Dermoid cyst with dermal sinus tract complicated with spinal subdural abscess

Spinal subdural abscess caused by spread of infection with the dermal sinus tract is rare in children. This article reports on a 1-year-old male with prolonged fever, progressive paraplegia, and bowel and bladder dysfunction resulting from a spinal subdural abscess secondary to an infected spinal dermoid cyst with a dermal sinus tract. This is the youngest patient to be reported having this condition. Surgical intervention was performed to find a tumor that had capsule and keratinlike contents. Culture of the abscess was positive for Escherichia coli and Bacteroides vulgatus. He received 6 weeks of parenteral antibiotic treatment. This patient illustrates the importance of urgent radiologic examination, immediate surgical resection, and appropriate antibiotic therapy for spinal subdural abscess.     

Occipital dermal sinus associated to a cerebellar abscess. Case

Congenital dermal sinuses are tubular tracts which communicate the skin with deeper structures. It is a manifestation of defective separation of the ectoderm and neuroderm. The incidence is 1/2500-3000 births alive. Almost 10 % of congenital dermal sinuses are localized in the occipitocervical region. They are usually asymptomatic, unless an infectious process is concurrent (meningitis, abscess). We are presenting the case of a 12 months girl with unnoticed cutaneous stigmata in the occipital region, who was admitted with a meningeal syndrome and secondary neurological impairment. She had a cerebellar abscess and was treated with decompression by puncture of the abscess and antibiotics. When infection was resolved, congenital dermal sinus was excised. Process solves without morbidity. We reviewed the clinical and therapeutic features in cases reported previously in the literature.     

Unusual cause of cerebellar abscess: occipital dermal sinus and dermoid cyst

Cerebellar abscess induced by a contiguous dermal sinus is a rare event. In a large series of acute posterior fossa abscedation, otogenic suppuration is the cause in 93% of the cases, while hematogenous infection is infrequent, probably because of the relatively weak bloodflow in this part of the central nervous system. The authors present an occipital dermal sinus and dermoid cyst revealed by a cerebellar abscess. The literature is reviewed briefly, treatment and prophylactic measures are discussed.     

Intramedullary abscess--a rare complication of spinal dysraphism.

Two cases are reported of patients with spinal dysraphism who developed abscesses within the spinal cord. In one case the infection had spread to the cord through a dermal sinus, as in the six cases previously recorded in the literature. In the other patient the sepsis developed within an intramedullary epidermoid tumour, but the route of infection was not clear. Each patient made a virtually full neurological recovery after open drainage of the abscess.     

Intramedullary abscess of spinal cord as a complication of existing dermal sinus

The paper describes a rare case of intramedullary spinal cord abscess in an infant after dermal sinus. In discussion with literature review the contemporary approach to patients with this pathology is highlighted.     

Proteus mirabilis abscess involving the entire neural axis

Intramedullary spinal cord abscesses are rare and potentially devastating lesions usually associated with other infective processes such as bacterial endocarditis, or pulmonary or urogenital infection. We describe a 2-year-old girl who presented with an infected dermal sinus leading to an intraspinal abscess. This abscess eventually spread and involved the entire neural axis leaving her quadriparetic. Drainage of the abscess resulted in recovery and the child regained normal function of her limbs. To our knowledge this is the first documented case of an intramedullary abscess involving the entire neural axis.     

Timing of surgery in patients with infected spinal dermal sinuses: report of two cases

In the past, if infection was present at the time a spinal dermal sinus was discovered, the sinus opening and cerebrospinal fluid were sterilized with a course of appropriate antibiotic therapy prior to the surgical intervention. The cases of two children with an infected spinal dermal sinus are reported here. One patient developed acute paraplegia during the initial stage of antibiotic therapy, and the second patient, who was admitted only with signs of fever and irritability, experienced after antibiotic therapy a complete myelographic blockade secondary to a large infected intradural dermoid tumor. The authors stress the importance of early neuroradiological and neurosurgical management of these lesions, even when an infection is present.     

