Dr.Sree Lekshmy.S,Dr.Jayasree,Dr.Perraju, Dr.Aswin, Dr.Lekshmi(Neonatology), Dr.Naveen (Ped surgery)DrC Jayakumar Thirty three week+ 4day old male ba, first child of Non consangenousparentage born to a G8A7 mother (IVF conception) with antenatal scans showing dilated fetal stomach and proximal small bowel loops, born Emergency LSCS (indication: preterm premature rupture of membranes).
Ba cried immediately after birth. Ba developed respiratory distress soon after birth and in view of worsening of distress ba wasstarted on CPAP and was slowly weaned off to room air.
Ba developed recurrent episodes of bilious vomiting and abdominal distension and has not passed stools after starting feeds. And was referred to AIMS.
At admission to NICU, ba had stable vitals. Abdominal examination revealed mildly distended abdomen with sluggish bowel sounds. Ba was kept nil per oral and was started on fluids as per protocol.
Differentials considered:
Duodenal atresia
Jejunal atresia
Malrotation with midgut volvulus
Necrotising enterocolitis
Xray abdomen showed triple bubble appearance.
Contrast meal revealed no passage of contrast beyond duodenal jejunal flexure, suggestive of jejunal
atresia. Pediatric surgery consultation was availed and child was taken up for Laparotomy.
Surgical findings include multiple bowel atresia with dilated proximal jejunum.
First atresia at about 10 cm distal to D J Flexure – Type I,
Second atresia – Type II – 2 cm distal to the first one, multiple atretic areas – 10 in number between this area and ileocolic junction.
Small segments of bowel between these atretic segments excised and the remaining bowel joined together with four end to end anastomoses.
Ileocolic junction and large bowel intact.
Total length of small bowel after reconstruction is about 60 cm.
As multiple trial of feeds after anastomosis failed, child was taken up for feeding gastrostomy and as there was a long segment of stomach, duodenum and proximal jejunum through which infant feeding tube could not be negotiated- gastrostomy was closed and Feeding jejunostomy was done and infant feeding tube placed across the 2nd anastomosis. Ba is now on continuous jejunostomy feeds which we are slowly increasing.
Jejunoileal Atresia:
Introduction: Intestinal atresia can occur in any location on the small bowel as a solitary or even multiple lesions. Ileal and jejunal atresias are usually described together as jejuno ileal atresia (JIA). JIA is a common cause of intestinal obstruction in neonates.
Incidence: It is seen in 1 in 5000 to 1 in 14000 live births.
Epidemiology: About 33% of the affected children are born prematurely, and JIA occurs equally in both sex. Familial cases of JIA have been reported, but the majority of JIA occurs sporadically.
Etiology: The cause has been attributed to an intrauterine vascular accident involving branches of mesenteric vessels in the midgut. The resultant ischemic necrosis of the fetal bowel is resorbed in utero, leaving behind a blind proximal and distal end of the bowel with a mesentery defect between the ends. Compared to distal vascular disruption, the more proximal vascular disruption results in more extensive bowel defects. Thromboembolic occlusion of mesenteric vessels may have contributed in some situations, as showed the increased risk of small intestinal atresia in mothers exposed to vasoconstrictive drugs and cigarette smoking in the first trimester of pregnancy.
Classification: Grosfeld classification
Type I – internal membrane with serosa continuity and no mesenteric defect
Type II – proximal and distal blind pouch connected a fibrous cord with serosal discontinuity
Type IIIa – serosa discontinuity with a V-shaped mesenteric defect
Type IIIb – apple peel deformity which described proximal jejunal atresia
Type IV – short ileal segment coiled around the ileocolic artery while multiple atresia
Histology: proximal distended hypertrophied blind loop of the intestine with normal-appearing villi but has defective peristalsis. The mean thickness of the inner circular muscle and outer longitudinal muscle layers of the proximal segment is higher within 3cm-5cm of atretic end in both ileal and jejunal atresia. The interstitial cells of Cajal (ICC), which is important for gastrointestinal motility, are lesser in the proximal and distal atretic ends of JIA than in healthy bowel.
Associations: Cystic fibrosis, malrotation, congenital heart disease, Down syndrome (trisomy 21), anorectal, and vertebral reported for jejunal atresia compared with ileal atresia, where additional anomalies are rare
Evaluation: History includes gestational age at birth, complications during pregnancy, family history, co-morbidities, illicit drug use, the result of prenatal screening such as ultrasound evidence of obstruction like polyhydramnios, dilated bowel, and congenital anomalies such as down syndrome. Perinatal history, including problems with the delivery, feeding history, onset of abdominal distension, bilious or non-bilious emesis, and passage of meconium, should be documented.
Physical examination should include a general assessment of the severity of illness, abdominal distension, evidence of peritonitis, respiratory compromise from aspiration or splitting of the diaphragm, signs of dehydration, jaundice, and congenital anomalies down syndrome, congenital heart disease, and anorectal malformation.
Diagnosis: Plain abdominal X-ray echocardiogram, which shows air in the proximal small intestine and no air distally. Renal ultrasound, rectal biopsy, and cystic fibrosis screening may be used to evaluate patients for associated congenital anomalies.
Treatment: Preoperative management includes decompression with a nasogastric tube, fluid and electrolyte resuscitation, and intravenous broad-spectrum antibiotics in the event of perforation or evidence of infection.
The most common technique is resection of proximal dilated and atretic bowel with primary end-to-end anastomosis with or without tapering enteroplasty of the proximal bowel. Following surgical intervention, the infant is first stabilized rehydration and correction of electrolytes and placement of nasogastric or orogastric tubes to remove fluid from the obstructed stomach and small intestine.
Differential Diagnosis:
Intestinal malrotation with midgut volvulus, internal hernia,
Congenital small left colon syndrome, Hirschsprung disease,
Meconium ileus and colonic atresia.
Complications: Adhesive bowel obstruction and short bowel syndrome. Postoperative complications include sepsis and anatomic leak