Delayed Presentation of Malrotation: Case Series and Literature Review
Himanshu Menghwani1, Rajat Piplani1, Enono Yhoshu1
1Department of Pediatric Surgery, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India.
Insights
Intestinal malrotation presenting beyond infancy is rare and often misdiagnosed due to varied symptoms. Early diagnosis and intervention, like the Ladd procedure, are crucial to prevent complications such as intestinal ischemia.
Area of Science:
- Pediatric Surgery
- Congenital Anomalies
- Gastrointestinal Disorders
Background:
- Intestinal malrotation is a congenital anomaly of midgut rotation and fixation.
- While commonly seen in neonates, it rarely presents beyond infancy.
Purpose of the Study:
- To describe clinical features, radiological findings, and treatment outcomes of intestinal malrotation in patients presenting after infancy.
- To emphasize the diagnostic challenges and importance of early intervention in delayed malrotation cases.
Main Methods:
- Retrospective analysis of 11 consecutive cases of delayed intestinal malrotation presentation (2017-2021).
- Diagnostic modalities included ultrasonography, upper gastrointestinal contrast study, and contrast-enhanced computed tomography.
- Treatment involved the Ladd procedure, with some cases requiring resection anastomosis or cyst excision.
Main Results:
- Patients ranged from 14 months to 18 years, presenting with diffuse abdominal pain, unlike neonatal bilious vomiting.
- Five patients had associated anomalies (e.g., intussusception, nutcracker syndrome).
- Eight of eleven patients had favorable outcomes; two developed adhesive obstruction, and one had persistent hematochezia.
Conclusions:
- Intestinal malrotation in older children is uncommon and often overlooked due to nonspecific symptoms.
- A high index of suspicion and advanced imaging are vital for diagnosis, identifying characteristic vascular repositioning.
- Prompt surgical intervention can avert severe complications like volvulus and ischemia.
Background:
Intestinal malrotation is a congenital anomaly resulting from abnormal or incomplete rotation and fixation of the midgut during embryogenesis. It commonly presents in the neonatal period (75%) with sudden onset bilious vomiting and rarely beyond infancy (<10%).
Aim:
The aim of the study was to highlight the clinical features, radiological findings, and treatment outcomes of patients with malrotation presenting beyond infancy.
Materials And Methods:
Eleven consecutive cases of delayed presentation of malrotation presented over a period of 5 years (2017-2021). Data were analyzed retrospectively.
Results:
Out of the 11 patients, four were female and seven were male. The age of patients ranged from 14 months to 18 years. Patients beyond infancy present usually with diffuse pain abdomen compared to neonates which present with sudden onset bilious vomiting and therefore difficult to diagnose. Five patients had associated abnormalities such as intussusception or nutcracker syndrome or mesenteric cyst or jejunal stricture or mesenteric lymphadenopathy along with malrotation. Patients underwent ultrasonography, upper gastrointestinal contrast study, and contrast-enhanced computed tomography abdomen to confirm the diagnosis. All patients underwent the Ladd procedure with four requiring resection anastomosis and one requiring excision of the mesenteric cyst. Eight out of eleven patients had favorable outcomes, two develop adhesive intestinal obstruction and required re-exploration, and one had persistent complaints of hematochezia.
Conclusion:
Malrotation beyond infancy is a rare diagnosis. Malrotation in older children is usually not suspected because of the wide range of symptoms. A high index of suspicion on ultrasound or computed tomography is required to demonstrate the reversal of superior mesenteric artery and superior mesenteric vein position and related conditions. Early intervention and treatment can prevent catastrophic events such as intestinal volvulus and intestinal ischemia in these patients.
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