Paediatric Surgical Emergencies Presented by Dr Ayman A A Albatanony Associate Professor of Surgery

Don't forget: Trauma: (RTA, Burn,…..) Urinary emergencies ENT emergencies Ophthalmic emergencies Orthopaedic emergencies Cardiothoracic emergencies

Neonatal Surgical Emergencies 3 Paediatric Surgical Emergencies 1 The subject is divided into 2 main topics: Neonatal Surgical Emergencies 3 Paediatric Surgical Emergencies 1

Neonatal Surgical Emergencies 1. Neonatal intestinal obstruction 2. Neonatal major abdominal wall defects 3. Neonatal obstructive jaundice Neonatal Surgical Emergencies

Neonatal intestinal obstruction Necrotizing enterocolitis Atresia, stenosis Small Bowel Atresia Malrotation Hirschsprung’s Annular pancreas Antral web Meconium ileus Imperforate anus Complicated inguinal hernia Congenital Hypertrophic pyloric stenosis

In physical Exam, remember to: Remove diaper

Physical Exam Must perform rectal exam, not just look!

Initial Management NG or OG (NPO!!) Hydrate and replace losses Antibiotics if suspect perforation or necrosis Consult surgeon and/or transfer to appropriate facility

Bowel Obstruction Diagnosis is often age specific Bilious vomiting in the infant and child is a surgical emergency until proven otherwise Child may look surprisingly good until it’s too late

Atresia Usually presents the first few days of life Child may feed well for a day or two with distal atresia Duodenal atresia often diagnosed on antenatal U/S Atresias can occur anywhere in GI tract from pharynx to anus

Atresias Esophageal: aspirate feeds immediately, OG tube won’t pass (non-bilious, but still bad) Duodenal: bilious vomiting immediately, “double bubble” on KUB with absence of distal gas, Down’s Syndrome Jejunal: usually present 1st 24 hours, large dilated proximal loop or loops

Atresias Ileal: may take 24-48 hours before bilious emesis Colonic: rare, may present with bilious emesis after 2-3 days Anal: should be diagnosed at birth, often a perineal fistula is labeled normal

Atresias may be multiple

Jejunal Atresia

Apple Peel Deformity (IIIb)

Imperforate Anus: Anal atresia

Hirschsprung’s Disease Congenital colonic aganglionosis Physiologic obstruction May present first few days to weeks of life Starts at anus and extends proximally a variable distance

Hirschsprung’s Delayed passage of meconium at birth: Meconium plug syndrome, small left colon syndrome, Down’s syndrome Often present with distension Profoundly distended abdomen with dilated bowel Fever and WBC’s with colitis

Hirschsprung’s Rectal exam may seem normal until withdraw finger “Explosive” release of liquid stool almost diagnostic Barium enema while dilated Irrigate and dilate until decompressed Rectal biopsy

Hirschsprung’s Disease

Necrotising enterocolitis: Aetiology: Remains unknown Ischemia and/or reperfusion injury may play a role Translocation of intestinal flora across compromised mucosa may play a role Incidence and age at onset More common in premature infants But can also be seen in term babies Affected term neonates are usually systemically ill with other conditions such as birth asphyxia, respiratory distress or congenital heart disease Babies who are breastfed have a lower incidence of NEC than formula-fed babies

Imaging findings Plain film of the abdomen remains method in which disease is diagnosed most often Findings include Dilated loops of bowel Thickened bowel walls Fixed and dilated loop that persists is especially worrisome Absence of bowel gas Pneumatosis intestinalis Pathognomonic of NEC in newborn Linear radiolucency parallels bowel lumen within bowel wall Represents air that has entered from the lumen

Toxic Megacolon Severe enterocolitis NG decompression, IV fluids, IV antibiotics Mortality 20-30%

Toxic Megacolon

Malrotation Normal

Malrotation Most often presents during the first few days of life Infant with acute onset of bilious emesis Malrotation is a surgical urgency due to the possibility of volvulus VOLVULUS IS A SURGICAL EMERGENCY

Volvulus Malrotation most common condition resulting in midgut volvulus Can have volvulus with normal rotation omphalomesenteric remnant internal hernia Duplication Adhesive small bowel obstruction

Midgut Volvulus

Volvulus 75% First month of life Malrotation is the risk for volvulus Small and large bowel are not fixed Narrow mesentery  more likely to turn around itself Malrotation can cause or present with: Volvulus is dangerous Acute obstruction Chronic intermittent obstruction

Volvulus is lethal Malrotation  midgut volvulus  midgut intestinal death  surgery (resected)  short-gut syndrome  death C/F Bilious vomiting +/- pain if +pain (irritable)  likely volvulus +ischemia - pain (calm)  malrotation+obstruction

