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Robert L. Conway, Moise Danielpour, and John M. Graham Jr.

✓The authors report on three patients with a congenital brain overgrowth syndrome, macrocephaly–cutis marmorata telangiectatica congenita (M-CMTC), who experienced neurological sequelae associated with herniation of the cerebellar tonsils through the foramen magnum. In two of these patients, the authors document postnatal brain overgrowth that contributed to recurrent descent of the cerebellar tonsils and complicated the surgical treatment in one of the patients. The authors address the neurosurgical concerns related to this syndrome with special attention to acquired tonsillar ectopia and postulate that some patients may be at risk for progressive tonsillar herniation and consequent neurological symptoms due to cerebellar overgrowth. Ectopic cerebellar tonsils have been reported previously in cases of M-CMTC, and this phenomenon may be a secondary event associated with brain overgrowth rather than due to a congenitally small posterior fossa.

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Harold L. Rekate

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Robert M. Conway, Nathan C. Tu, Pedrom C. Sioshansi, Dennis I. Bojrab II, Jeffrey T. Jacob, and Seilesh C. Babu

Cochlear implantation (CI) has become an option for the treatment of hearing loss after translabyrinthine resection of vestibular schwannomas. The surgical video presents the case of a 67-year-old male who had translabyrinthine resection of vestibular schwannoma with simultaneous CI and closure with a hydroxyapatite (HA) cement cranioplasty. HA cement cranioplasty can be utilized in place of abdominal fat graft for the closure of translabyrinthine approaches with similar efficacy and complication profile. To the authors’ knowledge, this is the first reported case of a simultaneous CI and translabyrinthine resection of vestibular schwannoma with HA cement cranioplasty.

The video can be found here: