Epidural Hematoma of the Cauda Equina in a Child with Hemophilia A

Travis, Susan F.; Greenbaum, Barbara H.; Mintz, Mark; White, Robert M.; Stein, Sherman C.; Steckler, Robert E.; Hirsch, Kenneth M.; Donaldson, Milton H.

doi:10.1097/00043426-199608000-00016

Cited by 11 publications

(10 citation statements)

References 9 publications

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“…MRI not only offers relatively rapid, noninvasive imaging for a PWH, but also provides detailed information concerning the size, location, and duration of the hematoma. 7 The most important aspect of nervous system hemorrhage management is the early replacement therapy. A prompt treatment increases the chances of a better outcome.…”

Section: Discussionmentioning

confidence: 99%

Spontaneous Hematomyelia in a Child With Hemophilia A

Aulakh¹,

Panigrahi²,

Naranje³

et al. 2009

Journal of Pediatric Hematology/Oncology

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Hemorrhagic complications in patients with hemophilia have been occasionally reported in the spinal column and the spinal cord. Treatment is based on prompt replacement therapy as the occurrence and development of neurologic dysfunction are related to the length of time between the onset of symptoms and the factor replacement. We report case of a 7-year-old hemophilic boy who presented with flaccid paraparesis resulting from thoracic hematomyelia. The patient showed gradual improvement on medical management with cryoprecipitate infusions. This case calls attention to the need for prompt diagnosis of rarely reported spinal hematomyelia based on clinical manifestations and radiologic features and highlights its management options in patients with hemophilia.

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Section: Discussionmentioning

confidence: 99%

Spontaneous Hematomyelia in a Child With Hemophilia A

Aulakh¹,

Panigrahi²,

Naranje³

et al. 2009

Journal of Pediatric Hematology/Oncology

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“…In this patient, FVIII activity level was maintained between 50 and 100% for the first 7 days, then between 30 and 80% for the following 7 days [6]. In the patient with the epidural haematoma, FVIII was infused as a bolus infusion every 8–12 h to keep a trough FVIII of 50% (0.50 U mL −1 ) for a week, then daily for a total of 3 weeks [7]. All three cases resulted in complete neurological recovery.…”

Section: Discussionmentioning

confidence: 99%

Successful use of B‐domain deleted factor VIII for resection of pituitary adenoma in a paediatric patient with severe haemophilia A

Hanrahan¹,

McKinnell²,

Storrs

et al. 2003

Haemophilia

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“…Eleven cases have been managed with surgical laminectomy and clot evacuation. 1,12,16,18,23,25,39,43,46,48 Three patients in these cases had a complete neurological deficit preoperatively and persistent neurological deficits postoperatively; 18,23,48 the remaining 8 patients recovered fully. Thirteen cases, including our own, were conservatively managed with factor replacement, blood transfusions, and close observation.…”

Section: Discussionmentioning

confidence: 98%

Conservative management of an acute spontaneous holocord epidural hemorrhage in a hemophiliac infant

Kiehna¹,

Waldron

Jane³

2010

PED

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Central nervous system hemorrhages are an uncommon but severe complication of hemophilia, occurring in only 2–8% of children with hemophilia. Less than 10% of these CNS hemorrhages are intraspinal. The authors report on their care of an infant with hemophilia A who presented with irritability, meningismus, and decreased spontaneous movement. These symptoms prompted imaging studies, which revealed a spinal epidural hematoma (SEH) extending from C-1 through the cauda equina. The boy was treated with factor replacement and close monitoring. Repeat radiographic imaging 14 days later demonstrated complete resolution, and the patient had returned to his normal baseline status. A literature review in the modern treatment era revealed 24 cases of SEH in children with hemophilia. Of these 24 cases, 11 underwent laminectomy and 13 received conservative treatment. All conservatively treated patients, 5 of whom had presented with weakness, experienced a full recovery. Of the 11 laminectomy patients, 10 presented with weakness and all but 3 experienced full neurological improvement. These 3 patients were notable for having previously undiagnosed hemophilia. An increased index of suspicion facilitates the essential management features of prompt diagnosis and correction of coagulopathies in children who present with SEHs. The authors apply a multidisciplinary approach involving a pediatric hematologist, neurosurgeon, and pediatric intensive care unit to ensure timely correction of the coagulation disorder, maintenance of adequate factor levels, and close hemodynamic and neurological monitoring. Observation with aggressive correction of coagulopathy is a reasonable treatment choice for hemophilic patients presenting with SEH and a stable neurological examination.

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Epidural Hematoma of the Cauda Equina in a Child with Hemophilia A

Cited by 11 publications

References 9 publications

Spontaneous Hematomyelia in a Child With Hemophilia A

Spontaneous Hematomyelia in a Child With Hemophilia A

Successful use of B‐domain deleted factor VIII for resection of pituitary adenoma in a paediatric patient with severe haemophilia A

Conservative management of an acute spontaneous holocord epidural hemorrhage in a hemophiliac infant

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