Congenital Dermal Sinus Research Articles

The importance of timing: evaluating the optimal age for surgical intervention in asymptomatic dermal sinus tracts.

Dermal sinus tracts (DSTs) are rare congenital lesions resulting from errors of spinal cord disjunction, leading to a persistent connection between the cutaneous ectoderm and underlying neural elements. As patients are at increased risk of infection and irreversible neurological compromise due to tethering, resection and detethering are recommended as a prophylactic measure. The ideal timing of surgical intervention in an asymptomatic cohort, however, remains unclear. A retrospective chart review was performed at a single institution from 1998 to 2022 of all patients surgically treated for lumbar DST by 10 different surgeons. Demographic, presentation, operative, and postoperative details were obtained. Five patients who presented with infectious or neurological symptoms were excluded from primary analysis. The age at the time of surgery was then analyzed as a continuous variable against the operative details and postoperative outcomes utilizing Spearman's correlation coefficient and the Mann-Whitney U-test. Fifty-two patients underwent prophylactic surgical excision of their DST as well as detethering. Overall, 65% of patients were female and the median age at diagnosis was 3 months, while the median age at the time of surgery was 7 months. An additional cutaneous finding was identified in 71% of patients, with hemangioma being most frequent (40%). Additional spinal lesions were radiographically identified in 29% of patients, including lipoma (19%), dermoid and epidermoid inclusion cysts (8%), and an arachnoid cyst (2%). Postoperative complications occurred in 8% (n = 4) of patients, primarily related to wound healing (3/4), with significant risk attributed to younger age at time of surgery (mean 3 months, range 1-5 months) (p = 0.01). During 56 months of follow-up, long-term rates of retethering (6%, p = 0.02) and abnormal neurological examinations (8%, p = 0.001) were associated with concurrent lipomas, but not with age at operation. In a series of 52 pediatric patients undergoing prophylactic resection of a DST with detethering, the complication rate was 8% and was significantly associated with age < 6 months at the time of operation. Associated spinal lipomas significantly influenced long-term outcomes. When evaluating asymptomatic infants with DST, patient age is a significant factor in assessing the risks and benefits of surgical timing.

Computed tomography of the equine caudal spine and pelvis. Pathological findings in 56 clinical cases (2018-2023).

Computed tomography (CT) of the axial skeleton is increasing across many equine hospitals. CT of the pelvis and caudal spine in a large group of clinical cases has not been reported previously. To describe the pathological lesions identified in the caudal spine/pelvis in horses and ponies undergoing CT spine of this region. A retrospective case series. Horses with CT imaging of the caudal spine/pelvis were included. Horses aged under 6 months and those with CT examinations performed post-mortem were excluded. Fifty-six horses met the inclusion criteria. Ages ranged from 8 months to 20 years and bodyweights from 85 to 680 kg (mean 488 kg). Horses presented predominantly for lameness (30/56) and poor performance (18/56). Osseous pathology was identified in 41/56 horses; including osteoarthritis of the sacroiliac joint(s) (n: 28), pathology of the intervertebral disc joint(s) (n: 12), pelvic fractures (n: 9), osteoarthritis of the coxofemoral joint(s) (n: 8), ventral spondylosis (n: 6), acetabular rim fracture (n: 2), dislocation of coxofemoral joint(s) (n: 2), and dorsal dermal sinus of the sacrum (n: 1). The relationship between CT imaging findings and clinical signs in horses remains unclear. Further work is required to describe the relationship between congenital, developmental, and acquired changes in the caudal spine and pelvis, and clinical signs. CT of the caudal spine and pelvis can be utilised in horses and ponies for diagnosing a range of clinical disorders that may be causing 'lumbosacroiliac pain', poor performance, hindlimb lameness, and stiffness. The pathologies we observed were diverse and many patients had multiple lesions.

Transnasal Endoscopic Approach for Excision of Intracranial Nasal Dermoid Sinus Cysts.

