis of congestive heart failure depends on the presence of the cardinal triad of 3 T (tachycardia, tachypnea and tender liver). Diagnosis of the cause can be made by clinical evaluation and echocardiography. Presence of central cyanosis and/or significant murmur suggests a congenital heart disease. Hypoxia or shock indicates a myocardial ischemia while manifestations of sepsis indicates a myocarditis.
|Cardinal clinical triad of congestive heart failure|
Tachycardia: Heart rate above 180/minute.
Tachypnea: Respiratory rate above 60/minute.
Tender liver: The liver is enlarged and tender.
- Metabolic acidosis: Clinical diagnosis of metabolic acidosis depends on the presence of deep rapid respiration (acidotic breathing). In more severe cases, disturbed consciousness becomes evident. Clinical suspicion should be confirmed by blood gas analysis where all parameters are low (pH, bicarbonate and PaCO2). The severity of acidosis can be determined by the degree of lowering of pH and serum bicarbonate level.
Normal blood gases in newborn
Grades of metabolic acidosis
7.35 – 7.4
Below 16 mEq/liter
20 – 24 mEq/liter
Below 13 mEq/liter
35 – 40 mmHg
Below 10 mEq/liter
60 – 80 mmHg
Below 7 mEq/liter
• For assessment of acid-base status, venous samples are satisfactory.
The cause of acidosis can be identified by the clinical evaluation (hypoxia, sepsis, shock), evaluation of renal function (renal failure), aminogram (errors of metabolism) and calculation of the anion gap (normal in renal tubular acidosis and high in other causes of metabolic acidosis).
- Severe anemia or polycythemia: Measurement of hemoglobin level and hematocrit value should be a routine in every case of neonatal respiratory distress. Severe acute anemia (hemoglobin below 8 gm/dl) may result from severe hemolysis or severe blood loss. Polycythemia (hematocrit over 65%) may result from delayed cord clamping, maternofetal transfusion or placental insufficiency secondary to intrauterine hypoxia. The most common clinical manifestations of polycythemia are plethora, cyanosis, respiratory distress, lethargy, jaundice and poor suckling.
Early onset jaundice
Late onset and persistent jaundice
Jaundice in the newborn is a very common problem, which can be physiological or pathological. It is clinically useful to classify cases into two groups as the causes; clinical evaluation and investigations are different in both groups. In early onset jaundice, jaundice appears during the first week and usually subsides over one or two weeks. In late onset and persistent jaundice, jaundice either appears after the first week or persists for more than 2-3 weeks.
EARLY ONSET JAUNDICE
In this group, the main 3 causes are acute hemolysis, physiological jaundice and septicemia. Clinical differentiation between these conditions depends on:
- Onset and severity of jaundice.
- Presence or absence of anemia.
- General condition (activity and suckling power).
- Presence or absence of complications especially bilirubin toxicity (kernicterus).Some laboratory investigations are also necessary for accurate diagnosis.
|Possible investigations of early onset jaundice|
Serum bilirubin level (total, unconjugated, conjugated): In ALL cases.
Hemoglobin level, Coombs test, red cell morphology, reticulocytic count: In suspected hemolysis.
G6PD enzyme activity: In hemolysis not due to Rh or ABO incompatibility.
Sepsis screen (CBC, ESR, CRP, blood culture): In suspected septicemia.
- Acute hemolysis: In this condition, jaundice appears at birth or during the first day and it is commonly severe. Serum bilirubin level may rise rapidly to reach serious levels where kernicterus may occur. Anemia is evident clinically and hemoglobin level may reach below 6 gm/dl. The general condition is commonly affected especially with serious bilirubin levels. Kernicterus (see neonatal convulsions)is a real risk and it may occur when serum bilirubin exceeds the critical level, which depends on the birth weight and the condition of the baby. The critical level is lower in those with low birth weight and in sick neonates.
|Critical indirect bilirubin level at which kernicterus may occur|
Doing well baby
Above 2500 gm
2500 – 2000 gm
2000 – 1500 gm
1500 – 1250 gm
Below 1250 gm
• Manifestations of sickness include hypoxia, hypothermia, hypoglycemia, acidosis or infections.
• Some races (East Asians, Mediterraneans) are at a higher risk for developing neonatal indirect hyperbilirubinemia.
The cause of hemolysis can be identified by clinical and laboratory evaluation.
- Rh incompatibility: It is the commonest cause of hemolysis. It occurs in some Rh positive babies born to Rh negative mothers. Hemolysis occurs due to placental passage of maternal antibodies active against the fetal red cells. The first baby is usually not affected as maternal sensitization usually occurs during delivery of the first baby. Jaundice and anemia are usually severe and the baby may be born with the picture of hydrops fetalis (pallor, edema and hepatosplenomegaly). Kernicterus is a common complication, which occurs when serum bilirubin exceeds the critical level. Diagnosis is confirmed by the presence of positive Coombs test, anemia (hemoglobin level below 10 gm/dl), reticulocytosis and unconjugated hyperbilirubinemia. Exchange transfusion is always necessary to keep serum bilirubin below the critical level.
- ABO incompatibility: It is less common than Rh disease. The first baby may be affected. The disease is milder than Rh disease. Jaundice and anemia are not severe. Hydrops fetalis and kernicterus are rare. Coombs test may be negative. Diagnosis is confirmed by the presence of unconjugated hyperbilirubinemia and spherocytosis.
