Conservative management of typical pediatric postauricular dermoid cysts.

OBJECTIVE: Congenital dermoid cysts of the skull and face frequently arise in embryonic fusion planes. They may follow these planes to extend intratemporally or intracranially. Advanced imaging and operative techniques are generally recommended for these lesions. Postauricular temporal bone dermoid cysts seem to form a distinct subgroup with a lesser tendency toward deep extension. They may be amenable to more conservative management strategies. METHODS: With IRB-approval, we queried a prospectively-accrued computerized patient-care database to find all postauricular temporal dermoid lesions surgically managed by a single pediatric otolaryngologist from 2001 to 2014. We reviewed the English-language literature to identify similar series of surgically treated pediatric temporal bone dermoid cysts. RESULTS: Ten postauricular temporal dermoid cysts with pathological confirmation were identified in our surgical series. The average size of the lesions was 1.5 cm (0.3-3 cm). The average age at time of surgery was 4 years (6 months-17 years). No intracranial extension was observed at surgery. There were no recurrences noted on last follow-up (mean 65 months, range 10-150 months). A computerized literature review found no examples of intracranial extension among typical postauricular dermoid cysts. CONCLUSION: There was no intracranial or temporal extension in our series or among postauricular lesions described in the literature. Given the low incidence of deep extension we advocate neither advanced imaging nor routine neurosurgical consultation for typical postauricular lesions. Dissection in continuity with cranial periosteum facilitates intact removal of adherent lesions. Surgery is curative if the dermoid is removed intact.

The spectrum of musculoskeletal problems in lipomyelomeningocele.

PURPOSE: Patients with lipomyelomeningocele (LMMC) represent a unique population within the spectrum of spinal dysraphism. The natural history of LMMC remains poorly defined. The description and prevalence of the presenting orthopaedic clinical signs and symptoms for LMMC have been infrequent and often documented only in general terms. The goal of this study is to define the patterns and prevalence of presenting clinical musculoskeletal signs and symptoms in LMMC patients. METHODS: This study was a retrospective review of charts of all patients identified as having LMMC in our spina bifida clinic. Patient charts with incomplete data or diagnoses other than LMMC were excluded from the analysis. Data collected included age at initial tethered cord release (TCR); repeat TCR; limb length discrepancy; foot deformities; asymmetry of motor and sensory deficits; presence of scoliosis; orthotic needs; assistive devices; functional status. RESULTS: We identified 32 patients with LMMC (21 female and 11 male patients). The majority of patients had their primary TCR by ≤1 year of age (59 %), with 22 and 19 % having primary TCR at ages 1-15 and >15 years, respectively. Fifteen patients had at least one repeat TCR, with ten of these having more than one repeat TCR. A significant relationship was noted between low back/radicular pain and repeat TCR (p < 0.001). Ten patients (31%) had a limb length discrepancy of >2.5 cm, and 53 % of patients had asymmetric involvement. Nine patients (28 %) had scoliosis of whom only one required operative treatment. Fifteen patients had foot deformities. Thirteen patients (41 %) had two or more orthopaedic procedures in addition to other neurologic or urologic procedures. CONCLUSION: The presenting musculoskeletal clinical signs and symptoms in patients with LMMC are uniquely different in terms of both pattern and frequency compared to myelomeningocele and other forms of spinal dysraphism. We noted a high prevalence of asymmetrical involvement, a high operative burden, and a high rate of repeat symptomatic tethered cord syndrome requiring TCR. As previously noted by others, TCR in LMMC does not prevent long-term functional deterioration. These findings may be important to our colleagues providing counsel to their patients with LMMC and to their families.

Congenital malformations of the skull and meninges.

The surgery and management of children who have congenital malformations of the skull and meninges require multidisciplinary care and long-term follow-up by multiple specialists in birth defects. The high definition of three-dimensional CT and MRI allows precise surgery planning of reconstruction and management of associated malformations. The reconstruction of meningoencephaloceles and craniosynostosis are challenging procedures that transform the child's appearance. The embryology, clinical presentation, and surgical management of these malformations are reviewed.

Potential complications of posterior spine fusion and instrumentation in patients with cerebral palsy treated with intrathecal baclofen infusion.

STUDY DESIGN: The authors evaluated a case series of 5 patients with cerebral palsy and a previously placed subcutaneous reservoir for continuous intrathecal baclofen infusion to control spasticity who underwent spinal arthrodesis with posterior instrumentation for progressive neuromuscular scoliosis deformities. OBJECTIVES: To present our preliminary experience and complications with posterior spine fusion in patients with cerebral palsy and continuous intrathecal baclofen infusion and provide a literature review. SUMMARY OF BACKGROUND DATA: Numerous studies have reported complications associated with intrathecal baclofen infusion, many of these catheter-related. Few reports address complications associated with the intrathecal baclofen infusion, including progressive spinal deformities and complications following spinal arthrodesis. METHODS: Five patients with spastic quadriplegia cerebral palsy had spine fusions for progressive neuromuscular scoliosis. The mean age at surgery was 14.5 years. The mean preoperative major curve was 73 degrees and mean pelvic obliquity was 19 degrees . The patients' medical records were reviewed for complications following reinsertion of the intrathecal catheter following posterior spine instrumentation and arthrodesis, and for progression of the neuromuscular scoliosis, before and after intrathecal baclofen infusion was initiated. RESULTS: Two patients presented with low-pressure headaches in the postoperative period, resulting from cerebrospinal fluid leak following subarachnoid catheter reinsertion and posterior instrumentation. Four patients documented progression of the major scoliosis curve after intrathecal baclofen infusion was begun. A mean progression of 44 degrees occurred over a mean period of 11 months before the spinal arthrodesis. CONCLUSIONS: Low pressure headaches resulting from a cerebrospinal fluid leak following catheter reinsertion may occur in the postoperative period. Preoperative concerns with the baclofen pump reservoir placed subcutaneously and pressure sores were not seen with careful prone positioning on a 4-poster frame. Progression of scoliosis in patients with cerebral palsy requiring spinal arthrodesis was demonstrated in 4 of the patients after continuous intrathecal baclofen was started. The progression of the spinal deformity as a consequence of growth, natural history, or the intrathecal baclofen infusion is unknown at the present time.

Reflections on shunt infection.

The placement and revision of ventriculoperitoneal (VP) shunts remains a mainstay in the surgical treatment of hydrocephalus. While the North American infection rate averages nearly 8-10%, published infection rates for VP shunt infection below 1% have been reported. We retrospectively reviewed shunt operations by a single surgeon over 62 months to analyze the infection rate. In 62 months, we performed 526 shunt placements or revisions in patients up to 18 years of age. There were 7 shunt infections (1.33%). In 5 cases, the organism was Staphylococcus epidermidis, and a single shunt each was infected with Haemophilus influenzae and Staphylococcus aureus. Each infection was treated with external ventriculostomy drainage and intravenous antibiotics. The new shunt was placed at a new incision site after at least 5 days of sterile spinal fluid cultures. The mean follow-up among these patients after shunt insertion was 25 months. VP shunting remains the most common operation for hydrocephalus. Infections are linked with seizures, higher future risks of shunt infection and malfunction, and reduced IQ and school performance. Our infection rate during 62 months was limited to 1.33%. Uniform surgical technique, limited hardware and skin edge manipulation and double gloving may be important factors in limiting shunt infections.