|LETTER TO EDITOR
|Year : 2015 | Volume
| Issue : 7 | Page : 461-462
Perioperative management of a patient with severe Haemophilia B for abdominal pseudotumour Surgery
Lakshmi Kumar1, Rekha Varghese1, Ramachandran Narayana Menon2, Neeraj Siddharthan3
1 Department of Anaesthesiology and Critical Care, Amrita Institute of Medical Sciences, Kochi, Kerala, India
2 Department of Gastrointestinal Surgery, Amrita Institute of Medical Sciences, Kochi, Kerala, India
3 Department of Haematology and Medical Oncology, Amrita Institute of Medical Sciences, Kochi, Kerala, India
|Date of Web Publication||16-Jul-2015|
Department of Anaesthesiology and Critical Care, Amrita Institute of Medical Sciences, Kochi, Kerala
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Kumar L, Varghese R, Menon RN, Siddharthan N. Perioperative management of a patient with severe Haemophilia B for abdominal pseudotumour Surgery. Indian J Anaesth 2015;59:461-2
|How to cite this URL:|
Kumar L, Varghese R, Menon RN, Siddharthan N. Perioperative management of a patient with severe Haemophilia B for abdominal pseudotumour Surgery. Indian J Anaesth [serial online] 2015 [cited 2021 Jan 25];59:461-2. Available from: https://www.ijaweb.org/text.asp?2015/59/7/461/160978
A 53-year-old male presented to the hospital with history of increasing abdominal distention and breathlessness over the last 4 years. He had been diagnosed with severe Haemophilia B (factor IX ≤1% activity) since childhood. A year earlier inferior vena caval filter was placed after detection of thrombosis of both leg veins. His abdominal swelling was a pseudotumour resulting from repeated bleed into soft-tissue due to uncorrected coagulopathy. He had pericardial effusion and 300 ml of haemorrhagic fluid was drained with pericardiocentesis 1-week after presentation. (Factor IX levels 80%). He was scheduled for surgery as he was symptomatic with limitations in activity. The coagulopathy was corrected with transfusions of factor IX to achieve levels close to 100% during the surgery as per the recommendations.  Accordingly, 7000 U of factor IX amounting to 100 U/kg (Immunine ® -Baxter) was administered 45 min before the surgery and factor IX activity of 120% was confirmed. At laparotomy, the left ureter, the left common iliac and the inferior mesenteric vessels were found to course through the tumour. Hence, it was decided to only debulk the tumour, and not attempt complete removal. The abdomen was packed for a relaparotomy 24 h after for control of bleeding and he was extubated after confirmation of adequate haemostasis. Post-operative management included monitoring of factor IX assays and administration of factor IX concentrates to target a level of 60-80% on the day of surgery. The patient had a prolonged stay in the hospital following surgery with bleeding from the abdominal drains. He was managed with factor IX transfusions (intermittent bolus doses) to target a trough level of 40-60% in the 1 st week, 20-30% in the next 2 weeks and was discharged after 30 days in the hospital in a stable state. After discharge, he was well for a brief period. Six months after the limited resection of the tumour he developed an intra-abdominal collection that needed percutaneous drainage and which was complicated by a large bowel fistula. He succumbed to sepsis complicating intra-abdominal infection 8 months later.
Haemophilia B is a disease with an estimated incidence of 1 in 25,000 males  and perioperative management of such patients can often be challenging.  The most common symptoms at presentation relate to bleeding into the joints with inflammation leading to deformities.  Abdominal pseudotumours are rare and limited reports are available. The management of bleeding mainly involves use of factor concentrates, whole blood and plasma but the use of antifibrinolytics, factor VIIa and immunoglobulins have also been described. , Prophylactic treatment for haemophilia B has reduced the incidence of pseudotumours. Our patient, however, was on an episodic treatment schedule and on account of inadequate product replacement, had evidences of bleeding and deformities in major joints. There is limited literature on the surgical management of abdominal pseudotumour in haemophilia B. ,
The rarity of the condition and the multi-systemic involvement presented challenges for management. Although the bleeding during surgery was not significant, we could not be sure of the anticipated magnitude of bleed particularly if the resection had been more extensive. The occurrence of a bowel fistula in our patient is similar to the case report described by Pakala et al. 
We present this case to emphasise the vigilance needed for these patients undergoing surgery, anticipation of problems, management of bleeding and potential long-term outcomes. The anaesthetic issues were related to management of coagulopathy as outlined in the intra- and post-operative care.
| References|| |
Jayandharan GR, Srivastava A (2011) Hemophilia: Disease, Diagnosis and Treatment. J Genet Syndr Gene Ther S1:005. doi: 10.4172/2157-7412.S1-005.Accessed on 29 th
Tagariello G, Iorio A, Santagostino E, Morfini M, Bisson R, Innocenti M, et al.
Comparison of the rates of joint arthroplasty in patients with severe factor VIII and IX deficiency: An index of different clinical severity of the 2 coagulation disorders. Blood 2009;114:779-84.
López-Gómez J, Contreras JS, Figueroa-Ruiz M, Servín-Torres E, Velázquez-García J, Bevia-Pérez F, et al.
Management of the hemophilic pseudotumor of the abdomen: A rare pathological entity. Int J Surg Case Rep 2014;5:789-92.
Pakala A, Thomas J, Comp P. Hemophilic pseudotumor: A case report and review of literature. Int J Clin Med 2012;3:229-33.