Noncontiguous Double Spinal Lipoma with Tethered Cord and Polydactyly: Two Different Embryological Events in One Patient.

Lumbosacral lipoma is reported to occur in 4-8 of 100,000 patients. Sixty-six percent of lipomyelomeningocele in young patients have accompanied by hypertrophic filum terminale. It is rare to find two isolated spinal lipomas simultaneously. Embryological origin of dorsal and filar lipomas is different from each other and hence rarer to find them together. Radical resection is now being preferred for better long-term progression-free survival. We report an interesting case of spinal dysraphism in a 4-month-old female child with protruding, nontender, soft, subcutaneous 5 cm × 7 cm mass of the lumbosacral area that had been present since birth. Other anomalies included polydactyly of left hand. Magnetic resonance imaging demonstrated two isolated spinal lipomas, a transitional type and a terminal type filum lipoma with an interval of normal filum between the two. The findings were confirmed at surgery and detethering done along the white plane with neural placode reconstruction as described by Dachling Pang. The child had an uneventful postoperative recovery.

Pediatric encephaloceles: A series of 20 cases over a period of 3 years.

BACKGROUND: Encephalocele is the protrusion of the cranial contents beyond the normal confines of the skull through a defect in the calvarium and is far less common than spinal dysraphism. The exact worldwide frequency is not known. AIMS AND OBJECTIVES: To determine the epidemiological features, patterns of encephalocele, and its postsurgical results. MATERIALS AND METHODS: The study was carried from year July 2012 to June 2015. Patients with encephalocele were evaluated for epidemiological characteristics, clinical features, imaging characteristics, and surgical results. RESULTS: 20 encephaloceles patients were treated during the study period. Out of these 12 (60%) were male and 8 (40%) female. Age range was 1 day to 6 years. The most common type of encephalocele was occipital 12 (60%), occipito-cervical 4 (20%), parietal 2 (10%), fronto-nasal 1 (5%), and fronto-naso-ethmoidal 1 (5%). One patient had a double encephalocele (one atretic and other was occipital) with dermal sinus tract and limited dermal myeloschisis. Other associations: Chiari 3 malformation (2), meningomyeloceles (4), and syrinx (4). Three patients presented with rupture two of whom succumbed to meningitis and shock. Seventeen patients treated surgically did well with no immediate surgical mortality (except a case of Chiari 3 malformation who succumbed 6 months postsurgery to unrelated causes). Shunt was performed in 4 cases. CONCLUSION: The most common type of encephalocele is occipital in our set up. Early surgical management of encephalocele is not only for cosmetic reasons but also to prevent tethering, rupture, and future neurological deficits.

Extended costotransversectomy to achieve circumferential fusion for pathologies causing thoracic instability.

BACKGROUND CONTEXT: Conventional circumferential stabilization for pathologies causing instability of the thoracic spine requires a two or even a three-staged procedure. The authors present their tertiary care center experience of single-staged procedure to establish a circumferential fusion through an extended costotransversectomy approach. OBJECTIVE: To demonstrate neural canal decompression, removal of the pathology, achieve circumferential fusion, and correcting the deformity through a single procedure. STUDY DESIGN: Prospective and observational. PATIENT SAMPLE: Forty-six patients with pan thoracic column instability due to various pathologies. OUTCOME MEASURES: Neurologic condition was evaluated using American Spinal Injury Association and Eastern Cooperative Oncology Group grading systems. Outcome was evaluated with regard to the decompression of neural canal, correction of deformity, and neurologic improvement. All patients were evaluated for neural canal compromise and degree of kyphosis preoperatively, early, and late postoperatively. METHODS: All patients had severe spinal canal compromise (mean, 59%±9%) and loss of vertebral body height (mean, 55%±10%). A single-stage circumferential fusion was performed (four-level pedicle screw fixation along with a ventral cage fixation after a vertebrectomy or corpectomy) through an extended costotransversectomy approach. RESULTS: The pathologies included trauma (21), tuberculosis (18), hemangioma (2), aneurysmal bone cyst (1), recurrent hemangioendothelioma (1), solitary metastasis (1) and plasmacytoma (1), and neurofibromatosis (1). Thirty-five of 46 patients (76%) demonstrated improvement in the performance status. The major complications included pneumonitis (3), pneumothorax (3) and neurologic deterioration (3; improved in two), deep venous thrombosis (2), and recurrent hemoptysis (1). No implant failures were noted on last radiology follow-up. There were two mortalities; one because of myocardial infarction and another because of respiratory complications. CONCLUSIONS: The following study demonstrated that extended costotrasversectomy approach is a good option for achieving single-staged circumferential fusion for correcting unstable thoracic spine due to both traumatic and nontraumatic pathologies.

A rare case of split pons with double encephalocoele, dermal sinus tract, and lipomeningomyelocele: a case report and review of literature.

INTRODUCTION: Multiple neural tube defects and pontine anomalies are relatively rare. Cases of split pons and double encephalocoeles in combination with other spinal anomalies are even rarer. CASE REPORT: Here, we present a very rare case of split pons, twin encephalocoeles (one of which was atretic), lumbar dermal sinus tract, lumbosacral lipomeningomyelocele, thickened filum, and syringomyelia that was managed successfully at our institute. CONCLUSION: Cases of split pons and double encephalocoeles in combination with other spinal anomalies are very rare, and to the best of author's knowledge, this is the first case report of these multiple anomalies in world literature.

A rare case of giant terminal lipomyelocystocele with partial sacral agenesis.

Terminal myelocystoceles (TMCs) are a rare form of occult spinal dysraphism. They constitute approximately 5% of skin covered lumbosacral masses. The TMC is composed of a low-lying conus medullaris with cystic dilatation of caudal central canal, a surrounding meningocele and a lipoma that extends from the conus to a subcutaneous fat collection. A 6-month-old female child presented with a large lumbosacral mass (14 cm × 12 cm × 10 cm) and weakness at the ankle joints since birth. Magnetic resonance imaging and computed tomography scan revealed a low-lying terminal lipomyelocystocele with holocord syrinx and partial sacral agenesis. The child was operated on successfully. This is an interesting and rare case of giant terminal lipomyelocystocoele with syrinx and a partial sacral agenesis. Terminal lipomyelocystocele should be included in the differential diagnosis of congenital lesions presenting as a lumbosacral mass and operated early.