Arquivos Brasileiros de Neurocirurgia: Brazilian Neurosurgery 2016; 35(03): 228-233
DOI: 10.1055/s-0036-1586155
Case Report | Relato de Caso
Thieme Publicações Ltda Rio de Janeiro, Brazil

Treatment of Craniodiaphyseal Dysplasia Presenting with Chiari Type-I

Tratamento de displasia craniodiafisária com Chiari tipo I
Rafet Özay
1   Clinic of Neurosurgery, Diskapi Yildirim Beyazit Training and Research Hospital, Ankara, Turkey
,
Ebru Doğan Doruk
1   Clinic of Neurosurgery, Diskapi Yildirim Beyazit Training and Research Hospital, Ankara, Turkey
,
Mehmet Serdar Balkan
1   Clinic of Neurosurgery, Diskapi Yildirim Beyazit Training and Research Hospital, Ankara, Turkey
,
Mehmet Fikret Ergüngör
2   Department of Neurosurgery, Cumhuriyet University, Sivas, Turkey
› Author Affiliations
Further Information

Publication History

29 September 2015

01 December 2015

Publication Date:
26 July 2016 (online)

Abstract

Objective Chiari malformation type-I (CM-1) is described radiographically as a simple displacement of the cerebellar tonsils at least 5 mm below the foramen magnum (FM). If CM-1 exists due to hyperostosis of the cranial bones, the authors were not able to determine a common consensus for the treatment of CM-1 and syringomyelia.

Methods A 31-year-old-female presented to our hospital with bilateral facial paralysis, hypoesthesia and motor loss of the extremities. The patient had bilateral gag reflex loss, phonation disorder and dysarthric speaking. Sensory and motor deficits were available at the bilateral upper and lower extremities. The skeletal radiographs revealed extensive thickening and sclerosis of the calvarial and facial bones, moderate widening and sclerosis of the clavicles and ribs, and that the internal auditory canal (IAC) and the optic foramen (OF) were narrowed. CM-1 and syringomyelia secondary to the small posterior fossa were due to calvarial hyperostosis. The patient underwent posterior fossa decompression and duraplasty. In addition, a syringosubarachnoid shunt was placed at the level of C7-T1. The symptoms of lower cranial nerve palsy and motor loss were recovered, but the symptoms of the foraminal stenosis, such as visual and auditory losses and facial paralysis were not recovered in any way.

Conclusion We described in this case report CM-1 as a late complication of craniodiaphyseal dysplasia (CDD), and the difficulties in its treatment. In the treatment of these patients with CDD, posterior fossa decompression and syringosubarachnoid shunting are necessary, in spite of all the risks of these procedures.

Resumo

Objetivo A malformação Chiari tipo-I (MC-1) é descrita radiograficamente como um simples deslocamento da tonsila cerebelar de pelo menos 5 mm abaixo do forame magno (FM). Se houve MC-1 em função da hiperostose dos ossos do crânio, os autores não foram capazes de determinar um consenso para o tratamento de MC-1 e siringomielia.

Métodos Paciente de 31 anos deu entrada com paralisia facial bilateral, hipoestesia e perda motora das extremidades. A paciente apresentou perda de reflexo de vômito bilateral, desordem fonética e disartria. Deficiência motora e sensorial foram identificadas em ambas as extremidades superiores e inferiores. Radiografias do esqueleto revelaram extensa espessamento e esclerose dos ossos calvários e faciais, ampliação moderada e esclerose das clavículas e costelas, além de estreitamento do canal auditivo interno (CAI) e do forame ótico (FO). A MC-1 e siringomielia secundária à pequena fossa posterior ocorreram devido à hiperosteose calvarial. A paciente foi submetida a descompressão da fossa posterior e duroplastia. Somado a isso, um shunt siringo-subaracnóideo foi colocado na altura da C7-T1. Os sintomas de paralisia do nervo craniano inferior e a perda motora foram recuperados, mas os sintomas de estenose do forame, como por exemplo perdas auditivas e visuais, ou paralisia facial, não foram recuperados em nenhum nível.

Conclusão descrevemos neste relato de caso a MC-1 como uma complicação posterior da displasia craniodiafisária (DCD), além das dificuldades em seu tratamento. No tratamento desta paciente com DCD, são necessários a descompressão da fossa posterior e o shunt siringo-subaracnóideo, apesar de todos os riscos neste procedimento.

 
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