Midgut volvulus Infant + Bilious vomiting is EMERGENCY Investigate (if infant is not sick) Upper GI series (look for malrotation) No duodenal C-loop Duodeno-jejunal junction (ligament of Treitz) to the right of Vertebral col. Duodenal obstruction Whirlpool or corkscrew sign (volvulus) U/S Can’t R/O volvulus Can Dx volvulus

Midgut volvulus Pt should go directly for surgery if: Time = Bowel If can’t do investigation immediately Pt is sick + bilious vomiting Time = Bowel Surgery: Untwist (counter clock wise)  assess viability If extensive ischemia  close 2nd look 24-48 hrs Viable SB  close and observe Ladd’s procedure

Meckel’s

Duplication

Intussusception Inversion of the bowel upon itself secondary to a lead point Juvenile intussusception most often idiopathic Also secondary to Meckel’s Presents 6 months to 2 years of age As early as 1 month

Intussusception Acute painful episodes followed by periods of lethargy When incarcerated progress to continuous lethargy May have “currant-jelly” stool But often stool is heme positive Rule out with a left lateral decubitus film

Left-lateral Decubitus Film

Intussusception

Intussusception

Bad Intussusception

Intussusception 7% chance of recurrence after hydroststic reduction May recur in 48 hours Operative exploration warranted on second recurrence to R/O pathologic lead point Recurrence after surgery rare but possible Post-op intussusception can occur after any surgery

Congenital Hypertrophic Pyloric Stenosis

Pyloric stenosis Presentation: vomiting occurs after all feeds copious, no bile in vomitus may be blood, initially child wants to feed again, later becomes weak, listless, metabolic alkalosis, failure to thrive Signs-may palpate pyloric tumour 9 in epigastrium just above umbilicus or between liver edge and right rectus, may see peristalsis presents in first few months of life-between 3-6 wks of age rare if <10days or older than 11 wks Affects 1:450 children Males ( 85%)more common than females Tx: NG tube, Rehydration, electrolyte correction, Ramstedt procedure

Incarcerated Hernia

Incarcerated Inguinal Hernia

Hernia Reduction From Surgery of Infants and Children, Oldham, et. al., 1997

Incarcerated Hernia Most can be reduced in clinic or ED Bowel usually OK if able to reduce Surgical consultation if reduction difficult Repair with 1-2 days of incarceration Beware the “inguinal node’ in females incarcerated ovary

Incarcerated Hernia If unable to reduce: urgent operative exploration (NPO) If able to reduce without sedation: urgent surgical referral with repair soon If extremely difficult (sedation, surgical referral): repair next day Watch child for obstructive symptoms

Perforated Appendix Children still die from complications of perforated appendicitis Resuscitation is critical Diagnosis difficult...Why??

Perforated Appendix Suspect in children 3-5 years old with history suggestive of appendicitis “Bowel obstruction” in a 3-5 year old without obvious etiology is perforated appendix until proven otherwise

Perforated Appendix

Resuscitation NG tube, NPO 20 cc/kg boluses until UOP > 1 cc/kg/hr and VS stable 1.5-2 times maintenance fluids Broad Spectrum Antibiotics

Perforated Appendix

? Summary Atresias Hirschsprung’s Malrotation Volvulus Intussusception Incarcerated Hernia Perforated Appendix ?

Neonatal major abdominal wall defects Omphalocele Gastroschisis

OMPHALOCOELE Anterior abdominal wall defect at the base of the umbilical cord with herniation of the umbilical contents

Incidence Small omphalocoele 1:5000 Large omphalocoele 1:10000 Male to female ratio 1:1

Pathophysiology Failure of the midgut to return to abdomen by the 10th week of gestation

Clinical Findings Covered clinical defect of the umbilical ring Defect may vary from 2-10 cm Sac is composed of amnion, Wharton’s jelly and peritoneum

50% have accompanying liver, spleen, testes/ovary Cord attachment is on the sac

Presentation : AFP level at 12 wks is elevated Detected at routine morphology USS

GASTROSCHISIS herniation of bowel contents through a defect in the anterior abdominal wall,not related to the umbilicus, not in the midline and organs not confined to peritoneal sac

Incidence 1:20,000-30,000 Sex ratio 1:1 10-15% have associated anomalies 40% are premature

Pathophysiology Abnormal involution of right umbilical vein Rupture of a small omphalocoele Failure of migration and fusion of the lateral folds of the embryonic disc on the 3rd-4th week of gestation

Clinical Findings Defect to the right of an intact umbilical cord allowing extrusion of abdominal content No covering sac

Bowels often thickened, matted and edematous 10-15% with intestinal atresia

Management (both) ABC Heat Management Fluid Management Sterile wrap or sterile bowel bag Radiant warmer Fluid Management IV bolus 20 ml/kg LR/NS D10¼NS 2-3 maintenance rate

