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Vivek A. Mehta, Chetan Bettegowda, George I. Jallo and Edward S. Ahn

Craniosynostosis, the premature closure of cranial sutures, has been known to exist for centuries, but modern surgical management has only emerged and evolved over the past 100 years. The success of surgery for this condition has been based on the recognition of scientific principles that dictate brain and cranial growth in early infancy and childhood. The evolution of strip craniectomies and suturectomies to extensive calvarial remodeling and endoscopic suturectomies has been driven by a growing understanding of how a prematurely fused cranial suture can affect the growth and shape of the entire skull. In this review, the authors discuss the early descriptions of craniosynostosis, describe the scientific principles upon which surgical intervention was based, and briefly summarize the eras of surgical management and their evolution to present day.

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Vivek A. Mehta, Chetan Bettegowda, Sebastian A. Ahmadi, Petra Berenberg, Ulrich-Wilhelm Thomale, Ernst-Johannes Haberl, George I. Jallo and Edward S. Ahn

Object

Symptom response to spinal cord untethering, and the impact of duraplasty and scoliosis on retethering, are poorly understood in tethering after myelomeningocele (MMC) repair. In this retrospective study, the authors examined the outcomes of children who developed first-time spinal cord tethering following MMC repair. The response of symptoms to untethering and the role of duraplasty and scoliosis in retethering are explored.

Methods

The authors performed a review of 54 children with first-time symptomatic spinal cord tethering following MMC repair to determine the impact of untethering on symptoms, the impact of dural repair type on retethering, and the role of scoliosis on the prevalence and time to retethering.

Results

The average patient age was 10.3 ± 4.9 years, and 44% were males. The most common presenting symptoms of tethered cord syndrome were urinary (87%), motor (80%), gait (78%), and sensory (61%) dysfunction. The average postoperative time to symptom improvement was 2.02 months for sensory symptoms, 3.21 months for pain, 3.50 months for urinary symptoms, and 4.48 months for motor symptoms, with sensory improvement occurring significantly earlier than motor improvement (p = 0.02). At last follow-up (an average of 47 months), motor symptoms were improved in 26%, maintained in 62%, and worsened in 11%; for sensory symptoms, these rates were 26%, 71%, and 3%, respectively; for pain, 28%, 65%, and 7%, respectively; and for urinary symptoms, 17%, 76%, and 7%, respectively. There was no difference in symptom response with type of dural repair (primary closure vs duraplasty). Symptomatic retethering occurred in 17 (31%) of 54 patients, but duration of symptoms, age at surgery, and type of dural repair were not associated with retethering. Scoliosis was not associated with an increased prevalence of retethering, but was associated with significantly earlier retethering (32.5 vs 61.1 months; p = 0.042) in patients who underwent additional untethering operations.

Conclusions

Symptomatic retethering is a common event after MMC repair. In the authors' experience, sensory improvements occur sooner than motor improvements following initial untethering. Symptom response rates were not altered by type of dural closure. Scoliosis was associated with significantly earlier retethering and should be kept in mind when caring for individuals who have had previous MMC repair.

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Vivek A. Mehta, Chetan Bettegowda and George I. Jallo

Tumoral calcinosis (TC), a calcium hydroxyapatite–based mass, is common in the extremities and hips, but has rarely been reported in the spine, and has never been reported within the spinal cord. It may occur sporadically, in familial form, or as a consequence of disorders that promote soft-tissue calcification. Gross-total resection appears to be curative, but the diagnosis of TC is rarely considered prior to surgery. In this report, the authors describe the management of the first case of intramedullary TC located at the T-5 level in a 20-month-old boy who presented with lower-extremity spasticity. Additionally, salient features of the TC diagnosis, radiological patterns, histological findings, treatment, and outcomes are discussed.