- Glucose 6 phosphate dehydrogenase (G6PD) deficiency: This X-linked disease is occasionally the cause of severe hemolysis and neonatal hyperbilirubinemia. The condition should be suspected in any male newborn with acute hemolysis not due to Rh or ABO incompatibility. Diagnosis is confirmed by the presence of low enzyme activity below 20 unit/1012 RBC (normal activity is 100 – 200 unit/1012 RBC).
- Large cephalohematoma: Hemolysis may occur in a large cephalohematoma and leads to jaundice. It should be excluded in every case of anemia and jaundice.
- Physiological jaundice: It is the commonest cause of jaundice as it occurs in up to 40% of normal newborns and 70% of prematures due to transient immaturity of hepatic conjugation of bilirubin and increased production of bilirubin following breakdown of fetal red cells. Jaundice appears in the second or third day and is usually not severe (less than 12 mg/dl in full terms and 15mg/dl in prematures). It usually subsides within one week of onset. Anemia is absent and the general condition is fair with good activity and suckling power. Kernicterus does not occur, as serum bilirubin does not reach critical levels except in premature babies where kernicterus may occur at lower levels. However, jaundice may be occasionally severe with serum bilirubin level above 15 mg/dl (exaggerated physiological jaundice). The main risk factors leading to exaggerated jaundice are male sex, race, cephalohematoma, polycythemia, breastfeeding and drugs as vitamin K3. Physiological jaundice may be also prolonged and persists for more than 2-3 weeks (see below).
- Neonatal septicemia: Jaundice in septicemia, if present, usually appears between the fourth and seventh day or later and is usually moderate in severity. Anemia, if present, is usually not severe. The most important clinical signs are the markedly affected general condition. The baby is not doing well with lethargy, poor suckling, vomiting, fever or hypothermia. In severe cases, serious complications may occur as septic shock, renal failure and DIC. With clinical suspicion, sepsis screen should be immediately made. It includes blood culture and other simple tests as complete blood count, erythrocyte sedimentation rate and C-reactive protein (CRP). Immediate hospitalization and combined parenteral antibiotic therapy are important.
|Laboratory findings suggestive of neonatal septicemia|
Total white cell count below 5000/mm3or above 30000/mm3.
Band count above 10% or band/total neutrophil ratio above 0.2.
Toxic granulations in neutrophils.
Erythrocyte sedimentation rate (ESR) above 15 mm/first hour.
C-reactive protein (CRP) above 20 mg/liter.
• Positive 2 tests indicate infection in 90% of cases and 3 tests in 97% of cases.
• Recently, elevated serum cytokines (as Interleukin- 6 and interleukin-8) are highly suggestive.
LATE ONSET AND PERSISTENT JAUNDICE
Jaundice appearing after the first week or persisting more than 2 weeks is produced by another group of causes and it may be unconjugated (indirect) or conjugated (direct). Three clinical questions are important:
Is it unconjugated or conjugated jaundice?
In unconjugated jaundjce, the skin color is bright yellow or orange and the stool is of normal color while in conjugated jaundice the skin is greenish yellow or muddy yellow and the stool is commonly pale or clay colored. Accurate differentiation is only made by determination of serum bilirubin level and its type.
What are the associated clinical manifestations?
In unconjugated group, jaundice is usually the only clinical finding. Anemia, if present, may suggest chronic hemolysis or hypothyroidism.
In conjugated group, jaundice is usually associated with hepatomegaly and pale or clay colored stool and the condition is known as “neonatal cholestasis”. Other clinical findings may be present and can suggest the causative disease.
|Possible clinical findings in neonatal cholestasis|
History of severe hemolysis in the first week: Inspissated bile syndrome.
Low birth weight and/or microcephaly: Congenital infections.
Cataract: Galactosemia or congenital rubella.
Congenital heart disease: Congenital rubella or Alagille's syndrome.
Anemia, purpura: Congenital infection.
Hepatosplenomegaly: Congenital infection.
Not doing well (lethargy, poor suckling, vomiting): Neonatal septicemia.
Facial and/or skeletal anomalies: Alagille's syndrome.
What is the cause?
With determination of serum bilirubin level and its type, causes are classified into two groups:
- Prolonged physiological jaundice: Persistence of physiological jaundice for more than 2 – 3 weeks should suggest congenital hypothyroidism and measurement of T4 and TSH or X-ray on knee region for determination of bone age should be made. Other conditions as constipation and pyloric stenosis are also associated with increased enterohepatic circulation and prolongation of physiological jaundice.
- Breast milk jaundice: It occurs insome breast-fed infants due to presence of certain substances (as pregnandiol), which inhibit bilirubin conjugation. Jaundice appears in the seventh day and it gradually increases in severity till it reaches its peak during the third week. There are no other clinical signs and the jaundice may persist for several weeks. A therapeutic withdrawal of breastfeeding for 3 days results in a rapid decline of bilirubin level and on resuming breastfeeding, bilirubin level will not rise to its previous levels.
