Case Reports

Indian Pediatrics 1999;36: 1151-1153

Acute Transverse Myelitis Due to Spinal Epidural Hematoma_First Manifestation of Severe Hemophilia

Case Report

A 10-year-old male presented with neck pain followed by sudden symmetrical onset of weakness involving all four limbs and accompanied by loss of all sensations over the trunk and limbs of one week duration. There was loss of bladder and bowel sensations with retention. There was no history of preceding febrile illness, recent intramuscular injections, local trauma or tuberculous contact. He was fully immunized. The child had received no treatment apart from symptomatic care at a local hospital prior to presentation.

On clinical evaluation, all vital parameters and anthropometry were normal. Neuro-logically, higher functions and cranial nerve examinations were normal. There was flaccid quadriplegia, deep tendon reflexes were exaggerated and plantar response was absent bilaterally. Sensory system examination revealed a loss of all modalities at and below C₅ spinal dermatome. There was a band of hyperesthesia at C₄ level, just below the clavicles. Sensations over the face and neck were preserved. Neck stiffness could be elicited on flexing, though there was no local tenderness over the cervical spine. All other systemic examination was normal.

Clinically, a diagnosis of acute transverse myelitis was considered and treatment with intravenous methyl prednisolone was begun, but the presence of neck stiffness prompted an early referral for MR imaging. MRI of the spine revealed an anterior epidural hematoma extending from C₂ to C₆ vertebral segments with its epicenter at C₅ level and causing spinal cord compression (Figs. 1 & 2).

Subsequently the coagulation profile was discovered to be abnormal: Activated PTT was prolonged (patient_74 seconds, control_47 seconds) which was corrected on mixing (patient-49 seconds, control-47 seconds). The prothrombin time was normal (patient_16 seconds, control_14 seconds). Factor VIII assay revealed a level of less than 1%. Other routine hematological parameters were normal.

Retrospectively, on careful enquiry the boy denied history of indirect trauma. There were no complaints of easy bruising, hematomas after DPT vaccine, prolonged oozing from loose avulsed teeth or mucous membrane bleeds. One of the maternal uncles had been noted to develop recurrent swelling of the knee followed by crippling deformity, but had never sought medical advice.

The child was managed conservatively with infusions of factor VIII concentrate. There was some improvement by the end of 2 weeks, power had returned in the distal joints to 2/5 Other neurological signs had not changed. Repeat MRI after 12 weeks showed complete resolution of the hematoma. Neurologically, a flicker of movement could be elicited at proximal joints, but no appreciable improve-ment was noted distally. Anesthesia persisted and bladder sensations had not returned. Lack of complete recovery was attributed to delay in diagnosis and treatment.

Discussion

Spontaneous CNS bleed is an uncommon  manifestation of hemophilia, and is usually precipitated by trauma, however trivial. Intraspinal bleeds account for 2-8% of CNS hemorrhages(2). Spinal epidural hematomas are rare; especially in children and exceptionally present as the first manifestation of severe hemophilia(3). The unusual feature of this case was the spontaneous nature of the epidural bleed and the complete absence of any manifestations prior to this event at the age of 10 years, despite severe factor VIII deficiency. Epidural hematomas are more common posterior to the spinal cord, anterior hematomas being relatively infrequent(4). The anterior location of the bleed in our child was the other unusual feature. Most of the cases reported have been infants or children less than 5 years of age(2,3,5-9), the youngest being 3 months old(7). Signs and symptoms are often atypical in infants, making the diagnosis difficult(2,3,6). In a literature review, less than 20 pediatric patients were reported with spinal hematomas(2,3,5-9) and none from India.

Surgical evacuation of hematomas by decompression laminectomy has its inherent complications and mortality as high as 22%(5). Recent reviews support treatment of selected cases with mild or stable neurological deficit conservatively with factor VIII concentrate infusions begun as early during the course as possible(2,5,6,8,9). Neurological recovery is gradual and complete recovery is possible over several weeks(2,5,6). However decompression laminectomy needs to be considered where diagnosis and treatment have been delayed and in cases where symptoms do not resolve with conservative management or are progressive(6,9).

This presentation stresses the need for prompt neuroimaging in every pediatric patient, especially a male, presenting with signs of acute transverse myelitis for accurate diagnosis of the underlying disorder so that specific treatment can prevent or reduce neurological sequel. Non-invasive modalities like CT or MRI are preferable(5,6,9).

Acknowledgement

The authors thank Dr. P.M. Pai, Dean of the Seth G.S. Medical College & K.E.M. Hospital for giving permission to publish the article.

References

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2. Hutt PJ, Herold ED, Koenig BM, Gilchrist GS. Spinal extradural hematoma in an infant with hemophilia A: An unusual presentation of a rare complication. J Pediatr 1996; 128: 704- 706.

3. Margraf S, Abel M. Epidural hematoma as the initial manifestation of moderately severe hemophilia A. Klinische Paediatrie 198 6; 198: 497-499.

4. Patel H, Boaz JC, Phillps JP, Garg BP. Spontaneous spinal epidural hematoma in children. Pediatr Neurol 1998; 19: 302-307.

5. Noth I, Hutter JJ, Meltzer PS, Damiano ML, Carter LP. Spinal epidural hematoma in a hemophiliac infant. Am J Pediatr Hematol Oncol 1993; 15: 131-134.

6. Travis SF, Greenbaum BH, Mintz M, White RM, Stein S, Steckler RF, et al. Epidural hematoma of a cauda equina in a child with hemophilia A. J Pediatr Hematol Oncol 1996; 18: 308-310.

7. Faillace WJ, Warrier I, Canady AI. Paraplegia after lumbar puncture in an infant with previously undiagnosed Hemophilia A: Treatment and peri-operative considerations. Clin Pediatr 1989; 28: 136-138.

8. Narawong D, Gibbons VP, McLaughlin JR, Bonhasin JD, Kotagal S. Conservative management of spinal epidural hematoma in hemophilia. Pediatr Neurol 1988; 4: 169- 171.

9. Hamre MR, Haller JS. Intraspinal hematomas in Hemophilia. Am J Pediatr Hematol Oncol 1992; 14: 166-169.