The Dermal Sinus: About 29 Cases and Review of the Literature

The term spina includes several types of congenital malformations including closed spinal dysraphisms, among these 10% are dermal sinuses.Our series includes 29 patients, the majority of which were asymptomatic, but these can present with different clinical pictures including neurological and / or infectious signs indicating a neglected dermal sinus and which therefore requires urgent treatment in the environment. Neurosurgical.MRI confirmed the diagnosis showing the dermal sinus tract communicating from the surface of the skin to the intra-dural space.Once the diagnosis has been made, surgical treatment becomes imperative and urgent because, despite the benignity of the lesion, the majority of which are located in the lumbar region, can lead to formidable neurological sequelae compromising the functional prognosis or even the vital prognosis in some cases.All of our patients were operated on and we obtained 90% good results, however we deplore one case of aggravation and one death.

P.234 “Inappropriate” spinal ultrasounds for suspected occult spinal dysraphism in northern Alberta

Background: Occult spinal dysraphism (OSD) may be associated with visible cutaneous manifestations. A common non-pathological mimic of a dermal sinus tract is an incidental sacrococcygeal dimple. Choosing Wisely Canada Guidelines recommend these dimples not be imaged. Methods: This study assessed the appropriateness of spinal ultrasounds performed for the investigation of an OSD. We interrogated our local imaging system to analyze spinal ultrasounds being performed in babies less than 6 months of age, from 2017-2018. Results: 429 children had spinal ultrasounds ordered by pediatricians (55%), family doctors (21%), and neonatologists (20%). 183 children (43%) had imaging indications that was deemed “inappropriate”. Some of this cohort had further MRI imaging (5/183) or neurosurgical referral (8/183). None of these children underwent neurosurgery. 231 (54%) children had appropriate indications for imaging. Within this cohort MRI imaging (23/231), neurosurgical referral (24/231) and neurosurgical intervention occurred (4/231). All four “surgical children” harboured either a dorsal appendage or a subcutaneous lipoma. We estimated, declining inappropriate scans would save $22, 500 annually. Conclusions: A significant portion of local spinal ultrasound requests for OSD are inappropriate. Collaboration with other sites is ongoing to investigate if the Choosing Wisely guidelines for imaging this patient population are being practiced more efficiently.

Intramedullary spinal abscess associated with intramedullary dermoid and infected dermal sinus:case report and review of the literature

Intramedullary spinal abscesses are extremely rare. Most occur secondary to cardiopulmonary spread;contiguous origin is less frequent. Few intramedullary spinal abscesses have been reported secondary to dermal sinus tracts. A dermoid sinus is a congenital lesion (closed simple dysraphism) with rare incidence.Dermoid sinuses can ease local invasion and spinal infections (meningitis, intradural extramedullary and intramedullary abscesses), usually with thoracolumbar involvement. Here we describe acase of a 20-month-old boy who presented with fever and refusal to walk 2 days prior to admission. On examination of the lumbar spine, a small sinus with skin stigmata was noted.Neurological evaluation revealed paraparesis more prominent on the left side, reduced tone and reflexes, left leg hypoesthesia,reduced anal tone, and urinary retention.MRI scan demonstrated intramedullary abscess extending from L2 to S1 level. The patient was urgently admitted for drainage of intramedullary abscess and excision of the dermal sinus tract. Proper antibiotic treatment was completed for 6 weeks with gradual improvement and ultimately full ambulation ability.

Isolated extramedullary intradural thoracic epidermoid cyst: A rare case report

Spinal epidermoid cyst is a rare entity. It may be congenital with associated spinal dysraphism such asspina bifida ,dermal sinus tract and other neural tube defects or acquired due to repeated lumbar puncture and trauma. Congenital spinal epidermoid cysts without spinal dysraphism are rare. Here we are reporting a rare and interesting case of thoracic extramedullary intradural epidermoid cyst with no associated features of spinal dysraphism or history of iatrogenic trauma in a young male patient.

A rare case of Diastematomyelia with syrinx and tethered cord in a newborn

Diastematomyelia or Split cord syndrome is a rare form of spinal dysraphism characterized by longitudinal splitting of spinal cord, conus medullaris or filum terminale to a variable extent. Presence of SCM is suggested by certain superficial markers like skin pigmentation, hemangioma, lipoma, dermal sinus and hypertrichosis. Meningocele or myelomenigocele may also be present. Affected children usually present with progressive sensorimotor symptoms and bowel and bladder dysfunction. Development of sensorimotor symptoms and progressive loss of function emphasis the need for antenatal diagnosis of the spinal deformities which paves way for early intervention and management thus minimizing the morbidity.

Pushing the Limits of Prenatal Ultrasound: A Case of Dorsal Dermal Sinus Associated with an Overt Arnold-Chiari Malformation and a 3q Duplication

We present a case of a fetus with cranial abnormalities typical of open spina bifida but with an intact spine shown on both ultrasound and fetal MRI. Expert ultrasound examination revealed a very small tract between the spine and the skin, and a postmortem examination confirmed the diagnosis of a dorsal dermal sinus. Genetic analysis found a mosaic 3q23q27 duplication in the form of a marker chromosome. This case emphasizes that meticulous prenatal ultrasound examination has the potential to diagnose even closed subtypes of neural tube defects. Furthermore, with cerebral anomalies suggesting a spina bifida, other imaging techniques together with genetic tests and measurement of alpha-fetoprotein in the amniotic fluid should be performed.

Pediatric spinal dermoids - the silent legacy of dysraphism: Diagnosis, management and prognosis – a case series with review of literature

Paediatric spinal dermoids are a subtype of cutaneous inclusion tumours of the spine consisting of ectodermal derivatives. They are relatively rare conditions with associated with the stigmata of spinal dysraphism with possible devastating consequences. We present a case series, in which we study the clinical, radiological and surgical aspects and follow up of the tumour in the last one year. All patients presented with either para or tetraplegia with or without sensory involvement. Contrast MRI was done in all cases, of which 3 were intradural extramedullary lesions and one was intramedullary lesion. The age ranged from 1 year to 4 years. 2 cases were associated with tethering of cord, 2 cases with dermal sinus and another with dandy walker malformation. One case had infected discharging sinus with dermoid. Gross total excision of tumour was done in all cases with Neuro monitoring. All had good post operative outcome without worsening of deficits. All patients received rehabilitation. Follow up study ranged from 3 months to 11 months. No residual tumour noted in follow up MRI.