Kingdom of Bahrain Arabian Gulf University College of Medicine and Medical Sciences

1. Kingdom of BahrainArabian Gulf UniversityCollege of Medicine and Medical Sciences Subspecialty Surgery Pediatric Surgery Ali Jassim Alhashli

2. Fluid Management • How to calculate maintenance fluid in pediatric age group? • First 10 kg = 4 cc/kg. • Second 10 kg = 2 cc/kg. • Every additional kilogram after 20 kg = 1 cc/kg. Notice that you have to monitor urine output = 1-2 ml/kg/hour. • Pediatric blood volumes: • Newborn: 85 cc/kg. • Infant (1-3 months): 75 cc/kg. • Child: 70 cc/kg.

3. Neck Masses/Lesions • Classified depending on the location: • Midline: • Thyroglossal duct cyst. • Enlarged thyroid gland (goiter). • Dermoid cyst. • Lateral: • Inflamed lymph nodes. • Branchial cleft cyst. • Cystic hygroma. • Carotid body tumor. • Thyroglossal duct cyst: • Normally, thyroid gland migrates from base of the tongue (foramen cecum) downwards through hyoid bone to reach its final destination. If a remnant of that tract persists, this will turn into a thyroglossal duct cyst. • Signs and symptoms: midline neck swelling which moves with swallowing and tongue protrusion. • Diagnosis: usually at age of 5 years via ultrasound. • Complications: mainly enlargement, infection and fistula formation. • Treatment: • Sistrunk procedure: removal of the cyst + tract + part of hyoid bone. • If the cyst is infected: treat with antibiotics first.

4. Neck Masses/Lesions • Branchial cleft cyst and sinus: • Remnants of the four branchial clefts in anterior neck can form cysts with tracts leading through to the skin. • Signs and symptoms: • Painless, mobile, lateral neck mass which may present with drainage. • Notice that second branchial cyst is the most common (presenting at the anterior border of SCM muscle). • Diagnosis: ultrasound. • Treatment: Surgical removal of the cyst and its tract. If it becomes infected, antibiotics must be given. • Cystic hygroma: • It is a congenital abnormality of a lymph sac resulting in lymphangioma (mostly discovered at time of birth). • Signs and symptoms: lateral, painless, soft, mobile neck mass which transilluminates brightly. • Diagnosis: CT-scan. • Treatment: must be removed surgically because it can enlarge and compress on the airway. If the lesion is unresectable → sclerosis.

5. Neck Masses/Lesions

6. Congenital Deformities of Mediastinum and Thorax • Pectusexcavatum: • Definition: it is the posterior disfigurement of sternum and xyphoid process. • Signs and Symptoms: • Funnel-chest appearance. • May Cause respiratory compromise. • Associated with mitral valve prolapse. • Treatment: • Surgery at 6 years of age by (Nuss procedure): strut placement without osteotomy. • Pectuscarinatum (less common): • Definition: it is the anterior disfigurement of sternum and xyphoid process. • Signs and symptoms: • Pigeon chest appearance. • Patients are usually asymptomatic. • Treatment: brace to correct the deformity which can be used until 19 years of age.

7. Congenital Deformities of Mediastinum and Thorax Pectusexcavatum Nuss procedure Pectuscarinatum

8. Congenital Deformities of Mediastinum and Thorax • Tracheoesophageal (TE) fistula: • Definition: incomplete separation of esophagus and trachea. • Signs and symptoms: • Respiratory distress and chocking following the first feed. • Postprandial regurgitation. • Excess drooling and salivation. • There are 5 types of TE fistula: • Type-A: pure esophageal atresia. • Type-B: esophageal atresia with proximal TE fistula. • Type-C (most common): esophageal atresia with distal TE fistula. • Type-D: esophageal atresia with proximal and distal TE fistula. • Type-E: no esophageal atresia with TE fistula. • Diagnosis: • Inability to pass feeding tube. • CXR: tube coiled in upper esophagus. • AXR: presence of air in the stomach. If air is not present, this might indicate the presence of esophageal atresia without TE fistula. • Notice that TE fistula is associated with: • Polyhydramnios. • Pre-term birth. • Small for gestational age. • Treatment: • Decompression of the blind esophageal pouch with constant suction. • Prophylactic antibiotics (to prevent complications of aspiration). • Search for other anomalies (especially cardiac and renal). • Surgical repair: • Ligation of fistula and insertion of gastrostomy tube. • Anastomosis of the two ends of esophagus (jejunal or colonic grafts are used).

