Karthikeyan A.S.
History Taking and Pediatric Eye Examination
Pediatric Oculoplasty and Lacrimal Disorders
Pediatric Corneal Disorders
Iris and Anterior Chamber Abnormalities
Pediatric Cataract and Lens Abnormalities
Pediatric Glaucoma
Recognized as the founder of pediatric ophthalmology, Dr Marshall M Parks dedicated his life to improving vision care for children. No contemporary physician has had a greater impact on pediatric ophthalmology. A prolific writer, respected lecturer and dedicated teacher, and as the founder of the Costenbader Society, Parks trained more than 160 of the world's leading pediatric ophthalmologists.
He was born in Michigan in 1918 and he received his medical education at the School of Medicine, St. Louis University. He studied pediatric ophthalmology under his mentor Frank D Costenbader, and together at Children's Hospital in Washington DC, they initiated the first fellowship- training program of any ophthalmology subspecialty. He proudly and bravely served his country during World War II as a US Naval Medical Officer.
Dr Parks first entered ophthalmology in 1946 when there were no ophthalmology subspecialties. He had seen many children with vision problems, and was concerned that there was no specific source of ophthalmic treatment for children. To meet their special needs, Dr. Parks expanded the pediatric ophthalmology program at the Children's National Medical Center in Washington DC.
Dr Parks is a founding member and first President of the American Association for Pediatric Ophthalmology and Strabismus. Dr Parks has presented 45 named lectures, authored several textbooks, contributed chapters to 26 others, and has over 65 published papers. Dr Parks’ seminal contributions include the diagnosis and treatment of strabismus and amblyopia, description of the monofixation syndrome, the benefits of early strabismus surgery, the management of infantile cataracts and innovative surgical techniques.
Dr Parks left the world in 2005 but his contribution to the speciality lives forever and his legacy is an inspiration for every pediatric ophthalmologist worldwide.
- HISTORY TAKING
- Informant
- Reliability
- Referral Details
- Chief Complaint
- Other Complaints Pertaining to Eye
- Elaborating the History of Complaints
- Birth History
- Vaccination Status and Vaccination History
- Antenatal History
- Developmental History
- Glasses
- Patching
- Medical History
- Some Important Quest1ons
- History of Allergy
- History of Prior Surgeries 7 Treatment History
- Family History
- Rapport
- PEDIATRIC EYE EXAMINATION
- Basic Instruments for Examination
- Instruments for Neuro-ophthalmological Examination
- Some Valuable Sterile Surgical Instruments at the Outpatient Department
- Eye Examination
- Making a Routine Examination Technique
- Scan the Face
- Examining the Glasses
- Examination of the Head
- Shape of the Head
- Dysmorphic Features
- Anterior Fontanelle
- Head Nodding
- Head Posture
- Anomalous Head Posture
- Subtarsal Foreign Body
- Facial Asymmetry
- Examine for Specific Behavior
- Bruckner Test
- Sequence of Examination 28 Some Practical Tips
- Sleeping Child
- Crying Child
- Injured Child
- Examination with Restraint
- Glance from Distance
- Examining a Dummy
- Geffing Assistance from the Parents
- Patching
- Examining Old Photographs
- Importance of Taking Photographs in Clinical Practice
- Scanning the Patient 454
HISTORY TAKING
History taking is an art and very critical in all fields of medicine. We all know that in neurology the clinician can almost pinpoint the anatomical level of lesion, etiology and diagnosis by history alone. The eye is an extension of the brain and many ophthalmic conditions too, can be diagnosed based on a very good history alone.
In pediatric ophthalmology, the general consensus is that a good history cannot be taken from children. This is usually true in preverbal children. An attempt to ask questions to a verbal child should always be made and one might be surprised to get very useful information from a 3-year-old child too. (Figures 1.1A to C) The clinician should then decide the usefulness or the reliability of the child's answer in context to the clinical findings.
The mother is usually a good observer of her child and will invariably be right with her observations on how the child sees by intuition.
Simple straightforward questions like
- Does your child make eye to eye contact?
- Does your child look and smile at you?
- Does your child move the head, when you/shadow disturbs her?
- Does your child like sleeping with the room light on? (Children with night blindness prefer having a night light on)
Children with poor vision since birth usually do not complain of poor vision. Children with unilateral visual loss do not complain of poor vision unless they close their normal eye for playing a game or so. What is complained as sudden visual loss is often a long-term visual loss that is suddenly recognized. Hence, how a child detected visual loss is a key question. Did you close one eye and check?
Figures 1.1A to C: Importance of asking children.(A)This 3-year-old child points to his affected eye when asked by the examiner. Direct questioning of the child should always be attempted and clinicians will be surprised by the intelligent answers or attempts made by the child which will give important clues. (B) A child with right Brown syndrome while evaluating ocular motility complained of pain and precisely points to the site of pain when asked by the examiner. This shows how most children facilitate the examiner if they are given an opportunity. (C) A child at 2 weeks follow-up following cataract surgery to her right eye is trying herself to open the eyes to facilitate the examination for an initial penlight examination (An initial rapport established during the first visit, first meeting with the clinician goes a very long way in the care of the child).
A continuous and conscious effort should be made by the clinician to make the visit for the child, the parents and all patients as a pleasant and memorable visit.
Never underestimate what children can express. It is how the examiner interacts with the child and makes good interpretation paves way for an efficient examination
Children with poor night vision often avoid playing in the dusk and might avoid dark. They might be misunderstood by parents as “afraid to go in the dark”. Hence, parents should be questioned as to whether their child is afraid to go in the dark. It might give a clue of a possible night blindness. In contrast, children with photophobia might avoid going in daylight and might stay preferably indoors.
Sometimes children are afraid of parents, teachers and conceal facts especially involving trauma, or conflict between siblings or friends or peers at school. Hence, interviewing the child separately is critical (telling the child that clinician would not tell about it to the parents/teachers). Once the truth of etiology of trauma is known, it should not be disclosed to the parents or teachers in front of the child as it breaks the trust; but once the examination is completed and management line is discussed, the parents should be told when the child is with another attendant or staff (not in the scene), and they should also be told as not to scold the child (This might affect subsequent compliance with treatment).
History should be elicited regarding systemic well-being of the child, and general systemic concerns should be addressed for one to pickup a diagnosis of systemic syndromes with ocular manifestations. Some parents often think that it might not be important to tell about hearing problems to an eye specialist. 5There are several conditions which can affect hearing and eyes and if the clinician does not specifically ask about each system at least as a checklist, the parents are either likely to forget or conceal the information thinking it is not necessary.
It is a good practice to start examining the child, even when the history is being taken, by making casual glances at the child's eye and visual behavior. Several observations need to be made, which could be altered or modified, once the becomes aware of what clinician is looking for. Head posture, and eye blink are some examples. The initial rapport can be established during the history taking which makes subsequent examination easier. Making the child feel comfortable is the first step for a successful examination.
The clinician has to be sensitive and careful in framing questions to parents, especially when symptoms are suggestive of genetic, congenital malformation or related to maternal events during pregnancy. Improper questioning might result in feeling of guilt and misunderstanding between parents and family members. Especially in underdeveloped and developing countries, it could even result in the mother being blamed for the child's condition resulting in harassment by her husband and family members.
History of leukocoria, finger waving and oculodigital poking (Child presses the eye with his or her fingers or the knuckles) can provide important clues in examination.
Many times, there are several of family members who come for consultation as any problem with children in general causes greater anxiety in the family than health problems in adults. The clinician should carefully select the informants (mother, grandmother and father are best in giving the history in that order). When both parents are working the grandmother gives important clues as she takes care of the child most of the time.
The clinician should never show resent if there are lot of family members. They should be explained gently that two to three members who have more information about the child can stay in the consultation room, and others can wait in the waiting area and would be called if any information is required. Too many informants in the consult room can sometimes provide a lot of overlapping information and might prevent the clinician from getting a well-directed history. Children also tend to get distracted.
In toddlers, it is important to have the family member with whom the child is most comfortable with, in the consultation room, as it helps greatly during the examination and also those members who spend more time with the child and can make useful contributions by providing their observations.
Elder siblings often like to be given responsibility in taking care of their younger siblings and can be great asset in assisting for an examination of their younger sibling. The elderly sibling can often engage in games with the patient while important history is being discussed with the parents and, at the same time, provides an opportunity for the clinician to observe the patient as well, and also examine the sibling if a genetic condition is suspected.
History taking is an art and an entire textbook can be dedicated for this topic, but here in this chapter, the basics of history taking is discussed and the finer aspects of history taking pertaining to individual clinical conditions are mentioned under the protocols for different clinical conditions in appropriate chapters (Figure 1.2).
Informant
Who has come with the patient?
It is important to note who provided the history. Sometimes, the child is accompanied by attendant who does not even know anything about the child's ocular or general health in detail but accompanied the child as the mom was busy and there was no one in the family to accompany. Such history might be very unreliable. The clinician should stress the importance of parents accompanying the child for the next visit if not possible in the same visit. A telephonic talk can sometimes help, but it might be prudent to have a visit of the parents or grandparents. This is preferable in non-emergency situations, though the examination is completed for that particular visit.
Reliability
Is this history reliable?
One has to mark the reliability of the informant and the importance that can be given to the relevance in history to clinical examination.
Referral Details
Who has referred this patient?
Self-referral, other family members, pediatrician, physician or ophthalmologist.
