Pediatric Surgery: Diagnosis and Management (2 Volumes) Devendra K Gupta, Shilpa Sharma, Richard G Azizkhan
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1Basic Pediatric Surgery2

Pediatric Surgery— Overview of the SpecialtyCHAPTER 1

DK Gupta
The mere formulation of a problem is far more essential than its solution, which may be merely a matter of mathematical or experimental skills. To raise new questions, new possibilities, to regard old problems from a new angle requires creative imagination and marks real advances in science.
—Albert Einstein
Recently, when pediatrics was separated from the general medicine, a strong need was also felt, though much later, that children need the services of a specialized surgeon to deal with their surgical problems. The reasons being the different anatomical and physiological parameters, smaller size of the organs needing intricate surgical skills, special dosage schedules, difficult venous access, and especially the spectrum of surgical diseases much different from those seen in adults.
The World Federation of Associations of Pediatric Surgeons had signed a document during the council meeting of the World Federation of Association of Pediatric Surgeons (WOFAPS) held in Kyoto, 2002, to protect the rights of the surgical child. It is popularly known as “Kyoto Declaration”, reproduced as below:
  • Children are not just small adults and have medical and surgical problems and needs that are often quite different from those encountered by adult physicians. Infants and children deserve the very best medical care available. Every infant and child who suffers from an illness or disease has the right to be treated in an environment devoted to their care by a pediatric medical or surgical specialist.
  • Pediatric Surgeons are specially trained physicians with extensive experience and expertise in treating infants and children of all ages (from birth to adolescence) with surgical disorders. Because of their unique training, pediatric surgical specialists provide a wide range treatment options and the highest quality care to children.
  • Pediatric Surgeons diagnose, treat, and manage children's surgical needs including: surgical repair of birth defects, serious injuries in children, childhood solid tumors, conditions requiring endoscopy and minimally invasive procedures, and all other surgical procedures in children.
  • In order to provide the best surgical care for infants and children, complex pediatric surgical procedures should be carried out in specialized pediatric centers with appropriately equipped intensive care facilities staffed 24 hours per day seven days per week. In addition to the trained pediatric surgeons, these facilities should be staffed with other pediatric specialists including radiologists, anesthesiologists and pathologists. These specialized centers should provide postgraduate education and research.
There are many children's hospitals and pediatric centres in the developed world that are celebrating their 200 years of establishments. However, developing world is yet to have adequate facilities to treat large volume of work related to congenital malformations, trauma, tumors, transplants, infections and many other pediatric surgical problems.15
 
WHO SHOULD DO PEDIATRIC SURGERY?
Only the Pediatric Surgeons who are qualified by virtue of their training in the field, should tackle the surgical cases in newborns, infants and children up to 4adolescents. There are however, many variations depending on local circumstances, practices and compulsions. Many institutions with a heavy workload, would accept children only up to 12-year of age. However, all newborns with emergencies (Acute Intestinal obstructions, respiratory distress) would normally be referred to the qualified pediatric surgeons who are all trained in the sub-specialty of newborn surgery.6 In absence of availability of the trained pediatric surgeons in all the centers, at all times, in all sub-specialties, the common pediatric surgical problems have been treated by general surgeons. This is true even in developed world. In the best interest of the patient, the treating general surgeon dealing with such routine cases should not only be familiar with the latest approach but also should have enough experience dealing with their special requirements.
 
WHAT IS THE SCOPE OF PEDIATRIC SURGERY?
Parents are becoming now more aware about the scope and the postsurgical results. Pediatric surgery is a wide field and deals with most body parts and organs in newborns, infants and children. These include various sub-branches like; neurosurgery (spina bifida, hydrocephalus, cranisynostosis), head and neck lesions, thoracic surgery (various congenital lung anomalies, empyemas, hydatid cysts, eventration of diaphragm, congenital diaphragmatic hernia, tracheo-esophageal fistula, esophageal replacements using gastric tube, colon transposition, stomach pullup, and rarely the tumors), abdominal surgery related to the disease of the liver, biliary tract, spleen, large and small bowel, anorectal malformations, Hirschsprung's disease, abdominal wall defects, undescended testis, hernia, torsion of testis, and others), plastic surgery (dealing with defects like cleft lip and palate, hypospadias, syndactyly, reconstruction of various flaps), urologic surgery (pyeloplasty, ureteric reimplantation, reconstruction of epispadias and exstrophy complex, vesico-intestinal fissure, partial and complete nephrectomies, urinary bladder augmentation, colon conduits and many more). Even in centers with well developed sub-specialties, a general Pediatric Surgeon is allowed to operate on patients with neonatal emergencies, trauma and routine surgical procedures to remain in stream.
Thus, the spectrum of the pediatric surgery is very wide and covers whole body except the open heart surgery, brain tumors, orthopedic anomalies, ophthalmology and ear, nose and throat. Presently, there are only few centers, mostly in developed nations, where the pediatric surgical specialists devote most of their time to a particular sub-specialty and they are satisfied with the volume as well the returns. Others would like to continue as general pediatric surgeons to cater to large spectrum of diseases. In the interest of patient care it is important now to define and develop the sub-specialties as the distinct disciplines, e.g. urology, neurosurgery, hepatobiliary, oncology, thoracic surgery, and may be in the neonatal surgery also.7 This may be feasible only in fully funded centers and the teaching institutes of national importance.
 
