Neonatal bilious vomiting Mekdelawit M. (MD, Pediatric Surgeon FCS-ECSA)
A 3 days old neonate presented with bilious vomiting of 2 days duration. 1. what hx would you like to take? 2. what pertinent physical examination would you like to do? 3. what are your differentials?
Intestinal atresia and stenosis Duodenal atresia/stenosis Occurs in 1/5000-10,000 births (25-40% of intestinal atresias ) Failed recanalization of the bowel lumen. 50% of infants are premature and have LBW. 50% have chromosomal abnormalities of which Trisomy 21 identified in 1/3 of pts 35-50% of infants have associated anomalies.
Classification Type I –( 92%)= Mucosal and submucosal obstructing septum/web with no defect in the muscular wall. Intact mesentery Type II- ( 1%) – short fibrous cord connecting 2 blind ends with intact mesentery. Type III ( 7%)- discontinuous two blind ends with V-shaped mesenteric defect.
Diagnosis Prenatal- Maternal polyhydramnios – 30-65% “double bubble “ sign Postnatally feeding intolerance with repeated bilious vomiting in 12-24hrs Subtle upper fullness , Jaundice in 1/3 of infants. May pass meconium 12-24 hrs followed by constipation. Gastric aspirate of >30ml bile stained. Plain abdominal X-ray- Double bubble sign .
Management Initial stabilization and workup (diagnostic and study of life threatening associated anomalies) Prevent hypothermia ,hypoglycemia Central line line for PN since EN is delayed for several days Surgical exploration : web excision VS Bypass anastomosis
Jejuno -ileal atresia and stenosis Most common congenital anomalies of Small intestine. Atresia - 95% Vs Stenosis - 5% Estimated incidence 1/1000-5000 births M:F= 1:1 25-35% have associated anomalies, more in cases of JA. mesenteric vascular occlusion in late gestation of pregnancy (segmental infarction and resorbtion of fetal intestine) = Widely accepted theory
Classification Pathologic Finding 10/16/2024 8
Clinical presentation The presence of small-bowel atresia is suspected when US reveals multiple distended loops of proximal bowel with polyhydramnios 10/16/2024 9
DIAGNOSIS Triple bubble sign Proximal jejunal atresia patients can have a few gas-filled loops of small bowel, but the remainder of the abdomen is gasless The lower the obstruction, the greater the distended loops of bowel and the more fluid levels will be observed. 10/16/2024 10
Management Pre operative – fluid and electrolyte management and proper resuscitation of the neonate Surgical management Resection and anastomosis of the atretic part
Ano -rectal malformations Worldwide incidence is 1 in 4000-5000 live births No specific cause has been described Slight male preponderance Classifications
Associated Anomalies Cardiovascular anomalies Present in 30% of patients. Only 1/3 require treatment Gastrointestinal anomalies like TEF occurs in 8-10% of cases Spinal, sacral & vertebral anomalies ( Hemivertebrae , MMC, Short sacrum, Hemisacrum,Tethered cord) can occur in 1/3 to ½ of the patients Genitourinary anomalies Hydronephrosis, VUR, renal agenesis, megaureter, Cryptorchidism, Hydrocolpos , Mullerian anomalies …. VACTREL anomalies
Clinical presentation Abdominal distention Failure to pass meconium meconium stained urine Vomiting Bowel perforation and peritonitis Sepsis, shock Signs and symptoms related to associated anomalies
Management First 24 hours Resuscitation and stabilization Make proper diagnosis Evaluation and investigations for associated anomalies Surgical management colostomy anoplasty
Hirschsprung Disease (HSD) characterized by the absence of ganglion cells in the myenteric and sub mucosal plexuses of the intestine. Upstream bowel becomes dilated secondary to functional obstruction Prevalence: 1/5000 births 3-5% of pts have Down’s syndrome 80% affected are boys
Level of involvement : Short segment HSD ( ~ 80% of cases) transition zone at rectosigmoid Long segment HSD- transition zone is proximal to splenic flecture Total Colonic Aganglionosis -sometimes involving distal small bowel Total intestinal aganglionosis – involving the whole small and large intestine 18
Presentation and diagnosis 50-90% present at neonatal period delayed passage of meconium 90% Enterocolitis, Chronic constipation Abdominal distention, Vomiting Perforation and peritonitis blast sign on PR examination X-ray :- distended bowel loops, airfluid level, free peritonial air Barium Enema :- transition zone, reverse rectosigmoid ratio, retained contrast after 24 hours
Rectal Biopsy - G old standard Absence of ganglion cells in the submucosal and myenteric plexuses Hypertrophied nerve trunks Increased acetylcholinesterase staining. 20 Management Resuscitation and stabilization Antibiotic therapy Surgical management Colostomy Definitive pullthrough procedures