Figure 1.2: Photograph shows history taking in progress. The mother is seen sitting with the pediatric patient
This would give to certain extent the degree of reliability of the earlier person's clinical findings, and also to ensure at the end of consultation that the patient and the parents get the direction/answer to the reason for which they have been referred.
Chief Complaint
What is the main concern that brings you here?
A good method is to allow the parents or the patient to talk (all of which they remember) without interrupting, and start questioning once they complete their initial complains.
Other Complaints Pertaining to Eye
What other problems do you have?
Elaborating the History of Complaints
The following information should be sought for each complaint.
- Onset
- Duration
- Progression
- Associated factors
- Aggravating factors
- Relieving factors.
Birth History
Some important clinical implications regarding information obtained from the birth history include:
- Full-term
- Preterm: Retinopathy of prematurity (ROP), increased risk of strabismus, refractive errors, amblyopia, congenital nasolacrimal duct obstruction (CNLDO)
- Normal vaginal delivery (ocular infections) as in ophthalmia neonatorum
- Forceps (forceps injury)/vacuum extraction/cesarian section
- Birth weight: Retinopathy of prematurity
- Cried immediately (Apgar score), tachypnea in Joubert syndrome, neurological status
- Duration of stay in hospital: Prolonged in ROP, prolonged neonatal jaundice, systemic abnormalities, sepsis, etc.
Vaccination Status and Vaccination History
- It will give clues regarding the overall health of the child
- It will also give clues as to how educated and informed are the parents in terms of child care, and a judgment can be made on their efforts to comply to treatment subsequently advised
- Can suggest possible clues to cause of visual loss, e.g. papillitis following vaccination.
Antenatal History
- Fever and rashes (congenital rubella)
- Maternal systemic history (diabetes, eclampsia, seizures, etc.)
- Maternal medications (effects of medications on the growing fetus and newborn)
- Alcohol and drug abuse (fetal alcohol syndrome)
- Anemia
- Prior pregnancy losses (possible chromosomal abnormality).
Developmental History
If the child has older siblings, asking the mother to compare the child with her previous children, which can be easier and bring out relevant history
- Normal
- Developmental delays (neurological insult and chromosomal abnormalities)
- Developmental regression (suspected metabolic disorders and loss of learned skills)
- Assessment for speech delay, motor delay or a global developmental delay is valuable.
Glasses
- When started wearing glasses?
- When started wearing the most recent prescription?
- Compliance with glasses
- Family history of glasses
- Power of glasses.
Patching
- When was patching started?
- Which eye was patched?
- How many hours was patching done?
- Was there an improvement in vision?
- Was it anytime discontinued and if so, why?
Medical History
Systemic history addressing all systems (simple words that the lay person can understand should be used). It may not be sufficient to ask just one blanket question “Does your child have any systemic problem “and be satisfied with a yes or no response. Parents often forget and usually recollect if questioned as below.
Does your child have any problems with?
- Heart
- Lung/breathing
- Skin
- Ear, nose and throat
- Bone and joints
- Muscle
- Stomach
- Endocrine or hormonal problems
- Bowel and bladder habits (one should avoid questioning about incontinence and enuresis in front of a child who can understand, as it will embarrass the child and break the trust and confidence in clinician)
- Neurological problems
- Psychological.
Some Important Questions
- Does he or she sleep well?
- How is his or her eating habit? (Clues regarding pica such as eating of mud)
- Does he or she read well?
- Does he or she play or mingle with other children? (Social interaction)
- Does he or she participate in sports activities?
These questions can give very important clues regarding the well-being of the child.
As an example of importance of history taking, a child had complained of headache and had consulted many pediatricians, neurologists, ENT (ear, nose and throat) specialists and thorough investigations including repeated neuroimaging had been done, and everything was found normal. Complete and comprehensive ocular examination was also normal. Finally, the mystery was revealed by a simple history. The child wanted glasses as her best friend was prescribed a beautiful pair of pink glasses following a complaint of headache and poor vision. It is important to talk about their best friends and peers at school. The child was given a beautiful plano glasses as a temporary measure and the headache disappeared in no time! The child was weaned of glasses later. It is also very important to rule out all organic causes before contemplating a diagnosis of malingering.
History of Allergy
Medications/food/dust.
History of Prior Surgeries
A specific history of prior other surgeries, nature of surgeries, their outcome, ocular surgeries and their outcome needs to be noted. Any adverse events related to anesthesia or similar events in other family members are critical. (Malignant hyperthermia is a serious life-threatening condition which is inherited as an autosomal disorder and is characterized by hyperthermia during anesthesia). Strabismus and oculoplasty procedures are prone to have a higher risk.
Treatment History
One should note the response to treatment of the other family members in hereditary disorders.
Family History
Pedigree
A three-generation pedigree in all suspected genetic conditions need to be taken. It is very imperative to take a thorough family history as it can help correlate the findings and also enable screening of other family members at-risk.
A good history can help to get a focused and targeted examination helping in obtaining a precise diagnosis early in the course of a disease which can make a huge difference in vision and sometimes quality of life of these patients
Rapport
Establishing a good rapport and also an early rapport is a key to successful examination in examining children (Figures 1.3A to E).
Some general concepts on child psychology are very important to make the best out of the examination. Different strategies have to be adopted in examining children of different age groups.
There is a brief description of someway on how the environment can make examinations easier and some practical tips are given. Many practical tips are provided in this chapter under the heading “Pediatric Eye Examination” also. This discussion here provides some guidelines. It is discussed under the following areas:
- Department
- Examiner
- Safety measures
- Rapport with the childern
- Trust of parents.
Department (Figures 1.4A to F)
- A child friendly pediatric eye care department is very important in making the atmosphere more conducive for pediatric eye examination
- A safe play area, where the child can be monitored by parents adds the visit a more fun experience than a doctor's visit
- A television with a cartoon program in the waiting area plays wonders
- An aquarium adds color to the environment as well as greatly reduces the tension of parents especially if genuine waiting time is anticipated8Figures 1.3A to E: Developing rapport. (A) Photograph shows a child who, when got an opportunity (when the examiner started writing the findings), was not hesitant to take the torch and show it toward the examiner. (Hey Doctor! This is what you do to us!). (B) This 2-year-old child is ready for a boxing bout with the examiner.(C) Even this child less than one year of age wants to say something.(D) An array of interesting toys to make the examination more a fun filled game.(E) This small mannequin is a very great asset in the pediatric ophthalmology department as it instantly attracts children and makes the examination room more of a play school, and makes many subsequent ocular examinations surprisingly and amazingly simple (discussed in this chapter under the heading “Making a Routine Examination Technique”)
- Colorful walls, cartoons and paintings on the wall add a more pleasant experience to the child and family
- Anything threatening (like syringes, medications, needles should not be anywhere in the vicinity where the child can see or have access)
- Remember some of the following do's and don'ts. Practically, this applies to everyone even in life for all ages. Probably many of us, as adults have forgotten these golden rules
- Respect and treat the child (everyone) as an individual
- Never tell lies to the children.
There are very few children who are not able to cooperate for an examination and if so, the examiner is more likely at fault at some stage of examination or the underlying clinical condition forces the child, or fear more than anything contributes to a failed examination.9
Figures 1.4A to F: (A) Reception and waiting area. A flow chart showing the sequence of examination to help a better preparedness.(B) Play area and an aquarium to keep children and parents relaxed during waiting hours. (C) Second waiting area with a television displaying images of the play area located at front of the department by a closed-circuit television (CCTV). (D) Closer view of the monitor showing the play area.(E) A picture from the Lion King on walls of the counseling room. (F) A friendly environment and also displaying important patient awareness information
Different methods need to be adopted when interacting with children of different age groups.
- Infants
- Toddlers
- 3–5 years
- 5–12 years
- Teenagers.
Examiner
- Wearing a doctor's coat is optional. It is better not to wear a doctor's coat especially when examining children with chronic diseases who have been to many health care centers and undergone several invasive procedures
- Wearing a tie with a smiley face or a badge with a cartoon can make the examiner appear friendlier and allay any fears of the child even at first sight. It is not necessary to dress too funny as parents can mistake the appearance for being less competent or less serious in their job. A good balance need to be maintained
- Children do not have much inhibition and tell the truth loudly, openly and instantaneously. Personal hygiene is very critical to avoid any embarrassment for the examiner as childern might just yell out that examiner has a bad breadth (when examiner is doing direct ophthalmoscope), the child may say doctor has long dirty nails, or your coat is dirty, etc.
Safety
- The examiner should ensure that he or she does not have any toy that can inadvertently cause injury like small beads, pencils or pens. Safety is critical. The children should not develop any untoward problem because of their visit!
- The examiner should basically ensure common sense and anticipation and whatever he/she provides the child to play with should not be likely to cause any form of injury
- The child should not be left unattended at the examination room with all equipments nearby
- In examining children with psychological issues, the examiner should show compassion but at the same time anticipate any violent behavior anytime to avoid injury to self, parents and other staff
Rapport with the children (Figures 1.3A to E)
- Say “Hi”, when you (examiner) meet children. Shake-hands or a high five can be a good way to start your conversation with the child
- Ask the parents, how they call their child at home and call by that name. The child feels more comfortable
- Ask the child if he or she has any pets at home. Talk about them
- Talking about cartoon characters that child likes is a great way to communicate
- Have some magic tricks under your sleeve. There is no child who cannot resist or not get excited by a trick or magic. Author finds it very useful to get the compliance for patching treatment for amblyopia
- Having a dummy child (mannequin) is of great help as discussed in this chapter under the heading “Pediatric Eye Examination”
- Comment positively on the T-shirt, dress or hair do, etc.
- Use whispering sometimes to ask questions, and children often whisper when they talk something secret to their friends, and hence examiner can become an instant friend
- Always give the elder sibling a supporting role in examination, and they are the best assistant sometimes as the younger child is not afraid when the elder sibling takes the responsibilities wonderfully in getting their younger sibling to cooperate
- Never tell the parents that their child is malingering in front of the child. It simply breaks all trust that examiners have developed during their initial rapport
- Paper toys are a great substitute to retrieve the toy at the end of the examination
- The examiner should ensure that the toy is got back, but he or she should ensure the burden of collecting it should be given to one of his or her paramedical team member and not by himself
- Dilating drops are best administered by the paramedical staff.
Rapport and Trust of the Parents
- Never address the child as it. This annoys the parents. Moreover, we do not use it for living people and only inanimate objects are named as it. So always refer to the child as him or her
- Some parents understandably feel upset if the child is addressed wrongly as boy instead of girl or vice versa. It greatly undermines the confidence of the parents in the examiner as a clinician
- A good clinician should be able to judge the age of every pediatric patient reasonably close even before asking the child or parents. Correct judgment tells the parents indirectly the clinician's experience in judgment
- Make regular eye contacts with the important members of the family when talking. Avoiding one person or special focus to only one parent all the time might make the other left out and less comfortable
- Examiner has to be sensitive to issues when talking about genetic disorders, serious ocular and systemic conditions, congenital malformations, etc. and should not communicate in a negative way that creates a feeling of guilt or argument between the parents or family members
PEDIATRIC EYE EXAMINATION
The presence of instruments and necessary set-up is important for a good pediatric eye examination. There should also be a good child friendly environment.
Basic Instruments for Examination (Figure 1.5A)
- Penlight
- Direct ophthalmoscope
- Streak retinoscopy and trial lenses
- Lens rack
- Flippers
- Trial frame and lenses
- Toys and dummy child for examination
- Occluder, accommodative targets, Maddox rod
- Optokinetic nystagmus (OKN) tape
- Indirect ophthalmoscope and 20 diopter lens
- Perkins tonometer, tonopen, applanation tonometer
- Gonioscope
- 90 diopter lens and 60 diopter lens
- Box of prisms
- Digital camera.
Instruments for Neuro-ophthalmological Examination (Figure 1.5B)
- Red colored mydriatic caps
- Knee hammer (Giraffe shaped knee hammer is an excellent substitute instead of the routine scary ones)
- Tuning fork
- Hardy-Rand-Rittler (HRR) color vision test
- Vision charts
- Inch tape
- Neutral density filter
- OKN tape, OKN drum.
Some Valuable Sterile Surgical Instrumentsat the Outpatient Department
- Lid retractors
- Speculum
- Cotton tipped applicator
- Medications
- Epilation forceps
- Toothed and non-toothed forceps
- Vectis for scleral depression.
Eye Examination
A comprehensive yet complete examination is mandatory in all patients. The sequence of examination at times needs to be slightly modified, but all the parts of examination need to be completed. Persistence in examination should be as much as the persistent behavior of a child, but the approach should be as gentler as possible. Examination with restraint needs to be done when indicated. If necessary examination under anesthesia should be planned rather than giving up, saying patient is not cooperative or compromising with an incomplete examination. If clinicians compromise in their examination or if clinicians compromise in their findings, of course they will land up with compromised treatment and compromised quality in care. So never compromise.
Sometime residents and fellows perform part of examinations during their training, e.g. indirect ophthalmoscopy only. Hence, he or she should also master to examine the anterior segment with 20 diopter lens prior examining the fundus. This practice will help enormously especially when one does a ROP screening where, neovascularization of the iris (NVI), ectropion uvea, persistent pupillary remnants and iris congestion could be recognized by an experienced examiner.12
Making a Routine Examination Technique
It happens many times, the patient comes with a particular problem but the clinician detects other problems other than the one complained by the patient. This happens more often only if the clinician makes a thorough and systematic complete examination with more area of focus on the complaint and not just observing only those areas relevant to complaint. A collateral thinking and examination is always necessary to pick up subtle and early signs of serious ocular disorders. Remember that always more than one disease can coexist. Just clinching one is not our accomplishment, giving total care that it needs recognizing and treating all the problems seen and unseen.
Author is suggesting a simple method of his examination technique irrespective of the complaint of the patient. Visual assessment is discussed in a separate chapter. Initially this might appear to take time but with time this becomes fast and very efficient, and of course it is not necessary to make the same routine for the same patient in close follow-ups if there are no new symptoms.
- Scan the face
- Observe the head and head posture
- Examination of glasses
- Perform a Bruckner's test
- Look for lagophthalmos
- Hirschberg, cover/uncover test/alternate cover test
- Access motility examination
- Pupil evaluation
- Anterior segment examination
- Undilated fundus examination with 90 diopter/direct ophthalmoscope examination
- Cycloplegic refraction
- Dilated indirect ophthalmoscope examination.
Depending on the findings that the clinician obtains at each level, he or she may have to make small side trips into other examination sequences.
With time one can develop their own way of systematic examination. With increasing experience one should resist the temptation to skip the basic steps in the sequence of examination which some senior consultants with time try to by pass few important basic steps and finally burn their fingers. Basics are basics!
Unfortunately, there is no short cut in medicine. Fortunately, if clinicians follow a systematic approach, rarely they miss the diagnosis. If clinicians probably follow short cuts and if they are successful, probably their day to fall into trap is not very far. When clinicians want to bypass a step, they should ensure that it would not affect the outcome of their examination or management. They should ensure that they complete it in their subsequent examination. The first few examination techniques and practical examination tips are described in this chapter.
Scan the Face
Scanning the face gives spot diagnosis of many clinical conditions and helps to focus on relevant ocular findings. It is important to scan the face in good illumination (Figures 1.6 to 1.16).
It is important to request the patient to remove caps, burkas, or any other scarfs prior examination. Some of the photographs explain the story (Figures 1.17A to D). A methodological scanning may increase clinicians’ accuracy.
- Head size (hydrocephalus, microcephaly)
- Head shape (craniosynostoses, plagiocephaly)
- Scan forehead for scars (traumatic optic neuropathy, prior ptosis surgery)
- Look for subtle dysmorphic features
- Quickly judge interpalpebral/intercanthal distance.
Some of the following clinical conditions can be recognized by good observation of the face. A window through which good daylight can be shone into the clinic, when required, is a prerequisite for examination. Some of the conditions where daylight is best for recognizing clinical signs are below:
Conditions that can be Better Appreciated/Diagnosed Best in Natural/Daylight
- Jaundice
- Capillary hemangioma
- Heterochromia
- Anisocoria
- Spontaneous dissociation in intermittent exotropia
- Effect of light on nystagmus intensity increases in albinism and cone dystrophy
- Faint port-wine stains in Sturge-Weber syndrome and hemangioma
- Nevus of Ota.
A window in the clinical examination room opens the door for much correct diagnosis.
Examining the Glasses
Examining the patient's glasses gives a lot of clues on the following:
- Nature of refractive error
- Severity of refractive error
- Anisometropia
- Condition of the glasses
- Presence or absence of prism
- Placement of bifocal segment
- Maintenance and clue for noncompliance (mechanical causes) and optical causes.
13After examining the glasses, observe the blink of the patient. Observe for the completeness and frequency of blink.
Examination of the Head
Though the following areas are under the specialty of pediatrics, patients in life do not obey textbooks and might present in many scenarios with eye problems first.
Hence, knowing the subtle signs (which we had already learnt earlier as medical residents) is very useful in correlating the eye findings and will help in timely referral to the pediatrician and pediatric neurologist. as sometimes children might present with a benign eye symptom not even related to the underlying major neurological problem. One has to note the following:
- Size
- Shape
- Status of fontanelle
- Asymmetry
- Dysmorphism features
- Prominent veinsFigure 1.6: Xeroderma pigmentosa. The Figure shows a child with pigmented lesions on the face. One can also observe the conjunctival lesion in both eyes. Xeroderma pigmentosa is a genetic disorder which increases the risk of cutaneous and ocular surface malignancies due to defective DNA repair following UV radiation induced DNA damageFigure 1.7: Tuberous sclerosis. The Figure shows a boy with multiple lesions on the face (adenoma sebaceum). Tuberous sclerosis is an autosomal dominant disorder. Epiloia encompassing the triad of epilepsy, low intelligence and adenoma sebaceum14Figures 1.8A to C: Coarse facial features (Hypothyroidism). (A and B) Cretinism or congenital hypothyroidism is characterized by alopecia, madarosis, closure of anterior fontanelle, mental retardation and a coarse face with a hoarse cry. This child presented with the above features and can observe the eyebrows have been drawn with an eyebrow pencil by the parents. (C) A patient with mucopolysaccharidosis showing coarse facial featuresFigures 1.9A and B: Down syndrome (discussed in detail in the chapter 18 “Systemic Disorders”). (A) Photograph shows a child with Down syndrome. (B) Another photograph shows female child with more prominent features of Down syndrome. The prominent features of Down syndrome are recognizable immediately are mongoloid slant and broad nasal bridgeFigures 1.10A and B: Waardenburg syndrome.(A) Photograph shows a boy with Waardenburg syndrome. Note the following features: Hypochromic heterochromia, synophrys, upturned nose and Cupid's bow shaped upper lip. (B) Photograph of the same patient with his mother. Note the striking similarity between the mother and the son. The hearing, however, was minimally impaired
- Document and look for old photographs.
It is very important to remember that the head of a child is significantly bigger in proportion to the child's body even in a normal child. This has two important implications:
- Hence, it might be difficult to comment on hydrocephalus if the child had microcephaly at birth or if the child developed hydrocephalus before presentation to the clinician (Figures 1.21A to C).15Figure 1.11: A 15-year-old female child with blepharophimosis syndrome (discussed in detail in the chapter 2 “Pediatric Oculoplasty and Lacrimal Disorders”). Features include ptosis, blepharophimosis, prominent eyebrows, hypoplastic supraorbital ridges, telecanthus and epicanthus inversusFigure 1.12: Sturge-Weber syndrome (discussed in detail in the chapter 18 “Systemic Disorders”). Photograph shows an infant with port-wine stain involving the left face in the distribution of ophthalmic and maxillary division of trigeminal nerveFigure 1.13: Goldenhar syndrome/hemifacial microsomia (discussed in detail in the chapter 18 “Systemic Disorders”). The photograph shows a boy with hemifacial microsomia. One can observe the mandible hypoplasia and preauricular tags. This child did not have any other features suggestive of Goldenhar syndrome
- One might miss a microcephaly if the head size is proportional to the body size and not bigger as it should be normally.
History of nature of delivery (Cesarean section as to normal delivery), difficulty during delivery, family history of macrocephaly is critical in reaching the diagnosis. Head circumference in doubtful cases should be documented, and a pediatrician consult obtained for evaluation.
Some of the clinical conditions where there is:
- Microcephaly (Figures 1.22A and B)
- Cerebral palsy
- Periventricular leukomalacia
- Intraventricular hemorrhage
- Macrocephaly
-
Figure 1.14: Congenital syphilis (discussed in detail in the chapter 18 “Systemic Disorders”). This child presented with interstitial keratitis. Note the triangular face, saddle nose appearance and frontal balding which are classical of congenital syphilisPhoto courtesy: Dr Cidan YangkyiFigure 1.15: Wildervanck syndrome (discussed in detail in the chapter 18 “Systemic Disorders”). The photograph shows facial asymmetry, limbal dermoid of the left eye and preauricular tags. She had features of Goldenhar syndrome and Duane retraction syndrome17Figure 1.16: Möbius syndrome (discussed in detail in the chapter 18 “Systemic Disorders”). This child has a mask-like face and left esotropia18Figures 1.17A to D: Hidden truths! under the scarf. (A) This patient presented with headache in a winter when most of them wear caps and scarfs. Asking him to remove the scarf reveals the cause for his headache (herpes zoster ophthalmicus). (B) Another girl child with scarf shows extensive scalp infection. (C) A surgical scar for a neurosurgery for tuberculoma of the brain in a patient with tuberous sclerosis is well-concealed in a cap. (D) This child presented for a different ocular complaint but the words on the head band surprised and shocked the author (SEX). As part of the first step of the clinician's examination, the author asked him to remove the scarf and it revealed TEXAS! infolding. Many relevant clinical findings will be missed if one does not ask the patient to remove the cap/scarf/burka, etc.Figure 1.18: Oculocutaneous albinism (discussed in detail in the chapter 18 “Systemic Disorders”). The photograph shows a female child with severe oculocutaneous albinism. One can observe the red reflex filling the center pupillary area due to iris transillumination. Note the skin and hair colorPhoto courtesy: Dr Arjun Mallah BhariFigure 1.19: Oculocutaneous albinism (mild). This child has a milder form of oculocutaneous albinism with brown hair19Figures 1.20A and B: Father and son with albinism. (A) Photograph shows a boy with lightly pigmented iris and light brown hair. (B) Same boy with his father. Note the father has a dark hair (hair dye) but his moustache is still light brown (undyed) and the light colored irisFigures 1.21A to C: Hydrocephalus. (A) Side view showing the enlarged size of the head of a boy who presented with vomiting. (B) CT scan of the same child showing Hydrocephalus. (C) Another child with larger head but this child did not have Hydrocephalus
- Infantile hyperostosis (Figures 1.23A and B)
- Craniosynostoses (Figures 1.24A and B)
- Cretinism
- Familial (Figures 1.25A and B).
-
The parents head size should be compared to rule out a familial macrocephaly.
Shape of the Head
- Dolichocephaly
- Brachycephaly
- Plagiocephaly (Figure 1.26)
-
Figures 1.23A and B: Infantile hyperostosis. (A) The photograph is of a boy with parent's complaint of poor vision and hearing impairment. (B) One can observe the larger head size and imaging showed hyperostosis with diffuse enlargement of the entire calvariaFigures 1.24A and B: Craniosynostosis. (A) External photograph showing the prominent eyes, maxillary hypoplasia, exorbitism due to shallow orbits, and low-set ears, divergent squint, some amount of frontal bossing, hypertelorism suggesting Crouzon's syndrome. (B) Side profile of the same patient showing the classic parrot-beak appearance and the shallow orbit and exorbitism
Dysmorphic Features
It is useful for the pediatric ophthalmologist to be familiar with the facial appearance of several systemic syndromes. A spot diagnosis will quickly help the clinician to draw attention to the eye findings associated with the specific condition.
Anterior Fontanelle
- Prominent pulsations could indicate increased ICT. Since in small children papilledema do not appear early due to skull expansion, collaborative clues like this can be very valuable
- Bulging fontanelle is also a feature of hypervitaminosis A, which causes benign intracranial hypertension in children
- The anterior fontanelle normally closes by 2 years of age in most children. Delayed closure occurs in conditions like cretinism, achondroplasia, and Down syndrome
- Early closure happens in fetal alcohol syndrome, hypoxic ischemic encephalopathy, dysmorphic syndromes and craniosynostosis
-
Figures 1.25A and B: Familial macrocephaly. (A) This picture shows the comparison of head and body size of a female child. The size of the head is larger as compared to the body. (B) Photograph of her mother shows a similarly larger head size. It is very important to compare the parents and siblings head size before planning an imaging especially if other visual parameters are normal
- Children might develop subdural hematoma without fracture of skull due to nipping of middle meningeal artery by overriding skull bones at the time of impact. Delayed onset loss of consciousness and seizures can occur
- Metastasis to skull bones can be picked up on palpation (usually less common in children).
Head Nodding
In the presence of head nodding, one should suspect spasmus nutans, head bobbing, typical congenital nystagmus. Head thrusting is also seen in oculomotor apraxia and gaze palsies.
Head Posture
One has to observe for abnormal head posture. If any abnormal had posture is noted, it is documented. Three things to be noted are head tilt, face or head turn and chin position. The following section gives guidelines regarding history taking and examination of a child presenting with anomalous head posture.
Anomalous Head Posture (Figures 1.27 to 1.31)
History
- When anomalous head posture is noticed?
- Birth history focusing on difficult delivery. Use of forceps or cephalopelvic disproportion
- Does the head posture maintain during sleep?
- Did the parents notice any squinting or nystagmus?
- Family history of strabismus and nystagmus?
- Does the head posture increase during periods of visual and mental attention?
- Any hearing impairment?
- Ask for old photographs. Idea on age of onset and clue regarding decompensated strabismus.
First step is to differentiate between ocular and non-ocular causes (Table 1.1, Figures 1.29 and 1.30).
Examination
- Ask the patient to stand straight with the hand by the sides and look at the distance visual acuity chart. If the patient is sitting, ensure that the patient does not lean on the parents or lean on the hand rest for support
- Ask the patient to read down the chart as head postures might become more prominent during increased visual demands (reading down the visual acuity chart)
- Observe for any facial asymmetry that indicates long standing etiology
- Document abnormal head posture as head tilt, face turn (head turn) and chin position for all head postures
- Check vision, binocularity, stereopsis with the adapted head posture, straight ahead and forced opposite head posture
- Now force the head straight and look for development of strabismus, nystagmus or dissociated vertical deviation (DVD)
- Ask for double vision in acquired head postures (acquired paralytic strabismus)
- Look for any head posture for near vision
- Perform a cover-uncover test. Watch out for alternating head postures during cover test as in nystagmus blockage syndrome (NBS)
- Perform ocular motility
- Perform a hearing assessment
- Perform confrontation fields to rule out congenital cause of hemianopia as a cause of abnormal head posture
- Feel the neck for secondary muscular and skeletal changes.
Causes of Abnormal Head Posture
Ocular
- Nystagmus
- Congenital motor nystagmus: To attain binocularity and optimize visual acuity
- Nystagmus blockade syndrome: To dampen the nystagmus by attempted convergence
- Manifest latent nystagmus
- Strabismus
- Strabismus syndromes (Brown syndrome, Duane retraction syndrome), binocularity
- Alphabetical pattern strabismus (binocularity)
- Paralytic strabismus (oculomotor, abducens and trochlear), binocularity and to avoid diplopia
- Restrictive strabismus (entrapment, pseudotumor, dysthyroid ophthalmopathy), binocularity and to avoid diplopia
- Amblyopia: Patients might adopt a head posture to the opposite side while reading
- Uniocular blindness: To center the visual field
- The face turn is ipsilateral to the side of blind eye
- Astigmatism: To use the bridge of the nose as a pinhole/slit effect
- Congenital or long-standing homonymous hemianopia
- Allow some binocularity (slightly controversial)
- Patient also develop an exotropia of the affected eye
- Right homonymous hemianopia patient has right exotropia, right face turn (all same side)
- Horizontal gaze palsy: To attain binocularity
- Ptosis: Chin elevation to clear the droopy lid from the visual field.
Figures 1.27A to C: Anomalous or adapted head posture. (A1) Child with right face turn, chin elevation and left head tilt. Figure A2 shows limitation of elevation of the left eye in adduction (left Brown syndrome). (B) Another child presenting with right Brown syndrome. (C) Right face turn in a patient with limited abduction. In this patient, forced duction was positive, and there was narrowing of palpebral fissure on abduction suggesting a medial rectus restrictive etiology. A CT scan of same patient showing inflammation and enlargement of right medial rectus. Same patients after 2 weeks of steroid therapy showing a lesser face turn. He also had better adduction.
One can observe that the head moves in a direction where the normal muscle (now affected) would actually move the eye so that the muscle is not brought into action. The patient in Figure C again moved his face in a direction where his diplopia would be minimal. There will be a similar right face turn in right abducens nerve palsy. The difference is that, in abducens nerve palsy, the face turn is to keep the affected lateral rectus muscle at rest. In medial rectus limitation, it is to prevent further stretch of the medial rectus muscle caused by abduction
Figure 1.28: Nystagmus. Child has a gross left face turn as she tries to comment on the finer details of the toy in the examiners hand. Typical congenital nystagmus often presents with anomalous head posture due to better visual acuity obtained at the null point. For this child, the null point is located in dextroversion causing the child to adapt a left face turn to bring the object of interest closer to the null point. It is often important to record the visual acuity by the ophthalmologist himself specifically looking for the dynamic changes as the child starts reading the lower case letters or an interesting toy
Figures 1.29A to G: Differentiating ocular and nonocular cause of head posture. Figure B shows a boy with left face turn. On patching the right or the left eye (Figures A and C), the face turn disappears. Figure D shows the same patient as a child with similar left face turn. This patient had Duane retraction syndrome of the left eye. One can also appreciate; he prefers blue color dresses! (E to G) Differentiating ocular and nonocular cause of head posture.
Photograph shows a boy with nonocular cause of head posture. One can observe the tight and contractured right sternocleidomastoid muscle
Figures 1.30A to D: Causes of abnormal head posture. (A) Patient with ptosis having chin elevation. (B) Child with Möbius syndrome and left face turn with left esotropia, and limitation of abduction in both eyes (discussed more about this finding in the chapter 13 “Strabismus”). (C) A patient with limitation of abduction caused by lateral rectus entrapment after an injury with foreign body (wooden). Figure C2 shows limitation of adduction, and Figure C3 shows the embedded foreign body that was removed. (D) An adult patient with congenital right superior oblique palsy with long-standing superior rectus contracture. There is no adoptive head posture as there is no binocularity. Figure D2 shows the marked under action of the right superior oblique
Figures 1.31A to C: Subtarsal foreign body causing abnormal head posture. (A1 and A2) Photograph shows a boy who presented (in 2006) with a right face turn, subtle chin elevation and a left head tilt. There was history of trauma of 2 days duration. Ocular examination revealed a right face turn and minimal left head tilt. The boy resisted all attempts to straighten his face by the examiner. Attempts were associated with the boy opening the lids wide open. There was no corneal abrasion but minimal nasal bulbar conjunctival congestion. (A2) Eversion of the upper lid revealed a subtarsal foreign body (encircled area).
(B1 and B2) Photograph shows a teenager who presented with irritation and watering in his left eye. On examination he had a right face turn and a chin elevation. He also resisted attempts to straighten his face. It was accompanied by widening of the palpebral fissure and a tendency to avoid blinking. Also note that the eyebrows are also raised. Eversion of his left upper lid showed a caterpillar hair in the lid margin (encircled area). There was minimal nasal bulbar conjunctival congestion and no corneal abrasion even on fluorescein staining.
(C1 to C3) Photograph shows a girl who presented with irritation in her right eye. She had a left face turn and a chin elevation. One can observe the wetly appearance of the right eye and the nasal bulbar congestion. Rest of the ocular examination was normal. As in the patient in Figure B, eversion showed a caterpillar hair and was removed. Figure C3 shows the site of lodged foreign body with surrounding area of induration. Also note the nasal bulbar conjunctival congestion. Figure C2 shows restoration of normal head posture on the subsequent day after removal of foreign body
Subtarsal Foreign Body
- It is described by the author as a new ocular cause of torticollis in children.
- Subtarsal foreign body (This as a cause of abnormal head posture has not been described in literature and is the authors personal observation and describes it as a new clinical sign) “Abnormal head posture due to subtarsal foreign body” (this new sign is described by the author in detail below and in the Figures 1.31A to C)
Mechanism for Adopted Head Posture
Reasons for face turn: When a patient has a subtarsal foreign body, he or she resists blinking as with each blink, the foreign body abrades the cornea causing lacrimation (windscreen wiper effect). This is more pronounced when the foreign body is located in the middle one-third of the upper lid as it abrades on the central cornea. Cornea is very sensitive tissue as compared to conjunctiva. Also the central cornea is more sensitive than the peripheral cornea due to both density of nerves and also pattern of innervation (stromal for the central cornea as against epithelia plexus of the periphery). A patient can tolerate repeated minor abrasions/insults to the conjunctiva but not to the cornea.
One should remember that the eyes move in the opposite direction of the face turn when the point of fixation is maintained. One should also understand that the lids do not move and only the eyeball moves. By adopting an opposite face turn, the foreign body is kept away from the cornea (now instead abrades the nasal bulbar conjunctiva) during blinking. Hence, nasal bulbar congestion was observed in all the above patients.
The degree of face turn depends on the location of the foreign body. The face turn is more when the foreign body is closer to midline (Figure 1.31A) as the patient has to adopt a large face turn to keep it away from the cornea during blinking. If it is farther away from the midline closer to the nasal side, the face turn is comparatively less (Figure 1.31B).
If the foreign body is temporal to the midline, a contralateral face turn is absent as it can worsen the abrasion with blinking. A subtle ipsilateral face turn might be seen (not shown in photographs).
Reasons for chin elevation: Normally, the upper lid normally covers 2 mm of the superior limbus. The lower eye lid is in level with the limbus.
A chin elevation results in eyes moving down or creates lid lifting effect (normal adaptive mechanism seen in ptosis to clear the visual area of the ptosis). However, in these patients with subtarsal foreign body, a chin elevation keep the foreign body further away from the superior limbus or cornea providing better comfort levels. The amount of chin elevation depends on the proximity of the foreign body to the lid margin. Chin elevation is more prominent in Figures 1.31B and C (lid margin) than in (Figure 1.31A).
Importance of everting the upper lid (also discussed at the end of this chapter): Everting the upper lid is often overlooked step especially by residents, beginners and will prove embarrassing, if he or she misses a foreign body and someone else picks it up. To add to more embarrassment to the clinician, it might be possible that a teenager patient can evert and locate it! Most importantly, beyond embarrassment, the timely relief in symptoms for the patient will be delayed and can cause harm if retained for longer periods.
This new sign can probably be added to the existing ocular causes of face turn. Though experienced examiners will as a protocol evert the lid in such situations, beginners often usually miss this step. A new ocular cause of abnormal head posture in children!
To summarize, the findings of this new clinical sign “abnormal head posture caused due to upper lid subtarsal foreign body”
- A history of trauma may or may not be elicited
- Face turn (contralateral)
- Chin elevation
- Nasal bulbar congestion
- No abrasion or healed abrasion in the cornea (nasally)
- Patient resists attempted measures to straighten his face
- Attempts to straighten the face are accompanied by an immediate attempt to avoid blinking, widening of the palpebral fissures in both eyes and lacrimation on blinking
- The severity of the face turn depends upon the location of the foreign body. More central is associated with more severe face turn
- The severity of the chin elevation is dependent on how close or far the foreign body is located from the lid margin
- Subtarsal foreign body on eversion
- Restoration of normal head posture following removal of foreign body
- Presents or mimics as gaze palsy in infants with subtarsal foreign body!
Children have an amazing adapting ability and innovation. What an amazing idea!
This sign is described by the author after his personal observations of several patients over several years.
Nonocular Causes
- Unilateral hearing impairment
- Sternocleidomastoid contracture
- Habitual
- Psychogenic.
Practical Points
- Presence of ocular cause of head posture in a child usually indicates presence of binocularity and is a good sign
Facial Asymmetry
There are many important considerations in patients with facial asymmetry (Figures 1.32A to F).
- Anisometropia is common with facial asymmetry
- Asymmetric orbital growth due to microphthalmos and anophthalmos results in facial asymmetry
- Long-standing or congenital IV cranial nerve palsy results in facial symmetry
- Patients with facial asymmetry tend to have strabismus more common, e.g. Duane syndrome
- Patients with plagiocephaly (premature unilateral closure of the coronary suture) present with a pseudoparalysis of the ipsilateral superior oblique due to retroplaced position of trochlea
- Children postenucleation if not fitted with orbital implants tend to develop facial asymmetry
- Unilateral radiation therapy to head and neck tumors in a young age can result in facial asymmetry
- Facial asymmetry can be confirmed by measuring the distance between outer canthus and angle of mouth on either side
- Perform Parks’ three-step test in all patients with facial asymmetry. It could pick up congenital superior oblique palsy.