Nasal dermoid sinus cysts (NDSCs) with intracranial extension in the form of dermal sinus tracts require careful and complete resection to prevent recurrence. Resection techniques necessitate adequate intracranial exposure, but morbidity associated with historical resection approaches has presented unique multidisciplinary challenges for surgeons treating cysts with intracranial extension. The authors primarily employed a transnasal approach through a midline nasal incision, utilizing endoscopic or microscopic access between the lateral cartilages for resection of NDSCs with intracranial extension. A retrospective review was completed for patients treated for NDSCs at the authors' pediatric quaternary referral center from 2017 to 2023. Data collection included demographics, comorbidities, perioperative data, pre and postoperative imaging, surgical outcomes, and complications. Eighteen patients with NDSCs with possible or confirmed intracranial extension were surgically treated from 2017 to 2023. Fifteen were treated with resection performed through a midline transnasal approach with endoscopic assistance, achieving successful total resection while avoiding nasal osteotomy or frontal craniotomy. One patient had a slow cerebrospinal fluid leak from an operative durotomy, successfully treated with a lumbar drain. No other complications occurred. No patients required transfusion. Incision length and postoperative scar burden were less than approaches that used osteotomies or craniotomies and demonstrated excellent cosmetic results. No patients have had cyst recurrence or required reoperation. A transnasal approach through a midline incision with endoscopic assistance is an effective approach for resection of NDSC with intracranial extension, but utility may vary with cyst size and complexity. This approach leverages appropriate exposure for resection with decreased morbidity and decreased incision length through avoidance of osteotomies.

P.068 Malignant transformation of a spinal dermoid cyst into carcinosarcoma

Background: Spinal dermoid cysts are uncommon, benign tumours of ectodermal origin, often associated with spinal dysraphism. Malignant transformation of spinal dermoid cysts is an exceptionally rare entity, with transformation into carcinosarcoma not previously reported. Methods: Case report and literature review Results: A 41-year-old male presented with a recurrent lumbar intradural mass, 28 years after resection of a dermal sinus tract and associated dermoid cyst. Intraoperative appearance and subsequent pathology were again consistent with a dermoid cyst. The patient re-presented 2 weeks after surgery with diplopia and headache due to hydrocephalus, thought to be due to chemical meningitis. Following ventriculoperitoneal shunt implantation, the patient rapidly deteriorated with progressive neurologic deficits and widespread leptomeningeal enhancement. A repeat spinal leptomeningeal biopsy was pursued, which revealed malignant transformation of the dermoid cyst into invasive carcinosarcoma. Without curative treatment options, the patient was palliated and died 85 days after admission. Conclusions: Malignant transformation of spinal dermoid cysts should be considered in the differential diagnosis of patients with dermoid cysts and progressive leptomeningeal enhancement. False negatives can occur with initial tumour pathology and repeat sampling may be warranted for diagnostic clarity. To the authors knowledge, this is the first report of a spinal dermoid cyst with malignant transformation into carcinosarcoma.

Results of Surgical Treatment of Occult Spinal Dysraphisms-A Single Centre Experience.

Occult spinal dysraphisms (OSDs) are caused by various defects in the embryogenesis of the spinal cord and represent an obstacle to the ascent of the conus, which allows the conus to pass from the lower levels of the spinal canal to the final position between L1 and L2 during normal foetal life. When an OSD tethers the spinal cord at the lower levels, it can lead to neurological symptoms, better known as tethered cord syndrome. Surgical treatment of OSD is primarily aimed at untethering the spinal cord. In asymptomatic patients, this can protect against the long-term development of neurological deficits. In symptomatic patients, this can halt or limit the progression of existing symptoms. The aim of this study is to examine all paediatric and adult patients diagnosed with OSD and treated in the Department of Neurosurgery at the University Medical Centre Ljubljana during the 5-year period of 2016-2021. All patients diagnosed with OSD during this period were included in the study. Patient characteristics, treatment modalities and outcomes were studied with the aim of describing the differences between the paediatric and adult population and defining the rationality of treating these pathological conditions. We included in the study 52 patients with 64 occult dysraphic lesions. Adults (>18 years old) represented 15/52 (28.8%) of all patients, while 37/52 (71.8%) were children. The most common OSDs were conus lipomas, followed by dermal sinus tracts, filum terminale lipomas and split cord malformations. Surgical treatment was performed in 35/52 (67.3%) cases, while conservative management was chosen in 17/52 (32.6%) cases. The preoperative presence of symptoms was statistically higher in adults than in children (p = 0.0098). Surgery on complex spinal cord lipomas was statistically related to a higher rate of postoperative neurological complications (p = 0.0002). The treatment of OSD is complex and must be based on knowledge of the developmental anomalies of the spine and spinal cord. Successful surgical treatment relies on microsurgical techniques and the use of neuromonitoring. Successful treatment can prevent or limit the occurrence of neurological problems.