- Chronic hemolytic anemias: Several types of chronic hemolytic anemias (congenital spherocytosis, sickle cell anemia or thalassemia) may uncommonly present in the neonatal period. Persistent anemia and reticulocytosis should suggest the diagnosis (see hematology). Identification of the causative disease requires study of red cell morphology (spherocytosis) and hemoglobin electrophoresis (sickle cell anemia and thalassemia).
- Crigler-Najjar syndrome: This inherited condition is the main cause of chronic nonhemolytic unconjugated hyperbilirubinemia. It has two types, which differ in mode of inheritance and severity. Type I is autosomal recessive and is caused by complete absence of glucoronyl transferase enzyme activity. Jaundice is severe (bilirubin exceeds 20 mg/dl) and kernicterus may occur. Phenobarbital is not useful and repeated exchange transfusions are necessary. Type II is autosomal dominant and is caused by deficient glucoronyl transferase enzyme activity. Jaundice is mild to moderate (5-15 mg/dl) and kernicterus does not occur. Phenobarbital is useful in lowering serum bilirubin level and exchange transfusion is not needed. Mild type II should not be confused with Gilbert's disease, which is characterized by low grade unconjugated hyperbilirubinemia (below 5 mg/dl) with episodic exacerbations in response to infection. It is a benign condition, which is also inherited as an autosomal dominant disease and caused by an uptake defect of the liver cell membrane.
Conjugated or Mixed Hyperbilirubinemia (Neonatal Cholestasis)
Clinical differentiation between different causes of neonatal cholestasis is impossible and a laboratory approach is always necessary to reach a precise diagnosis.
|Laboratory approach of neonatal cholestasis|
1. Search for treatable conditions
2. Search for congenital infections
3. Search for other metabolic conditions
4. Search for choledochal cyst
5. Differentiation between idiopathic hepatitis and extrahepatic biliary atresia
In spite of multiplicity of the conditions causing neonatal cholestasis, most cases are caused by idiopathic hepatitis and extrahepatic biliary atresia. Early differentiation between these 2 conditions is important because in case of biliary atresia early surgical correction will prevent further hepatic injury and subsequent development of biliary cirrhosis.
- Inspissated bile syndrome: It is a form of transient cholestasis that may follow a severe hemolytic disease (post-hemolytic cholestasis). The severe unconjugated hyperbilirubinemia in the first week is gradually replaced by conjugated or mixed hyperbilirubinemia and pale stool. The condition is benign as liver functions are normal and gradual complete recovery will occur over few or several weeks.
- Neonatal hepatitis: In a small percentage of cases, neonatal hepatitis is caused by a congenital infection. The possibility should be considered in presence of other clinical findings as low birth weight, microcephaly, cataract or congenital heart disease. Fundus examination may reveal chorioretinitis and skull X-ray may show intracranial calcification. TORCH screening is useful for diagnosis and identification of the causative disease. In another small group of cases, hepatitis occurs as a complication of neonatal septicemia. In this case, the baby is sick with lethargy, poor suckling, vomiting, fever or hypothermia. Sepsis screen is essential in every case of cholestasis to exclude septicemia. However, most cases of neonatal hepatitis are of unknown cause (idiopathic or giant cell hepatitis). It is the commonest cause of neonatal cholestasis as it accounts alone for up to 50% of cases. The condition has both sporadic and familial varieties. In sporadic cases, about 70% of patients will recover over several weeks or months. Prognosis of familial cases is worse and recovery rate is as low as 20%. Cases who will not recover will develop chronic liver cell failure and portal hypertension. Radionuclide scanning and liver biopsy are essential for accurate diagnosis and differentiation from other causes.
|Diagnostic investigations of idiopathic neonatal hepatitis|
Conjugated or mixed hyperbilirubinemia.
Markedly elevated serum transaminases ± prolonged prothrormbin time.
Radionuclide scanning (Hida scan): Some excretion of the dye into the intestine occurs.
Liver biopsy: Hepatocellular necrosis, giant cell transformation, inflammatory infiltrate of portal
tracts and bile duct proliferation.
- Extrahepatic biliary atresia: It is the second most common cause of neonatal cholestasis. Jaundice usually appears after the first week and it gradually increases in severity. Pale stool and firm to hard liver are always present. The baby is usually a full term and he does not look sick in spite of the progressive jaundice. Early recognition is important, as early surgical correction will prevent further hepatic damage. Without surgery, biliary cirrhosis and death usually occur during infancy. Radionuclide scanning and liver biopsy are needed for accurate diagnosis.
|Diagnostic investigations of extrahepatic biliary atresia|
Conjugated or mixed hyperbilirubinemia: Conjugated fraction is more than 80%.
Radionuclide scanning (Hida scan): No excretion seen in bowel over 24 hours.
Liver biopsy: Wide portal tracts, distorted angulated bile ducts, periportal fibrosis and some giant cell transformation.
Laparotomy and intraoperative cholangiography may be needed in doubtful cases.
- Intrahepatic biliary hypoplasia: Hypoplasia or paucity of interlobular bile ducts is occasionally the cause of neonatal cholestasis. The condition may occur as an isolated disease (nonsyndromatic intrahepatic biliary hypoplasia) or may be associated with cardiac, facial and skeletal anomalies (syndromatic intrahepatic biliary hypoplasia or Alagille's syndrome). As peripheral pulmonary artery stenosis is the most common cardiac anomaly, the disease is also called arteriohepatic dysplasia. Liver biopsy is essential for diagnosis and it demonstrates paucity of interlobular bile ducts with variable portal fibrosis.
