In utero Diagnosis of Spinal Dermal Sinus.

INTRODUCTION: Congenital dermal sinus (CDS) is an open neural tube defect (NTD) that occurs in 1 in 2,500 births a year and often goes undetected until patients present with complications like infection and neurological deficits. Early diagnosis and repair of CDS may prevent formation of these complications. In utero diagnosis of these lesions may improve long-term outcomes by enabling referral to specialty services and planned postnatal repair; however, only 2 such cases have been reported in the literature. We present a third case of in utero diagnosis of CDS with a description and discussion of findings from surgical exploration and pathology. CASE PRESENTATION: Routine prenatal ultrasound scan detected a tethered cystic structure arising from the back of the fetus at 20 weeks of gestation. Dedicated fetal ultrasound confirmed the presence of a cystic lesion protruding through a lamina defect, while fetal magnetic resonance imaging showed an intact spinal cord and meninges, suggesting a diagnosis of CDS. Neurosurgery followed along closely and took the child for surgical exploration on day 2 of life. A fibrous stalk with an intradural component and associated cord tethering was excised. Histology showed fibrous tissue without an epithelial-lined lumen. CONCLUSION: CDS is a form of NTD that occurs from nondisjunction of the cutaneous ectoderm and neuroectoderm during formation of the neural tube. Slight differences in how this error occurs can explain variations seen in this spectrum of disease, including CDS without an epithelial-lined lumen as seen in this case. Newborns with CDS can go undiagnosed for years and present with long-term complications. Fetal imaging can assist in early recognition and surgical excision of CDS in newborns.

Morphological changes of the dorsal contour of the corpus callosum during the first two years of life.

BACKGROUND: In the medicolegal literature, focal concavities or notching of the corpus callosum has been thought to be associated with fetal alcohol spectrum disorders. Recent work suggests corpus callosum notching is a dynamic and normal anatomical feature, although it has not yet been defined in early life or infancy. OBJECTIVE: Our purpose was to characterize the dorsal contour of the corpus callosum during the first 2 years of life by defining the prevalence, onset and trajectory of notching on midsagittal T1-weighted images. MATERIALS AND METHODS: We reviewed retrospectively 1,157 consecutive patients between birth and 2 years of age. Corpus callosum morphology was evaluated and described. A notch was defined as a dorsal concavity of at least 1 mm in depth along the dorsal surface of the corpus callosum. Patient age as well as notch depth, location, number and presence of the pericallosal artery in the notch were noted. RESULTS: Two hundred thirty-three notches were identified in 549 patients: 36 anterior, 194 posterior and 3 patients with undulations. A statistically significant (R2=0.53, Beta=0.021, P=0.002) positive correlation between posterior notch prevalence and age in months was noted. A positive correlation between age and depth of the posterior notch was also statistically significant (r=0.32, n=179, P≤0.001). A trend for increased anterior notch prevalence with age was identified with significant correlation between visualized pericallosal artery indentation and anterior notching (r=0.20, n=138, P=0.016). Sub-analysis of the first month of life showed corpus callosum notching was not present. CONCLUSION: The presence of posterior notching increased significantly with age and was more frequent than that of anterior notching. Corpus callosum notching was absent in the first week of life, building on prior studies suggesting corpus callosum notching is acquired. This study provides baseline data on normative corpus callosum notching trajectories by age group during early life, a helpful correlate when associating corpus callosum morphology with disease.

Combined Laminoplasty and Posterior Fusion for Cervical Spondylotic Myelopathy Treatment: A Literature Review.

STUDY DESIGN: A literature review. PURPOSE: To explore the utility of laminoplasty in combination with instrumented fusion, with a focus on neurological outcomes and changes in kyphotic deformity. OVERVIEW OF LITERATURE: Management of cervical spondylotic myelopathy (CSM) to reduce morbidity within the neurosurgical population. METHODS: A US National Library of Medicine PubMed search was conducted for manuscripts pertaining to cervical laminoplasty and fusion for the management of CSM. Several relevant studies were shortlisted for review, and the bibliographies of the articles were searched for additional references. The search was limited to human studies, English-language literature, and reports on more than one patient. RESULTS: Combined laminoplasty and fusion was found to provide at least comparable, if not superior, neurological outcomes in specific patient populations with CSM. The Japanese Orthopedic Association scores, local kyphosis, and C2-C7 angle have been reviewed in several manuscripts, and improvement in each of these categories was found with laminoplasty and fusion. CONCLUSIONS: The treatment of CSM necessitates an individualized approach based on the pathoanatomical variation. Laminoplasty and fusion can be appropriately used for patients with CSM in a setting of local kyphotic deformity, ossification of the posterior longitudinal ligament, associated segmental instability, and the need for strong stabilization.

Catecholamine-secreting paraganglioma of the thoracic spinal column: report of an unusual case and review of the literature.

BACKGROUND AND IMPORTANCE: Paragangliomas are rare tumors of neuroendocrine origin that arise from paraganglionic tissue of the extrachromaffin cell system. These lesions may be seen at various sites along the neuraxis. Primary thoracic paragangliomas have rarely been reported in the literature, with secretory thoracic lesions being exceedingly rare as only 3 previous cases have been cited. CLINICAL PRESENTATION: A 49-year-old woman presented with episodes of hypertension, palpitations, and diaphoresis. Workup revealed positive urine catecholamines and a thoracic spine mass extending into the thoracic apex. Preoperative α-blockade with phenoxybenzamine was used followed by posterior decompression and tumor resection. Arthrodesis from C5 to T4 was subsequently performed, and the patient received postoperative radiation. CONCLUSION: Two years postoperatively, the patient has continued to have regression of her symptoms. We report a rare case of a catecholamine-secreting primary thoracic paraganglioma in a 49-year-old woman. These tumors should be treated carefully by the neurosurgeon with preoperative assistance from endocrinology for α-blockade, followed by gross total resection and postoperative radiation if residual tumor remains.