Perioperative management of a child with von Willebrand disease undergoing surgical repair of craniosynostosis: looking at unusual targets.

We report the successful management of a craniosynostosis repair in a child with severe Type I von Willebrand disease diagnosed during the preoperative assessment and treated by coagulation factor VIII and ristocetin cofactor. Collaboration among the anesthesiologist, the neurosurgeon, the clinical pathologist, and the pediatric hematologist is important for successful management.

Management of diffuse glioma in children: a retrospective study of 27 cases and review of literature.

Gliomas are the most common CNS tumours in children and present either as circumscribed tumours or diffusely infiltrative neoplasms. Diffuse gliomas develop both in the cerebral hemispheres and the brainstem and have a poor prognosis. Guidelines for the therapy of these tumours are still debated. In this study, we reviewed the clinical features of 27 consecutive patients with diffuse gliomas admitted to the Department of Paediatrics of CHR Citadelle, University of Liège, between 1985 and 2005. We review their clinical presentation, diagnosis, treatment and outcome with reference to the published literature.

Reversible tonsillar prolapse and syringomyelia after embolization of a tectal arteriovenous malformation. Case report and review of the literature.

The authors report the case of a 21-year-old woman who presented with headaches, frequent sensations of loss of equilibrium, and intermittent strabismus. A tectal arteriovenous malformation (AVM) was diagnosed based on magnetic resonance (MR) imaging findings. The AVM drained toward the straight sinus and was associated with a tonsillar prolapse (Chiari malformation Type I [CM-I]) and cervical syringomyelia. The tectal AVM was embolized with N-butyl cyanoacrylate, and disconnection of about 80% of the lesion was obtained. All clinical symptoms resolved after embolization, and radiosurgery was proposed to treat the malformation remnant. A control MR image confirmed the regression of the tonsillar prolapse and the disappearance of the syrinx. This report emphasizes that CM-I and syringomyelia may be acquired and related to hydrovenous disorders.

Shunting of Syringomyelic Cavities by Using a Myringotomy Tube: Technical Note and Long-Term Results.

BACKGROUND: Syringomyelia is a progressive cerebrospinal fluid disorder that can lead to irreversible spinal cord injury. To date, the optimal management of syringomyelic cavities remains controversial. Multiple studies have emphasized the importance of the craniocervical decompression or shunting procedures; however, except for syrinx related to Chiari malformation, nearly one-half of patients need to undergo reoperation. The purpose of the present study was to describe a simple and efficient surgical technique and to report the long-term radioclinical outcomes. MATERIAL AND METHODS: We report a series of 17 consecutive patients (10 male, 7 female) with symptomatic syringomyelia treated by myringotomy tube between January 1999 and January 2014. The mean follow-up was 43.6 months (5-118). For each case, a laminectomy was carried out at the level of the most expanded part of the syrinx. The myringotomy tube was then placed through a puncture myelotomy. RESULTS: Clinical examination showed disappearance of symptoms in 3 cases, a significant improvement in 8 cases, stabilization in 5 patients, and continuation of neurologic deterioration in 1 case. Thus, favorable long-term outcomes were observed in two-thirds of patients. No permanent postoperative complication was reported. The postoperative imaging showed complete or almost complete disappearance of the syrinx in 11 cases and a reduction over 80% in 4 cases. In 2 cases, the cavity remained stable. Moreover, just 2 patients had to undergo reoperation with the placement of a syringoperitoneal shunt. CONCLUSION: A myringotomy tube is a simple surgical technique that seems to be an efficient and safe treatment for syringomyelic cavities.

Epidural hematoma after cervical spine surgery.

The authors report an acute epidural hematoma after the surgical removal of a cervical C6-C7 disc herniation through an anterolateral approach of the cervical spine. Clinical history consisted of respiratory distress and flaccid tetraplegia that appeared 2.5 hours after surgery. Without any complementary radiologic investigation, the patient was immediately transferred to the operating room for a second look, which was unsuccessful. Magnetic resonance imaging performed after this second surgical procedure showed an anterior cervical hematoma extending from C3 to T3 without significant spinal cord compression. A cervical laminectomy was performed to evacuate the hematoma. The patient was extubated the next morning and discharged from the hospital after 5 days with no residual neurologic deficit. An extensive postoperative investigation revealed no coagulation disorder.

Familial colloid cyst of the third ventricle: neuroendocrinological follow-up and review of the literature.

Colloid cysts of the third ventricle are rare, benign cysts of endodermal origin. Between 1989 and 1999, eight patients with this lesion (five females, three males), with a mean age of 40.5 years (range 20-54), were identified out of 1354 operated for tumours of the central nervous system. Among the eight, two were familial. They were half sisters 38 and 28 years-old, who were diagnosed to have colloid cysts of the third ventricle on CT scanning. Transcortical excision yielded 10 and 15 mm sized colloid cysts, respectively. Moreover, both sisters developed a multinodular goiter associated with these congenital tumours. The second sibling developed hyperprolactinemia associated with macroprolactinemia. Pregnancy was only possible after bromocriptine treatment. These cases provide further evidences that colloid cysts probably have an autosomic recessive pattern of inheritance with variable penetrance.