Examine for Specific Behavior
- Oculodigital sign (Figures 1.33A and B)
- Finger waving.
These signs indicate poor vision. Compare the eye size and look for asymmetry in orbit size, eye size, heterochromia, anisocoria and other indices (Figures 1.34 and 1.35).
Bruckner Test
Bruckner test (Figures 1.36A to C) is a simple test to detect
- Media opacities: Cataract, vitreous opacity
- Gross refractive error: Dull reflex
- Hirschberg test can be performed
- The deviating eye appears brighter
- Anisometropia: Difference in refractive error.
In a dimly illuminated room, in an undilated state, from a distance of 1 m, distant direct ophthalmoscopy is performed with light falling on both the pupils at the same time, and the examiner observing looking through the ophthalmoscope.
The following are observed:
- Corneal reflex (Hirschberg)
- Brightness comparison between the eyes
- Media opacity.Pupil reaction can also be performed from a safe distance as part of the examination by quickly moving the ophthalmoscope.
- In a child with eye injury, it is easy to assess the red reflex from a distance
- Additional information can also be obtained by examining the patient in different gaze positions in dilated state. This can be done at the end of the clinical examination after cycloplegic refraction
- It is one of the tests that can be performed by pediatricians as a screening test for refractive errors, strabismus, media opacity and leukocoria.
Sequence of Examination
The sequence of examination is shown in a continuous sequence of photographs. The readers are advised to read the following sequence first and then look at the photographs in a continuous flow to ensure best results (Figures 1.37 to 1.48). The detailed technique is described in corresponding chapters.
- Bruckner test (Figures 1.36A to C)
- Corneal light reflex test (Hirschberg test) (Figure 1.37)
- Look for lagophthalmos (Figure 1.38)
- Cover/uncover test and alternate cover test (Figure 1.39)
- Ocular motility (Figure 1.40)
- Pupil examination
- Anterior segment examination (penlight) (Figures 1.41A and B)
- Specifically looking for punctum position and apposition
- Anterior segment examination by slit lamp (Figures 1.42A and B)
- Undilated fundus examination (Figures 1.43A and B)
- Noncontact tests (fields, color vision, intraocular pressure assessment) (Figures 1.44, 1.45A and B)
- Diplopia charting/fusional amplitudes (Figures 1.46A and B)
- Gonioscopy in indicated patients
- Dilation
- Cycloplegic refraction (Figures 1.47A and B)
- Dilated fundus examination with indirect ophthalmoscope (Figures 1.48A and B). Examiner should be aware of the side effects of ocular medication (Figure 1.47B)
Examination: Practical Tips
- The anterior segment can be examined by the magnification provided through aphakic glasses of the patient
- A magnifying device can be used to examine the eye
- When the anterior segment of a small child is examined with restraint, the pupil reaction can also be observed by closing and opening the lid with the light focused on the eye continuously rather than moving the penlight to and fro from the eye2930Figures 1.32A to F: Facial asymmetry. (A) A patient with persistent fetal vasculature of the right eye (pseudophakic) and microphthalmos. Note that the facial asymmetry on the right side. (B) An adult patient with congenital right superior oblique palsy. Note the gross facial asymmetry and it appears, as if the patient has been photographed from side despite the patient has been photographed from straight ahead. (C) The patient is same as in Figure 1.15. (D) A patient with right superior oblique palsy and left head tilt. Note the distance between the outer canthus and angle of mouth is lesser on the normal side. The face is flatter on the normal side. (E) Facial symmetry caused by clefting syndrome. (F) Progressive hemifacial atrophy (considered as a localized form of scleroderma) causing a facial asymmetry predominantly affecting the upper half of the faceFigures 1.33A and B: Oculodigital sign. (A) A child with Leber congenital amaurosis. (B) A child with bilateral microcornea and congenital cataract.Even before starting the examination, one can observe these findings, which gives a clue as to the cause of poor vision. Oculodigital sign is usually common in Leber congenital amaurosis/retinal causes of blindness, and sometimes in cataract also. Very poor vision sometimes can result in this behavior31Figures 1.34A and B: Eye size differences. (A) Right eye phthisical eye—this presented with bilateral retinoblastoma in child, with phthisis of the more affected right eye (Group E). (B) High myopia in left eye (anisometropia) making the left eye obviously larger than the right eye. This child also had myelinated nerve fibers of the left eye (rabbit eye syndrome)Figures 1.35A and B: Heterochromia iridis. (A) Child with persistent fetal vasculature and cataract of the right eye with heterochromia. Note the iris is darker in the left eye. (B) Child with heterochromia iridis. There is no anisocoria. Both heterochromia and anisocoria can be genetic and hence on should examine the siblings and parents.A simple way to remember the terminology is iridiS is used if with in the same eye—Singular—S for singular, and iriduM is used if it is in both eyes—M for multiple.Sequence of examination:Each of them is described in detail under different chapters. A brief note to provide the sequence of examination is given here in this chapter32Figures 1.36A to C: Bruckner test. (A) Note that the round light of the direct ophthalmoscope falling on both of eyes. (B) A child with retinoblastoma showing leukocoria of the left eye. (C) Another child with nystagmus and esotropia of the left eye and a left face turn showing more brightness in the left eyeFigure 1.37: Hirschberg test/Corneal reflex test. After observing head posture, facial asymmetry, ocular asymmetry one starts the following examination. By this stage ptosis, heterochromia, anisocoria, nystagmus, conditions associated with facial asymmetry are picked up. The corneal light reflex is observed for any asymmetry and any deviationFigure 1.38: Lagophthalmos. This is one of the most commonly missed findings, if one does not specifically look for, in every patient as a routine part of examination. At this stage, one picks up lagophthalmos and subtle cranial nerve VII palsy. Blinking is observedFigure 1.39: Cover/uncover/alternate cover test. It is done for distance, near, with and without correction, with an accommodative target. The presence/absence of strabismus, eccentric fixation, pseudostrabismus, Angle kappa is detected at this stage. Diagnostic positions are looked specifically if there is a strabismus33Figure 1.40: Ocular motility. Following cover test, one checks the motility looking for pursuits, saccades, convergence and also noting any palpebral fissure changes with motility. Any pain, discomfort or complaints of double vision is asked and documented. Ductions are performed if there is any limitation of motility on versions. The chances of missing a Brown syndrome are very high if one does not examine the motility regularly in all gaze positions. Each time the examiner should bring the penlight to the primary position before moving to the respective diagnostic positionsPupil examination Anisocoria is specifically looked for and any signs of aberrant regeneration are noted. A relative afferent pupillary defect (RAPD) is specifically looked. At this point of time one gets the severity of the eye problem. One has to correlate the presence of RAPD with the visual acuity. Horner syndrome, third cranial nerve palsy, traumatic mydriasis, RAPD, Holmes-Adie pupil, accommodative spasm are picked up in this stageFigures 1.41A and B: Examine the lacrimal puncta in naked eye and pressure on the sac area. Normally, the lower lid puncta should not be visible without everting the lid margin and should be visible to naked eye. The photograph shows examination of the puncta in a patient with complains of wateringFigures 1.42A and B: (A) Slit lamp examination. Most of the lid, corneal, ocular surface disorders, and iris and lens abnormalities are picked up at this stage. (B) Handheld slit lamp examination is very valuable as an examination tool in smaller children. Only draw back is that, since the hands are not free, procedures like foreign body removal cannot be done with slit lamp held in one hand. Slit lamp examination will tell the need of gonioscopy examination. Performed as a last examination prior dilation
- Fundus examination with restraint is most of the times quick and comfortable on a couch than examining in the lap of the parent, and examiner technique for indirect ophthalmoscope as the child constantly wriggles making examination more prolonged and emotionally disturbing to the parents. There may be limitations imposed by the countries’ medical laws regarding using restraintsThe following examination procedures are described under corresponding chapters.
- Cover test/uncover test and alternate cover test are discussed in the chapter 13 “Strabismus”
- Direct ophthalmoscope, indirect ophthalmoscope and 90 diopter fundus examinations are discussed in the chapter 7 “Pediatric Retinal Disorders and Uveitis”, slit lamp examination is discussed in the chapter 3 “Pediatric Corneal Disorders” and chapter 4 “Iris and Anterior Chamber Abnormalities”, and pupil evaluation is discussed in the chapter 16 “Neuro-ophthalmology (Pediatric and Adult)”
- Intraocular pressure (IOP) assessment, gonioscopy, confrontation fields do not form a part of routine examination of every patient and is discussed in the chapter 6 “Pediatric Glaucoma” and chapter 16 “Neuro-ophthalmology (Pediatric and Adult)”
- Vision and refraction are discussed in the chapter 11 “Visual Acuity Assessment and Amblyopia Management” and chapter 12 “Pediatric Refraction Errors, Contact Lens and Low-Vision Rehabilitation”.
Figures 1.43A and B: Fundus examination (undilated). (A) Direct ophthalmoscopy examination in undilated state. (B) Slit lamp with 90 diopter examination.
This stage of examination will give a clue as to how to proceed next. For example color vision, confrontation fields, orthoptic evaluation if fundus is normal and symptoms point toward orthoptic exercises (near point of convergence and accommodation, and fusional amplitude measurement)
Figure 1.44: Pulsair tonometry. A noncontact pulsair tonometer is used as a baseline in most outpatients. Applanation tonometry in elderly children, tonopen in sleeping child, Perkins in cooperative children and those planned for examination under anesthesia. In patients where intraocular pressure (IOP) monitoring is mandatory like in trauma, glaucoma, uveitis, applanation is preferred modality of IOP measurement
Some Practical Tips
The points to be noted when a child is sleeping is discussed below. Sometimes, a sleep examination can be as valuable as an examination under anesthesia (anesthesia at no cost and no risk!).
