Occipital Dermal Sinuses

Abstract

Occipital dermal sinuses (ODS) are rare dysraphic lesions resulting from defective separation of the ectoderm and neuroderm. These lesions are often diagnosed at birth and should be treated early because of potentially life-threatening complications. However, the pathogenesis of these rare lesions is still poorly understood, and there is no consensus about the optimal timing of surgery. We reviewed 9 pediatric cases of ODS operated in our department since 1982 and 79 other cases published in the literature during the last 50 years. Thirty-eight percent of these had neurological infection (meningitis or abscess), 22% had purely local infection and 40% were uncomplicated at the time of diagnosis. The incidence of infection was low during the first year of life. MRI was highly valuable to study the extent of the sinus and the content of the cyst. The outcome was regularly favorable after surgery. Surgery should however be undertaken with caution on account of the intimate relationship with venous structures, aggravated by postinfectious scarring when the patient is operated late. Considering the risks associated with surgery in neonates and the low incidence of infection during the first year, we advise performing surgery at around 6 months.

Unusual Clinical Presentation and Association of Cranial Dermoid Tumor and Tethered Cord Syndrome

“…Groen and van Ouwerkerk [16] observed a genetic determination on a pair of twins, one with CDT and the other with occipital meningocele, and suggested that CDT and meningomyelocele originate from the same pathology, an adhesion between the ectoderm and neuroderm. The probability of genetic factors is also reported by Vinchon et al [7]. …”

“…The defective cleavage between the ectoderm and the neuroderm during the 3rd to 5th gestational week is responsible for the pathogenesis. However, due to the fact that it is an uncommon disease, the pathophysiology of CDT is not well defined [7]. The tumor generally sits on the midline of the posterior fossa, vermis, and spine, which are on the closing line of the neural tube [6].…”

“…It is a rare dystrophic lesion that grows slowly and is characterized by progressive epithelial desquamation and gland secretion within the cyst [7]. The CDT sits on the midline of the cranium, mostly in the occipital region.…”