Melorheostosis: segmental osteopoikilosis or a separate entity?

PURPOSE: Melorheostosis is a progressive hyperostotic bone disease that commonly affects the appendicular skeleton. Melorheostosis has a significant degree of overlap with other hyperostosis conditions including osteopoikilosis and likely represent varying degrees of a clinical spectrum. METHODS: This is a report of 2 patients with melorheostosis who presented with different clinical presentations and involvement of different anatomic locations. RESULTS: One of the patients presented with foot size asymmetry along with intermittent foot pain and limping. This patient also had irregular nonblanching yellow plaque-like lesion on the forehead. The second patient presented with abnormal gait but no pain. Radiographs demonstrated endosteal hyperostosis, which were consistent with a mixed picture of melorheostosis and osteopoikilois. Genetic testing of the LEMD3 gene from a blood sample was negative in both cases. CONCLUSIONS: The diagnosis of melorheostosis can be made based on the clinical and radiographic features and can be challenging to differentiate from other hyperostosis conditions. LEVEL OF EVIDENCE: Level V.

Scoliosis in Children With Aicardi Syndrome.

BACKGROUND: Aicardi syndrome (ACS) is a rare neurodevelopmental disorder that was classically characterized by the triad of agenesis of corpus callosum, infantile spasms, and chorioretinal lacunae. As new cases emerge and new common phenotypic features are being described in subsequent reports, new modified diagnostic criteria have been proposed that now classify the observed costovertebral abnormalities as supporting diagnostic features. To our knowledge there are no previous studies focusing and describing the scoliosis observed in children with ACS. METHODS: We screened billing lists to identify patients seen in the Division of Orthopaedic Surgery at our institution with a diagnosis of ACS that were treated for scoliosis after 2001. A total of 5 patients were identified. Medical records and radiographs were retrospectively reviewed in all cases. In all of the patients the diagnosis of ACS had been confirmed through complete genetic evaluation and advanced imaging. RESULTS: The mean age when scoliosis was first noticed was 3.9±4.2 years (range, 0.5 to 10.5 y) with a mean Cobb angle of 22.5±6.7 degrees (range, 10 to 27 degrees). The mean age at the first orthopedic visit was 5.8±5.0 years (range, 1.5 to 13 y) with a progressed mean Cobb angle of 39.5±17.3 degrees (range, 15 to 57 degrees). Congenital vertebral anomalies were observed in 1 patient. Three patients were treated surgically; 1 of the 3 patients had a surgical complication with loss of intraoperative neuromonitoring signals. Two patients had not undergone surgery at the last visit with a mean Cobb angle of 75.5 degrees. The mean postoperative follow-up for the surgical group (cases 1 to 3) was 3±3.6 years (range, 0.6 to 7.2 y) and the mean total follow-up for both groups was 6.6±2.5 years (range, 2.6 to 8.6 y). CONCLUSIONS: Scoliosis in ACS can represent a clinically significant problem that is underdiagnosed and overshadowed by the other severe medical complications associated with the syndrome. Our data suggest that scoliosis in patients with ACS is rapidly progressive and bracing is ineffective; early screening, close observation, and low threshold for referral to an orthopedic surgeon are crucial. LEVEL OF EVIDENCE: Level IV-case series.

Pinless halo in the pediatric population: indications and complications.

BACKGROUND: The use of the conventional halo is accompanied by the possibility of serious complications, especially in the pediatric population. Complications could include penetration of pin into the skull, pin loosening, pin tract infection, cranial nerve palsies, and vest-related pressure sores. The noninvasive "pinless" halo was introduced in an attempt to mitigate these problems while retaining the effectiveness of the conventional halo. The purpose of this study is to determine the indications and complications related to pinless halo application. METHODS: We retrospectively reviewed 61 patients, whose treatment included the use of a pinless halo device, presenting to our institution between 2004 and 2012. RESULTS: There were 35 male and 26 female patients with an average age of 6.04 years. Indications of pinless halo application included postoperative immobilization for congenital muscular torticollis in 38 cases, conservative management of atlantoaxial rotatory subluxation in 11 cases, postoperative immobilization following cervical fusion in 10 cases, and immobilization for occipital condyle fracture in 2 cases. The average duration of the pinless halo application was 32.68 days. Thirteen patients had complications, among which major complications were seen in 2 patients, each of whom developed a pressure sore; one on the scalp and the other on the chest. Both the pressure sores responded to local treatment; however, 1 resulted in permanent alopecia. CONCLUSIONS: The use of the noninvasive pinless halo was found to be safe with few complications in our study. The complications were infrequent and patients were compliant to treatment, indicating that this modality is patient-friendly. Effectiveness of this treatment in comparison with invasive halos and other cervical orthoses was not determined and is a limitation of this study. LEVEL OF EVIDENCE: Level IV-Case series.