Unusual Presentation of Congenital Dermal Sinus on Lateral Buttock

Unusual Presentation of Congenital Dermal Sinus on Lateral Buttock

Holocord Abscess with Bulbo-Medullary Extension Associated with a Congenital Dermal Sinus in an 18-Month-Old Child

Holocord Abscess with Bulbo-Medullary Extension Associated with a Congenital Dermal Sinus in an 18-Month-Old Child

Dorsal Dermal Sinus with Intramedullary Epidermoid Cyst in a Toddler: A Tiny Clue to Underlying Major Neurological Condition

Background: Skin dimples at specific sites of the body at birth are a clue to underlying congenital malformation and intimate the need for extensive evaluation. A midline dimple in the spinal region may be a sign of occult spinal dysraphism in a neonate. Methods: A toddler presented with clinical symptoms and signs of acute paraparesis. The child also had thoracic dermal sinus on clinical examination. The child was evaluated with hematological and radiological investigations to diagnose the cause of acute paraparesis. Results: Hematological investigations were normal. Magnetic resonance imaging of the spine showed an intramedullary epidermoid cyst at the D1 vertebral level to D4 vertebral level with dorsal dermal sinus at the D3 vertebral level. Conclusion: We are reporting this case to insist on clinical examination of all neonates for skin dimples and detailed evaluation of neonates with suspicious skin dimples to prevent future neurological disability. A clinically significant skin dimple implies impending neurological problems in a patient.

Developmental Abnormalities of the Pediatric Spine: A Review of the Correlation Between Ultrasound and MRI Findings.

A broad spectrum of spinal pathologies can affect the pediatric population. Ultrasound (US) is the primary modality for pediatric spine assessment due to its widespread availability, non-requirement of sedation, and absence of ionizing radiation. Supplementing this, MRI offers an in-depth exploration of these conditions, aiding in preoperative strategizing.In this review, we examine the clinical indications, methodologies, and protocols for US and MRI scans of the pediatric spine. Additionally, we illustrate normal pediatric spinal anatomy, highlighting several examples of normal variants that are often misinterpreted. Through a series of case-based illustrations, we offer a comprehensive overview of various pathological conditions such as tethered cord, spinal dysraphism, spinal lipoma, diastematomyelia, and dermal sinus tract, among others. Furthermore, we explore the correlation between US and MRI findings for these lesions, employing real-world cases to enhance our understanding of this topic.

Posterior cervical congenital dermal sinus tract: case report and review of literature

Background and importanceCongenital dermal sinus tract (DST) is a rare spinal dysraphism characterized by a persistent tract lined by epithelial cells, beginning at the epidermis and terminating in deeper tissue layers. With 1% of all congenital DST cases found in the cervical region, only 4% of all cases are diagnosed after the age of 20.Clinical presentationIn this case, a 65-year-old woman with a congenital DST at the cervical level presented with symptoms of neck and some arm pain, suboccipital headaches, and unique external characteristics. Neck Disability Index and visual analog scale were used to assess the patient’s preoperative and postoperative pain, and quality of life. Patient underwent an operative intervention, where the DST was surgically removed followed by interlaminar decompression at C1–C2, excision of the epidural component, and biopsy followed by plastic surgical repair. Pathology analysis indicated a squamous epithelial-lined sinus tract interacting with the dura. Most notably, a meningothelial proliferation with associated psammomatous calcifications was identified, similar to a meningioma.ConclusionA review of literature was conducted to further discuss clinical and radiological presentation as well as to document the novel appearance of this congenital DST. As one of the oldest cases of DST, it demonstrated unusual pathological characteristics with a meningothelial proliferation, compatible with meningioma, reported at the epidural level.

