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Intestinal necrosis from congenital hypercoagulopathy

T M Crombleholme¹, B H Harris, C G Rosenfield

¹Department of Pediatric Surgery, Tufts University School of Medicine, Boston, MA.

Journal of Pediatric Surgery

|February 1, 1994

Summary

Protein-S deficiency, a rare congenital disorder, can cause severe blood clots in children, leading to critical conditions like intestinal necrosis. Early diagnosis is vital to prevent potentially fatal outcomes.

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Area of Science:

Pediatric Hematology
Congenital Coagulopathies

Background:

Protein-S deficiency is a rare genetic disorder causing a hypercoagulable state.
It typically manifests as recurrent venous thrombosis in adults, with limited pediatric case reports.

Observation:

A 2-year-old girl presented with septic shock and extensive ischemic intestinal necrosis requiring extensive surgery.
Surgical intervention included total gastrectomy, pancreaticoduodenectomy, and partial small bowel resection.

Findings:

Postoperative evaluation revealed an unrecognized Protein-S deficiency as the underlying cause.
The patient underwent complex reconstruction to preserve intestinal length.

Implications:

This case highlights that severe thrombotic events in children can be linked to Protein-S deficiency.

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Consideration of Protein-S deficiency in pediatric thrombotic episodes is crucial for timely intervention and preventing mortality.