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Erik C. Brown, Kunal Gupta and Christina Sayama

Lateral meningocele syndrome (LMS) is a rare genetic connective tissue disorder. It is associated with morphological changes similar to those of other connective tissue disorders, with the unique distinction of multiple, often bilateral and large, lateral meningoceles herniating through the spinal foramina. In some cases, these lateral meningoceles can cause pain and discomfort due to their presence within retroperitoneal tissues or cause direct compression of the spinal nerve root exiting the foramen; in some cases compression may also involve motor weakness. The presence of lateral meningoceles imposes unique challenges related to CSF flow dynamics, especially with concurrent Chiari malformation, which also occurs with increased frequency in individuals with LMS.

The authors present the case of a 6-month-old female with LMS with multiple lateral meningoceles throughout the thoracic and lumbar spine. The infant experienced a focal neurological abnormality due to enlargement of her lateral meningoceles following decompression of a symptomatic Chiari malformation and endoscopic third ventriculostomy. The finding was reversed through implantation of a ventriculoperitoneal shunt, which reduced the burden of CSF upon the lateral meningoceles. Such a case compels consideration that CSF flow dynamics in addition to altered connective tissue play a role in the presence of lateral meningoceles in patients within this and similar patient populations.

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Diana Ko, Daniel Blatt, Chafic Karam, Kunal Gupta and Ahmed M. Raslan

Nusinersen (Spinraza) is a US Food and Drug Administration–approved intrathecal medication for the treatment of spinal muscular atrophy (SMA). Adult patients with SMA often undergo thoracolumbar fusion to treat neurogenic scoliosis, preventing thecal access. The authors report a laminotomy technique and the ease of intrathecal access in three SMA patients with prior thoracolumbar fusions.

Patients were positioned in the lateral decubitus position or prone. Lumbar laminotomy was performed below the conus, between the lateral longitudinal rods, to preserve mechanical stability. Fluoroscopy provided real-time identification of instruments. Hardware was contoured with a carbide drill bit to develop the surgical window. Fiducial screws were placed along the perimeter for demarcation. Sublaminar wire removal caused dural defects that were repaired with a layer of dural substitute onlay and sealant. All patients successfully received nusinersen thecal injections via lumbar puncture by an interventional radiologist. Fluoroscopy time ranged from 6 to 36 seconds. No postoperative pseudomeningoceles, cerebrospinal fluid leaks, or wound complications occurred.

For patients with SMA and posterior fusion from prior scoliosis treatment, lumbar laminotomy is an effective method for creating thecal access for the administration of nusinersen.

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Kunal Gupta, Hatem A. Sabry, Aclan Dogan, Nicholas D. Coppa, Sean McMenomey, Johnny B. Delashaw and Ahmed M. Raslan

OBJECT

Middle fossa floor dehiscence (MFFD) can present as multiple syndromes depending on dehiscence location, tissue herniation, and dural integrity. The authors propose a classification system for MFFD with the potential to guide clinical decision making.

METHODS

A retrospective analysis of the electronic medical records (years 1995–2012) of patients who had undergone temporal craniotomy for the surgical repair of an MFFD syndrome at a single institution was undertaken. Reviewed data included demographic, operative, presentation, and outcome details. Middle fossa floor dehiscence was classified as follows: Class A, bony dehiscence without herniation of the brain and/or meninges; Class B, herniation of the brain and/or meninges through the middle fossa floor without CSF leakage; Class C, dehiscence with CSF leakage without meningitis; or Class D, dehiscence with meningitis.

RESULTS

Fifty-one patients, 22 males and 29 females, were included in the analysis. The mean age was 48.7 ± 15.5 years, mean body mass index was 32.65 ± 6.86 kg/m2, and mean symptom duration was 33 ± 42 months. Seven patients underwent repeat surgery for symptomatic recurrence; therefore, there were 58 surgical encounters. Repair included bony reconstruction with hydroxyapatite with or without resection of encephaloceles and/or repair of a dural defect. According to the MFFD classification system described, 15, 8, 27, and 8 cases were categorized as Class A, B, C, and D, respectively. The prevalence of hearing loss was 87%, 63%, and 70% in Classes A, B, and C, respectively. Vestibular symptoms were more prevalent in Class A. Seven patients reported persistent symptoms at the last follow-up. Transient complications were similar in each classification (13%–25%), and a single permanent complication related to anesthesia was observed. There were no mortalities or severe neurological morbidities in the series.

CONCLUSIONS

Middle fossa floor dehiscence has a spectrum of clinical presentations. A classification system may help to clarify the diagnosis and guide therapy. Surgery, the mainstay of treatment, is safe and well tolerated.