Pertinent findings on autopsy included a macerated male fetus whose anthropometrical measurements were normal for age and sex. Development of facial features, thorax, and extremities appeared appropriate for the gestational age. No anatomic defects were identified on gross evaluation. Head, chest and abdominal circumference were normal for 35 weeks gestational age. Cephalhaematoma with overriding of skull bones was noted. On opening the body, the most remarkable finding was marked distension, dilatation and congestion of the duodenum and jejunum that ended in a blind pouch (Figure 1B,C). The distal small intestine was shortened and coiled around a fibrous band (apple peel deformity) (Figure 2A). There was malrotation of gut with appendix lying on the left side (Figure 2B). The distal small intestinal segment although shortened was patent and contained meconium. Gross examination of other organs including the heart, lungs, kidneys was unremarkable.

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Figure 1: A) Ultrasound scan showing dilated gastric fundus bubble with dilated proximal bowel. B) Dilated and congested stomach, duodenum and jejunum. C) Scalpel head showing atretic intestinal segment.

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Figure 2: A) Distal small intestine coiled around a fibrous band (apple peel deformity). B) Malrotation of gut with appendix lying on left side. C) Section from dilated duodenum proximal to site of atresia showing partially autolysed mucosa (blue arrows) with thickened muscle layers (red arrows) (H&E; x100). D) Section from distal small bowel behind the atretic site showing partially autolysed mucosa (blue arrows) along with normal thickness of muscle layers (red arrows) (H&E; x100).

Histopathological examination of most of the organs showed autolytic changes Sections from the dilated duodenum showed autolytic mucosa with a markedly thickened muscle layer (Figure 2C). Sections from the distal small bowel showed partial autolytic changes in the mucosa with normal thickness of the muscle layer (Figure 2D). Sections from the atretic segment showed fibromuscular tissue only. Examination of placental tissue was unremarkable. Umbilical cord showed normal threevessel morphology without any evidence of funisitis.

Fetal tissue submitted for cytogenetic analysis showed normal cytogenetics.

It is certified that ethical approval of ethics committee of Command Hospital Air Force Bangalore and consent of next of kin of deceased was sought prior to publication of this study.

Jejunoileal atresia could be single (>90%) or multiple and may be familial ⁹. There was a history of fetal demise in a previous pregnancy in this case; however, it was not investigated. There may be associated intrauterine growth restriction, gastroschisis, volvulus, malrotation of the gut, and cystic fibrosis ¹⁰. Our case had malrotation of the gut as an associated anomaly. Jejunoileal atresia is traditionally classified as per Grosfeld subtypes. Type I is mucosal in which the lumen is blocked by an intact membrane. Type II has atretic bowel ends which are connected by a fibrous cord. Type III has two variants. Type IIIA has an obvious gap between disconnected segments. Type IIIB is the rarest form with apple peel deformity while Type IV has multiple obstructions resulting in sausages on string appearance and is the least common variant ¹¹. This neonate also had type IIIB deformity. Almost one-third of JIA and duodenal atresia cases can be identified during the prenatal period with ultrasonography ¹². In our case, maternal ultrasound up to the 29th week was reported as normal but routine third trimester ultrasound showed dilated bowel loops. Dilated bowel loops are highly indicative of bowel obstruction due to a congenital disorder, intestinal malrotation, volvulus, and meconium plug syndrome ¹³.

The most commonly accepted theory for pathogenesis of intestinal atresia is based upon work by Louw and Barnard ^6,14. They demonstrated and reproduced all models of atresia after ligation of mesenteric vessels at various levels in canine models, thus supporting their theory of vascular insufficiency. Our case had malrotation of gut that possibly led to vascular insufficiency leading to jejunoileal atresia.

What might have caused intrauterine fetal demise in the current case? It is postulated following obstruction, the raised bile acids in fetal bloodstream, may have triggered a fetal arrhythmia, or marked dilatation of the stomach, duodenum and proximal jejunum in this case may have triggered bradycardia and ultimately sudden death resulting from high vagal tone ^15,16.

With respect to prenatal diagnosis, repeated ultrasound imaging is useful to assess the progress and development of atresia in the foetus. If undetected, the neonate may present with signs and symptoms of high bowel obstruction several hours after delivery. Despite the high mortality, it can be treated and managed with appropriate early surgical intervention ¹⁷.

Our autopsy case report presents a rare and complicated variant of jejunoileal atresia associated with malrotation of gut. This case highlights the importance of early diagnosis and intervention.

CONFLICT of INTEREST
The authors declare no conflict of interest.

FUNDING
None

AUTHORSHIP CONTRIBUTIONS
Concept: DM, SS, Design: DM, Data collection or processing: DM, SS, Analysis or Interpretation: DM, SS, Literature search: DM, SS, Writing: DM, SS, Approval: DM, SS.

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