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Sarah C. Jernigan, Jay G. Berry, Dionne A. Graham, Stuart B. Bauer, Lawrence I. Karlin, Nedda M. Hobbs, R. Michael Scott and Benjamin C. Warf


Although survival for patients with myelomeningocele has dramatically improved in recent decades, the occasional occurrence of sudden, unexplained death in young adult patients with myelomeningocele has been noted by the authors. This study was undertaken to determine risk factors for sudden death in this population.


The authors performed a retrospective chart review of patients born between 1978 and 1990 who received care at Children's Hospital Boston. The relationship between sudden death and patient demographics, presence of CSF shunt and history of shunt revisions, midbrain length as a marker for severity of hindbrain malformation, seizures, pulmonary and ventilatory dysfunction, body mass index, scoliosis, renal dysfunction, and cardiac disease was evaluated using the t-test, Fisher exact test, and logistic regression analysis.


The age range for 106 patients in the study cohort was 19–30 years, with 58 (54.7%) women and 48 (45.3%) men. Six patients, all of whom were young women, experienced sudden death. In multivariate analysis, female sex, sleep apnea, and midbrain elongation ≥ 15 mm on MR imaging remained significantly associated with a higher risk of sudden death. These risk factors were cumulative, and female patients with sleep apnea and midbrain length ≥ 15 mm had the greatest risk (adjusted risk ratio 24.0, 95% CI 7.3–79.0; p < 0.05). No other comorbidities were found to significantly increase the risk of sudden death.


Young adult women with myelomeningocele are at significantly increased risk of sudden death in the setting of midbrain elongation and sleep apnea. Further investigation is needed to determine the benefit of routine screening to identify at-risk patients for closer cardiopulmonary monitoring and treatment.

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Bram P. Verhofste, Michael P. Glotzbecker, Michael T. Hresko, Patricia E. Miller, Craig M. Birch, Michael J. Troy, Lawrence I. Karlin, John B. Emans, Mark R. Proctor and Daniel J. Hedequist


Pediatric cervical deformity is a complex disorder often associated with neurological deterioration requiring cervical spine fusion. However, limited literature exists on new perioperative neurological deficits in children. This study describes new perioperative neurological deficits in pediatric cervical spine instrumentation and fusion.


A single-center review of pediatric cervical spine instrumentation and fusion during 2002–2018 was performed. Demographics, surgical characteristics, and neurological complications were recorded. Perioperative neurological deficits were defined as the deterioration of preexisting neurological function or the appearance of new neurological symptoms.


A total of 184 cases (160 patients, 57% male) with an average age of 12.6 ± 5.30 years (range 0.2–24.9 years) were included. Deformity (n = 39) and instability (n = 36) were the most frequent indications. Syndromes were present in 39% (n = 71), with Down syndrome (n = 20) and neurofibromatosis (n = 12) the most prevalent. Eighty-seven (48%) children presented with preoperative neurological deficits (16 sensory, 16 motor, and 55 combined deficits).

A total of 178 (96.7%) cases improved or remained neurologically stable. New neurological deficits occurred in 6 (3.3%) cases: 3 hemiparesis, 1 hemiplegia, 1 quadriplegia, and 1 quadriparesis. Preoperative neurological compromise was seen in 4 (67%) of these new deficits (3 myelopathy, 1 sensory deficit) and 5 had complex syndromes. Three new deficits were anticipated with intraoperative neuromonitoring changes (p = 0.025).

Three (50.0%) patients with new neurological deficits recovered within 6 months and the child with quadriparesis was regaining neurological function at the latest follow-up. Hemiplegia persisted in 1 patient, and 1 child died due a complication related to the tracheostomy. No association was found between neurological deficits and indication (p = 0.96), etiology (p = 0.46), preoperative neurological symptoms (p = 0.65), age (p = 0.56), use of halo vest (p = 0.41), estimated blood loss (p = 0.09), levels fused (p = 0.09), approach (p = 0.07), or fusion location (p = 0.07).


An improvement of the preexisting neurological deficit or stabilization of neurological function was seen in 96.7% of children after cervical spine fusion. New or progressive neurological deficits occurred in 3.3% of the patients and occurred more frequently in children with preoperative neurological symptoms. Patients with syndromic diagnoses are at higher risk to develop a deficit, probably due to the severity of deformity and the degree of cervical instability. Long-term outcomes of new neurological deficits are favorable, and 50% of patients experienced complete neurological recovery within 6 months.