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Rare Coexistence of Christmas Tree Ileal Atresia with Intestinal Malrotation in a Neonate

Case Studies 4 min 114

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Created On: 01/04/2026

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Poster abstract

This case report describes a rare coexistence of Type IIIb (“Christmas Tree”/Apple Peel) ileal atresia with intestinal malrotation, presenting as neonatal intestinal obstruction in a 15-day-old male infant. The neonate presented with worsening abdominal enlargement, greenish vomiting, and failure to pass stool, consistent with small bowel obstruction. Radiological evaluation suggested obstruction, although the ultrasound was inconclusive. Exploratory laparotomy confirmed Type IIIb ileal atresia marked by a coiled distal bowel around a single arterial vessel, along with intestinal malrotation evidenced by abnormal duodenojejunal junction positioning and a floating cecum.

Surgical management included resection of the nonviable bowel segments and creation of a double stoma (colostomy and ileostomy). The postoperative recovery was uneventful with good stoma function and recovery. This case underscores the diagnostic difficulty and operative challenges posed by the coexistence of these rare anomalies, which heighten the risk of volvulus and short bowel syndrome. Early clinical suspicion, timely exploratory laparotomy, and tailored surgical management are critical for improving neonatal outcomes, particularly in resource-limited settings.

Complaints

A 15-day-old male neonate presented with:

Bilious vomiting (green-stained vomit)
Severe abdominal distension
Inability to pass stool
Abdominal bloating

Anamnesis

Introduction

Neonatal intestinal obstruction is a life-threatening surgical emergency, most commonly caused by congenital anomalies such as jejunoileal atresia. These atresias present in various anatomical forms, with Type IIIb (“Christmas Tree” or “Apple Peel” deformity) being one of the rarest and most complex. Its incidence is low, representing only 5–10% of all small bowel atresias. It is marked by extensive mesenteric loss, multiple interloop adhesions, and a markedly shortened, coiled bowel, resulting in severe functional impairment and substantial surgical and postoperative challenges.

An exceptionally rare condition involves Type IIIb atresia occurring alongside intestinal malrotation, a developmental anomaly caused by incomplete 270° midgut rotation, resulting in abnormal bowel positioning and increased risk of volvulus. The simultaneous presence of these abnormalities aggravates detection owing to overlapping clinical features, frequently delaying timely intervention. Intraoperative findings can be particularly cumbersome, especially in resource-limited settings lacking advanced imaging and neonatal intensive care support.

This report describes a 15-day-old neonate presenting with intestinal obstruction due to this rare combination.

Medical history

The birth history was normal, though the symptoms gradually became more severe over time.

Examination

(a) Physical examination

The abdomen was markedly distended, suggesting intestinal obstruction due to gas or fluid buildup.

(b) Laboratory findings

Hyperkalemia: 7.9 mmol/L
Metabolic acidosis: pH 7.28
Base excess: −5.4
Bicarbonate: 18 mmol/L (mild metabolic acidosis)

(c) Imaging findings

Abdominal X-ray showed multiple air-fluid levels with dilated proximal bowel loops.
Ultrasound was inconclusive, unable to clearly assess mesenteric vessel orientation.

(d) Intraoperative findings

Grossly dilated proximal small intestine with blind-ending loop.
Type IIIb ileal atresia with a classic “Christmas tree” pattern.
Distal collapsed bowel loops spiraling around a single arterial supply.
Abnormally positioned duodenojejunal junction on the right side.
Freely mobile cecum in the right upper abdomen, confirming intestinal malrotation.

Diagnosis: Type IIIb ileal atresia associated with incomplete intestinal malrotation causing neonatal distal intestinal obstruction.

Treatment

(a) Surgical procedure

Emergency exploratory laparotomy was performed due to clinical and radiological suspicion of intestinal obstruction.
Intraoperatively, the collapsed distal ileum, cecum, and proximal ascending colon were surgically removed.
In the right lower quadrant, an end ileostomy with a colostomy was created.
Adequate hemostasis was ensured, and the abdominal wall was closed in layers.

(b) Postoperative care

The neonate was closely monitored in the neonatal surgical ward postoperatively.
Parenteral nutrition was initiated, and electrolyte imbalances were corrected.
Enteral feeding was gradually introduced once ileostomy function was established.
The stoma demonstrated adequate output and good functional status.

(c) Outcomes and follow-up

The neonate showed satisfactory recovery and was discharged in stable condition.
Follow-up was scheduled for potential stoma reversal and long-term assessment of bowel length.

Results

Discussion

This case illustrates the complexity of managing dual congenital intestinal anomalies. Type IIIb atresia is associated with significant mesenteric deficiency and shortened bowel length, increasing postoperative nutritional risk. The additional presence of malrotation heightens the potential for volvulus and further ischemic injury. Clinically, bilious vomiting and progressive abdominal distension in neonates strongly suggest obstructive pathology. Imaging may be nonspecific, and ultrasonography may fail to confirm malrotation, particularly in unstable patients.

Early surgical exploration remains both diagnostic and therapeutic when suspicion is high. Compared with isolated atresia, combined pathology increases operative complexity and influences decisions regarding resection and diversion. In this case, staged reconstruction minimized operative risk and allowed metabolic stabilization. The patient demonstrated favorable early recovery with functional stomas and planned follow-up for bowel length assessment and potential stoma reversal.

Key learnings

Bilious vomiting in neonates mandates urgent evaluation for distal intestinal obstruction.
Type IIIb (“Christmas tree”) ileal atresia is an uncommon but severe cause of neonatal intestinal obstruction with high risk of reduced bowel length.
Coexisting intestinal malrotation substantially increases diagnostic complexity and surgical risk.
Early exploratory laparotomy is fundamental when imaging is inconclusive. However, clinical suspicion remains strong.
Bowel preservation and staged surgical management are key to preventing short bowel syndrome and improving survival.
Comprehensive postoperative nutritional and metabolic support is crucial for optimal neonatal outcomes.

Source:

International Journal of Surgery Case Report

Link:

http://sciencedirect.com/science/article/pii/S2210261225011344

Article:

Christmas tree pattern ileal atresia with intestinal malrotation in a 15-day-old infant: A rare dual congenital anomaly in the pediatric population – A case report