Nutrition Gastric Distention Infection Control Associated Defects NPO and TPN Gastric Distention OG/NG tube Infection Control Associated Defects

Conservative treatment Reduction by squeezing the sac Painting sac with escharotic agent 0.25% Silver nitrate 0.25% Merbromin (Mercurochrome)

Surgical Management Skin Flaps Primary Closure Staged Closure Staged repair using silo pouch

Skin Flaps

Primary Closure

Staged Closure

Omphalocoele Gastroschisis Incidence 1:6,000-10,000 1:20,000-30,000 Delivery Vaginal or CS CS Covering Sac Present Absent Size of Defect Small or large Small Cord Location Onto the sac On abdominal wall Bowel Normal Edematous, matted

Omphalocoele Gastroschisis Other Organs Liver often out Rare Prematurity 10-20% 50-60% IUGR Less common Common NEC If sac is ruptured 18% Associated Anomalies >50% 10-15% Treatment Often primary Often staged Prognosis 20%-70% 70-90%

Neonatal obstructive jaundice Biliary atresia Choledochal cyst Inspissated bile syndrome Sepsis

Biliary Atresia Incidence 1/20,000 Obliterative process of the extrahepatic bile ducts Associated with hepatic fibrosis Arrest of development during the solid stage of bile duct formation. Aetiology unclear

Atretic ducts – solid fibrous cords that may contain occasional islands of biliary epithelium Three patterns: minimal, partial complete Over time the failure to excrete bile results in progressive periportal fibrosis and obstruction of the intrahepatic portal veins

Presentation & Diagnosis Grey or acholic stools – secondary to obstructed bile flow Failure to thrive Liver failure and portal hypertension Bilirubin > 3 mg/dl Alk phos 500-1000 GGT > 300

Technetiun-99m iminodiacetate (HIDA) after pretreatment with phenobarbital ( promotes tracer uptake) If radionucleotide appears in the intestine then the biliary tree in presumed to be patent Ultrasound can exclude choledochal cyst

Treatment Excise scarred bile ducts and gall bladder and Portoenterostomy

Biliary atresia (60%) Neonatal Hepatitis (35%) hepatic inflammation that can be secondary to several different causes: CMV, syphilis, herpes, toxoplasma Metabolic defects : Alpha 1-antitrypsin deficiency, galactosemia Choledochal cyst (5%)

Continued Spontaneous perforation of extrahepatic bile ducts Ascites, mild jaundice, failure to thrive Usu occurs at the junction between the cystic and common bile ducts Inspissated bile syndrome – bile plug syndrome Extrrahepatic obstruction of the bile ducts by biliary sludge Associated with massive hemolysis, hemorrhage, TPN, cystic fibrosis and other intestinal diseases such as Hirchsprungs

Diaphragmatic hernia Diaphragmatic hernia 95

Congenital diaphragmatic hernias occur in about 1 out of 2,500 live births with a 2 to 1 male to female ratio. Herniation of abdominal viscera occurs through a defect in the diaphragm caused by failure of the pleural peritoneal canal to close completely during embryonic development. Varying degrees of herniation can occur. These patients will often have hypoplastic lungs due to crowding of the thoracic space.

They may show signs of severe respiratory distress such as dyspnea and cyanosis if herniation of abdominal contents is to such an extent as to cause hypoplastic lungs. Signs and symptoms of acute intestinal obstruction can also occur. The diagnosis is usually made by radiographic examination. Emergency treatment: respiratory support, Nasogastric intubation with suction. Extracorporal membrane oxygenation (ECMO) may improve prognosis although mortality rate remains about 50%.

TracheoEsophageal Fistula

TracheoEsophageal Fistula 5 Types (Gross and Vogt) 7.7% 0.8% 86% 0.7% 4.2%

Tracheoesophageal Fistula Incidence: 1:4000 live births M > F (25:3) 10-40% are preterm Antenatal history: polyhydramnios (60%) Etiology: failure in mesenchymal separation of upper foregut

Tracheoesophageal Fistula Clinical Presentation choking on 1st feed coughing cyanosis excessive salivation aspiration pneumonia

Tracheoesophageal Fistula Diagnosis inability to pass a suction catheter into the stomach CXR: coiled orogastric tube in the cervical pouch; air in the stomach and intestine

Tracheoesophageal Fistula Emergency management NPO head-up position sump tube on low continuous suction ± gastrostomy under local anesthesia Antibiotics 12-L ECG and Echocardiogram : mandatory??? IV access ± arterial line

Pediatric surgical emergency 1 Differential diagnosis of an acutely painful scrotal swelling in a 6 years old boy Testicular torsion Acute epididymitis (with or without orchitis) Orchitis (e.g., mumps) Trauma eg testicular haematoma Testicular tumor (hemorrhage within tumor) Incarcerated inguinal hernia

Thank you