Sleeping Child
- If the child is sleeping, do not wake the child immediately
- Red reflex, refraction, anterior segment examination, IOP assessment by pulsair tonometer, and sometimes indirect and direct ophthalmoscopic examination can be completed with ease in the sleeping child. Do not forget to check the presence of Bell's phenomenon (Figures 1.49A and B)
- Do not ever see a sleeping child and manage the patient without waking him or her. The single most important factor on which the entire treatment is made is vision and clinicians need to wake the child
- Ask the parents to wake the child and ask them to come after 5–10 minutes. Imagine how anyone would feel, if someone wakes him or her from a deep sleep and shows a bright torch at their eyes. Anyone would be irritated and so does the child. “Pupil evaluation needs to be done in awake state” though some information can be available in a sleeping child.
Crying Child (Figures 1.50 and 1.51)
- When a child starts crying, residents and beginners often panic. Do not panic, and ask the parents to bring the child after sometime35Figures 1.45A and B: Confrontation fields examination. (A) The child observes a toy in the right hand of the examiner and slowly another toy is brought in the periphery. (B) The child suddenly shifts attention to the toy in the examiners left hand indicating the fields are grossly normal. Proper confrontation fields are done in children suspected to have neurological and neuro-ophthalmological conditions. Note that the mother was even faster to remove the child's cap when the child was still seeing the toy held in the left hand. It was ensured and reconfirmed that the child did not move to see the object on his right, because of the distraction caused by removal of cap
- Try to examine for things like deviation of angle of mouth, Bell's phenomenon, whether tears roll
- Subtle features of Down syndrome can be brought out when the child cries
- Ask the mother to breastfeed and usually the child goes to sleep if the cause was hunger. In fact, this technique is used by many pediatric ophthalmologists routinely
- If child cries incessantly, give a break to the child and clinician should also take a break. It might work sometime later. Author usually asks the parents to stay in his visibility, and take sudden glances to examine the child.
Injured Child
- First rule out any life-threatening injuries
- Then reassure the child
- Get history from parents and from the child if the child can speak. Many children are afraid of parents and hide the truth. Ask leading questions to the child and parents will be shocked when their children elaborate the nature of injury. If necessary, interview the child alone especially in adolescents36Figures 1.47A and B: Cycloplegic refraction. (A) Photograph shows refraction being done. (B) Flushing due to medication is obvious in this patient with albinism. Cyclopentolate, tropicamide and once again cyclopentolate is used, and refraction is performed at about 45 minutes. Cyclopentolate is not used in children with seizures, and undiagnosed and progressive neurological disorders. Atropine is not used in Down syndrome (homatropine and tropicamide is used). Digital occlusion of the lacrimal pathway is advised in children to reduce the systemic absorption and subsequent side effects of ocular medicationsFigures 1.48A and B: Indirect ophthalmoscope examination. (A) Photograph shows the indirect examination couch in the form of elephant, making it more child friendly asking the child to get on the elephant for a ride. (B) The height of the patient is compared on the table, and stars on the ceiling help in getting cooperation for examination. Indirect ophthalmoscopy being performed
- Red reflex as a first step in a child with trauma and will tell many things before touching the child like vitreous hemorrhage, cataract, red glow, white glow as in endophthalmitis. Even relative afferent pupillary defect (RAPD) could be detected with experience (Cox method) and all from a safe distance!
- Rule out an open globe injury. If open globe, ask immediately for the time of intake of food or water. The parents should be advised to have their child NPO (nil per oral) and the clinician should document the time at which clinicians have examined the patient as NPO time. Do not take the time mentioned by parents unless they have been specifically instructed by a health care professional who had explained the requirement of surgery and hence the NPO status.
Feeding the Child
Giving a chocolate to the child and observing the child chewing or eating the chocolate can help the clinician in the following situations:37
Figures 1.49A and B: Bell's phenomenon. (A) Child sleeping with eyes closed. There is no nocturnal lagophthalmos. (B) Gentle opening of the eyes show upward rotation of the eyeballs (Bell's phenomenon intact)
Figure 1.50: Left facial cranial nerve palsy. Photograph shows a crying child with deviation of the angle of mouth to the normal right and also note the severe exposure keratopathy with secondary infective keratitis in the left eye
- Marcus Jaw winking phenomenon
- Crocodile tear in Duane retraction syndrome
- Getting the child to sleep
- To measure the intraocular pressure
- When there is incessant cry due to hunger. No child with hunger will be able to cooperate for a good examination. Ask the parents to get the child something to eat and then examine. Ensure that the patient does not require an examination or procedure under general anethesia before saying so.
Examination with Restraint
Sometimes it might not be possible to get the cooperation of the child despite the best efforts of the examiner and examination cannot be compromised quoting difficulty in cooperation.38
Figures 1.51A and B: Down syndrome. The features of Down syndrome become more obvious in a child when the child cries. One should not make the child purposefully cry as most of the time it is evident. It is of academic interest
If all methods of playing and cajoling fail, one has to examine the child with restraint. Though in some countries, laws might be different regarding such procedures including restraint for fundus examination. The risk of general anesthesia should be weighed (Figures 1.52, 1.53A to E).
Indications
- Examination of anterior segment
- Removal of superficial foreign body
- Examination of fundus.
Contraindications
- Suspected open globe injury
- History suggestive of vitamin A deficiency and severely malnourished child
- Children above 3 years.
In the conditions that have been mentioned as contraindications, there is risk of extrusion and expulsion of intraocular contents if the child is restrained. Examination with constraint should be avoided in children of more than 3 years as they are strong enough and may not be able to apply restraint.
Children between 6 years and 8 years can be usually be convinced by friendly talk and briefing the procedure, and cooperation can most of the time be obtained. It is usually difficult to examine children between 3 years and 6 years who have an acute painful eye condition. The ophthalmologist should avoid anytime if he or she thinks, it might not be possible to examine with restraint and prefer an examination under anesthesia. The most important thing that one needs to know should quickly be assessed.
Glance from Distance
Sometimes children might already be crying before they come to examination chair. In that situation, after observing those features to be examined in a crying child, one can ask the parents to take the child and stay within the room but away from the examiner with the mother holding the child, and then slowly turning away from the examiner but showing the child as shown in (Figures 1.54A and B). It gives an opportunity for the clinician to see the child from a safe yet observable distance. Examine the parents and siblings if one is examining a child with a possible dysmorphic or genetic disorder.
Examining a Dummy
Many times, it becomes easier to get the cooperation of the child after faking an examination with a dummy.39
Figure 1.52: Examination with restraint. The child's hands are raised above the head as shown and gentle restraint is applied with the thumb on the forehead preventing head movement. The parent's consent for such an examination is obtained. The parents are explained that it causes minimal discomfort but no pain to the child though the child might cry.
Quick examination of anterior segment, minimal invasive procedures like removal of pseudomembranes, superficial foreign body removal are carried out in this fashion. Fundus examination can be performed in a similar fashion. Intraocular pressure (IOP) measurement should not be done by this method as it defeats the purpose as IOP increases on straining
Figures 1.53A to E: Examination with restraint. The photograph is self-explanatory and shows how an infant can be restrained for examination
It works wonders to have a small dummy child in the examining room (Figures 1.55A to E).
Getting Assistance from the Parents
Sometimes, it is necessary to get the help of the parents in assisting for examination. Some children feel comfortable if the mother holds the head (Figures 1.56A to C).
- This is especially valuable in examining ocular motility and measurement of deviations
- Holding objects during fundus examination
Holding of the head is done after the head posture has been assessed.
Patching
Patching of an eye with a patch as part of an outpatient examination is done in the following clinical situations:
- To differentiate and demonstrate to parents, as to whether the adopted head posture is ocular or non-ocular
- To demonstrate to parents that there is no visual potential in a blind eye or an eye with the tumor to achieve better compliance with treatment protocols
- To measure the maximum deviation in intermittent exotropia
- To judge the child's behavior during patching therapy at home by an office assessment.
Examining Old Photographs
Tips
- Ask for old photographs of different ages, so that clinician can approximate the age of onset of problem
- Most adults might have a driving license or National Identity Card with them
- Children might have school ID photographs. But most of them are of recent and may not be very helpful
- Parents might have their children's photographs stored in mobile cameras
- Examine the photographs with a magnifying lens
Examining the old photograph is useful in the following conditions:
- Ptosis: Chronic progressive external ophthalmoplegia (CPEO) (Figures 1.61A and B), ocular fibrosis syndrome, oculopharyngeal muscular dystrophy and myasthenia
- Strabismus: To know whether intermittent exotropia or constant exotropia (Figures 1.58A to D). Decompensated microtropia presenting as an acquired esotropia as complained by parents, age of onset of strabismus (Figures 1.62A to D), photophobia and eye closure (Figures 1.58A to D), planning for strabismus re-surgery where the patient does not remember the direction of deviation prior surgery
- Abnormal head postures (Figures 1.57A to F, 1.60A and B)
- Anisocoria
- Heterochromia
- Any affected siblings or family members
- Leukocoria
- Comparing size of lesions.