Spinal congenital dermal sinus: an experience of 23 cases over 7 years

Spinal congenital dermal sinus is a rare entity, which supposedly results from the failure of neuroectoderm to separate from the cutaneous ectoderm during the process of neurulation. The present study was undertaken to know the clinical profile of these patients, to study associated anomalies and to assess the results of surgical intervention. We had 23 patients with male : female ratio of 9:16. Only 2 patients were below 2 years of age and most cases (16) were between 2-16 years (mean age =10.2 years). Lumbar region (17 cases) was most frequently involved, followed by lumbosacral and thoracic region in 3 patients each. Only three patients were asymptomatic at the time of presentation. Most of the cases presented with evidence of neural compression or tethered cord syndrome. Only one case presented with spinal abscess. The motor, sensory and autonomic deficits were seen in 20, 11 and 12 patients respectively. Scoliosis and CTEV (congenital talipus equino varus) were the common associated anomalies. MRI revealed associated dysraphic state of spinal cord in 21(>90%) cases. All patients underwent surgical exploration and repair of dysraphic state and excision of the sinus. None of the asymptomatic patients deteriorated. Overall 8 patients improved, 14 got their neurological status stabilized, including 3 asymptomatic cases. Only one patient deteriorated. Postoperative wound infection was seen in 2 cases. As age advances, the chance of developing neurological deficit increases. Associated dysraphic state should be looked for and treated simultaneously, using microsurgical technique, whenever possible. It is better to treat all these cases with aggressive surgical intervention before the neurological deficits appear.     

Recovery after tetraplegia caused by dermal sinus infection: intramedullary abscess and tetraparesis

Congenital dermal sinuses result from abnormal neurulation, and are uncommon. A spinal intramedullary abscess secondary to an infected dermoid cyst is very rare, and the functional prognosis is usually quite poor. We report on a 16-month-old child with tetraplegia secondary to intramedullary abscesses because of a dermoid cyst infection associated with a dermal sinus. The abscesses were drained, and the dermoid cyst was removed. Antibiotics were administered for 6 weeks after neurosurgery. The child was followed at a pediatric rehabilitation department. After 1 year, he was able to walk quickly and had regained appropriate upper limb motor function for his age. However, bladder sphincter dyssynergia persisted, requiring intermittent catheterization. This case highlights the importance of early diagnosis for surgical intervention and prolonged antibiotic therapy. Long-term follow-up by a multidisciplinary team allowed for the effective management of related neurologic, orthopedic, and bladder disorders.     

Catastrophic Intramedullary Abscess Caused by a Missed Congenital Dermal Sinus

Congenital dermal sinus (CDS) is a type of occult spinal dysraphism characterized by a midline skin dimple. A 12-month-old girl presented with fever and ascending quadriparesis. She had a midline skin dimple in the upper sacral area that had been discovered in her neonatal period. Imaging studies revealed a holocord intramedullary abscess and CDS. Overlooking CDS or misdiagnosing it as benign sacrococcygeal dimple may lead to catastrophic infection and cause serious neurological deficits. Therefore, further imaging work-up or consultation with a pediatric neurosurgeon is recommended following discovery of any atypical-looking dimples in the midline.     

The spinal dermal-sinus-like stalk

Objects  In this study, a disjunction anomaly mimicking the spinal congenital dermal sinus (DS) is described. This anomaly is referred to as the dermal-sinus-like stalk. Dissimilarities between a true dermal sinus and a dermal-sinus-like stalk are discussed. Clinical material  Three cases in which a spinal congenital dermal sinus was suspected are presented. A similar anatomical configuration, different from that of a dermal sinus, was found. All cases presented with a skin-covered dimple from which a solid tract was seen continuing intramedullary in two cases and intraspinally in one case. None of the patients presented with signs of infection or an associated dermoid–epidermoid tumor. Clinical, radiological, and surgical findings are discussed. A hypothesis is made on the pathological genesis of this malformation. Conclusion  A dermal-sinus-like stalk is a malformation similar to a spinal congenital dermal sinus but is not associated with DS-related complications. Despite important clinical, radiological, surgical, and histopathological differences, it is difficult to distinguish this malformation from a true DS based on clinical and radiological examination alone. Therefore, surgical intervention, at the time of diagnosis, is recommended in all cases.     

