Congenital Dermal Sinus Research Articles

Dermal sinus secondary to spinal abscess

A 19-month-old female patient was brought into our clinic because of fever (over 39°C) for 17 days, as well as vomiting and weakness which were not resolved by antibiotherapy. On physical examination, respiration sounds were rough and a dimple was detected on the skin at the sacral level. Acute phase reactants were found higher on blood analysis and the patient had no history of chronic disease. Upon development of quadriplegia and glob vesicle on the second day of hospitalization, contrast thoracolumbar magnetic resonance imaging and contrast abdominal computed tomography for etiology of fever were performed. The contrast abdominal computed tomography showed hypodense abscess formations extending from the lower lumbar level to the sacrum with a peripheral contrast uptake, and a dermal sinus tract extending to the skin at the sacral level with dural contrast. Furthermore, the urinary bladder was hydropic (Fig. 1). The contrast thoracolumbar magnetic resonance imaging, on enhanced sagittal T1-weighted imaging, showed multiple abscesses presenting peripheral contrast uptake extending from the lower thoracic level to the sacrum, as well as diffuse contrast uptake on dural surfaces and neural roots, whereas on sagittal T2-weighted imaging significant expansion in the spinal canal was noted (Fig. 2).Based on these findings, the patient was diagnosed with spinal intramedullary abscess due to dermal sinus and dural infection. Fig. 2(Left) There is increased signal intensity and expansile spinal cord on sagittal T2-weighted image. (Middle and Right) There is peripheral enhanced abscess cavity on enhanced sagittal T1-weighted images. View Large Image Figure Viewer Download Hi-res image

Prenatal diagnosis of tethered spinal cord associated with sacrococcygeal teratoma

Tethered spinal cord is mostly caused by myelomeningocele and lipomyelomeningocele, while dermal sinus tract, diastematomyelia, lipoma, tumor, thickened/tight filum terminale, spinal trauma, and spinal surgery are among the other causes. Prenatal diagnosis of tethered cord has been reported, and it is usually associated with neural tube defects. We present an atypical presentation of a tethered spinal cord, which was associated with a sacrococcygeal teratoma and was diagnosed in the 23rd week of pregnancy by ultrasonography. © 2016 Wiley Periodicals, Inc. J Clin Ultrasound 44:506-509, 2016.

Ciddi Nörolojik Patolojilerin Nadir Görülen Bir Kutanöz Bulgusu: Torakal Dermal Sinüs

A 39 year old adult female patient was reported with a rare combination of thoracal dermal sinus tract, diastematomyelia, idiopathic syringomyelia and multipl vertebral anomalies in this patient. Spinal dermal sinus tracts were located most frequently in the lumbar and lumbosacral regions. Thoracal localization of congenital dermal sinus tracts is considerably infrequent. Because of this unusual localization, the lesion was confused with the chronic epidermal cyst which we mostly come across in clinical practice and further radiological examination was not thought. Our case has the property of being a rare case, showing the association of thoracal dermal sinus tract with diastematomyelia, idiopathic syringomyelia and multipl vertebral anomalies without any neurolojic deficit clinically. This case showed us that severe neurological pathologies could be found under a lesion considered as a simple cyst.

Case of congenital diastematomyelia type 1: a rare presentation

Diastematomyelia is a rare congenital anomaly that results in the splitting of the spinal cord in a longitudinal (sagittal direction). It is also known as a split cord malformation, refers to a type of spinal dysraphism (spina bifida occulta) when there is a longitudinal split in the spinal cord. We report a case of new born female baby with Diastematomyelia type 1 associated with low lying conus, tethered cord and dorsal dermal sinus.

Epidermoid Cyst Of Posterior Cranial Fossa Associated With Congenital Dermal Sinus Tract – A Case Report And Review Of The Literature Drinking

Summary Epidermoid cysts are benign lesions composed of ectodermal structures. They account for less than 2% of primary intracerebral tumors. They can be present at birth (congenital cysts), or can be acquired cysts (after surgery or trauma). Some cysts are asymptomatic but depending on their location, they may cause symptoms such as headache, vertigo, seizures, focal deficits, increased intracranial pressure. The major differential diagnosis of epidermoid cysts are dermoid cyst, any cystic neoplasm, craniopharyngioma, arachnoid cyst. A 39-year-old female patient with complaints of headache, vertigo, nausea and vomiting was admitted to the hospital. Radiological data suggested intracranial tumor in fossa posterior. Craniotomy was performed and the biopsy specimen demonstrated the mass lesion to be an epidermoid cyst of the intracranial dermal sinus tract. Intracranial cystic-appearing masses can be problematic for both radiologists and physicians. Important characteristics demonstrated by computed tomography scans and classical magnetic resonance imaging sequences are significant for the accurate diagnosis, but the imaging appearances of intracranial cystic lesions are sometimes nonspecific, and the diagnosis is usually established by histological examination.

