Hemophilia A and Spinal Epidural Hematoma in Children

Dear Editor

With great interest I read the paper by Dr. Morsing et al. entitled “Hemophilia A and spinal epidural hematoma in children” [1]. The authors described a 13-months-old boy with Hemophilia A with irritability and unspecified pain, that refused to walk, but showed a normal neurological examination (except for a Horner syndrome on the right side) at presentation in the hospital, 48 h after the onset of the complaints. MRI of the spine resulted in the diagnosis of a large subacute posterior spinal epidural hematoma at C2-S1. Because of the absence of spinal cord compression symptoms, conservative treatment with correction of the coagulopathy and follow-up MRI was instituted. The boy did not need operative treatment, and the hematoma resolved completely within 3 weeks. The authors explained that this case illustrates that rapid and aggressive factor replacement and correction can lead to complete neurological recovery with conservative therapy in Hemophilia patients with SSEH.

At present there is a significant amount of literature about the treatment of patients with spontaneous spinal epidural hematomas (SSEH) [2] [3]. Most patients require acute operative decompression, however, also patients with spontaneous resolution have been described. The role of anticoagulants/coagulopathy in the etiology of SSEH still remains unsolved, but it is evident that, in general, hematomas in patients with clotting abnormalities are larger in size. However, larger hematomas are not equivalent with more severe neurological deficit [3]. On the contrary, in patients with coagulopathy, hematomas were larger in the conservative group (SSEH^cons) when compared with operative cases (SSEH^oper) [3]. It has been speculated that spreading of the hematoma along the spinal epidural space in the acute stage of hemorrhage (before clotting) plays a role in decompression of the intradural neural structures, promoting the spontaneous relief of neurological deficit [3].

In Morsing's case, clinical signs (irritability, pain, unwilling to walk) were present 48 h before MRI was made. MRI showed a very large sub-acute SSEH. Consequently, from the imaging perspective, the hematoma existed for more than 24 h [4]. Most SSEHs present with acute severe pain (in neck, back, arm or leg) followed by (rapidly) progressive sensory and/or motor deficit. In this patient alarming neurological signs or symptoms apparently were absent, despite of the very large and extensive hematoma. The referral to the paediatrician/neurologist has been in the “subacute” stage (after 24–48 h), which is in line with MRI findings. Therefore, the statement by the authors that aggressive factor replacement and correction has been vital in preventing operative treatment is purely speculative and very unlikely. In case of an ongoing bleeding, symptoms would have been severe and rapidly progressive. Such a patient would end up with a para- or tetra-plegia, and in this particular case also with respiratory insufficiency.

In the past, we have analysed a series of 333 patients with SSEH [2]. A total of 89 patients suffered coagulopathy (caused by medication in 77, and by disease in 12). In 81 (91%) of these patients solid hematomas were reported during surgery, and in 8 (9%) patients “semi-solid hematomas” were encountered. Of the remaining 244 patients (without coagulopathy), solid hematomas were reported in 238 (97.5%), a “liquid hematoma” was reported in only 1 patient [5], and in 5 patients profuse bleeding occurred during the removal of the epidural blood clot. This means that the intra-operative finding of an active spinal epidural bleeding is exceptional [6].

As such, the case reported by Morsing et al. is another example of a benign course of a SSEH. It illustrates that, fortunately, also in patients with a serious coagulopathy, spontaneous resolution of SSEH may occur. Nevertheless, most cases of SSEH, as is supported by the large number of cases reported in the literature, require urgent operative decompression. Conservative treatment of SSEH may only be considered in mild cases with a benign clinical course and provided that the patient is closely followed neurologically and with MRI, as in the present case by Dr. Morsing et al.

R. J. M. Groen

References

• 1 Morsing IE, Brons P, Draaisma JMTh. et al . Hemophilia A and spinal epidural hematoma in children.  Neuropediatrics. 2009;  40 245-248
• 2 Groen RJ, van Alphen HAM. Operative treatment of spontaneous spinal epidural hematomas: a study of the factors determining post-operative outcome.  Neurosurgery. 1996;  39 494-509
• 3 Groen RJ. Non-operative treatment of spontaneous spinal epidural hematomas: a review of the literature and a comparison with operative cases.  Acta Neurochir. 2004;  146 103-110
• 4 Chen CJ, Hsu WC. Imaging findings of spontaneous spinal epidural hematoma.  J Formos Med Assoc. 1997;  96 283-287
• 5 Huff JS. Spinal epidural hematoma associated with cocaine abuse.  Am J Emerg Med. 1994;  12 350-352
• 6 Groen RJ. Operative treatment of spontaneous spinal epidural hematomas: a study of the factors determining post-operative outcome.  Neurosurgery. 1997;  41 1219-1220

Correspondence