WHERE SHOULD PEDIATRIC SURGEONS FUNCTION?
Congenital anomalies differ from region to region and so the spectrum of anomalies seen in a developed country differ from those in a developing countries. Ideally each newborn should have the facility of being seen by a pediatric surgeon without having to travel long distances. In many countries, it is not the distance but the mode of transport what is most important. To travel even small distances (from hills, remote areas which are not well connected with roads, air and rail network), parents might take many hours or even days to bring the baby to the nearest center for medical care.
Generally, a group of 3 Pediatric Surgeons is required for a center catering to the general population of one million. Similarly, there should be a neonatal surgical ICU for the same sized population. Though, the future need would be to have Pediatric Surgeons even at the district level to manage general pediatric surgical problems, presently, all medical colleges, referral centers and institutes of national importance should have a teaching department of pediatric surgery with at least 3–5 teachers and supporting staff (resident, nursing and technical staff).
In urban areas, with the good antenatal ultrasonography, a large percentage of anomalies, can be diagnosed well in time and thus can be referred to a tertiary center as the case may be. The types of patients managed in a rural center are different from 5those seen in an urban center. The facilities in a government center would also differ from those in a private set up and similarly the type of surgeries would also be quite different in different places.
 
TRAINING IN PEDIATRIC SURGERY
The pediatric surgical specialty is different from others, requiring involvement, commitment and dedication. Presently, it is neither paying nor glamorous. Only those with a zeal and interest to serve the delicate babies should opt for it. The pattern of training differs in different parts of the world.8 The learning becomes meaningful with the depth of involvement, large volume of workload faced and the surgical experience gained in managing various types of anomalies during the limited period of surgical training.
In most setups the surgeons first trains himself/herself in general surgery to acquire the basic principles and the skill of general surgery. One should then get training in pediatric surgery to acquire experience and finer skills. The period of training also varies from place to place. In India, it is minimal period of 12 years including the medical graduation, to become a qualified pediatric surgeon. However, this does not include the period required for the fellowships (urology, neonatal surgery, oncology, laparoscopy, etc.). As there may be more than one technique for diseases like Hirschsprung's disease, hypospadias, pyeloplasty, esophageal replacement and bladder augmentation. It is ideal that a pediatric surgeon is posted to work under the supervision of at least 3–4 pediatric surgeons to get exposed and experience the different techniques. Then he can adopt the one he feels most comfortable. Training courses should also be imparted to teachers to keep them updated on the subject.
 
AGE GROUP FOR A PEDIATRIC SURGICAL PATIENT
This varies from 12–18 in different parts of the world. Most developed countries however limit the upper age to 18 while developing countries keep it as 12. This depends on the doctor to patient ratio. Countries with limited workload, allow children up to 18 years to be included in the pediatric surgical group, while others with major workload, would like to restrict the admissions only up to 12 or 14 years of age. Despite all this, many a times, patients present very late with pediatric surgical index diseases like Hirschsprung's disease, anorectal anomalies, hepatobiliary disorders, lung pathologies, genitourinary requiring reconstructions and others. These have either been missed or mismanaged in the past. Due to their expertise, it is desirable that the pediatric surgeons should either accept the responsibility to treat index cases, irrespective of age, and operate upon them. Else, they should help their counterparts to achieve the best surgical results.
 