- Metabolic liver disease: It is occasionally the cause of neonatal cholestasis. Galactosemia is the most common and it should be routinely excluded. Tyrosinemia and alpha one antitrypsin deficiency may be also responsible. Rare causes include Gaucher disease, Niemann-Pick disease and cystic fibrosis (see genetics).
- Cholestasis with total parenteral nutrition: It occurs as a complication of parenteral nutrition, which may occur due to high protein or lipid load. The condition is more common with prolonged nutrition for more than few weeks especially in the very low birth weight premature babies. Discontinuation of parenteral nutrition results in gradual recovery.
- Familial recurrent Cholestasis: Recurrent cholestasis may occur with several familial conditions. Dubin-Johnson syndrome is characterized by recurrent cholestasis and dark brown pigment in liver cells. Rotor syndrome is also characterized by recurrent cholestasis but without a pigment in liver cells. Benign recurrent cholestasis is characterized by complete remission between the recurrent episodes of cholestasis. Unlike the above 2 syndromes, the condition is benign and the liver function is not impaired.
Neonatal Sepsis or Septicemia
Early onset sepsis
Late onset and nosocomial sepsis
Main causative organisms
Main risk factors
Main causative organisms
Main risk factors
Prematurity is a risk factor in both groups and it is associated with 5-10 times greater risk of sepsis
Neonatal sepsis or septicemia is a clinical syndrome resulting from a serious infection in the first month of life. It is a common serious problem with mortality rate ranging from 10 to 40%. In early onset disease, the infection is mostly acquired from the mother and clinical manifestations appear during the first three days or up to the 7th day. It is mainly caused by organisms present in the cervix or vaginal canal. In late onset and nasocomial disease, the infection is acquired postnatally from the community or the hospital and clinical manifestations appear after the first three days or the first week. It is mainly caused by other more virulent organisms at Staphylococcus and Pseudomonas. Nonbacterial infections with viruses (herpes simplex, cytomegalovirus) and fungi (Candida albicans) may be the cause of late onset sepsis.
• How to diagnose neonatal sepsis?
• How to confirm the diagnosis?
How to diagnose neonatal sepsis?
It should be emphasized that diagnosis of neonatal sepsis is a clinical diagnosis, which can be confirmed by laboratory investigations.
Clinical suspicion of neonatal sepsis should be considered in presence of the risk factors (see above). Presence of more than one risk factor especially in association with prematurity makes sepsis more likely. It should be also suspected in any mechanically ventilated baby who shows a clinical deterioration or persistent metabolic acidosis.
Early manifestations of neonatal sepsis are usually vague and nonspecific. The condition should be considered in any baby who is not doing well or sick with lethargy, poor suckling, vomiting, fever or hypothermia. In these clinical situations, hospitalization, investigations and immedia te parenteral combined antibiotic therapy are indicated.
Late manifestations or complications of neonatal sepsis are usually related to different systems. Serious focal infections may become evident as CNS (meningitis), respiratory (pneumonia), urinary (pyelonephritis), digestive (hepatitis, necrotizing enterocolitis) or skeletal (septic arthritis). Careful search for all these infections should be made. Septic arthritis of the hip should be considered when the movement of one lower limb is limited or painful and diagnosis can be confirmed by ultrasound. Other serious manifestations or complications should be expected and excluded. Sclerema is particularly fatal.
|Late manifestations or complications of neonatal sepsis|
Serious focal infections: Meningitis, pneumonia, pyelonephritis, hepatitis, septic arthritis.
Septic shock: Cold extremities, skin mottling, poor capillary refill, tachycardia, hypotension.
Septic renal failure: Oliguria, edema, acidotic breathing.
Serious bleeding (DIC): Purpura, bleeding from puncture sites, necrotic skin patches.
Sclerema: Skin hardening starting over limbs and extending to trunk and face (fatal).
How to confirm the diagnosis?
With clinical diagnosis of neonatal sepsis, laboratory investigations are indicated to confirm the diagnosis and to identify the causative organism.
Laboratory confirmation is usually made by simple tests as complete blood count (CBC), erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). Positive 2 tests indicate infection in 90% of cases and 3 tests in 97% of cases.
|Laboratory findings suggestive of neonatal sepsis|
Total white cell count below 5000/mm3 or above 30000/mm3.
Band count above 10% or band/total neutrophil ratio above 0.2.
Toxic granulations in neutrophils.
ESR above 15 mm/first hour.
CRP above 20 mg/liter.
Elevated serum level of cytokines (as interleukin-6 and interleukin-8).
Identification of the causative organism is made by appropriate cultures (blood, urine, CSF and tracheal aspirate). It is important to emphasize that positive cultures are not necessary for diagnosis especially when clinical suspicion is considerable and nonspecific laboratory tests are suggestive.
Vomiting in doing well baby
Vomiting in sick baby
Swallowed maternal blood
Congenital pyloric stenosis
Cow's milk protein intolerance
Serious medical conditions
Any normal neonate or infant may regurgitate or vomit once or even twice a day. This occasional vomiting should not be mistaken with the frequent or persistent vomiting which should be taken with concern.