Congenital cervical dermal sinus tract caused tethered cord syndrome in an adult: a case report.

The objective of this study was to report on a 34-year-old woman who presented with tethered cord syndrome due to dermal sinüs tract. A 34-year-old woman had got dermal sınüs tract admitted to our hospital with swelling on the neck, pain and numbness on the left upper limb. She was treated by surgical removal of dermal sinuses and untethering the spinal cord which is stretched by the dermal sinus. Congenital dermal sinus tracts are uncommon types of cranial and spinal dysraphisms. They can occur in the midline of the craniospinal axis from the occiput to the sacral region. For dermal sinuses, cervical region is very rare location that is reported in the literature. They are diagnosed usually in childhood with skin signs, neurological deficits, local infections and meningitis. We present a rare case of dermal sinus tract located in cervical region. Early diagnosis and treatment of cervical dermal sinus tract are important to prevent neurological deficits.

Tethered cord syndrome in children: a single-center experience with 162 patients.

Tethered cord syndrome (TCS) is not an uncommon clinical problem in children. The aim of this retrospective study is to document our experience on the surgical treatment of TCS in childhood. The data of 162 children who underwent surgical treatment for TCS in a 15-year period were reviewed retrospectively. Their demographic, clinical, radiological, and surgical features were documented. They were divided into two groups as primary and secondary TCS, and the surgical technique for each group was demonstrated. Untethering the spinal cord and correction of the associated malformation were the standard surgical technique for each patient. The results of the treatment were summarized. Among the 162 children, 101 (62.3%) of them were female and 61 were male with a mean age of 62 months. Primary TCS was detected in 43 patients while secondary TCS was found in 119 (73.4%) patients. Hypertrichosis was the most common physical finding while back pain was the common complaint. Lipoma, split cord malformation, dermal sinus tract, and myelomeningocele were the associated malformations for secondary TCS. Children should be individualized for the treatment of TCS. Each patient must be evaluated neurologically and radiologically for the accurate diagnosis. Surgical untethering is the safe and effective method of treatment for children with TCS.

Unusual Presentation of Congenital Dermal Sinus

Congenital dermal sinus tract is a rare dysraphism with an incidence of 1/2500-3000 ratio. The sinus ostium located in the midline of the posterior of the body from the occiput to the lumbosacral region. Paramedian congenital dermal sinus tract with paramedian ostium are rare. Here, we describe a 2-year-old boy who presented with paramedian pigmented lesion with an ostium on the left lumbosacral region. During surgery we noted that sinus tract attached to thick lipomatous filum terminale.

Congenital spinal dermal tract: how accurate is clinical and radiological evaluation?

OBJECT A dermal sinus tract is a common form of occult spinal dysraphism. The presumed etiology relates to a focal failure of disjunction resulting in a persistent adhesion between the neural and cutaneous ectoderm. Clinical and radiological features can appear innocuous, leading to delayed diagnosis and failure to appreciate the implications or extent of the abnormality. If it is left untreated, complications can include meningitis, spinal abscess, and inclusion cyst formation. The authors present their experience in 74 pediatric cases of spinal dermal tract in an attempt to identify which clinical and radiological factors are associated with an infective presentation and to assess the reliability of MRI in evaluating this entity. METHODS Consecutive cases of spinal dermal tract treated with resection between 1998 and 2010 were identified from the departmental surgical database. Demographics, clinical history, and radiological and operative findings were collected from the patient records. The presence or absence of active infection (abscess, meningitis) at the time of neurosurgical presentation and any history of local sinus discharge or infection was assessed. Magnetic resonance images were reviewed to evaluate the extent of the sinus tract and determine the presence of an inclusion cyst. Radiological and operative findings were compared. RESULTS The surgical course was uncomplicated in 90% of 74 cases eligible for analysis. Magnetic resonance imaging underreported the presence of both an intradural tract (MRI 46%, operative finding 86%) and an intraspinal inclusion cyst (MRI 15%, operative finding 24%). A history of sinus discharge (OR 12.8, p = 0.0003) and the intraoperative identification of intraspinal inclusion cysts (OR 5.6, p = 0.023) were associated with an infective presentation. There was no significant association between the presence of an intradural tract discovered at surgery and an infective presentation. CONCLUSIONS Surgery for the treatment of spinal dermal tract carries a low morbidity. While it seems intuitive that tracts without intradural extension carry a low risk of spinal cord tethering, it is not possible to reliably detect these cases using MRI. Similarly, intraspinal dermoid cannot be reliably excluded using MRI and carries an increased risk of infection. These points justify excision together with intradural exploration of all spinal dermal sinus tracts.