9. Congenital Deformities of Mediastinum and Thorax

10. Congenital Deformities of Mediastinum and Thorax • Congenital Diaphragmatic Hernia (CDH): • Definition: herniation of the bowel through a defect in the diaphragm resulting in ipsilateral pulmonary hypoplasia and later in pulmonary hypertension. It is more common to occur on the left side because liver blocks the right side. • There are two types: • Bochdalek (most common): postero-lateral hernia. • Morgagni: antero-medial hernia. • Signs and symptoms: • Significant respiratory distress. • Scaphoid abdomen. • Bowel sounds heard in the chest. • Diagnosis: • CXR: bowel gas in the chest + shifting of mediastinum. • Treatment: • Endotracheal and nasogastric tubes. • Surgical repair: hernia reduction and repair of the defect (by primary closure or use of a synthetic patch). • Prognosis: • Survival rate = 50%. • Predictors of mortality: • Pulmonary hypoplasia. • Pulmonary hypertension: reduced by inhalation of nitric oxide (a pulmonary vasodilator).

11. Congenital Deformities of Mediastinum and Thorax

12. Congenital Deformities of Mediastinum and Thorax • Pulmonary sequestration: • Definition: it is the presence of embryologic, non-functioning (no communicating with airways), cystic pulmonary tissue which is receiving its own blood supply from systemic circulation. • There are two types: • Intralobular (contained within a normal lung): • Not covered by pleura. • Presenting with infection. • Venous drainage via: pulmonary veins. • Extralobular (separated from normal lung; can be under the diaphragm): • Covered with pleura. • Associated with diaphragmatic hernia. • Vanous drainage via: systemic veins. • Diagnosis: CXR and chest CT-scan. Always document blood supply by doppler ultrasound. • Treatment: • Intralobular: lobectomy. • Extralobular: surgical resection.

13. Congenital Deformities of Mediastinum and Thorax

14. Congenital Deformities of Mediastinum and Thorax • Congenital Cystic Adenomatoid Malformation (CCAM): • Definition: presence of abnormal cystic lung tissue. • Signs and symptoms: • Neonatal respiratory distress. • Recurrent respiratory infections. • Diagnosis: • CXR: swiss-cheese appearance. • You differentiate it from CDH by passing the NG tube (in which the tip will reach the abdomen). • Treatment: surgical removal.

15. Abdominal Wall Defects • Gastroschisis: • Congenital, full-thickness defect of the abdominal wall which is found right to the umbilicus and resulting in exposed bowel (with no covering). • Diagnosis: • Often detected by pre-natal ultrasound. • Associated with ↑ alpha-fetoprotein (AFP). • Not associated with other anomalies. • Treatment: • Temperature regulation. • Sterile covering with a plastic wrap. • NG decompression with Total Parenteral Nutrition (TPN). • Broad-spectrum antibiotics. • Surgical correction and closure of the abdomen. • Prognosis: 20% of cases are complicated with necrotizing enterocolitis. • Omphalocele: • Herniation of abdominal contents into the base of umbilical cord (with a covering made of peritoneum and amnion). • Associated anomalies: • Beckwith-Wiedmann syndrome (gigantism, macroglossia, hypoglycemia, umbilical defect and organomegaly). • Trisomy 13 and 18. • Extrophy of urinary bladder. • There are two types: • Small: containing only intestine. • Large: liver, spleen and GI tract. • Treatment: • Intact sac: not urgent. • Ruptured sac: similar to gastroschisis. Emergency surgical repair is required.

16. Abdominal Wall Defects • Inguinal hernia: • Definition: Herniation of a viscus or part of a viscus through inguinal canal. In children, most commonly an indirect inguinal hernia from patent processusvaginalis. Remember that indirect inguinal hernia is lateral to inferior epigastric vessels. • Epidemiology: • More common among males (Especially premature infants). • More common on the right side because right testicle descends later. • Signs and symptoms: • Scrotal or groin mass which becomes more prominent with increased intrabdminal pressure (example: coughing, crying). • Diagnosis: • Physical examination reveals (silk glove sign): after hernia is reduced, sac and thickened spermatic cord can be palpated and rolled underneath examiner’s finger. • Treatment: • Surgical repair is required because there is increased risk for bowel incarceration/strangulation. • Herniotomy is performed without repair of abdominal wall (pediatric cases are usually not repaired with a mesh).

17. Gastrointestinal Defects • Pyloric stenosis: • Definition: it is the narrowing of pyloric canal due to hypertrophy of smooth muscle. This is more common among males, those with family history and firstborn males. • Signs and symptoms: • They will appears between 2 weeks – 2 months of age. • Olive-like mass in the midepigastric area. • Projectile, non-bilious vomiting (obstruction is proximal to ampulla of vater). • Visible peristalsis. • Hypokalemic, hypochloremic metabolic alkalosis with paradoxical aciduria. • Diagnosis: ultrasound which will show: • Elongation of pyloric canal (< 14 mm). • Thickened pyloric wall (< 4 mm). If ultrasound is not diagnostic, barium swallow shows string sign. • Treatment: • Fluid replacement, correction of electrolytes and acid-base disturbance. • Surgery: Ramstedtpyloromyotomy. Remember that vein of Mayo crosses the pylorus. • Biliaryatresia: • Definition: complete obliteration of extrahepaticbiliary tree. • Signs and symptoms: • Neonatal jaundice (< 1 week). • Hyperbilirubinemia (increased both direct and indirect bilirubin). • ↑ alkaline phosphatase level. • Signs of portal hypertension (hepatosplenomegaly and ascites). • Diagnosis: • Ultrasound. • HIDA scan. • Treatment: • Surgical repair which is optimally done > 8 weeks of age (Kasai hepatoportoenterostomy). If Kasai procedure fails → liver transplantation.

18. Malrotation and midgutvolvulus: • Definition: incomplete rotation of intestine during fetal development. • Normal embryology: midgut starts migrating back to the abdomen at 12 weeks by rotating a total of 270 degrees counterclockwise around the axis of superior mesenteric artery. • Signs and symptoms: • Abdominal distention. • Acute onset of bilious vomiting. • Hypovolemia and lethargy. • Bloody stool (late sign). • Diagnosis: • AXR: • Presence of bowel loops overriding the liver. • Double bubble sign. • No gas in GI tract distal to the volvulus. • Barium enema: cecum located in RUQ. • Treatment: Ladd procedure • Reduce with counterclockwise rotation. • Ligation of ladd’s bands. • Appendectomy. Gastrointestinal Defects

19. Duodenal atresia: • Definition: failure of duodenum to recanalize during fetal development. Mostly occurs at the site of ampulla of Vater. • Associated conditions: • Down syndrome. • Imperforate anus. • Esophageal atresia. • Polyhydramnios. • Small for gestational age. • Signs and symptoms: • Abdominal distention. • Bilious vomiting. • Diagnosis: • AXR: double bubble sign (air in stomach and duodenum). • Treatment: • Fluid replacement. • Gastric decompression. • Broad-spectrum antibiotics. • Surgery: • Duodenal atresia: side-to-side anastomosis. • Jejunoilealatresia: end-to-end anastomosis. Gastrointestinal Defects

20. Intussusception: • Definition: telescoping of one portion of the bowel into the other. Common site is ileocecal valve. • Epidemiology: • It is the most common cause of intestinal obstruction in males under 2 years of age. • Causes: • Idiopathic. • Hypertrophy of Peyer’s patches after viral infection (enterovirus in summer; rotavirus in winter). • Meckel’sdiverticulum. • Polyp. • Lymphoma. • Henoch-Schonleinpurpura. • Cystic fibrosis. • Signs and symptoms: • Intermittent colicky abdominal pain. • Currant-jelly stool. • Bilious vomiting. • Sausage-like mass in RUQ. • Diagnosis and treatment: • Abdominal x-ray and ultrasound: target sign. • Barium enema (both diagnostic and theraputic): • It will show: coiled spring appearance. • Rule of 3’s: • Barium column should not exceed a height of 3 feet. • 3 attempts. • 3 minutes/ each attempt • Recurrence: • Reduction with barium: 10% • Surgical reduction: 5% Gastrointestinal Defects

