Congenital spinal intradural arachnoid cyst associated with intrathoracic meningocele in a child

Abstract

Abstract:Congenital spinal intradural arachnoid cyst associated with intrathoracic meningocele is very rare. We report a case in a 9-year-old Chinese boy who presented with a two-week history of progressive paraparesis and gait ataxia. Magnetic resonance imaging revealed that a dorsal intradural extramedullary cystic lesion extended from T1 to T5 and compressed the spinal cord. A left lateral intrathoracic meningocele pouch was found incidentally at the level of T1. The arachnoid cyst as well as meningocele was removed and the spinal cord compression was relieved. Arachnoid cyst was confirmed by histological examination. The patient recovered well postoperatively. This is the second report of such a case in the world according to the available literature. The take-home message for our case is that the surgical approach should be individualized, depending on the size and location.

Surgical repair of a large intrathoracic meningocele associated with neurofibromatosis type 1 after failed cystoperitoneal shunts: illustrative case

“…Respiratory or neurological symptoms may supervene, like paraparesis, back pain, dyspnea, cough, and palpitations. 12 Described techniques of surgical treatment are broadly divided into three categories: (a) CSF diversionary procedures, namely cystoperitoneal or lumboperitoneal shunts; (b) intradural approach for meningocele neck repair; and (c) extradural approach for resection or plication of meningocele. Posterior laminectomies, costotransversectomies, or the transthoracic method by thoracotomy or thoracoscopy are variably used for exposure.…”

“…Respiratory or neurological symptoms may supervene, like paraparesis, back pain, dyspnea, cough, and palpitations. 12 …”

Large intrathoracic meningocele in a patient with neurofibromatosis type 1

“…3,5 However, conservative treatment carries the risk of progressive paralysis of the lower limbs, bleeding, and dyspnea. [6][7][8][9] Simple suction of the cysts usually results in recurrence, and intracranial pressure would be reduced when there is a large amount of cystic fluid loss. The spinal cord or nerve may be damaged by blind puncture.…”

Symptomatic Spinal Cord Compression from an Intradural Arachnoid Cyst with Associated Syrinx in a Child: Case Report

“…As to why some enlarge over time, possible mechanisms include a unidirectional ball-valve flow effect that leads to accumulation of a pocket of CSF, possible secretions from cells in the cyst wall, or possible changes in osmotic intracellular contents of cells in the cyst wall that lead to a transudative fluid accumulation [8]. Interestingly, two reports in the literature of spinal arachnoid cysts have been reported with associated intrathoracic meningoceles in children, as well [5]. …”

“…To our knowledge, of the previously reported pediatric cases of intradural extramedullary spinal arachnoid cysts, none have been reported to be associated with a spinal cord syrinx [1,2,3,4,5,6]. We present a case of such a lesion, treated with surgical fenestration and subtotal resection, followed by placement of a syringosubarachnoid shunt, which led to improvement in the patient's paraparesis.…”