Single-institution Series of Hirayama Disease in North America.

STUDY DESIGN: A retrospective chart review. OBJECTIVE: The aims of this study were to review pathophysiology, workup, and treatment for Hirayama disease (HD); and to assess outcomes from a single institution. SUMMARY OF BACKGROUND DATA: HD is a rare, painless, cervical myelopathy with distal upper extremity weakness, muscle wasting, and spinal cord atrophy. Disease progression-a consequence of repeat flexion injury-occurs up to 5 years from the initial diagnosis. METHODS: Single-institution review of pediatric HD patients from 2010 to 2020. RESULTS: Patients (n=10 male, n=2 female) presented in the second decade (14-20 y) with painless progressive distal upper extremity weakness and atrophy without sensory loss. Electromyography (n=12) demonstrated denervation in C7-T1 myotomes and flexion/extension magnetic resonance imaging showed focal cord atrophy and anterior displacement of the posterior dura with epidural enhancement in flexion. Treatment included observation and external orthoses (n=9) and anterior cervical discectomy with fusion (n=3). One of the 9 patients managed conservatively experienced further deterioration; no patient who underwent anterior cervical discectomy with fusion progressed. CONCLUSIONS: Patients with HD require a multidisciplinary approach to diagnosis and treatment to preserve function. Treatment is preventive and aims to minimize flexion injury by inhibiting motion across involved joints. First-line management is avoidance of neck flexion and use of rigid orthosis; in cases of failed conservative management and/or rapid clinical deterioration, surgical fixation can be offered.

Two-Surgeon Multidisciplinary Approach to Pediatric Cervical Spinal Fusion: A Single-Institution Series and Review of the Literature.

BACKGROUND: A collaborative 2-surgeon approach is becoming increasingly popular in surgery but is not widely used for pediatric cervical spine fusions. The goal of this study is to present a large single-institution experience with pediatric cervical spinal fusion using a multidisciplinary 2-surgeon team, including a neurosurgeon and an orthopedic surgeon. This team-based approach has not been previously reported in the pediatric cervical spine literature. METHODS: A single-institution review of pediatric cervical spine instrumentation and fusion performed by a surgical team composed of neurosurgery and orthopedics during 2002-2020 was performed. Demographics, presenting symptoms and indications, surgical characteristics, and outcomes were recorded. Particular focus was given to describe the primary surgical responsibility of the orthopedic surgeon and the neurosurgeon. RESULTS: A total of 112 patients (54% male) with an average age of 12.1 (range 2-26) years met the inclusion criteria. The most common indications for surgery were os odontoideum with instability (n=21) and trauma (n=18). Syndromes were present in 44 (39%) cases. Fifty-five (49%) patients presented with preoperative neurological deficits (26 motor, 12 sensory, and 17 combined deficits). At the time of the last clinical follow-up, 44 (80%) of these patients had stabilization or resolution of their neurological deficit. There was 1 new postoperative neural deficit (1%). The average time between surgery and successful radiologic arthrodesis was 13.2±10.6 mo. A total of 15 (13%) patients experienced complications within 90 days of surgery (2 intraoperative, 6 during admission, and 7 after discharge). CONCLUSIONS: A multidisciplinary 2-surgeon approach to pediatric cervical spine instrumentation and fusion provides a safe treatment option for complex pediatric cervical cases. It is hoped that this study could provide a model for other pediatric spine groups interested in implementing a multi-specialty 2-surgeon team to perform complex pediatric cervical spine fusions. LEVEL OF EVIDENCE: Level IV-case series.

The low utility of routine cranial imaging after pediatric shunt revision.

OBJECTIVE: Postoperative routine imaging is common after pediatric ventricular shunt revision, but the benefit of scanning in the absence of symptoms is questionable. In this study, the authors aimed to assess how often routine scanning results in a change in clinical management after shunt revision. METHODS: The records of a large, tertiary pediatric hospital were retrospectively reviewed for all consecutive cases of pediatric shunt revision between July 2013 and July 2018. Postoperative imaging was classified as routine (i.e., in the absence of symptoms, complications, or other direct indications) or nonroutine. Reinterventions within 30 days were assessed in these groups. RESULTS: Of 387 included shunt revisions performed in 232 patients, postoperative imaging was performed in 297 (77%), which was routine in 244 (63%) and nonroutine in 53 (14%). Ninety revisions (23%) underwent any shunt-related procedure after postoperative imaging, including shunt reprogramming (n = 35, 9%), shunt tap (n = 10, 3%), and a return to the operating room (OR; n = 58, 15%). Of the 244 cases receiving routine imaging, 241 did not undergo a change in clinical management solely based on routine imaging findings. The remaining 3 cases returned to the OR, accounting for 0.8% (95% CI 0.0%-1.7%) of all cases or 1.2% (95% CI 0.0%-2.6%) of cases that received routine imaging. Furthermore, 27 of 244 patients in this group returned to the OR for other reasons, namely complications (n = 12) or recurrent symptoms (n = 15); all arose after initial routine imaging. CONCLUSIONS: The authors found a low yield to routine imaging after pediatric shunt revision, with only 0.8% of cases undergoing a change in management based on routine imaging findings without corresponding clinical findings. Moreover, routine imaging without abnormal findings was no guarantee of an uneventful postoperative course. Clinical monitoring can be considered as an alternative in asymptomatic, uncomplicated patients.