Acute abdomen in a teenager with Factor XIII deficiency peri
The present case has been published in the Journal of Obstetrics and Gynaecology.

Surgeries in women with coagulation pathway disorders need good teamwork and communication between gynecologist, hematologist and anesthesiologist. Congenital factor XIII deficiency is a rare disorder.

The authors present a report of a 13-year-old female with known congenital factor XIII deficiency who presented with acute abdomen, diagnosed as an ovarian hematoma and was posted for laparoscopic evacuation of the same.

In the past, she had presented with umbilical cord bleed in the first week after birth and thereafter multiple bruises and hematoma till l yr of age when she was diagnosed as congenital factor XIII deficiency by the department of hematology and was enrolled for follow up, transfusion of fresh frozen plasma (15 ml/kg) monthly were initiated for which she reported irregularly due to which she suffered intracranial hemorrhage, pelvic hematoma, renal subcapsular hematoma and a subdural hemorrhage in the past which were managed conservatively with FFP transfusions.

Preoperatively her Hb was 7.1 g%, platelet count 1,60,000/cc and coagulation tests including clot solubility test were within normal limits. The team of hematologists, gynecologists and anesthesiologists meticulously discussed and planned her operation. The hematologist advised preoperative coagulation profile and clot solubility test, which were normal. Infusion of two units of FFP and 1 unit of blood were advised a day prior to the operation.

In the operation theatre, she was found to have a difficult intravenous access and two 20 G cannulas could be secured on the dorsum of her wrist. Ultrasound guided machine was kept standby for central cannulation, in case needed. Supplementation of analgesia by regional anesthesia was avoided to prevent hematoma.

Read in detail here: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3425690/
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