Indian J Pediatr (August 2014) 81(8):791–796 DOI 10.1007/s12098-013-1273-7 PEDIATRICS IN GENERAL PRACTICE Guest Editor: Bhim S. Pandhi Emergencies in Pediatric Surgery Anup Mohta Received: 15 August 2013 / Accepted: 16 October 2013 / Published online: 17 November 2013 # Dr. K C Chaudhuri Foundation 2013 Abstract Primary care physicians are often required to initially manage the children with surgical emergencies. Many neonates with congenital malformations delivered without supervision may also be managed initially by the family physicians. The role of the primary care physician in such cases should be to diagnose the condition correctly, provide immediate care and then refer the newborn or child to higher centre for appropriate management. Keywords Surgical emergency . Neural tube defect . Anorectal malformation . Intussusception . Congenital diaphragmatic hernia Spinal Dysraphism and Hydrocephalus Spinal dysraphism or neural tube effects are a major cause of morbidity and mortality, and have long term consequences on quality of life of the patients as well as the care givers. These defects occur due to abnormal neurulation during fetal development. The defect can occur from head to the sacral region. Cranial lesions may include a) anencephaly; b) encephalocele (Fig. 1), or c) cranioschisis. Amongst the many types described, common varieties of spinal lesions include a) meningomyelocele; b) meningocele; c) rachischisis (Fig. 2) d) lipomeningomyelocele; and; e) congenital dermal sinus [1]. Most common type seen in clinical practice is meningomyelocele as many severe types result in perinatal mortality. Most lesions can be diagnosed on antenatal ultrasound and appropriate management should be advised to the parents after counseling about the prognosis of the lesion. Severe anomalies are associated with intrauterine death. If seen for the first time after birth, diagnosis can be made on clinical examination. The lesion can appear as a cystic lesion, or open defect with leak of cerebrospinal fluid. Spina bifida occulta may present as skin covered lesion, a sinus, or tuft of hair on the back. Special attention is required to look for neurological sequelae like paresis, fecal and urinary incontinence, and associated hydrocephalus. Head circumference should be measured and monitored for evidence of hydrocephalus. Required investigations include ultrasonography and magnetic resonance imaging [2]. After proper neurological assessment and explaining the prognosis, surgical repair of the lesion can be performed in most cases. It must be explained to the parents that the neurological deficit and hydrocephalus may not improve after surgery but may become apparent or worsen in postoperative period. Hydrocephalus may require insertion of ventriculoperitoneal shunt before or after correction of spinal defect. The parents must be counseled that the condition may occur again in the next sibling but incidence can be reduced by administration of folic acid before conception. Esophageal Atresia with Tracheoesophageal Fistula A. Mohta Department of Pediatric Surgery, Chacha Nehru Bal Chikitsalaya, Delhi, India A. Mohta (*) 28-B, Pocekt—C, S.F.S. Flats, Mayur Viahr Phase-III, Delhi 110096, India e-mail: mohtaanup@hotmail.com This is one of the common but most severe anomaly of the upper gastrointestinal tract. This occurs due to a developmental defect in the foregut. In the most common type, the upper end of esophagus is blind while the lower esophagus communicates with the trachea or bronchus leading to trachea-esophageal fistula. The anomaly is 792 Indian J Pediatr (August 2014) 81(8):791–796 and frothing at mouth. The child may have rapid respiratory rate due to aspiration pneumonia. X-ray after insertion of a nasogastric feeding tube will show coiling of the tube in the neck. Such a child requires immediate suction of all the contents in the oral cavity and throat; stopping of all the feeds, initiation of intravenous fluids and oxygen, and transfer to a tertiary care centre under supervision where surgery can be performed by the pediatric surgeons [4]. Respiratory Distress Fig. 1 Encephalocele associated with many other congenital anomalies described by the acronym VACTERL (Vertebral, Anal, Cardiac, TracheoEsophageal, Renal/Renal and Limb defects). Therefore, in a child suspected or diagnosed to have esophageal atresia, it is important to perform clinical examination and investigations to rule out other associated anomalies [3]. If delivered in the hospital setting, the diagnosis is usually made in the immediate postnatal period. But in case of unsupervised delivery, the diagnosis may be missed and the baby generally presents with failure to accept feeds, cyanosis, Apart from pneumonia and other medical causes, surgical causes of respiratory distress in a newborn include congenital diaphragmatic hernia, trachea-esophageal fistula, pneumothorax, and congenital lung malformations like congenital cystic adenomatoid malformation and congenital lobar emphysema. The child presents with signs of respiratory distress like tachypnea, cyanosis, chest retractions etc. [5]. In case of congenital diaphragmatic hernia, the abdomen appears scaphoid in shape. The apex beat may be shifted to opposite side and one can appreciate bowel sounds in the thorax on the side of hernia [6]. If a case of suspected congenital diaphragmatic hernia requires resuscitation, it is advised to intubate the trachea and ventilate in preference to bag and mask ventilation because the later may worsen the respiratory distress. The x-ray findings are quite suggestive of individual lesions. In diaphragmatic hernia, bowel may be seen in the chest, heart is