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LETTER TO THE EDITOR
Year : 2012  |  Volume : 7  |  Issue : 3  |  Page : 239-240
 

Perioperative management of a hemophilia B infant with intracranial hemorrhage


1 Department of Anaesthesiology and Intensive Care, GB Pant Hospital and Maulana Azad Medical College, J L Nehru Marg, New Delhi, India
2 Department of Neurosurgery, GB Pant Hospital and Maulana Azad Medical College, J L Nehru Marg, New Delhi, India

Date of Web Publication25-Jan-2013

Correspondence Address:
Pragati Ganjoo
Department of Anesthesiology and Intensive Care, GB Pant Hospital and Maulana Azad Medical College, J L Nehru Marg, New Delhi - 110 002
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1817-1745.106494

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How to cite this article:
Juneja S, Ganjoo P, Tandon MS, Sharma A. Perioperative management of a hemophilia B infant with intracranial hemorrhage. J Pediatr Neurosci 2012;7:239-40

How to cite this URL:
Juneja S, Ganjoo P, Tandon MS, Sharma A. Perioperative management of a hemophilia B infant with intracranial hemorrhage. J Pediatr Neurosci [serial online] 2012 [cited 2019 Jul 19];7:239-40. Available from: http://www.pediatricneurosciences.com/text.asp?2012/7/3/239/106494


Dear Sir,

A 2-month-old, 4.5 kg, infant was admitted with irritability, refusal of feeds, and seizures for 3 days with an altered sensorium, bulging anterior fontanel, right limb weakness, and pallor. There was no history of fever, trauma or drug use; his maternal grandfather had hemophilia B. Computed tomography of the head showed a left parietal subdural hematoma with the midline shift; cerebral angiography was normal. Hematology revealed a hemoglobin (Hb) of 4 g%, prolonged activated partial thromboplastin time (aPTT) of 120 (32.6) sec, low-plasma factor IX (FIX) activity of 4.3%, adequate platelets and normal bleeding, clotting and prothrombin times. Hemophilia B was diagnosed and 400 international units (IU) of FIX and 50 ml of packed red cells (PRC) were administered; his Hb and aPTT improved to 9.7 g% and 30/28 sec, respectively. Emergency craniotomy and hematoma evacuation was undertaken; 400 IU of FIX was repeated half-an-hour before surgery. The operation was meticulously performed to minimize surgical blood loss; extra care was taken to prevent bleeding and hematoma formation at non-operative sites that included, gentle oropharyngeal and tracheal suctioning, adequate pressure point padding and avoiding intramuscular injections. The child had an uneventful intraoperative course; blood loss averaged 50 ml and 50 ml PRC and 50 ml fresh frozen plasma (FFP) were transfused. Postoperatively, he was ventilated overnight and intubated uneventfully the following day; FIX (200 IU/day) was administered for two more weeks raising his plasma FIX activity to 60%. He improved neurologically and had no re-bleeding.

Hemophilia B is an X-linked, recessive coagulation disorder secondary to deficiency or defect of FIX; it affects 1:25,000 males and accounts for 15% of all hemophilia cases. Patients with mild (FIX ~5-40%) and moderate disease (FIX ~1-5%) have excessive bleeding following minor trauma, while severe hemophiliacs (FIX < 1%) often manifest with spontaneous hemorrhages. [1] Intracranial hemorrhages (ICH) are known (incidence ~2.2-7.8%), and are among the commonest causes of death in hemophiliacs (mortality ~34%); [2],[3] ICH may be the first manifestation of hemophilia, [2],[4] also seen in our patient.

Surgery for ICH in hemophiliacs is a high-risk procedure though use of perioperative clotting factor has markedly improved results. [5] Preoperative normalization of FIX activity up to 60-80%, and its postoperative maintenance to over 30% for at least 14 days is recommended. [1] Factor doses (IU) are calculated by the desired rise in factor activity (%) × body weight (kg), and once daily dose of FIX is adequate; monitoring is by FIX assays or serial aPTT (acceptable values ~patients' aPTT/normal ≤1.2).

Thus a safe surgical outcome in hemophiliacs with ICH is possible with perioperative clotting factor and FFP therapy besides the routine precautions.

 
   References Top

1.Guidelines for the Management of Haemophilia. World Federation of Haemophilia 2005. Available from: http://www.wfh.org/2/docs/Publications/Diagnosis_and_Treatment/Guidelines_Mng_Hemophilia.pdf. [Last Accessed on 2011 Dec 24].  Back to cited text no. 1
    
2.Rodriguez V, Schmidt KA, Slaby JA, Pruthi RK. Intracranial haemorrhage as initial presentation of severe haemophilia B: Case report and review of mayo clinic comprehensive hemophilia center experience. Haemophilia 2005;11:73-7.  Back to cited text no. 2
[PUBMED]    
3.Ljung RC. Intracranial haemorrhage in haemophilia A and B. Br J Haematol 2008;140:378-84.  Back to cited text no. 3
[PUBMED]    
4.Gerlacher GR. Spontaneous intracranial hemorrhage as the presenting sign of hemophilia B in a 3-month-old infant. Pediatr Emerg Care 1999;15:416-8.  Back to cited text no. 4
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5.Agrawal D, Mahapatra AK. Spontaneous subdural hematoma in a young adult with hemophilia. Neurol India 2003;51:114-5.  Back to cited text no. 5
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