Surgical pathway proposal for severe paralytic scoliosis in adolescents with myelomeningocele.

PURPOSE: The increased life expectancy of patients with myelomeningocele led to the necessity of new management for addressing complex spine deformities with severe pelvic obliquity. Tethered cord release (TCR) and spinal fusion were described as treatment options. However, nowadays, the surgical strategy is not well defined and high rates of mechanical failures and complications are reported. Our aim was to propose a new two-stage surgical pathway to treat a selected group of severe myelomeningocele scoliosis. METHODS: This is a retrospective series of myelomeningocele scoliosis in paralytic adolescent patients treated with concurrent TCR and posterior spinal fusion (PSF), followed by delayed anterior fusion (ASF) through minimally invasive lateral approach. Inclusion criteria were as follows: evolutive scoliosis in tethered cord syndrome and paraplegia, main curve >70°, pelvic obliquity >20°, and age between 10 and 18 years. Clinical, surgical, and radiographic parameters were evaluated preoperatively and at a mean follow-up (FU) of 2.8 years. RESULTS: Six patients out of 58 met the inclusion criteria and were included in the study (mean age 12.3 years old). The mean curve and pelvic obliquity correction were 68.8% and 82%, respectively. No loss of correction was recorded at FU. One case of cerebrospinal fluid leakage requiring revision surgery was recorded. CONCLUSION: To the best of our knowledge, this is the first study proposing a two-stage surgical pathway for severe MMC spinopelvic deformity treatment. Concurrent TCR and PSF with delayed minimally invasive ASF permitted to minimize complications providing good fusion rates without loss of correction and implant failure.

Hydrocephalus due to a Cavernoma-Like Lesion of an Obliterated Cerebral Arteriovenous Malformation Treated by Embolization and Radiosurgery.

BACKGROUND: Gamma-Knife radiosurgery can be the treatment of choice for small cerebral arteriovenous malformations (AVMs) in eloquent brain areas or, in association with endovascular treatment, for large and complex AVMs. Among the possible delayed complications occurring after radiosurgery of AVMs, de novo formation of a cavernoma has only recently been described. The authors report a unique case of communicating hydrocephalus with highly proteinaceous cerebrospinal fluid due to a cavernoma-like lesion of an obliterated cerebral AVM treated by embolization and radiosurgery. CASE DESCRIPTION: A 43-year-old female patient with a left parieto-occipital AVM (Spetzler-Martin grade III) underwent a multimodality treatment comprising several endovascular procedures and Gamma-Knife radiosurgery. At the follow-up angiograms, the AVM was completely obliterated. Twelve years later, she came back to our attention for headache and vomit with the radiological evidence of brain edema, severe hydrocephalus, and a chronic encapsulated intracerebral hematoma. At the beginning, the drainage of hydrocephalus proved ineffective to improve symptoms and edema. Therefore, the surgical resection of both the obliterated AVM and the cavernoma-like lesion-histologically confirmed-was necessary. The hydrocephalus resolved as well as the cerebral edema after 3 weeks, without necessity of a ventriculoperitoneal shunt. CONCLUSIONS: In previous neurosurgical literature, the association of a caveroma-like lesion in an obliterated AVM and communicating hydrocephalus with highly proteinaceous cerebrospinal fluid has not yet been discussed. We believe that only the surgical resection of both the obliterated AVM and the cavernoma-like lesion can lead to complete clinical recovery of the hydrocephalus.

Paediatric spinal Langerhans cell histiocytosis requiring corpectomy and fusion at C7 and at Th8-Th9 levels.

An 11-year-old girl was treated by corpectomy and anterior fusion because of the destruction of the C7 vertebral body. Pathological studies were not conclusive. The outcome was excellent, but 18 months later, she required thoracic corpectomy with anterior fusion owing to the impending kyphotic fracture of the Th8 vertebral body. Langerhans cell histiocytosis was now recognised and chemotherapy was given. 3 years later, the disease appears well controlled with normal shape of both the operated vertebral levels and maintenance of the movements of the adjacent vertebrae.