Clinical evaluation should include the general condition, onset and characters of vomiting and the associated clinical manifestations.
Laboratory evaluation is individualized and depends on the most likely clinical diagnosis.
|Possible investigations of neonatal vomiting|
Barium swallow or radio-opaque catheter into the stomach: Suspected tracheoesophageal fistula.
Plain X-ray abdomen, erect position: Suspected intestinal obstruction.
Cranial ultrasound: Suspected increased intracranial pressure.
Sepsis screen: Suspected neonatal septicemia.
Metabolic screen: Suspected inborn errors of metabolism.
Barium esophagography under screen: Suspected gastroesophageal reflux.
Barium meal: Suspected pyloric stenosis.
VOMITINGIN DOING WELL BABY
In this group, vomiting is the only clinical manifestation and it is never bile-stained. Otherwise, the baby looks well with good activity and suckling power. There is no fever, abdominal distention, disturbed consciousness, convulsions or respiratory distress.
- Amniotic gastritis: Swallowed amniotic fluid may lead to gastric irritation and vomiting during the first few feeds. The vomiting is usually mucoid but it may be blood-streaked. The condition usually subsides during the first day or two.
- Swallowed maternal blood: Bloody vomiting during the first day or two is mostly due to swallowed maternal blood during delivery. The condition is usually associated with bloody stool. The condition is not associated with anemia or bleeding from other sites (see neonatal bleeding).
- Feeding disorders: Overfeeding and/or faulty feeding is the most common cause of neonatal vomiting. In case of overfeeding, vomiting occurs shortly after feeding and may be accompanied with colics and frequent bowel movements. Failure of eructation of swallowed air or vigorous manipulations after feeding may lead to vomiting. Careful dietetic history is very important in every case of neonatal vomiting in doing well babies.
- Gastroesophageal reflux: This common problem of neonates and infants characterized by persistent relaxation of the lower esophageal sphincter and reflux of gastric contents into the esophagus. Other terms as chalasia or hiatal hernia (partial thoracic stomach) may be used. Vomiting usually starts during the first week. It characteristically occurs after feeding when the infant is in horizontal position and after he has been returned to his crib. The duration and effects of vomiting are related to the severity of the condition: In mild cases, the condition may improve over several weeks with simple positioning and medical treatment. In moderate cases, symptoms persist throughout infancy with frequent recurrent aspiration pneumonia. Other causes of recurrent aspiration, as H-type tracheoesophageal fistula and cricopharyngeal incoordination should be considered. In severe or persistent cases, symptoms continue in early childhood may be up to the age of 4 years. In these patients, chronic cough and wheezing may be the main presentation.Radiological diagnosis is made by a barium esophagography under screen. Retrograde filling of the dilated esophagus can be demonstrated. Partial thoracic stomach (hiatal hernia) may be demonstrated.
- Congenital pyloric stenosis: In this condition, vomiting usually starts in the second or third week and it becomes projectile within another week. It occurs shortly after feeding and it contains only gastric contents but never bile-stained. It may be accompanied with constipation, dehydration and weight loss. On examination, the gastric peristaltic waves may be visible immediately after feeding and before vomiting. A pyloric mass may be palpated midway between the umbilicus and right costal margin just lateral to the right rectus. Diagnosis is confirmed by barium meal, which demonstrates the dilated stomach, fine elongated pyloric canal (string sign) and the hypertrophied pylorus (pyloric tumor).
- Cow's milk protein intolerance: It is occasionally the cause of vomiting in neonates and young infants. It should be considered in bottle-fed infants especially when vomiting is associated with colic and diarrhea. Diagnosis is confirmed by the response to a therapeutic trial of withdrawal of cow's milk and prescribing a hypoallergic soy protein milk.
VOMITING IN SICK BABY
In this group, vomiting may be bile-stained and is usually associated with other clinical findings as poor suckling, fever, disturbed consciousness, convulsions or abdominal distention. The baby is sick and usually necessitates hospitalization. Vomiting is caused by either a surgical emergency or a serious medical condition.
- Tracheoesophageal fistula (TEF): It should be suspected at birth when there is unusual drooling from the mouth or with inability to pass a catheter into the stomach. Vomiting occurs with the first feed in a characteristic way: after one or two swallows there is vomiting, coughing, choking and cyanosis. The picture is repeated with every trial of feeding leading finally to aspiration pneumonia. Immediate radiological studies are essential. Radiological diagnosis is made by failure to pass a radio-opaque catheter into the stomach and the catheter is shown coiled in the upper esophageal pouch. There are 5 types of tracheoesophageal fistula (see Basic Pediatric Radiology). The most common type is atresia with lower fistula (85%) followed by atresia without fistula (10%). Other associated anomalies are common including the VATER syndrome (vertebral and vascular defects, tracheoesophageal fistula with esophageal atresia, renal dysplasia and renal defects).