shifted to opposite side and bowel shadows in the abdomen are decreased. The x-ray in congenital lobar emphysema may mimick pneumothorax. This sometimes results in insertion of intercostal tube drainage without any clinical improvement. In both conditions, it is recommended that the primary care physician should initiate the child on intravenous fluids and oxygen, and transfer the baby to a higher centre for management. Contrary to previous belief of immediate surgery, it is necessary to resuscitate and stabilize the hemodynamic status of the neonate before repairing the diaphragmatic defect [6]. Idiopathic Hypertrophic Pyloric Stenosis Fig. 2 Rachischisis Idiopathic hypertrophic pyloric stenosis is a condition wherein functional gastric outlet obstruction occurs secondary to hypertrophy and hyperplasia of the muscular layers of the pylorus. The condition is more common in males and first born babies. The incidence is about 2–4/1000 in western countries and is less common in India. They usually present with progressive, non-bilious, projectile vomiting in the first month of the life. Untreated, the child may develop hematemesis and a small percentage may develop jaundice. Indian J Pediatr (August 2014) 81(8):791–796 793 Clinical examination reveals a malnourished child with varying degree of dehydration. Due to continued vomiting of gastric contents, there is hypokalemic, hypochloremic, metabolic alkalosis. Urine output is reduced. Abdominal examination shows visible peristalsis moving from left to right, and the pyloric mass may be felt in 3/4th of the cases. For this, an empty stomach child is put to the left breast of the mother, and an attempt is made to feel the mass in the right hypochondrium on deep palpation using left hand as the child is feeding. Ultrasound examination confirms the diagnosis in most cases and contrast studies are usually not required. It is important to adequately resuscitate the infant and correct the electrolyte imbalance before contemplating surgery [7]. Pyloromyotomy remains the standard treatment of choice and can be performed laparoscopically. Intestinal Obstruction Intestinal or bowel obstruction is one of the most common emergencies seen by the primary care physician. The causes of intestinal obstruction are different in neonates and older children but the principles of management remain the same. Intestinal obstruction in the neonates is usually due to congenital anomalies and includes Hirschsprung’s disease, duodenal and intestinal atresia, malroation and volvulus, meconium ileus etc. Common causes of intestinal obstruction in infants and older children include Hirschsprung’s disease, congenital bands and malrotation, intussusceptions (Fig. 3), obstructed internal and external hernia. Tuberculosis and ascariasis (Fig. 4) still remain common causes of intestinal obstruction in India [8]. Detailed description of individual conditions is beyond the scope of this review. Acute intestinal obstruction may present with bilious projectile vomiting, pain, distension of abdomen and failure to pass stools and may vary with the condition. A neonate who presents with projectile bilious vomiting with or without blood, abdominal distension and features of shock should be considered to have malrotation with volvulus and managed accordingly. It cannot be overemphasized that bilious projectile vomiting is a definite sign of acute intestinal obstruction. Occasionally, paralytic ileus associated with other conditions like sepsis, pneumonia, and some drugs may mimick dynamic intestinal obstruction but should be managed conservatively. Plain x-ray in erect position demonstrates multiple air-fluid levels and may show gas under the dome of diaphragm if there is an intestinal perforation. Any child suspected to have acute intestinal obstruction should be kept nil orally; nasogastric tube should be inserted to aspirate the intestinal contents; and the child should be put on appropriate intravenous fluids. A severely dehydrated child is catheterized to monitor urinary output. The child is then transferred to a facility for the appropriate surgical procedure Fig. 3 Operative photograph showing intussusception by a pediatric surgeon. Surgical intervention depends upon the individual condition. Abdominal Wall Defects: Omphalocele and Gastroschisis Omphalocele and gastroschisis are congenital abdominal wall defects which present in neonatal period and require Fig. 4 Intestinal obstruction due to ascariasis 794 Indian J Pediatr (August 2014) 81(8):791–796 closure and gradual reduction followed by closure of abdominal wall [10]. Ventilatory support is required in many cases. immediate surgical intervention. Omphalocele is a defect in which the abdominal contents fail to return to abdominal cavity during fetal development. The contents commonly include intestine and liver which are covered by a membranous sac (Fig. 5a). In gastroschisis, on the other side, intestines protrude through a defect to the right of umbilicus and are usually edematous, thickened (Fig. 5b) and may be atretic [9] . If diagnosed on antenatal ultrasound, the parents should be advised to have the delivery at a health care facility where surgery can be performed for these defects. Initial management of both the conditions includes covering of the lesions and the contents with a moist sterile dressing or a sterile bag; nasogastric tube to decompress the intestinal tract; maintenance of temperature and asepsis; and intravenous fluids to adequately hydrate the neonate. The child with gastroschisis is nursed in lateral position during stay in the hospital and transfered to a centre with facility for neonatal surgery. The treatment