RECENT ADVANCES
Since 1980, the Stem cell research has taken a lead and is currently recognized as one of the main areas of interest to explore its potential in congenital anomalies, renal dysplasias, tumors, cirrhosis, testicular dysgenesis and many more.9 The very fact that the infant's bone marrow is quite rich with mononuclear cells (mostly hemopoetic and less of mesenchymal cells), which if modulated by appropriate regulators (unfortunately not well known till yet) may have the potential to repair or regenerate the damaged tissues in the body.
Minimal invasive surgery and endoscopy with much finer instruments have become popular to suite the pediatric surgical patients.10 However, their indications in newborns and infants still remain limited in general practice. Robotic surgery, though really useful and a preferred tool for deep pelvic surgery (e.g. radical prostectomy in adults and anorectal anomalies in children) has been used even for other procedures like pyeloplasty. Parents are aware of the global developments and demand for laparoscopy and even robotic surgery. These are becoming popular for use in pediatric surgical practice. Telesurgery has also become feasible with surgeons operating from distant places even across continents.
Laser technology has been applied to bronchial, esophageal and urethral valves, renal, ureteric and bladder stones and surface anomalies like hemangiomas. The harmonic scalpel has enabled liver resection, tumor surgery without much blood loss.
Most infants with biliary atresia would ultimately require Liver transplant (Ltp) in almost 80–90% children by 2-year of age, even after an initial successful porto-enterostomy. Currently, biliary atresia is the most common indication for Ltp, followed 6by other indications like, tumors, trauma, and hepatic failure. However, the facility remains limited to developed nations, supported by the governmental or other program for an otherwise very expensive life long proposition for surgery and maintenance of immunosuppression. Though, renal transplant can now be performed even in smaller babies, the problem remains to find a suitable donor to fit in the recipient's small abdomen. With the ongoing research and interest in tissue engineering, it might just take another 5–10 years when the most of the organs in the body of the appropriate sizes and shapes would be made available on shelf for children also.
Indications of fetal surgery have now become well defined with the lessons learnt from the experiences during the past two decades.11 There are few conditions where fetal intervention can improve the morbidity and even the mortality. Small instruments assisted by minimal access surgery have been utilized for thoracic procedures, urinary tract decompression in babies with obstructive uropathy, ligation of the main vessel supplying the large tumors (e.g. Sacrococcygeal tumors), tracheal occlusion in babies with diaphragmatic hernia associated with lung hypoplasia and so on with the development of newer anesthetic agents and proper tocolysis, fetal surgery has been considered safe without much risks to both the fetus and the mother.
The management of pediatric tumors has been revolutionized both in investigations and in treatment. Positron Emission Tomography has been recognized as an important new modality of treatment.12 It is now possible to cure at least some of these if not all. Renal tumors, lymphomas, are a good example with almost 80–95% survival. Newer chemotherapeutic drugs and regimes are not only more effective but with less side effects also. Sarcomas need not have amputations any more. Radiotherapy is also considered only in selected cases if the chemotherapy is not sufficient. Intraoperative implants or brachytherapy can be considered in children with residual tumors, e.g. neuroblastoma, so as to have less of side effects usually associated with postoperative wider field of radiation. Detection of new microarrays may help in newer ways to classify tumors and detect their recurrences.
The electronic world of computers has not only improved the communication faster, easier and economical but also the skills amongst the experts. Many offices have already gone paperless. Telemedicine and teleconferencing has brought the whole world close to each other, enabling bilateral exchange of ideas from different centers and different parts of the world at an affordable cost.
 
PEDIATRIC SURGICAL SPECIALTY IN INDIA
The exact past of the specialty of Pediatric surgery is not known. However, in India, it may date back to ancient times. Sushruta, the father of Indian surgery and Plastic surgery had written large volumes of literature and recently retrieved from Turkey, may be as old as 3000 BC (Fig. 1.1). Others feel it is only 600 year BC and may well coincide with the Hippocratic era. Sushruta is known to have the knowledge about pediatric surgical conditions like anorectal malformations, intussusception, a condition resembling Hirschsprung's disease, bladder outlet obstruction in children, vesicoureteric reflux, vesical calculus, phimosis, congenital hydrocele, inguinal hernia and pelvic tumors. He had also reasoned the etiology of intersex due to non-dominance of either the sperm or the egg.
Sushruta also wrote, “Only the union of medicine and surgery constitutes the complete doctor. The doctor who lacks knowledge of one of these branches is like a bird with only one wing.”
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Fig. 1.1: Sushruta who lived in Kasi was an ancient Indian medical practitioner who was one of the first to study the human anatomy
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Thus the need for collaboration between a pediatrician and pediatric surgeon is evident. He understood the high mortality in pediatric surgery in absence of anesthesia and antibiotics and warned that any surgeon should operate upon children only after obtaining due permission from king, lest it will amount to the offense of infanticide.
The pediatric surgical specialty in India, first established in 1965, as a section of Association of Surgeons of India (ASI), has a present strength of more than 800 members and collectively 30–40 pediatric surgeons are added each year from 23 teaching departments. After the improvement seen in the care of patients with trauma, malignancy and serious infections, the infrastructure for providing care to the surgical. Newborns is also improving with the survival of the newborns even with major malformations. Our specialty is very demanding and the facilities, including the trained faculty, nursing and technical staff is limited. The services are also restricted mostly to the urban areas. Most patients have to travel long hours even for short distances.
 