- Congenital intestinal obstruction: Vomiting usually starts in the first day or two in high obstruction and few days later in low obstruction. The vomiting is frequent, copious and bile-stained and is usually associated with abdominal distention and constipation. An urgent X-ray of the abdomen (erect position) is essential for diagnosis. In high obstruction (e.g. duodenal atresia), the double-bubble and double-fluid level can be demonstrated. In low obstruction (e.g. malrotation and volvulus), multiple fluid levels and marked abdominal distention are evident (see Basic Pediatric Radiology). The condition should be differentiated from acquired intestinal obstruction whether mechanical or functional. Intestinal perforation and pneumoperitoneum may occur in neglected cases.
|Causes of congenital intestinal obstruction|
Jejunal or ileal atresia
Malrotation and volvulus
Congenital fibrous band of Ladd
• In meconium ileus, there is no multiple fluid levels but the distended small bowel may be granular appearance or may show tiny bubbles mixed with meconium.
- Acquired intestinal obstruction: Functional obstruction (paralytic ileus) is a common problem which may occur with severe hyaline membrane disease, neonatal septicemia, meconium or mucous plugs and necrotizing enterocolitis. Mechanical obstruction may also occur as in intussusception, strangulated inguinal hernia, mesenteric thrombosis and necrotizing enterocolitis. In acquired obstruction, the onset of vomiting is usually not during the first few days. Necrotizing enterocolitis is a serious disease affecting mainly the sick premature babies in neonatal intensive care units. Predisposing factors include perinatal asphyxia, polycythemia, early feeding, hyperosmolar feeds and umbilical vessel catheterization. The disease starts with increasing gastric aspirates and vomiting which is usually bile-stained. Abdominal distention and bloody diarrhea quickly follow. Intestinal perforation and peritonitis are common complications. Septic and hypovolemic shock may also occur and the baby may collapse or die. Plain X-ray of abdomen shows multiple fluid levels and may be intramural gas (pneumatosis intestinalis), which is characteristic. If perforation occurs, pneumoperitoneum can be demonstrated (see Basic Pediatric Radiology).
Serious Medical Conditions
- Serious infections: Septicemia, pneumonia and meningitis present in early stages with nonspecific manifestations as lethargy, poor suckling, vomiting, fever or hypothermia. The possibility should be considered in any baby who is not doing well and sepsis screen should be made (see neonatal sepsis).
- Increased intracranial pressure: Vomiting with bulging fontanel and other neurological manifestations as disturbed consciousness or convulsions should suggest the condition. Hypoxic ischemic encephalopathy, intracranial hemorrhage and neonatal meningitis are the main causes. Cranial ultrasound and lumbar puncture are important for diagnosis (see neonatal convulsions).
- Inborn errors of metabolism: The possibility should be considered in any newborn with unexplained vomiting. Exclusion of surgical emergencies and other serious medical conditions should be the first step. The baby is usually normal at birth and symptoms appear after a few days of milk intake. Organic acidemia and hyper-ammonemia are the main causes (see neonatal convulsions). Congenital adrenal hyperplasia should be considered in any newborn with severe vomiting leading to weight loss and dehydration. The possibility becomes great when serum electrolytes show severe hyponatremia and hyperkalemia. In females, virilized external genitalia (ambiguous genitalia) is usually evident but in males, the genitalia is normal and the diagnosis is more difficult (see also adrenocortical disorders and intersex).
Bleeding in doing well baby
Bleeding in sick baby
Swallowed maternal blood
Stress gastric ulceration
Hemorrhagic disease of the newborn
Inherited coagulation defects
Disseminated intravascular coagulation
Bleeding in the newborn is a common potentially serious problem. Causes are ranging from a benign transient condition to a serious fatal illness. Initial clinical assessment should include evaluation of the general condition, onset and sites of bleeding and other associated manifestations.
Laboratory evaluation should include platelet count, prothrombin time (PT) and partial thromboplastin time (PTT). It should be remembered that normal values of prothrombin and partial thromboplastin times are higher in premature and fullterms when compared to infants and children.
|Normal values of laboratory screening tests in neonatal bleeding|
PT (in seconds)
PTT (in seconds)
150.000 – 400.000
14 – 22
35 – 55
Full term newborn
150.000 – 400.000
13 – 20
30 – 45
Infants and children
150.000 – 400.000
12 – 14
25 – 35
BLEEDING IN DOING WELL BABY
In this group, bleeding is the main clinical manifestation. Otherwise, the infant is doing well with good activity and suckling power.
- Swallowed maternal blood: Bloody vomiting during the first day is mostly due to maternal blood swallowed during delivery. The condition is usually associated with bloody stool. There is no bleeding from other sites and laboratory screening tests are normal.
- Hemorrhagic disease of the newborn: It is a common condition that occurs in infants who are not given intramuscular vitamin K immediately after delivery. Bleeding occurs due to hepatic immaturity and transient deficiency of vitamin K dependant factors. Bleeding usually starts in the third or fourth day and it may be gastrointestinal (bloody vomiting or bloody stool), from umbilical cord, following circumcision or oozing from puncture sites. Laboratory investigations reveals prolonged prothrombin time and partial thromboplastin time and normal platelet count. The bleeding characteristically responds dramatically to I.V. injection of 5mg vitamin K1(coagulation defects are corrected within few hours).
- Inherited coagulation defects: The condition should be suspected when bleeding occurs in spite of routine vitamin K administration at birth. It should be also suspected when bleeding does not respond to vitamin K injection. Hemophilia A or B, Von Willebrand's disease or deficiency of other factors may be responsible. Coagulation defect depends on the deficient factor (see hematology).