includes reduction of the contents in to the abdominal cavity and closure. It is usually possible to close the defects primarily in small omphalocele and most gastroschisis; it may not be feasible to perform primary closure in some cases. In such cases, alternatives include skin closure and creation of a ventral hernia or silo Anorectal malformations occur with an incidence of about 1 in 5000 and are one of the most common congenital anomalies seen by pediatric surgeons. These include a wide spectrum of conditions in both males and females. Most cases present in the neonatal period, however some girls may present in later life with missed or untreated defects. While the most commonly used Wingspread classification divided the anomalies into low, intermediate and high anomalies, the recent Krickenbeck’s classification divides them as major clinical groups and rare regional variants. In males, there is generally complete absence of an anal opening (Fig. 6a) and the child may pass meconium with urine in case of a rectourinary communication. In the girls, there is usually a perineal fistula through which she may pass meconium and decompress (Fig. 6b). It is important to rule out other Fig. 5 a Omphalocele. b Gastroschisis Fig. 6 a Male anorectal malformation. b Female anorectal malformation Anorectal Malformations Indian J Pediatr (August 2014) 81(8):791–796 associated anomalies in a child with anorectal malformation. Anomalies like covered anus, anterior perineal anus, anocutaneous fistula, anovestibular fistula, and anal stenosis can be managed by a primary perineal procedure as a definitive treatment in neonatal age [11]. However, most other conditions require colostomy at birth and divided sigmoid colostomy is the preferred procedure. The child is subjected to radiological investigations like distal colostogram to define the anatomy, micturating cystogram to rule out reflux, ultrasound to rule out renal anomalies and echocardiogram to check for cardiac anomalies. While the low anomalies are managed by perineal anoplasty, posterior sagittal anorectoplasy is the most preferred surgical procedure for other common anomalies. It is important for primary care physician to not explore the local anal site as it may disturb the anatomy and inadvertently damage the sphincter system, and affect the results of definitive repair. Paraphimosis Paraphimosis is a condition wherein the prepuce cannot be pulled back over the glans after it has been retracted back. It generally results due to forcible retraction of the tight prepuce by the clinician or the parents for performing any procedure or cleaning. The retracted prepuce become swollen and edematous and cannot be brought back over the glans in the original position. The condition is quite painful and may be associated with retention of urine. The condition is best avoided by small precautions. A tight prepuce should not be retracted back forcibly with a mistaken diagnosis of phimosis. If retracted, it should be pulled back over the glans after cleaning or the procedure [12]. Paraphimosis can be treated by manual compression of the swollen foreskin followed by reduction of prepuce over the glans. In some cases, needle puncture may be done to express the edema fluid. An emergency dorsal slit or circumcision should be the last option for the management of paraphimosis. The child requires analgesics and antibiotics in some cases. Testicular Torsion Torsion of the testis is a surgical emergency where the spermatic cord twists and results in ischemic damage to the testis [13]. The event may occur even in antenatal period resulting in an atrophic or vanishing testis. In the pediatric age, torsion occurs more commonly in the prepubertal age. The condition presents as severe scrotal pain of acute onset, along with swelling and erythema of the scrotum. It is usually associated with nausea and vomiting. Examination reveals a high tender testis with an abnormal orientation. Opposite testis may also be abnormally positioned. 795 Clinical examination is most important in making a diagnosis of torsion of testis. Epididymoorchitis and some other testicular inflammatory conditions may mimick torsion. Torsion can be confirmed on Doppler ultrasound and nuclear scan. However, this is one emergency where surgical intervention may be required even with a doubtful diagnosis. Delay should not be done for the want of diagnostic investigations as this leads to loss of viability of testis. Scrotal exploration is done to untwist the cord, confirm the viability of the testis and orchiopexy. Orchidectomy is necessary for non-viable testis. Most surgeons fix the opposite testis at the same time. Take Home Message It is important to note that primary care physicians are often the first medical contacts for children with surgical emergencies and play a role in improving outcome of these cases [14]. Most conditions may be beyond their expertise but prompt diagnosis, optimal initial management and timely referral to a centre with facility for neonatal and pediatric surgery are necessary. One should ensure appropriate temperature maintenance, proper hygiene and cleanliness, insert nasogastic tube wherever appropriate, administer intravenous fluids, and start broad spectrum antibiotics wherever deemed necessary. The transfer of the child should be done with possible prior information to the centre so that preparations can be done for prompt management. Contribution AM conceived and wrote the manuscript. He will act as guarantor for this paper. Conflict of Interest None. Role of Funding Source None. References 1. Venkataramana NK. Spinal dysraphism. 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