A Case of Dermal Sinus Tract with Recurrent Meningitis in a 3-Month-Old Infant

Spinal dermal sinus tract (DST) is a rare congenital dysraphism that occurs in approximately one in every 2,500 live births. Many studies have reported that cutan­ eous stigmata overlying the posterior midline neuroaxis may be associated with dermal sinuses, including abnormal pigmentation, hemangioma, hypertri chosis, and benign sacrococcygeal dimples. Spinal DSTs that are connected with sinuses may have diverse clinical presentations and may involve clinical complica tions, occasionally caused by a neurologic defect, or infectious complications, in cluding life­threatening conditions such as meningitis. A 3­month­old girl had experienced recurrent meningitis without typical abnormal findings on physical and laboratory examinations. Magnetic resonance imaging (MRI) was performed to evaluate infec­ tious conditions, and DST was found incidentally. Although MRI findings revealed DST, we could not find any small opening on the skin on careful observation. Thus, first ­line physicians should keep several important factors in mind when initially evaluating patients with recurrent meningitis, such as cutaneous findings over the midline neuroaxis and early neuro imaging studies for congenital dysraphism.

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.

Spinal dorsal dermal sinus tract: An experience of 21 cases.

Background:Spinal dorsal dermal sinus is a rare entity, which usually comes to clinical attention by cutaneous abnormalities, neurologic deficit, and/or infection. 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.Methods:Medical records of 21 patients treated for spinal dorsal dermal sinus from September 2007 to December 2013 were reviewed.Results:We had 21 patients with male: female ratio of 13:8. Only 2 patients were below 1-year of age, and most cases (15) were between 2 and 15 years (mean age = 8.2 years). Lumbar region (11 cases) was most frequently involved, followed by thoracic (4 cases), lumbosacral, and cervical region in 3 patients each. All of our patients presented with neurological deficits. Three patients were admitted with acute meningitis with acute onset paraplegia and had intraspinal abscess. The motor, sensory, and autonomic deficits were seen in 14, 6, and 8 patients, respectively. Scoliosis and congenital talipes equinovarus were the common associated anomalies. All patients underwent surgical exploration and repair of dysraphic state and excision of the sinus. Overall, 20 patients improved or neurological status stabilized and only 1 patient deteriorated. Postoperative wound infection was seen in 2 cases.Conclusions:All patients with spinal dorsal dermal sinuses should be offered aggressive surgical treatment in the form of total excision of sinus tract and correction of spinal malformation, as soon as diagnosed.

Infratentorial Subdural Empyema Associated with Long Standing Occipital Dermal Sinus: Case Presentation

Infratentorial subdural empyema is a rare form of life threatening intracranial infection, requiring immediate neurosurgical intervention. We present this 4-years-old girl with posterior fossa subdural empyema which is associated with congenital occipital dermal sinus. A contrast-enhanced CT scan showed an infratentorial supracerebellar hypodense fluid collection with the peripheral rim enhancement to the left of the midline that raised suspicion of a subdural empyema with supratentorial mild ventricular dilatation which was confirmed by MRI with contrast. The patient was operated through sub-occipital decompression and drainage of the collection and the samples was sent for culture and sensitivity. Dermal sinus can be a cause for intracranial infection and should be investigated to rule out intradural connection. Infratentorial subdural empyema should be managed urgently by neurosurgical intervention to prevent further life threatening complications.

Congenital dermal sinus tract in the cervical region of a 16-year-old girl

Congenital dermal sinus tract in the cervical region of a 16-year-old girl

Dermal Sinus Tract: Clinical Presentation and Imaging Findings

Dermal Sinus Tract: Clinical Presentation and Imaging Findings

Congenital Dermal Sinus Tract of the Spine

Congenital dermal sinus tract is a rare entity which lined by epithelial cells and can end anywhere between subcutaneous planes to thecal sac. These tracts may be accompanied with other pathologies such as lipomyelomeningocele, myelomeningocele, split cord malformation, tethered cord, filum abnormality and inclusion tumors and treatment includes resection of tract with intradural exploration. The authors review their experience with 16 cases. Clinical, radiological appearance and treatment of these lesions discussed with literature review.

