The Association of the Epidermoid Cyst of the Filum Terminale, Intradural Spinal Lipoma, Tethered Cord, Dermal Sinus Tract, and Type I Diastematomyelia in a Child
Congenital midface anomalies are rare. Multiple congenital midface anomalies occur in children. Imaging techniques like computed tomography (CT) and magnetic resonance imaging (MRI) help in characterising the lesions, making definite diagnosis and knowing about intracranial extension. We present a case report of 8 year old female child with Frontonasal Epidermoid Cyst with patent dermal sinus tract opening on the dorsum of nose and intracranial extension through cribriform plate defect.
We report the use of diffusion-weighted magnetic resonance imaging to diagnose and manage a rare case of a symptomatic thoracic intramedullary congenital epidermoid cyst with associated dermal sinus in a girl. Congenital intramedullary epidermoid cysts with associated dermal sinuses are very rare occurrences and seldom present symptomatically in very young children. We present a case of a 32-month old with a draining dimpled skin lesion. Magnetic resonance images demonstrated an intramedullary epidermoid with a dorsal dermal sinus tract opening to the skin surface which was confirmed surgically. The patient was treated with debulking to prevent recurrent infection and progression of neurological symptoms. This case demonstrates the use of diffuse-weighted MRI to assist in the diagnosis and surgical management of an atypical presentation of a rare developmental abnormality, which is not well documented in the pediatric radiological literature. Failure to diagnosis may have significant neurological permanent debilitating consequences.
Dermal sinus tracts (DSTs) are an uncommon form of occult spinal dysraphism that is attributed to incomplete neural tube closure during fetal development. Dermal sinus tracts are found along the midline neuroaxis from the nasion to the coccyx, but they most commonly appear in the lumbar region. Dermal sinus tracts are more commonly associated with other developmental abnormalities such as skin tags, naevi, spinal dermoid cysts, meningocoele, lipomas and spinal cord tethering, and can be complicated by cerebrospinal fluid drainage, shedding of keratin from the epithelialized tract, and infection such as meningitis.
Introduction: MRI plays an important role in characterising spinal dysraphisms.The reason behind this is quality of MRI showing very high contrast resolution of soft tissuesand the ability to detect fat in the lesion.T2 weighted images provide excellent contrastbetween the sub-arachnoid spaces and neural tissue while evaluating spinal dysraphisms.Objectives: To determine the frequency of rare disorders of spinal dysraphism in view of MRIfindings of spinal dyspharism patients. Study Design: retrospective. Duration: the study wasconducted from 1st September 2015 to 30th November 2015. Settings: Radiology department,Allied hospital Faisalabad. Methods: A total of 20 consecutive pediatric age group patientspresented with clinical suspicion of spinal dysraphism were included. MRI L/S spine was carriedout on 1.5Tesla Philips and diagnosis of various disorders of spinal dysraphism was made.Conclusion: MRI is the first choice examination for the detection of occult spinal dysraphism.Its timely diagnosis improving surgical & medical outcome as well as better & long term careof patients with spinal dysraphism. Majority of patients in the current study had tethered cord(75%), myelomeningocele (20%), myelocele (10%), lipomyelocele (10%) Dermal sinus tract(10%) & diastematomyelia (10%) followed by associated spinal lipomas (5%), caudal agenesis(5%) & anterior meningocele (5%).
Object
The author studied the specific issue of ventral tethering in cases of type II split cord malformation (SCM). He also discussed the issue of ventral tethering in patients with type I SCM in whom a bony septum and dural sleeve have been resected.
Methods
Eleven (21%) of 52 patients with type II SCM were found to have a ventral fibrous septum tethering the hemicords to the ventral dura. Four categories of ventral septa were noted: 1) pure ventral intradural septa (three patients); 2) complete dorsoventral intradural septa (three patients); 3) dorsoventral septa continuous with a dermal sinus tract (two patients); and 4) ventral or complete septa continuous with ventral intestinal bands causing intestinal malrotation or diverticulum (three patients).
Excluding one autopsy case, all 10 patients had symptoms or progressive neurological syndromes at diagnosis. Magnetic resonance imaging was completely ineffective, and computerized tomography (CT) myelography was only partially effective in detecting ventral tethering in 50% of cases. The only other positive predictor of ventral tethering is the association of dermal sinus tract and intestinal malformations. Combining this predictor with CT myelography, only 60% of the cases of ventral tethering could be suspected before surgery.
Conclusions
The author recommends examining all patients with type II SCM, and at surgery the ventral surface of the split length and reunion site should be thoroughly inspected to reveal a ventral band.
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.
The association of tethered cord, syringomyelia, diasto - metamyelia, spinal epidermoid, spinal lipoma and dermal sinus tract in a child