Ectasia dural e hipotensión endocraneal en síndrome de Marfán / Dural ectasia and intracranial hypotension in Marfan syndrome

INTRODUCCIÓN: El síndrome de Marfán es un trastorno multisistémico del tejido conectivo de herencia autosómica dominante, de expresión variable. La ectasia dural es un compromiso frecuente, pero poco conocido, que puede asociarse a síndrome de hipotensión endocraneana (SHE). OBJETIVO: Pre sentar un caso de cefalea invalidante secundario a SHE, para advertir de esta rara complicación, que debe tenerse presente en niños portadores de conectivopatías, en especial síndrome de Marfán. CASO CLÍNICO: Adolescente femenina de 13 años, portadora de sindrome de Marfán, de diagnóstico clínico según criterios de Ghent 2010, que consultó por cefalea ortostatica invalidante de 6 meses de evolución. La Resonancia Magnetica (RM) de cerebro mostró múltiples signos de hipotensión endocraneana, mientras que la RM de columna total mostró una ectasia dural que determinó la dilatación del saco tecal y remodelación posterior de los cuerpos vertebrales, especialmente a nivel del sacro. Se realizó tratamiento con parche sanguíneo autólogo epidural con buena respuesta clínica. CONCLUSIONES: La ectasia dural, frecuente en el sindrome de Marfán, es una causa predisponente a fuga de líquido cefaloraquideo (LCR), que podría causar cefalea ortostática segundaria al SHE.

INTRODUCTION: Marfan syndrome is an autosomal dominant, multi-systemic connective tissue di sorder of different presentations. Dural ectasia is a common, but little known complication that can be associated with intracranial hypotension syndrome (IHS). OBJECTIVE: To present a case of severe headache secondary to IHS in order to warn about this rare complication, which must be considered in children carriers of connective tissue diseases, especially Marfan syndrome. CLINICAL CASE: 13-year- old female carrier of Marfan syndrome, clinically diagnosed according to the 2010 Ghent criteria, who consulted due to a 6-months history of severe orthostatic headache. Head magnetic resonance imaging (MRI) showed multiple signs of intracranial hypotension, while whole-spine MRI showed dural ectasia that caused the thecal sac dilation and subsequent remodeling of vertebral bodies, es pecially the sacral ones. Treatment with an autologous epidural blood patch was administered with good clinical response. CONCLUSIONS: Dural ectasia, frequent in Marfan syndrome, is a predisposing cause of cerebrospinal fluid (CSF) leakage, which could cause orthostatic headache secondary to IHS.

Spontaneous intracranial hypotension and its complications / Hipotensão intracraniana espontânea e complicações

ABSTRACT Spontaneous intracranial hypotension (SIH) is a syndrome that was unknown until the advent of magnetic resonance imaging (MRI). It is a cause of orthostatic headache, which remains underdiagnosed and, rarely, can result in several complications including dural venous sinus thrombosis, subdural hematoma and subarachnoid hemorrhage. Some of these complications are potentially life-threatening and should be recognized promptly, mainly by imaging studies. We reviewed the MRI of nine patients with SIH and describe the complications observed in three of these patients. Two of them had subdural hematoma and one had a dural venous sinus thrombosis detected by computed tomography and MRI. We concluded that MRI findings are of great importance in the diagnosis of SIH and its complications, which often influence the clinical-surgical treatment of the patient.

RESUMO Hipotensão Intracraniana Espontânea (HIE) é uma síndrome desconhecida até o advento das imagens de Ressonância Magnética (RM). É uma causa de cefaleia ortostática que permanece subdiagnosticada e raramente resulta em complicações, como trombose de seios venosos durais, hematoma subdural e hemorragia subaracnoidea. Algumas dessas complicações são potencialmente ameaçadoras à vida e devem ser prontamente reconhecidas pelos estudos de imagem. Nós revisamos as RM de 9 pacientes com HIE e descrevemos as complicações observadas em 3 casos. Dois deles tiveram hematoma subdural e um teve trombose de seio venoso dural detectados por tomografia computadorizada e RM. Concluímos que achados de RM são de grande importância no diagnóstico de HIE e suas complicações, frequentemente influenciando o tratamento clínico-cirúrgico do paciente.