Dr Varsha V S, Dr Jayasree,DrAswinPrabhu ,DrPerraju/ Dr Lakshmi S Nair/ Dr Smrithi Menon, (AIMS NEO),DrCJayakumar
Department of Pediatrics AIMS Kochi.
Early term/Symmetrical SGA/Male ba born to a 32 years old G2P1L1 mother with no comorbidities but scan s/o oligohydramnios and grade 1 IUGR. Ba was born via emergency LSCS (Indication: non-progression of labor). Ba cried immediately after birth and shifted to mother’s side. New born examination was normal. Ba passed urine and stools within 24 hours. On post natal day 4 onwards ba developed recurrent episodes of bilious vomiting post feed. Hence ba was referred to AIMS NICU for further evaluation and management.
Examination: No other gross congenital anomalies. Vitals stable.
Auxology Weight: 1.8 kg below 3rd centile HC: 30.5cm (at 3rd centile) Length: 46cm at 3rd centile.
Systemic examination done showed mildly distended abdomen with sluggish bowel sounds.
Ba was kept NPO and fluids were given according to NICU protocol.
X-ray abdomen showed mild distension of small bowel.
Differentials considered were:
1. Intestinal atresia
2. Jejunal atresia
3. Malrotation with midgut volvulus
4. Necrotizing enterocolitis
5. Meconium ileus
Labs CBC: TC: 9.86 K/uL/ N/L: 50%/ 31.4%, Hb: 14.4g/dl, Platelet count: 5.84 Lac.
LFT: TSB: 12.67mg/dL, DSB: 0.85mg/dL, ALT: 12.3IU/L,
RFT/ Serum electrolytes: normal.
Blood C/S: Sterile.
Thyroid profile: Normal,
TORCH screening: negative.
Barium meal s/o midgut volvulus.
USG abdomen showed inverted SMA –SMV axis. (Inversion of the superior mesenteric vein (SMV) and the superior mesenteric artery (SMA) means the SMV is typically located to the right of the SMA with a retroperitoneal position of the duodenum when a fluid bolus is given via a nasogastric tube)
Ba had Findings indicative of malrotation with midgut volvulus and was taken for emergency laparotomy and detorsion procedure.
Ba tolerated the procedure well and shifted back to NICU for further management.
Fig 1: Barium meal study showingcorkscrew pattern of the twisted duodenum and jejunum
Discussion: Malrotation with Midgut volvulus: Rotation anomalies occur as a result of arrest in the normal rotation of the embryonic gut. Symptomatic malrotation occur in neonates with a frequency of 1 in 6000 live births.
Associations: Over 50% of children have associated anomalies
1. Congenital diaphragmatic hernia
2. Congenital heart disease
3. Omphalocele
4. Prune Belly syndrome and VACTERL association.
Intestinal malrotation is described as abnormal positioning of bowel loops within the peritoneal cavity in the intrauterine life. It is caused defective rotation of primitive intestinal loop around the axis of SMA during embryogenesis and resulting in short mesenteric root which leads to twist around itself leading to midgut volvulus.
Clinical features: Classical feature is bilious vomiting with or without abdominal distension, abdominal tenderness, hematochezia, hypovolemia and hemodynamic instability.
Investigation: Mainly imaging which includes plain x-ray with or without contrast.
USG to look for abnormal position of SMA and SMV.
CT and MRI is used for diagnosis when Barium studies are inconclusive.
Classical whirlpool sign is seen CT scan and it is due to swirling shape created SMV and mesentery wrapping around SMA.
Diagnosis is confirmed upper GI contrast studies documenting the position of DJ flexure. The pathognomicfeature is the corkscrew pattern of the twisted duodenum and jejunum.
Management: This includes initially stabilizing the ba and definitive management includes emergency laparotomy, volvulus detorsion and a Ladd procedure.
If frankly necrotic bowel is present then it is resected and stomas are created.
Prognosis: The overall mortality rate after surgery for malrotation ranges from 3 to 10%.
Mortality is increased with volvulus, intestinal necrosis, prematurity and associated anomalies.
The Ladd procedure for malrotation reduces the risk of recurrent volvulus widening the base of mesentery. But the risk is not eliminated because the underlying embryologic defect has not been corrected.