- Congenital and inherited thrombocytopenias: These conditions should be suspected when bleeding in doing well baby is associated with thrombocytopenia. Two conditions are important. Thrombocytopenia absent radius syndrome (TAR) is characterized by bleeding in the first few days of life, thrombocytopenia and associated anomalies (mainly aplasia of the radius and thumb and cardiac or renal anomalies). The main defect is absent megakaryocytes in the bone marrow. Wiskott Aldich syndrome is an X-linked recessive disease characterized by bleeding, thrombocytopenia, cutaneous eczema and immunologic deficiency (mainly Ig M) leading to increased susceptibility to infection. Megakaryocytes in the bone marrow are normal and the low platelet count is due to abnormal platelet formation or release. Significant number of cases may develop lymphoreticular malignancies.
- Neonatal immune thrombocytopenias: These conditions occur due to passage of anti-platelet antibodies through placenta to fetal circulation, leading to platelet destruction. Maternal ITP or SLE may be the cause but more commonly it occurs due to isoimmune thrombocytopenia, which is characterized by presence of fetal antigens causing maternal sensitization and formation of platelet antibodies. The condition occurs in the first pregnancy and recurs in 80% of following pregnancies. Bleeding occurs only during the first few days but thrombocytopenia usually persists for 4 – 8 weeks. Diagnosis is confirmed by demonstration of anti-platelet antibodies. The condition is self-limited and exchange transfusion may be needed.
BLEEDING IN SICK BABY
In this group, bleeding is not the only manifestation. The baby is sick, often hospitalized, with poor activity and poor suckling.
- Stress gastric ulceration: It is a common condition that occurs in sick babies secondary to prolonged hypoxia, shock or septicemia. Intolerance to feeding with coffee ground aspirates and bloody vomiting are the main clinical manifestations. There is no bleeding from other areas and laboratory investigations reveal a normal platelet count and coagulation factors. The condition usually responds to correction of the precipitating factors in addition to simple measures as temporary withholding of feeding, gastric wash with cold saline and administration of antacids.
- Consumptive thrombocytopenia: With severe septicemia, destruction of the circulating platelets may occur and leads to bleeding from gastrointestinal tract or other areas. Clinically, purpura may be present and the baby looks sick. Laboratory investigations reveal a thrombocytopenia without any coagulation defect. Clinical and laboratory evidence of sepsis are usually evident. Congenital infections especially cytomegalovirus may lead to pinpoint purpuric eruption and thrombocytopenia at birth.
- Disseminated intravascular coagulation (DIC): It is aserious disease characterized by consumption of platelets and several coagulation factors in a process of formation of minute intravascular clots. An accompanying severe illness is usually present and the baby appears critically sick. The main precipitating factors are severe hypoxia, shock, acidosis, hypothermia and septicemia. Clinically, in addition to bleeding from puncture sites, purpura and necrotic skin patches are common. Laboratory diagnosis depends on 3 criteria: (i) thrombocytopenia, (ii) Prolonged thrombin, prothrombin and partial thromboplastin times, (iii) Demonstration of fibrin degradation products (FDPs) in the peripheral blood. Prognosis is generally poor as serious internal hemorrhage and intracranial hemorrhage are common complications. The outcome depends mainly on the extension of internal hemorrhage and the control of the original precipitating factors.
- Necrotizing enterocolitis: It is a serious disease affecting primarily the sick premature babies in neonatal intensive care units. Predisposing factors include perinatal asphyxia, polycythemia, early feeding, hyperosmolar feeds and umbilical vessel catheterization. Clinically, bile-stained vomiting, abdominal distention and bloody diarrhea are the main manifestations. Intestinal obstruction (functional or mechanical) occurs and plain X-ray on the abdomen shows multiple air-fluid levels and may be intramural gas (pneumatosis intestinalis). Intestinal perforation and peritonitis are common complications and X-ray of the abdomen shows a pneumoperitoneum. The condition should be differentiated from surgical emergencies causing bloody stool as volvulus and intussusception.
- Surgical emergencies: Volvulus or intussusception usually present with severe vomiting, abdominal distention and bloody stool or passage of blood and mucus from the rectum without fecal matter. Plain X-ray on the abdomen reveals multiple air-fluid levels. In case of intussusception, an abdominal mass may be felt in the right upper quadrant (see also neonatal vomiting and gastrointestinal bleeding).
Diminished RBCs production
Before or during delivery
External blood loss
Internal blood loss
Anemia in the newborn is a common problem that occurs due to several unrelated conditions. Diagnosis is made when hemoglobin level of venous sample is below 14 gm/dl. It is important to know that capillary blood samples can give hemoglobin concentration 1-2 gm/dl higher than simultaneous venous samples. In severe cases, hemoglobin level may reach below 8 gm/dl. Clinical evaluation should include the onset and severity of anemia, evidence of blood loss and associated clinical manifestations.
|Clinical evaluation of neonatal anemia|
Severe anemia with shock and poor perfusion: Acute blood loss.
Severe anemia with jaundice: Acute hemolysis.
Anemia in critically sick baby: Septicemia, DIC.
Hepatosplenomegaly: Chronic hemolysis, congenital infections, congenital leukemia.
Anemia only in doing well baby: Physiological anemia.