21. Meckel’sdiverticulum (true diverticulum): • Definition: persistent vitelline duct which does not obliterate. It contains heterotopic epithelium (gastric or pancreatic). • Epidemiology: it is more common in males > 2 years of age. • Rule of 2’s: • 2 feet away from ileocecal valve. • 2 inches (length). • 2% of population. • Symptomatic in 2% of patients. • 2 types of tissues (gastric and pancreatic). • Under 2 years of age. • Signs and symptoms: • Most important: intermittent painless rectal bleeding. • Intestinal obstruction. • Diverticulitis. • Diagnosis: Mickel’s scan (scintigraphy). • Treatment: surgical resection. • Imperforate anus: • Definition: it is the lack of anal opening of proper location or size due to failure of urinary and hindgut systems to separate. • Associated anomalies: • VACTERL association: (Vertebral, Anorectal, Cardiac, TracheoEsophageal, Renal, Limb). • Treatment: • High lesions (above puborectalis sling): colostomy. Neoanus is usually made at 1 year of age. • Low lesions (more common in females):perinealanoplasty. • Prognosis: higher lesions have poor prognosis. Gastrointestinal Defects

22. Hirschsprung’s disease: • Definition: it is the lack of Auerbach (myenteric) and Meissner (submucosal) plexi thus resulting in intestinal obstruction. • There are 3 types: • Rectal. • Rectosigmoid. • Entire colon. • Signs and symptoms: • Neonates: • Failure to pass meconium (< 24 hours). • Most common cause of mortality is enterocolitis. • Children: • Abdominal distention. • Bilious vomiting. • Chronic constipation. • Diagnosis: • AXR. • Barium enema: look for transition zone which may not appear before 1-2 weeks of age. • Definitive diagnosis: rectal biopsy (showing absence of ganglion cells). • Treatment: • Colostomy proximal to the transition zone. • Surgical resection when age reaches 6-12 months. • Colostomy kept after surgery for 1-3 months. Gastrointestinal Defects

23. Gastrointestinal Defects

24. Necrotizing enterocolitis: • Definition: immature gut (in pre-terms) necrosis due to combination of vascular, mucosal and toxic insults. • Signs and symptoms (similar to sepsis): • Systemic: • Fever or hypothermia. • Hypotension. • Feeding intolerance. • GI: • Abdominal distention. • Vomiting. • Bloody diarrhea. • Abdominal wall erythema (indicating perforation and abscess formation). • Diagnosis by AXR which shows: • Pneumatosisintestinalis. • Portal vein air. • Pneumoperitoneum (indicating perforation and is a surgical emergency!). • Treatment: • NPO and bowel decompression. • TPN. • Sepsis evaluation and antibiotics. • Monitor fluid intake and output. • Monitor vital signs. Gastrointestinal Defects

25. Neoplastic Disease • Wilm’s tumor: • It is the most common intra-abdominal malignancy in children between 2-4 years of age. • Associated conditions: • WAGR association: Wilm’s tumor, Aniridia, Genitourinary abnormalitis and mental Retardation. • Beckwith-Wiedmann syndrome. • Denys-Drash syndrome: Wilm’s tumor, pseudohermaphroditism and glomerulopathy. • Signs and symptoms (triad): • Abdominal mass (does not cross the midline). • Hematuria. • Hypertension (20%). • Diagnosis: • Abdominal ultrasound. • CT-scan with contrast. • CXR (to rule out pulmonary metastasis as lung is the most common site for metastasis). • Stages: • Stage-I: involves only kidney. • Stage-2: invading capsule and possibly perinephric fat. • Stage-3: no hematogenous spread; not completely resectable. • Stage-4: hematogenous spread to lung, brain and distal nodes. • Stage-5: involves both kidneys. • Treatment: • Surgical resection of the tumor. • Chemotherapy and radiation for later stages. • Prognosis: 90% survival rate.

26. Neoplastic Disease • Neuroblastoma: • Definition: tumor from neural crest cells which can arise in: adrenal medulla (65%), sympathetic ganglia or organ of Zuckerman (para-aortic ganglia). • Signs and symptoms: • Abdominal mass and pain. • Hypertension (25%). • Fever, lethargy and diarrhea. • Neurological symptoms. • Diagnosis: • Urine for catecholamines: VMA, HVA and metanephrines. • CT-scan with contrast. • Intravenous pyelography: inferior displacement of opacified calyces (drooping lily sign). • Stages: • Stage-I: Tumor is limited to one organ; completely resectable. • Stage-II: tumor does not cross the midline. • Stage-III: tumor crosses the midline. • Stage-IV: distant metastasis (it is the most common tumor which metastasizes to the orbit and cause proptosis). • Treatment: • Stage-I: surgical removal. • Stage-II and III: surgical resection with chemotherapy. • Stage-IV: surgical resection + chemotherapy + radiotherapy.