Delayed Diagnosis of a Duodenal Web and Concurrent Heterotaxy Syndrome

Introduction: Duodenal webs usually present in infancy, but partial obstruction may remain indolent. We report a delayed diagnosis of a duodenal web in a child with previously unrecognized heterotaxy syndrome.

Case Description: A neonate with an interrupted IVC presented with bilious vomiting. Initial upper GI fluoroscopy was normal; subsequent ultrasound showed proximal duodenal dilatation with to-and-fro peristalsis. Lost to follow-up, the patient returned at two years with chronic vomiting and massive gastric distension limiting ultrasound. Repeat fluoroscopy demonstrated a windsock sign. CT confirmed the web and revealed polysplenia and a truncated pancreas, consistent with heterotaxy syndrome. The web was treated endoscopically.

Discussion: Despite initially normal fluoroscopy, ultrasound had already suggested partial duodenal obstruction. At two years of age, repeat fluoroscopy clearly demonstrated the duodenal web with a characteristic windsock appearance, in the setting of marked proximal dilatation. The interrupted IVC, known since infancy, was also an important clue to heterotaxy that had not been pursued. Although duodenal webs are not a typical feature of heterotaxy, their coexistence has been described previously.

Conclusion: This case highlights the importance of pursuing discordant imaging findings and recognizing interrupted IVC as a clue to underlying heterotaxy.

Presenting Author: Dr. Anna Parail, Radiology Resident, Department of Radiology, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel.

Co-Author: Dr. Li-Tal Pratt, Pediatric Radiologist, Department of Radiology, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel.

Figure. Upper gastrointestinal fluoroscopy reveals proximal duodenal dilation with a peripheral ring-like filling defect, representing the windsock sign.

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