National Scenario
Our National Health Policy draft plan of 1983 was recently revised in the year 2002. The nation spends only 2.6% of its annual budget on health care. It increases to 5.2% if the expenditure incurred on providing preventive public health and hygiene is also included. The Government still has to focus on controlling the communicable diseases through various national health programs and treat malnutrition, diarrhea and infectious diseases on priority. Small pox has been eradicated. Polio is on the verge of eradication. There has been substantial drop in the total fertility rate and the infant mortality rate. Present population in India has already crossed 1 billion and we add 17–18 million newborns each year, almost equal to the existing population of Australia. The life expectancy has also increased from 36.7% in 1951 to 64.6% in year 2000.
Pediatric surgery is new and yet to be recognized for the role the specialty plays in providing quality care and reducing the national Infant Mortality Rate (IMR). Pediatric surgical services are presently limited only to the urban cities with very little or no pediatric facilities available in the rural sector. Also, almost 70–80% of pediatric surgical procedures are being performed by the general surgeons as the pediatric surgical specialists are not available in most centers or the medical colleges. It is aimed at developing pediatric surgery, in each and every Medical college in the country in the near future, and the facility needs to be extended later at the district level.
The main strength of pediatric surgery in India is the massive clinical work load dealing with more than 400 million children less than 14 years of age, constituting about 39% of population. This offers vast training opportunities to the residents in almost all aspects of surgical disciplines except open heart surgery and advanced neurosurgery.
 
Teaching and Training
The teaching program in pediatric surgery in India was started way back in 1969. It was only in 1972 that the American Board of Surgery was approved for the certification in Pediatric Surgery. This was followed by Canada and Great Britain in 1976. The Inter Collegiate Board of Royal Colleges in UK started its examination only in 1990s. India is proud of the postgraduate training program in pediatric surgery, following the British system, is now considered one of the best in the world. India, have not only the intense postdoctoral teaching program, dedicated curriculum and the syllabus but also have the expertise to offer in many sub-specialties.
 
