|Ahead of print
Congenital transmesenteric hernia – A rare presentation
Anilkumar Pura Lingegowda, B Amit, Pramod R Pillai, Ramachandra Chandrayya
Department of Paediatric Surgery, Sakra World Hospital, Bengaluru, Karnataka, India
|Date of Submission||26-May-2022|
|Date of Decision||09-Jul-2022|
|Date of Acceptance||21-Aug-2022|
|Date of Web Publication||12-Dec-2022|
Anilkumar Pura Lingegowda,
Flat 411, Wing 2, Sandeep Square Apartment, GEAR School Road, Bhoganahalli, Bellandur Post, Bengaluru - 560 103, Karnataka
Department of Paediatric Surgery, Sakra World Hospital, Bengaluru, Karnataka
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Congenital transmesenteric defect is a rare but serious condition which presents a diagnostic challenge as symptoms and investigations are nonspecific. Here, we present a case with fetal ascites and dilated bowel loops who underwent intervention within 6 h of birth. Laparotomy showed volvulus and gangrene of 45 cm of the distal ileum, which was herniating through mesenteric defect. Timely detection and intervention can help in preventing significant morbidity and mortality. Given the downside to delay of surgical intervention and lack of specific tests, open exploration continues to be the only way to establish a definitive diagnosis of a transmesenteric hernia.
Keywords: Congenital, gangrene, internal hernia, transmesenteric, volvulus
|How to cite this URL:|
Lingegowda AP, Amit B, Pillai PR, Chandrayya R. Congenital transmesenteric hernia – A rare presentation. J Indian Assoc Pediatr Surg [Epub ahead of print] [cited 2023 Mar 26]. Available from: https://www.jiaps.com/preprintarticle.asp?id=363427
| Introduction|| |
Congenital transmesenteric hernia (CTMH) is a rare but serious condition; diagnosing this is a challenge as symptoms and signs are nonspecific and radiological investigations may not give adequate insight. If this is not detected and intervened in the early stages, it may give rise to far-reaching implications.
CTMH constitutes 5%–10% of all internal hernias (IHs). In CTMH, IH of bowel occurs without a sac; bowel loops herniate through a window in the mesentery and lead to undesirable sequelae.
We are reporting a case of perinatal suspected fetal small bowel obstruction stressing on importance of prompt evaluation and exploration to manage effectively.
| Case Report|| |
A 32-week pregnant female noticed decreased fetal movement; fetal ultrasound showed normal cardiac activity with fetal ascites and edematous bowel loops. As patient presented with ascitis on antenatal scan, torch was done to rule out medical causes of ascitis, and hence, the possibility of small bowel obstruction was contemplated. After discussing the above findings and counseling regarding the need for intervention for the baby with the family, an emergency cesarean section was done.
At birth, the child was found to have abdominal distention with a mass felt on the right umbilicus; the child was shifted to the neonatal intensive care unit for stabilization. Upper gastrointestinal contrast study revealed normal C-loop which ruled out malrotation [Figure 1].
|Figure 1: Contrast X-ray showing normal C loop of duodenum with distal paucity of contrast|
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As the baby had altered nasogastric aspirates, and serial X-ray showed the absence of flow of dye beyond few proximal jejunal loops which was suggestive of obstruction, with hemoglobin of 6 gm%, clinical suspicion of bowel gangrene with volvulus was arrived at. Moreover, the patient was taken up for emergency laparotomy at 6 h of life.
Laparotomy showed volvulus and gangrene of 45 cm of the distal ileum, which was herniating through mesenteric defect [Figure 2] and [Figure 3]. Proximal healthy 75 cm of the small intestine was noted. Resection of gangrenous segment and ileostomy was done; ileocecal junction was preserved. After 75 days, the baby underwent elective stoma closure and had an uneventful hospital course. At present, the child is 5-year-old growing well without any sequel of her disease.
|Figure 2: Intraoperative photograph showing gangrenous bowel herniating through mesenteric defect|
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| Discussion|| |
Rokitansky initially described this entity as an autopsy finding in 1836. IH can be of two types: congenital or acquired. Congenital IH is named as per their locations; foramen of Winslow, paraduodenal, pericecal, transmesenteric, transomental, and intersigmoid hernias. Acquired causes are due to postsurgery and posttraumatic., Only case series and case reports representing IH due to mesenteric defects are present in the literature.
IH-causing intestinal obstruction accounts for 0.5%–5.8% of reported intestinal obstructions. Transmesenteric hernia is the most common location of IH in the neonatal and pediatric age groups. Toddler-age children most commonly present with symptoms of bowel obstruction due to CTMH. Of all IHs, the reported incidence of CTMH is 1%.
The etiology of mesenteric defects in congenital cases is vaguely understood. Other congenital bowel anomalies have been also reported with CTMH such as small bowel atresia, intestinal duplication, Hirschsprung's disease, and malrotation. This suggests that CTMH may share a common inciting event during embryogenesis such as vascular insult, regression of the dorsal mesentry, or hindrance during rotation of gut.,
Depending on the progression of pathology, symptoms may occur either early in neonatal period or later in life. Mesenteric defect causes herniation of bowel loops and progresses to bowel obstruction. Most mesenteric defects are 2–3 cm wide, occurring in small bowel mesentery, which usually traps a loop of the ileum. If the pathology is not addressed at this point, vascular compromise occurs which results in necrosis, perforation, volvulus of bowel, gangrene, and sepsis.
Radiological investigations play a limited role in CTMH as there is no specific sign which corroborates the diagnosis. The cornerstone to establish this pathology remains surgical exploration; any delay can have undesirable sequelae., Mortality rate up to 45% is because of delay in diagnosis. The outcome depends on the high index of suspicion and prompt surgical intervention as vascular compromise can be detrimental and leads to significant morbidity. Prognosis improved by early diagnosis and treatment.
| Conclusion|| |
We describe a perinatally suspected small bowel obstruction. High index of suspicion of neonatal small bowel obstruction should be kept when antenatal ultrasound shows bowel edema with fetal ascites. Timely detection and intervention can help in preventing significant morbidity and mortality. Regular clinical assessment and further progress of symptoms of the newborn babies remain the best possible guide to deciding further course of management.
Persisting abdominal distension and fall in hemoglobin should point in direction of bowel gangrene. Given the downside to delay of surgical intervention and lack of specific tests, open exploration continues to be the only way to establish a definitive diagnosis of a transmesenteric hernia.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]