Theodore S. Roberts

The differential diagnosis of posterior plagiocephaly : true lambdoid synostosis versus positional molding

&NA; The diagnosis and treatment of posterior plagiocephaly is one of the most controversial aspects of craniofacial surgery. The features of true lamibdoid synostosis versus those of deformational plagiocephaly secondary to positional molding are inadequately described in the literature and poorly understood. This has resulted in many infants in several craniofacial centers across the United States undergoing major intracranial procedures for nonsynostotic plagiocephaly. The purpose of this study was to describe the detailed clinical, imaging, and operative features of true lamhdoid svnostosis and contrast them with the features of positional plagiocephaly. During a 4‐year period from 1991 to 1994, 102 patients with posterior plagiocephaly were assessed in a large multidisciplinary craniofacial program. During the same period, 130 patients with craniosynostosis received surgical treatment. All patients were examined by a pediatric dysmorphologist, craniofacial surgeon, and pediatric neurosurgeon. Diagnostic imaging was performed where indicated. Patients diagnosed with lambdoid synostosis and severe and progressive positional molding underwent surgical correction using standard craniofacial techniques. Only 4 patients manifested the clinical, imaging, and operative features of unilambdoid synostosis, giving an incidence among all cases of craniosynostosis of 3.1 percent. Only 3 among the 98 patients with positional molding required surgical intervention. All the patients with unilambdoid synostosis had a thick ridge over the fused suture, identical to that found in other forms of craniosynostosis, with compensatory contralateral parietal and frontal bossing and an ipsilateral occipitomastoid bulge. The skull base had an ipsilateral inferior tilt, with a corresponding inferior and posterior displacement of the ipsilateral ear. These characteristics were completely opposite to the findings in the 98 patients who had positional molding with open lambdoid sutures and prove conclusively that true unilambdoid synostosis exists as a specific but rare entity. Awareness of the features of unilambdoid synostosis will allow more accurate diagnosis and appropriate treatment of posterior plagiocephaly in general and in particular will avoid unnecessary surgical intervention in patients with positional molding. (Plast. Reconstr. Surg. 98: 765, 1996.)

THE ROLE OF SELECTIVE DORSAL RHIZOTOMY IN cérébral PALSY: CRITICAL EVALUATION OF A PROSPECTIVE CLINICAL SERIES

This is a prospective observational study of a consecutive series of 34 children with spastic cérébral palsy treated at a single center. 10 had spastic quadriplegia and 24 had spastic diplegia. AH were followed for at least one year. After selective dorsal rhizotomy (SDR), all children received one month of physical therapy at the center and were prescribed a program of physical therapy in their community. The children were assessed before and one year after SDR and physical therapy, using the Ashworth Scale, deep tendon reflex response, range of motion and the Gross Motor Function Measure. The results show that there is often a decrease in lower‐extremity spasticity and functional improvement after SDR with physical therapy, but that there is considerable variability in outcome. Randomized prospective clinical trials with masked objective outcome measures are needed to determine the efficacy of SDR.

Spontaneous resolution of a cervicothoracic syrinx in a child. Case report and review of the literature.

A child with near complete spontaneous resolution of a cervicothoracic syrinx and improvement in a Chiari type I malformation without surgical intervention is presented. The child was followed clinically with serial magnetic resonance (MR) imaging and has remained neurologically stable over an 11-year period. To our knowledge, only 3 pediatric cases of spontaneous resolution of a spinal cord syrinx as documented by MR imaging without surgical intervention have been reported. This case contributes to the literature on the natural history of syringes.

Management of Intrinsic Gliomas of the Tectal Plate in Children

The natural history, management, and long-term outcome for patients with benign, intrinsic tectal plate gliomas remain controversial in spite of their propensity to cause late-onset hydrocephalus. A 10-year retrospective review has identified 11 consecutive children with tectal plate lesions. Headache, vomiting, a decline in school performance, tremor, and complex partial seizures were common presenting symptoms. All patients presented with signs and symptoms of hydrocephalus. Magnetic resonance (MR) imaging delineated an intra-axial mass lesion of the midbrain primarily localized to the tectal plate which uniformly was hyperintense on T2-weighted imaging and had a more variable appearance on T1-weighted imaging and rare enhancement with gadolinium. No patient underwent surgical resection, chemotherapy, or radiotherapy. Three of 11 patients (27%) showed evidence of progression in size or a new focus of enhancement on MR imaging, which was clinically asymptomatic. In this series, no patient with a tectal plate lesion less than 1.5 cm in maximal diameter and without gadolinium enhancement showed any evidence of clinical or radiological progression. Although intrinsic tectal lesions in children are clinically indolent and the initial management consists of CSF diversion, these lesions may eventually progress and still warrant long-term follow-up with serial MR imaging.

Electrophysiological monitoring during selective dorsal rhizotomy, and spasticity and GMFM performance

The relation between abnormal electrophysiological responses to intraoperative stimulation during selective dorsal rhizotomy (SDR) and the degree of spasticity and motor dysfunction was explored in 92 children with spastic cerebral palsy (CP) who underwent SDR at a single center. The proportion of abnormally responding rootlets was compared with the degree of spasticity measured with the modified Ash worth Scale (MAS) and with the spasticity measurement system (SMS) at discrete segmental levels. Motor impairment measured with the Gross Motor Function Measure (GMFM) was also compared with the proportion of abnormally responding dorsal rootlets. A consistent relation between the proportion of abnormally responding rootlets and the degree of spasticity and gross motor abnormality at the corresponding muscles could not be demonstrated. There was also no consistent association between the proportion of rootlets ablated during SDR and the change in spasticity measured with the MAS and SMS, or to the change in motor function as measured with the GMFM. These data suggest that the intraoperative monitoring technique most commonly used for SDR is unlikely to identify accurately those neural elements which contribute to spasticity in children with CP.

Efficacy of selective dorsal rhizotomy (SDR) in spastic diplegia: Changes in spasticity and mobility after 24 months |[dagger]| 1742

Objective: To study the efficacy of SDR in spastic diplegia. Design: Single center investigator-masked randomized clinical trial. Subjects: 43 children were randomized to receive SDR plus physical therapy (PT), or PT alone. Interventions: SDR was guided with electrophysiologic monitoring. All subjects received equivalent PT. Spasticity was measured by an electromechanical torque device (EMT) and the Ashworth scale. The Gross Motor Function Measure (GMFM) recorded changes in functional mobility. Investigator-masked outcome measures were collected at baseline, 12 and 24 months. Results: See Table. Conclusions: SDR is safe and reduces spasticity in children with spastic diplegia. SDR plus PT and equivalent PT without SDR result in equal improvements in mobility at 24 months.