Spinal intramedullary abscess with an epidermoid secondary to a dermal sinus

Congenital dermal sinuses are relatively uncommon, and result from abnormal neurulation. A lack of awareness about this problem causes these patients to manifest significant mortality and morbidity. Spinal intramedullary abscesses are rare and potentially devastating lesions. Intraspinal epidermoids are also unusual lesions. We report on a boy, aged 1 year and 5 months, who had a lower-back hairy hemangioma at birth without further evaluation, and dermal sinus tract without surgical intervention at age 7 months. He also manifested rapidly progressive paraplegia with urine and stool retention, and was found to have a spinal intramedullary abscess with an epidermoid as the result of a dermal sinus. This patient illustrates the importance of the recognition and evaluation of skin markers, and of an awareness and complete neurological assessment of all patients with a congenital dermal sinus, because of the potential for intradural extension and a frequent association with other dysraphic abnormalities. Prophylactic surgery is indicated to prevent dangerous and recurrent infections of the central nervous system.     

En bloc excision of a dermal sinus tract

Dermal sinus tracts are a form of spinal dysraphism that arises from a failure of dysjunction early in embryogenesis. They are diagnosed in pediatric patients and who present with a dimple, infection, or neurologic deficit. The tract is surgically excised en bloc to avoid contamination from the tract, which harbors bacteria. However, dermal sinus tracts typically terminate intradurally, rendering their en bloc excision difficult. To avoid entering the tract, allowing for an en bloc excision, we modified the usual technique employed for accessing the spinal intradural space. An en bloc excision of the dermal sinus tract was successfully performed. The patient recovered from the procedure neurologically intact and her postoperative course was uncomplicated. We conclude that en bloc excision of a dermal sinus tract down to the intradural space is feasible with modifications to standard operative technique.     

Congenital dermal sinus of the cervical spine in an adult.

Congenital dermal sinuses are epithelium-lined tracts that extend from an opening in the skin through deeper tissues. Most dorsal congenital dermal sinuses are located in the lumbosacral area; a cervical location is unusual. This report describes a 24-year-old woman who presented with neck pain of 6 months' duration. A dimple in the skin over her cervical spine and a stunted right foot (0.5 cm shorter than the left) were detected on physical assessment. Neurological examination revealed increased deep tendon reflexes in her lower extremities. Magnetic resonance imaging showed a dermal sinus extending from the dimple site to the spinal cord near the C4-C5 vertebrae. Laminectomies were performed at C3-C4 and the tract was totally excised. The patient's neck pain completely resolved and her deep tendon reflexes were normal at 12 months after surgery. Tethered spinal cord alone does not necessarily demand surgery; however, if a congenital dermal sinus is present then surgery is indicated to prevent spinal infection. Even if there are no neurological symptoms, the tract must be surgically removed and the cord must be fully released to prevent future infectious or neurological complications.     

Sacrococcygeal dermal sinus presented bacterial meningitis: a case report]

A rare case of sacrococcygeal dermal sinus suffering from relapsing meningitis is presented. In addition, deference of clinical figure and pathology between sacrococcygeal region and other regions are discussed. A 10 -month-old boy was suffering from relapsing meningitis. Spinal MRI was performed to find occult spinal dysraphism because he had a pit on his hip. The MRI revealed sacrococcygeal dermal sinus. He received surgical resection of dermal sinus based on this findings. Interestingly, different from other dermal sinuses, the dermal sinus runs to caudal direction, and was fused filum terminale with dermoid cyst. This interesting pathological feature of sacrococcygeal dermal sinus seems to come from developmental difference between sacrococcygeal dermal sinus and the other dermal sinuses. Only sacrococcygeal dermal sinus is related with caudal cell mass on its pathogenesis. Because filum terminale is also developed from caudal cell mass, sacrococcygeal dermal sinus seems to be related with filum terminale. Most important differential diagnosis is coccygeal pit that is found in 1 -4% of newborn baby. The clinical feature and pathology got from our case must be very helpful to distinguish sacrococcygeal dermal sinus from coccygeal pit.     