The Case Files

A ruptured dermoid can have many presentations, making it hard to diagnoseFigureFigureA 45-year-old man presented after a sudden transient loss of consciousness followed by excessive crying. He was conscious and oriented upon arrival. He had no fever, and his blood pressure was 130/80 mm Hg, heart rate was 90 bpm, respiratory rate was 20 bpm, oxygen saturation level was 100 percent, and blood sugar level was 103 mg/dL. No neurological deficit was found on examination. The patient had a sinus tract on his lower back at L2-L3. The rest of the exam was normal. The patient had no significant medical history and was not taking any medication. Neuroimaging, CT, and a brain MRI revealed disseminated lipid tissue in the frontal horn of the lateral, third, and fourth ventricles. Similar extra-axial fat intensities were also found in the frontal region and left sylvian, interpeduncular, and right CP angle cisterns. Two differential diagnoses were considered: ruptured dermoid tumor and traumatic pelvic or spinal fracture. Spinal MRI suggested an intramedullary mass lesion with lobulated margin (1.6 cm by 1.1 cm by 6.8 cm) in the conus medullaris and cauda equina, extending from the L1 to L3 vertebrae. The lesion appeared hyperintense on T2W and T1W images, which were suppressed on saturated T1 images. Evidence of intramedullary T1 and T2 hyperintense multiple foci adherent to the dorso-lumbar spinal cord suggested a ruptured dermoid. A dorsal dermal sinus tract at L2 to L3 vertebrae was noted. Rupture of a spinal dermoid cyst into the central spinal canal is rare. An intradural dermoid cyst may rupture with subsequent subarachnoid dissemination of lipid droplets. Dermoid cysts are benign tumor-like lesions comprising approximately one percent of intracranial and spinal tumors and occur slightly more often in men. Their high incidence occurs in the lumbosacral region involving the conus and cauda equina. Upper thoracic (10%) and cervical (5%) lesions are rare. Dermoids are associated with a dermal sinus in 20 percent of cases. The rupture of a dermoid cyst is a well-known phenomenon and can be spontaneous, iatrogenic, or traumatic in origin. A ruptured dermoid may have myriad presentations. Chemical and aseptic meningitis are the most common features after a rupture because of the irritating effects of the disseminated cholesterol debris. The patient may be asymptomatic or experience headache, vomiting, seizures, coma, infarct, and rarely death. A headache might occur because of the compression of adjacent structures, chemical meningitis, or hydrocephalus due to obstruction of cerebrospinal fluid pathways by lipid droplet. The mean age at presentation of such lesions is 40.6, and all cases have been reported in men. Treatment includes steroids, analgesics, antibiotics, and surgical resection of the spinal lesion.

A Case of Giant Dermoid Cyst at Sacrococcygeal in Adult at “X” Hospital in Bandung

Dermoid cysts are benign tumors that can be found anywhere in the body. There is no dermoid cysts clear data yet in Indonesia. In the spine, these tumors generally occur in the lumbar region and are often accompanied by congenital spinal dysrafism and/or dermal sinus passages. Dermoid cysts arise from abnormal growth of the ectodermal folds during pregnancy. Its incidence is 0.7 – 1.8% of all central nervous system tumors. The aim of reported this case is to highlight the role of a diagnostic and evaluate the surgical outcome. A 47 year old female patient with a lump in the sacrococcygeal area measuring 20 x 18 x 16 cm, with hard and soft consistency. MRI images show a hypo and hyperintense mass without spinal deformity. After the tumor was removed, the patient was allowed to go home for the 2nd day without complications. In conclusion, extraspinal dermoid cyst is a rare case that requires special diagnostic and surgical modalities to provide better clinical results.

Co-occurrence of subcutaneous myxopapillary ependymoma, dermal sinus tract, and filum terminale lipoma: a review of the pathobiology of caudal spinal cord development and spinal cord tethering. Illustrative case.

Myxopapillary ependymoma (MPE) is typically benign and found in the conus medullaris and/or filum terminale, although rare cases of subcutaneous and extra-axial MPE have been reported. The co-occurrence of MPE, tethered cord syndrome (TCS) with lipoma of the filum terminale, and a dermal sinus tract is extremely rare, with only 6 reported cases in the literature. Here, the authors present the first case, to their knowledge, of an extra-axial, subcutaneous MPE co-presenting with TCS, lipoma of the filum terminale, and a dermal sinus tract and discuss the underlying pathobiology. A 14-month-old male who presented for evaluation of a dermal sinus tract underwent magnetic resonance imaging, which revealed a tethered cord with associated lipoma. At 14 months, the patient underwent spinal cord detethering with resection of his sacral dimple and sinus tract. Histopathological evaluation revealed an incidentally found MPE within the dermal sinus tract. The authors review the underlying biology of MPEs, tethered cord syndrome, and dermal sinus tracts, and explore possible points of convergence within the developmental pathways that may result in this unique concomitant presentation. Additionally, they suggest that extra-axial MPE may be underappreciated and underdiagnosed; this case suggests that extra-axial MPE may be only effectively diagnosed with histological studies.