Infected lumbar dermoid cyst mimicking intramedullary spinal cord tumor: Observations and outcomes

We report two unusual cases of a 17-month-old boy with a previously undiagnosed lumbar dermal sinus tract terminating in an intradural dermoid cyst and holocord edema or syrinx, presenting with paraparesis and sphincter dysfunction secondary to an intramedullary abscess and a 26-month-old boy with a previously undiagnosed lumbar dermal sinus tract terminating in an infected dermoid cyst and intramedullary abscess, presenting with recurrent episodes of meningitis and hydrocephalus. Pre-operative magnetic resonance imaging (MRI) studies in these patients were initially confused for an intramedullary spinal cord tumor; however, the presence of an associated dermal sinus tract made this diagnosis of neoplasm less likely. Total excision of the dermal sinus tract, debulking of the dermoid cyst and drainage of the intramedullary abscess through an L1-L5 osteoplastic laminoplasty and midline myelotomy, followed by long-term antibiotic therapy resulted in a good functional recovery. Post-operative MRI of the spine showed removal of the dermoid cyst, decreased inflammatory granulation tissue and resolution of the holocord edema or syrinx. We also performed a literature review to determine the cumulative experience of management of intramedullary abscess in this rare clinical setting.

A Girl with Life Threatening Meningitis Caused by an Infected Dermoid Cyst Derived from an Occult Dermal Sinus

Congenital dermal sinuses are a rare entity of spinal dysraphism. Most patients have cutaneous markers of their presence, including nevus or port wine stains, dimple, hypertrichosis and subcutaneous lipoma. Inclusion tumors, such as dermoid tumor, are associated to dermal sinus tract; they are congenital, benign and slow-growing lesions of midline. Their clinical relevance is due to the possibility of causing infection, spinal abscess, signs of corde or nerve root compression. Spinal Magnetic Resonance Imaging (MRI) is the diagnostic tool of choice. We present the case of a child with atypical meningitis, resistant to large spectrum antibiotic therapy, caused by an infected dermoid cyst, undiagnosed in the first months of life despite repeated MRI.

Conflicting Diagnosis of Dermal Sinus Tract and Tethered Cord

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.

Limited dorsal myeloschisis: a not-so-rare form of primary neurulation defect

Limited dorsal myeloschisis (LDM) is a distinctive form of spinal dysraphism characterized by two constant features: a focal "closed" midline skin defect and a fibroneural stalk that links the skin lesion to the underlying cord. The embryogenesis is hypothesized to be incomplete disjunction between cutaneous and neural ectoderms, thus preventing complete midline skin closure and allowing persistence of a physical link (fibroneural stalk) between the disjunction site and the dorsal neural tube. We utilize the experience gained from the management of 63 patients with LDM to illustrate these features. All patients were studied with MRI or CT myelogram, operated on, and followed for a mean of 9.4years. There were 11 cervical, 16 thoracic, 8 thoracolumbar, and 28 lumbar lesions. Two main types of skin lesion were: saccular (26 patients; consisting of a skin base cerebrospinal fluid sac topped with squamous epithelial dome or a thin membranous sac) and nonsaccular (37 patients; with a flat or sunken squamous epithelial crater or pit). The internal structure of a saccular LDM could be a basal neural nodule, a stalk that inserts on the dome, or a segmental myelocystocoele. In nonsaccular LDMs, the fibroneural stalk has variable thickness and complexity. All fibroneural stalks contain glioneuronal tissues accompanied by variable quantities of nerves and mesodermal derivatives. In all LDMs, the fibroneural stalk was tethering the cord. Twenty-nine patients had neurological deficits. There was a positive correlation between neurological grade and age suggesting progression with chronicity. Treatment consisted of detaching the stalk from the cord. Most patients improved or remained stable. LDMs were associated with three other dysraphic malformations in more than coincidental frequencies: six LDMs were contiguous with dorsal lipomas, four LDMs shared the same tract or traveled in parallel with a dermal sinus tract, and seven LDMs were related to a split cord malformation. The embryogenetic implications of these associations are discussed. LDM is a distinctive clinicopathological entity and a tethering lesion with characteristic external and internal features. We propose a new classification incorporating both saccular and flat lesions.