Posterior fossa abscesses secondary to dermal sinus associated with dermoid cyst in children

We report on the rare disease of posterior fossa abscesses secondary to dermal sinus associated with dermoid cyst in 4 pediatric cases seen over a 15-year of period. All children had a small dimple or a pinhole on the skin of the occipital region. On admission they had the signs of increased intracranial pressure, while 3 of them still suffered from signs of meningitis resulting from a bacterial infection through the dermal sinus. CT scan and MR images revealed both the route of the dermal sinus and intracranial lesions. Total resection was successfully achieved before various treatments in all patients without nervous sequelae. In conclusion, a posterior fossa dermoid cyst should be considered in all children with an occipital dermal sinus. Cerebellar abscesses will arise once bacterial infection has occurred through the associated dermal sinus. Radiological studies such as CT scans and MR images can help confirm the diagnosis and locate the lesions. Early neurosurgical interventions including external ventricular drainage, external abscess drainage and primary removal should be planned as soon as possible in accord with the children's condition.     

Report of eight cases of occipital dermal sinus: an update, and MRI findings

OBJECTIVE: We report our experience with 8 patients who were ultimately diagnosed as having occipital dermal sinuses. Clinical presentation, imaging findings and outcome are discussed, along with a review of the literature. STUDY DESIGN: In eight patients with occipital dermal sinuses, we retrospectively collected the following data: time to diagnosis, presenting signs, dermal anomalies, location of bone defects, intraparenchymal extension, surgical techniques and clinical outcome. Conventional radiography was performed in all patients; computerized tomography (CT) scans and magnetic resonance imaging (MRI) in all but two. RESULTS: Time to diagnosis ranged from 3 months to 9 years. Presenting symptoms were: infection of a cutaneous mass (n = 4), occipital mass (n = 2), intracranial hypertension related to intra-cerebral abscess (n = 2). In all patients, dermal sinus was located near the external protuberance of the occipital bone. In all patients but one, a cutaneous orifice was associated. Other cutaneous anomalies were: hairy nevus (n = 2) and subcutaneous cysts (n = 4). One patient had associated cranio-facial malformations. Plain skull films revealed linear bony defects in all cases but one; CT scans revealed linear bony defects in all cases, osteitis and intra-parenchymal abscesses if present but they did not allow the delineation of the sinus tract. MRI performed in six patients allowed a better appreciation of the malformation revealing intracranial abscess (n = 1), the sinus tract (n = 2), inclusion cysts (n = 2), and associated venous anomalies (n = 1). Surgery was performed in all patients: a complete removal of the dermal sinus was obtained in all patients but one; inclusion cysts were found in six patients. Total recovery without sequelae was obtained in all patients, except one who suffered from developmental delay and a polymalformative syndrome. CONCLUSIONS: In newborns, a meticulous visual exploration of the midline skin along the craniospinal axis is necessary to detect congenital dermal abnormalities. Early surgery is required to prevent severe intracranial infection. MRI could help to determine the surgical approach by delineation of the sinus tract, its extension into deeper tissues and its association with cysts, abscesses or venous anomalies.     

Spinal ossifying lipoma.

A case of ossifying spinal lipoma is reported. The patient presented with a large swelling over the lumbar region with a dermal sinus in the centre. Imaging revealed an intradural lipoma connected with a large subcutaneous lipoma in which there was a prominent island of bone. The lipoma was excised and there were no postoperative neurological deficits. To the best of our knowledge, this is the first report of a spinal ossifying lipoma associated with a dermal sinus.     

Cervical intradural extramedullary ependymal cyst associated with congenital dermal sinus: a case report

Spinal intradural extramedullary ependymal cyst is a very rare entity with only few cases reported in the literature. Its association with congenital dermal sinus has not been described so far. We present a unique report of a 3-year-old male child who presented with spastic quadriparesis with a trophic ulcer in the right great toe of 1-year duration. He harbored a congenital dermal sinus in the cervical spine since birth. Intraoperatively, the sinus was associated with an intradural cyst which proved to be an ependymal cyst on histopathological examination. The clinical profile along with review of literature of this rare entity is presented.