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CC BY-NC-ND 4.0 · Indian J Radiol Imaging 2018; 28(01): 65-69
DOI: 10.4103/ijri.IJRI_303_17
Gastrointestinal Radiology and Hepatology

Primary presentation of Jeune’s syndrome as gastric motility disorder in an infant: A case report

Amit Katyan
Departments of Radiology and Imaging, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India
,
Shabnam Bhandari Grover
Departments of Radiology and Imaging, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India
,
Heena Rajani
Departments of Radiology and Imaging, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India
,
Deepak Bagga
Departments of Radiology and Pediatric Surgery, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India
,
Neha Antil
Departments of Radiology and Imaging, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India
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Abstract

We report a case of a 4-week-old female neonate with Jeune’s asphyxiating thoracic dystrophy (JATD) and coexistent situs anomaly, primarily presenting as gastric motility disorder. The child presented with abdominal distension and nonbilious vomiting since birth with failure to thrive. However, skeletal survey revealed JATD. Upper gastrointestinal contrast study showed situs inversus with delayed gastric emptying. Pyloric biopsy and intraoperative antro-duodenal manometry confirmed association of gastric motility disorder. Awareness of the unusual possibility of primary presentation of Jeune syndrome as gastric motility disorder will improve the management approach in such infants.

Keywords

Gastrointestinal motility disorder - Jeune’s asphyxiating thoracic dystrophy - situs inversus - skeletal survey - upper gastro-intestinal contrast study


Publication History

Article published online:
26 July 2021

© 2018. Indian Radiological Association. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).

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