Chest wall osteochondroma in children: a case series of surgical management.

BACKGROUND: Chest wall osteochondroma is a rare tumor in children. Even though the potential for malignant transformation or serious intrathoracic complications is low, it has led some centers to advocate surgical management of these bony tumors. We present our experience of the surgical management of costal osteochondromata. METHODS: Between January 1, 2006 and November 1, 2012 we saw 854 patients with solitary or multiple exostoses in our clinics. By reviewing our billing lists we found 7 children who had surgical management of chest wall osteochondromata. The indications for surgery were pain (3 patients), excision for confirmation of diagnosis (2 patients), recurrent pneumothorax (1 patient), and malignancy (1 patient). RESULTS: All patients made a good postoperative recovery with a median hospital stay of 1.8 days (range, 0 to 4 d). There was no recurrence of exostosis on follow-up (range, 8 mo to 2.6 y). One patient required surgery for excision of another chest wall osteochondroma at an adjacent location. No patient reported scar-related pain symptoms. No malignant transformation or intrathoracic complications occurred. We found ribs as the first site of presentation of multiple hereditary exostoses in 2 young patients. CONCLUSIONS: Surgical management of thoracic osteochondroma, with excision for painful, symptomatic, malignant lesions or lesions adjudged to be at risk of intrathoracic complications, yields good outcomes in terms of symptom control, establishing histologic diagnosis, and prevention of thoracic complications. LEVEL OF EVIDENCE: Level IV-case series.

Bilateral C6 spondylolysis with spondylolisthesis in 3 adolescent siblings.

BACKGROUND: Cervical spondylolysis with spondylolisthesis is a rare condition in the pediatric population. The nature of this condition and clinical presentation are important to provide appropriate management. METHODS: This is a case report of 3 adolescent siblings who had C6 cervical spondylolysis with spondylolisthesis. RESULTS: Two cases were diagnosed insidiously with absence of neurological deficits and no instability on imaging and were treated conservatively. One case had a traumatic presentation associated with instability on imaging and was managed with cervical fusion and instrumentation. All 3 patients were doing well on a follow-up of >2 years. CONCLUSIONS: These cases suggest that cervical spondylolysis could be familial. The treatment was offered based on clinical presentation and presence of instability on radiographic studies. LEVEL OF EVIDENCE: Level IV.

Adjunctive vancomycin powder in pediatric spine surgery is safe.

STUDY DESIGN: Therapeutic level II cohort study. OBJECTIVE: To evaluate the safety of adjunctive local application of vancomycin powder (VP) for infection prophylaxis in posterior instrumented thoracic and lumbar spine wounds in pediatric patients weighing more than 25 kg. SUMMARY OF BACKGROUND DATA: Spine surgeons have largely turned to vancomycin prophylaxis in an attempt to decrease the incidence of late surgical site infection and acute surgical site infection from methicillin-resistant Staphylococcus aureus. In adult patients, the adjunctive local application of VP with an intravenous cephalosporin has been shown to decrease postsurgical wound infection rates significantly; however, the safety of VP as an adjunct in pediatric spine surgery has not been reported. METHODS: We reviewed data collected under a systematic protocol specifically designed to monitor the safety profile of VP. We measured changes in creatinine and systemic vancomycin levels after intrawound application of 500 mg of unreconstituted VP during spine deformity correction surgery in patients weighing more than 25 kg (patients also received routine intravenous cephalosporin prophylaxis). Laboratory values were measured preoperatively and on postoperative days 1 and 4. Any adverse reactions and infections through available follow-up (2-8 mo) were recorded. RESULTS: Eighty-seven consecutive pediatric patients with spinal deformity weighing more than 25 kg who received intraoperative VP during a 9-month period were identified. Sixty-three percent of the patients in this series had adolescent idiopathic scoliosis, 15% congenital scoliosis, 15% neuromuscular scoliosis, and 5% spondylolisthesis. The average change in creatinine levels between the preoperative and postoperative day 1 draw was -0.03 and between the preoperative and postoperative day 4 draw was -0.075. The postoperative systemic vancomycin levels remained undetectable. None of the patients experienced nephrotoxicity or red man syndrome. Three of the 87 patients developed a surgical site infection. CONCLUSION: In this cohort there were no clinically significant changes in creatinine level or systemic vancomycin level caused by use of intraoperative VP. LEVEL OF EVIDENCE: 2.