Challenges
The clinical workload in the specialty is immense and is mostly directed to institutions, for seeking free, fair and seasoned service. The private sector is expensive especially for the newborn surgery and malignancy, remaining beyond the reach of the common man. The insurance cover is for less than 2% population. Antenatal care is limited and only 10–15% anomalies can be detected pre-natally. Folic acid prevention therapy is yet to take off. Challenging anomalies like spina bifida, exstrophy bladder, gross hydrocephalus, large abdominal wall defects, multiple anomalies incompatible with life, require ethical considerations. Finally, the job opportunities even for the most qualified and the talented staff are also rare. This has contributed to the professional dissatisfaction and 8sometimes even frustration amongst the younger generation opting for the specialty.
In a vast country like India, with lots of diversity in clinical workload, teaching and the research priorities, we have the following objectives:
  1. To provide specialized pediatric surgical services to over 400 million pediatric population at an affordable cost and safety, ethically and professionally.
  2. To provide rigorous and uniform training including sub-sections, at least in apex and centers of excellence.
  3. To utilize the available limited financial resources to conduct only need based research to understand the common diseases in the region.
India being a vast country, all facilities are not likely to be available in each and every center. The quality of teaching and training also differs from place to place. Most our residents become quite trained and adequately competent after receiving the rigorous 3 year training. To meet any shortcoming, interdepartmental and the interstate exchanges have been established to widen their horizons and also keep the specialty vibrant and attractive. The talented faculty can excel in any field of medicine with proper planning and utilization of services.
In the recent past, the trend has been reversed. Not only the residents but also the faculty from developing and developed countries have been visiting key departments like that at AIIMS, New Delhi for higher training and patient care. This is only going to grow in future, with many teaching departments improving their teaching and training programs.
It is important that all postgraduate students in pediatric surgery are exposed to the research methodology to make them better teachers of tomorrow.13,14 Unfortunately, Animal experiments are conducted only in a few institutions in India. Recently, various Govt. and the non-Government organizations are objecting to the experiments on animals. This has seriously affected the ongoing animal experimental research programs in major institutions. However, animal research is essential, and these should be performed following the strict guidelines as laid down by various ethical committees. Alternately, experiments in the field of molecular biology, stem cell research and Tissue Engineering are quite exciting.
Antenatal diagnosis is still not very common and only 10–20% anomalies are being diagnosed, that to mostly after 20 weeks of gestation, offering limited scope for termination even for major defects.15 Organ transplantation remains a major challenge due to lack of facilities, shortage of donors and the high maintenance cost involved. India also has infrastructure and the well developed expertise in the field of endoscopy, urology, oncology and neonatal surgery but again only in the limited centers.
There is acute need to develop pediatric surgery in all Medical Colleges for teaching and patient care. Also, there should be at least one major tertiary care level center, one in each state. There should be apex centers at the national level to train the teachers and offer advanced surgery like transplant programs, conduct clinical and experimental research in the field of molecular biology, tissue engineering, stem cell therapy and others. These centers should be independent with complete autonomy for efficient delivery of advanced patient care, quality teaching and need based research.
Thus, Pediatric Surgery has come a long way in last few decades.16 It has taken a better shape, yet we have to go a long way to establish ourselves and the specialty, solve many hurdles, develop infrastructure and refine treatment methodologies suiting to each nation.
It is not enough to begin. Continuance is necessary. The reason of failure in most cases is—lack of perseverance.
REFERENCES
  1. Chatterjee SK. Is Pediatric Surgery a sinking specialty? J Indian Assoc Pediatr Surg 2002;7:103–4.
  1. Dorairajan T. Future of Pediatric Surgery. J Indian Assoc Pediatr Surg 2002;7:115–16.
  1. Gupta DK. Pediatric Surgery. Is it a sinking or growing specialty? J Indian Assoc Pediatr Surg 2002;7:105–8.
  1. Rao KLN, Chowdhary SK. Shortcomings of Pediatric Surgery. J Indian Assoc Pediatr Surg 2002;7:109–14.
  1. Gupta DK, Editorial. The much awaited Baby is born with a smile. Journal of Pediatric Surgical Specialties March 2007;1(1):3.
  1. Upadhyaya P Foreword, in Textbook of Neonatal Surgery, Modern Publishers,  DK Gupta (Ed), New Delhi  2000.
  1. Gupta DK, Editorial. Sub-specialization in Pediatric Surgery—Who, When, Where ? Journal of Indian Association of Pediatric Surgeons 2006;11(2):70–72.

  1. 9 Gupta DK. Official document of the Indian Association of Pediatric Surgeons (IAPS)— Recommendations of the Curriculum Committee, for M Ch training Program in Pediatric surgery in India, submitted to Medical Council of India, 1998.
  1. Gupta DK, Sharma Shilpa. Stem Cell Therapy — Hope and scope in pediatric Surgery. Journal of Indian Association of Pediatric Surgeons. July - September 2005:10(3);138–41.
  1. Gupta DK, Editorial. Make Pediatric Laparoscopy a surgeon's armamentarium. Journal of Indian Association of Pediatric Surgeons. October - December 2006:11(4);198–200.
  1. Gupta DK, Sharma Shilpa. Fetal surgery, in Recent Advances in Surgery, Ed. Roshal Lal Gupta, 2006;chapter 6;10:116–29.
  1. Gupta DK, Pathak M, Kumar R, Sharma S, Agarwala S, Bajpai M, Bhatnagar VB. PET-CT in staging and determining treatment response in Neuroblastoma and Rhabdomyosarcoma, Abstracted in the proceedings of the 8th Congress of European Pediatric Surgeons Association. Turin Italy 2007;54.
  1. Gupta DK, Editorial. Research in Pediatric Surgery — Who should light the flame. Journal of Indian Association of Pediatric Surgeons. July-September 2006;11(3):127.
  1. Gupta DK Editorial. Research in Pediatric Surgery in the developing countries. African Jour Pediatr Surg, 2008 (in press).
  1. Sharma Shilpa, DK Gupta. Discrepancies between the Antenatal and Postnatal Diagnosis of Pediatric Surgical Conditions. Journal of Indian Association of pediatric surgeons 2006;11:183.
  1. Pathak IC. Editorial. Is'nt it time to get started? Journal of Indian Association of Pediatric Surgeons. July-September 2005;10(3):135–36.