Laboratory evaluation should include hemoglobin level, CBC, reticulocytic count, bilirubin level and Coombs test. Sepsis screen or screening for congenital infection may be done when clinical suspicion is strong. Bone marrow examination may rarely be needed. Skeletal survey may reveal osteopetrosis in unexplained cases.
Physiological anemia of infancy is characterized by progressive decline in hemoglobin level starting from the first week and persisting for about 8-10 weeks. The hemoglobin level, which is normally high at birth (16-18 gm/dl), gradually decreases and may reach to 10 gm/dl in full terms and 7 – 9 gm/dl in prematures. The condition is due to physiological absence of erythropoietin during this period. Folic acid or vitamin E deficiency may aggravate the anemia especially in prematures.
Newborns and young infants cry for several reasons. It is clinically useful to classify causes into 3 groups:
- Normal or physiological crying: A normal infants cries for about 2 hours a day at the age of 2 weeks and about 3 hours a day at 6 weeks then crying gradually decreases to one hour a day at 3 month. The main causes are:
- Physiological needs: A baby who is underfed sleeps for shorter periods and cries at shorter intervals. Thirst may be also responsible.
- Psychological needs: Some babies cry to gain sufficient or additional attention while others simply need to be held. Crying baby should always be picked up.
- Environmental causes: Babies cry when they are wet or soiled. Uncomfortable diapers, tight abdominal binders, too much clothing, hot or cold environment may be the cause. Failure of eructation of swallowed air after feeding is also responsible.
- Secondary excessive crying: Crying in excess of the expected normal range may be secondary to a transient or persistent illness:
- Transient causes include any acute illness as otitis media, early pneumonia or septicemia, acute febrile illness, incarcerated inguinal hernia or napkin dermatitis. Careful exclusion of these conditions is important.
- Persistent causes include milk allergy, lactose intolerance or maternal diet containing irritant foods as spices and chocolates. History should always include the maternal diet.
- Primary excessive crying (Infantile colic): Other terms as “evening colic”, “three months colic” or “paroxysmal fussing in infants” or are used to describe this common problem of newborns and young infants. Some infants seem to be more susceptible and generally described as “colicky infants”. The condition usually starts in the first or second week and usually persists till the infant is three of four months. It is characterized by repeated attacks or paroxysms of excessive crying occurring mainly in late afternoon and evening. The attack begins suddenly with loud and continuous crying which may last for several hours and during it, the face is flushed, abdomen is distended and tense, hands are clenched and knees are drawn up on the abdomen. The baby may look hungry but he is not quieted for long either by feeding or picking up. Apparent relief usually follows passage of feces or flatus. The cause of the condition is unknown. Recent theory incriminates three factors; feeding, psychosocial environment and intrinsic problem in the infant. Mothers should be reassured that the condition does not reflect any serious illness and it will subside gradually to disappear at the age of 3-4 months.
Neonatal Cutaneous Lesions
Several cutaneous problems are commonly encountered in neonates and young infants. The most common lesions are:
- Napkin or diaper dermatitis: Inflammation and erythema of the diaper area is a very common condition of neonates and young infants. The main 2 causes are contact dermatitis and monilial dermatitis:
- Contact napkin dermatitis: It occurs due to friction and prolonged contact with urine and stool. Retained soaps may be also responsible. The diaper area becomes erythematous with fissuring, erosions and may be macerations. The eruption involves mainly the convex surfaces of buttocks, medial side of the thighs and genital area but groin creases are usually spared. Secondary fungal infection (Monilia) is common.
- Monilial napkin dermatitis: It usually follows contact dermatitis. It is characterized by intense fiery red erythema with sharply demarcated edge, which may extend to involve the whole napkin area and rises on the trunk. Small satellite lesions outside the sharp edge are characteristic of monilial napkin dermatitis.
- Persistent diaper rash for weeks in spite of therapy should suggest other conditions as seborrheic dermatitis, acrodermatitis enteropathica and histiocytosis X.
- Seborrheic dermatitis: It is a common chronic inflammatory disease that may start during the neonatal period. The scalp is the most common involved site where diffuse or focal scaling and crusting occur (cradle cap). The inflammatory process may involve the face, neck, retroauricular area and diaper area. A generalized form (Leiner disease) is uncommon and it is usually associated with complement deficiency.
- Milia: They are tiny sebaceous retention cysts scattered over the face, mainly on the nose and around it. They appear as whitish opalescent pinhead-sized spots. Milia can be felt with the finger and it usually disappears within few weeks.
- Mongolian spots: These are blue macular lesions that occur most commonly in the presacral area. They may be solitary or multiple and usually involve large areas. These lesions usually fade during the first few years.
- Umbilical granuloma: The umbilical cord usually separates during the first or the second week. Mild infection may result in formation of moist granulation tissue at the base of the cord. The granuloma is soft, vascular and pinkish in color with mucoid or mucopurulent discharge. Cleaning with alcohol several times a day may be effective. Cauterization with silver nitrate may be necessary in persistent cases.
- Breast engorgement: It is a common physiological process in newborns due to transplacental passage of maternal hormones mainly estrogen. Manual expression or any manipulations should be avoided; otherwise infection may occur (mastitis neonatorum). Clinical manifestations of infection include redness, swelling and pain. If an abscess develops, it should be incised and drained.