In fact, it is important to ask the local photographers to tell the parents to take the child for an ophthalmologist consultation if they notice anything mentioned above. This helps in a very long way in picking up vision threatening, and sometimes life- threatening conditions.
Examining the family album, (Family Album Tomography)! FAT scan sometimes is more valuable than other scans.
Importance of Taking Photographs in Clinical Practice
With recent increase in medical litigations, it is absolutely necessary to document with photographs and the author also uses it as a very important tool in educating his patients.
“Seeing helps in believing, believing results in trust, trust enhances compliance with treatment, compliance with treatment maximizes successful outcome.”41
Figures 1.55A to E: (A) Dummy/mannequin. (B) Child following first postoperative day comfortable enough for a smiling photograph (the dummy baby does the trick). (C) The child is allowed to play with the dummy. (D) The dummy child is then examined with handheld slit lamp. (E) Subsequently the child cooperates/enjoys for a slit lamp examination
Figures 1.56A to C: Parents’ assistance. It is sometimes important to get the support of the parents for holding the head after assessing the head posture. (A) Photograph shows a child with left esotropia but the child also has a right face turn. (B) Photograph shows the correct method of stabilizing the head by the mother for further motility examination. (C) Another child whose head is stabilized by the mother. This child has right eye inferior oblique overaction caused by a right congenital superior oblique palsy. Parents’ assistance in holding the child's head is very useful for examining ocular motility and measuring the deviations
Figures 1.57A to F: Facial asymmetry in left congenital superior oblique palsy. (A to C) Sequential photographs of the same child with left congenital superior oblique palsy. One can observe that there is no significant facial asymmetry, but probably a subtle head tilt to the right in Figure A. (B and C) Photograph shows the gross facial asymmetry and right head tilt. (D and E) Photographs show despite patching either eye, there is no full correction of the head posture indicating secondary skeletal and muscular changes in the neck. Most importantly, the patient in Figures A to C had binocularity. He lost the adopted head posture in Figure D due to manifest deviation and loss of binocularity (note the left hypertropia). Sleeping with alternate face postures from early childhood possibly reduces the facial asymmetry
The digital camera is an important tool in any examination and the author considers it as one of his important ophthalmology equipment in addition to the array of instruments, lenses that all clinicians use.
Written informed consent or atleast an oral consent is obtained from every patient prior taking photograph. The author uses the digital camera for the following purposes. The list is very long but some of the most important reasons are:
- Demonstrate the problem that the patient has as in subluxated lens, microcornea, micropthalmos and facial asymmetry
- All injury patients as documentation for medicolegal purposes
- To compare the progress of treatment as in vernal keratoconjunctivitis (VKC) with supratarsal steroid injection
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44Figures 1.58A to D: Intermittent exotropia. (A) Photograph shows a child with Left eye exotropia. (B) A photograph taken a year before shows left exotropia. (C) Photograph of the same boy 3 years before shows a minimal exotropia. Just fusion breaking up at the time of photography flash. (D) Same boy 5 years before closing his left eye in bright sunlight, which indicates it was intermittent and increasingly with age, the loss of control and constant exotropia. He has amblyopia in his left eyeFigures 1.59A to C: Onset of head tilt. (A) Photograph of a 5–6 months old infant showing right esotropia. (B) Same child at 10 months of age showing left head tilt. This photograph was taken by parents with someone holding the child from behind the screen. (C) The same child at 2.5 years of age showing classical left head tilt in right superior oblique palsy. Same patient as in Figure 1.56C
- Sometimes in outpatient department (OPD), where prolonged examination of a child, for example a child with suspected supratarsal foreign body, is difficult even with restraint. A quick photograph of the upper tarsal conjunctiva and then examining at higher magnification in a computer monitor scanning every area is possible
- To explain the motility problems in strabismus
- Comparing the preoperative and postoperative photographs to show the results to the patient and attender. For some reason, some patients despite good alignment are not convinced with the outcome of the surgery. It will prove very useful if the photographs have been taken
- RetCam images, to educate the parents as in retinoblastoma and ROP where parents are not aware of what is happening inside the eye, and to update them above the course of treatment, response and the need for repeated lasers and cryotherapy
-
Figures 1.61A and B: Chronic progressive external ophthalmoplegia (CPEO) and mitochondrial myopathy. This patient presented with what he described as “sudden onset of ptosis, double vision and limitation of eye movements”. His old photograph showed he had mild ptosis earlier in both eyesFigures 1.62A to D: The time of onset of esotropia. (A) Photograph of a girl child at 6–7 months of age showing no obvious strabismus. (B) Same child at 2 years of age showing no squint. (C) Same child at 2.5 years of age showing right esotropia. (D) There is still an esotropia with full correction. The child has partially accommodative esotropia. Old photographs can document the onset of a deviation
- Preoperative counseling using photographs of similar eye conditions of earlier patients.
Scanning the Patient
Many times one has to quickly steal a look at the child's hands, toes for polydactyly, syndactyly, club foot and specifically scan if there are dysmorphic features (detail is discussed in the chapter 18 “Systemic Disorders”)
Clinical findings likely to be missed if:
- Clinician does not ask to remove the scarf/cap/head band
- Scalp infections
- Herpes zoster lesions
- Dysmorphic features
- Scars of prior neurosurgery
- The upper lid is not lifted (Figures 1.64 to 1.66)
- Bleb (Figures 1.65A and B)
-
Figures 1.64A to C: The importance of lifting the upper lid. Left eye hyperolean and band-shaped keratopathy. (A) Photograph shows exotropia of the left eye with cataract. (B) Lifting the lid shows silicone oil in anterior chamber. (C) Slit lamp photograph showing band-shaped keratopathy and inverse hypopyon or hyperolean47Figures 1.65A and B: Congenital glaucoma (bleb). (A) Photograph shows a child with bilateral increased corneal diameters and haziness of cornea in the right eye. This child has bilateral congenital/developmental glaucoma. (B) The same child showing the bleb in the right eye. A bleb can be easily missed in the examination if the lid is not lifted upFigure 1.66: A boy postcataract surgery with scleral thinning and cystoids cicatrix formation which is evident after lifting the upper lidFigures 1.67A and B: Eversion of the upper lid. (A) Cobblestone appearance; photograph shows cobblestone appearance in vernal keratoconjunctivitis. (B) Supratarsal foreign body; photograph shows a vegetable foreign body in the upper lid. It is important to scan the everted upper lid in slit lamp under high magnification and vary the direction of illumination to look for foreign body
- Silicone oil in anterior chamber (hyperolean) (Figures 1.64A to C)
- Tube shunt
- Floppy eye lid syndrome (when lifting, feel the laxity and look for spontaneous eversion)
- The upper lid is not everted (Figures 1.67A and B)
- Subtarsal foreign body
- Cobblestone
- Arlt's line in trachoma
- The lower lid is not pulled down (Figure 1.68)
- Symblepharon
- Subconjunctival fibrosis
- Trace hyphema and hypopyon
- Concretions.
Sometimes parents try to hide certain information and clinical findings (Figures 1.69A and B). Observe the brow for very subtle scars. Traumatic optic neuropathy (Figures 1.70A and B)
It is important to access how children with poor vision move around in clinic. It is very important to judge how independent the child is especially in nonprogressive cause of low vision (Figure 1.71).48
Figures 1.69A and B: Glancing. (A) A child with unilateral retinoblastoma affecting the left eye. (B) Note that the child also has absence of four fingers in the left hand (clue to probably genetic retinoblastoma). The father had purposefully covered the left hand to avoid the defect being seen. Many times parents try to conceal or may not volunteer to disclose other systemic abnormalities thinking that there might not be any association and therefore do not think it is necessary to tell to an eye specialist. A quick glance will help in making the further systemic examination that can be done after stressing the importance of systemic examination. This will help in collating the findings and arriving at a more meaningful diagnosis
Figures 1.70A and B: Brow scar in traumatic optic neuropathy. (A) This patient presented with poor vision in his right eye. Photograph shows an exotropia of his right eye. (B) Side profile of same patient showing brow scar in outer eye brow. A classic site for traumatic optic neuropathy. The patient also had a pale disk and a relative afferent pupillary defect (RAPD) of the right eye. It is important to search for brow scars in unilateral optic atrophy especially in smaller children as the injury perse need not cause loss of consciousness, to be remembered as an incident. A history of injury might be easily forgotten if there has been no bleeding, loss of consciousness or an injury not requiring hospital admission
Figure 1.71: Examining the ability of the child to move around. The photograph shows a child moving to the chair. The ability of the child to move around in unfamiliar surrounding without bumping indicates reasonable ambulatory vision. If the child is not able to move around in unfamiliar surroundings, the child will need someone to escort all the time
Figures 1.72A and B: (A) Photograph shows that most children are very cooperative for an examination. This child is attempting to push her head to aid the examination at the slit lamp. So if someone says that the child is not cooperative, probably the examiner needs to modify his or her approach to get better cooperation. (B) The fingers of the examiner can be placed as a chin rest which will help the examiner to control the height of the head. Here the child rests the chin on the examiners fingers which can lift the head of the child to the desired height for comfortable examination as some child feel uncomfortable to place the chin on the chinrest
Almost all children are very cooperative for an examination except a very few children (Figures 1.72A and B). That number also comes down if the examiner takes sometime to talk to the child. Only very few children because of their ocular status or because of the fear pose some difficulty to the examiner. The examiner should not hesitate to examine under sedation or anesthesia if the clinical situation necessitates so.