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Mesenteric venous thrombosis in familial free protein S deficiency
H Inagaki1, O Sakakibara, H Miyaike
1Department of Surgery, Nagoya City University, School of Medicine, Japan.
Abstract:
Protein S deficiency reportedly is associated with a high risk for thromboembolism, but mesenteric venous thrombosis is a rare manifestation of this hereditary disorder. We describe an additional case of mesenteric venous thrombosis in a 25-yr-old man with type I protein S deficiency. Total protein S level remained just below the normal range, but free protein S level was less than 10% of normal. Family study revealed that his father had had the same type of disorder. We have reviewed six well-documented cases of mesenteric venous thrombosis with protein S deficiency. It is particularly interesting that those with type I deficiency have had no previous thrombotic episodes (p < 0.05). Our patient was successfully treated with extended bowel resection, but unusual perioperative management was required. Because of extremely edematous mesentery and small bowel, we had to treat him with the abdomen wide open for 2 days between the primary and second laparotomies.
Insights
Protein S deficiency, a hereditary disorder, can lead to rare mesenteric venous thrombosis. This case highlights unusual perioperative management for a patient with type I protein S deficiency.
Area of Science:
- Vascular Medicine
- Hematology
- Genetics
Background:
- Protein S deficiency is a known risk factor for thromboembolism.
- Mesenteric venous thrombosis (MVT) is an uncommon but serious manifestation of inherited thrombophilias.
Observation:
- A 25-year-old male with type I protein S deficiency presented with MVT.
- His free protein S levels were critically low (<10% of normal), despite total protein S levels being near the lower limit of normal.
- A family history of similar disorder was noted.
Findings:
- This case adds to the limited literature on MVT in protein S deficiency.
- Analysis of six documented cases revealed that individuals with type I deficiency had no prior thrombotic events (p < 0.05).
- The patient required extended bowel resection and unique perioperative management due to severe mesenteric edema.
Implications:
- Highlights the importance of considering protein S deficiency in young patients with MVT.
- Suggests a potential link between type I protein S deficiency and initial thrombotic events.
- Underscores the need for specialized surgical and perioperative strategies in complex MVT cases associated with thrombophilia.