Meningite secundária a seio dérmico dorsal infectado em região torácica - um relato de caso

Infant presenting with clinical and laboratory features of meningitis with progressive neurological deficit leading to the diagnosis of intramedullary abscess due to dorsal dermal sinus in the thoracic region.

Asymptomatic Isolated Congenital Cervical Dermal Sinus

Asymptomatic Isolated Congenital Cervical Dermal Sinus

Cystic Posterior Neck Mass in a Premature Newborn

Congenital dermal sinus is a type of occult spinal dysraphism. It is a result of incomplete separation of the cutaneous ectoderm from neural ectoderm. MRI can aid in identifying the subtype of spinal dysraphism however, surgery is always recommended to dissect the intradural component.

Limited dorsal myeloschisis without extradural stalk continuity to coexisting congenital dermal sinus.

Limited dorsal myeloschisis (LDM) is characterized by a fibroneural tethering stalk linking the skin lesion to the underlying spinal cord. LDM without an extradural stalk is rare. A full-term boy was noted at birth to have a dimple in the upper back (dorsal skin of the lower thoracic region). Computed tomographic scan showed spina bifida at the T9-12 vertebral level and osteochondral tissue at the T10 level. Magnetic resonance imaging (MRI) demonstrated a tiny dorsal lipoma at the T8 vertebral level, but the intradural tethering tract was not apparent. At 18days of age, the congenital dermal sinus (CDS) tract started from the dimple and terminated at the osteochondral tissue, without continuity of the dura mater, and the osteochondral tissues were resected. At age 2years 8months, he developed spastic paresis of the right foot. On MRI, the tethering tract from the dorsal lipoma became apparent. During the second surgery at age 2years 11months, the intradural stalk started from the dorsal lipoma and joined the inner surface of the dura mater was untethering from the cord. Postoperatively, right spastic paresis was improved. Histological examination of the intradural stalk revealed the distribution of S100-immunopositive peripheral nerve fibers, which is one of the histopathological hallmarks of LDM. We speculated that the extradural stalk with coexisting CDS originally linked from the skin lesion subsequently regressed and was replaced by fibroadipose tissue with osteochondral tissue migration. Intradural exploration should always be seriously considered in these disorders of persisting neurocutaneous connection.

Early Surgical Intervention with Antibiotic Treatment for Congenital Dermal Sinus with Central Nervous System Infection: A Retrospective Study of 20 Cases

To investigate the efficacy of surgical intervention with antibiotic treatment for congenital dermal sinus (CDS) with central nervous system infection. Clinical data of 20 patients with CDS and central nervous system infection were retrospectively analyzed. All patients received early surgical treatment combined with postoperative antibiotic therapy after the diagnosis was confirmed. The infection control effect was evaluated according to the clinical symptoms, laboratory examination results, and improvement of neurological function. All 20 patients were treated with complete resection of the CDS and postoperative antibiotic therapy. No severe surgical complications occurred. Patients were followed for 3-81 months with a median follow-up time of 37.5 months. Postoperative infection was effectively controlled with no recurrence. Neurological dysfunction and related symptoms improved to varying extents after treatment, and the Spina Bifida Neurological Scale score at the final follow-up significantly increased. Patients with CDS and central nervous system infection should receive surgical management at an early stage with antibiotic treatment. This treatment algorithm was effective for control infection and protection of neurological function.

Congenital Dorsal Dermal Sinus, A Rare Entity: diagnosis and Management

Congenital dorsal dermal sinus is a rare occult spinal dysraphism. It may be associated with other cutaneous manifestations and spinal pathologies. Patients either presents with infection or neurological problems. We present a case presented with dorsal dermal sinus with discharging sinus with split cord with block vertebra with hypertrichosis and hairy melanocytic naevus. Bang. J Neurosurgery 2021; 11(1): 45-49

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.