Dermal Sinus Research Articles

Complicated congenital dermal sinus: Diagnosis and management

ObjectiveCongenital dermal sinus tracts of the spine are a rare form of spinal dysraphism, and should be considered in the differential diagnosis of lumbar subcutaneous abscesses, The CDS tracts can be associated with several pathological findings, including inclusion tumors (for example, epidermoid, dermoid, and teratoma), split-cord malformations, and tethered spinal cords. Excision of the sinus and its track with untethering of the cord should be done as early as possible to avoid intraspinal infection and abscess formation. Our study aims to find out the value of early diagnosis & rapid management of complicated CDS, to re-establish neurological function. Patients and methodsTen cases (2 females and 8 males), with age range (13 months −4 years) presented with acute paraparesis and one case of quadriparesis, MRI spine showed spinal abscess, operative drainage was done with excision of the dermal sinus and track. ResultsNeurological deficits improved in all cases, but 4 cases showed residual deficits, while the other 6 cases got complete recovery. ConclusionEstablishing diagnosis and rapid management of abscess complicating a congenital dermal sinus, is crucial to restore neurological function.

A Rare Case of Infected Cervicodorsal Intramedullary Epidermoid Cyst

Abstract Introduction Intramedullary spinal epidermoid cysts have a reported incidence of less than 1% of intramedullary spinal cord tumors; and out of these, intramedullary epidermoid lesions within the cervical spinal cord are even rarer. Here, we present the case of an adult patient with an infected intramedullary epidermoid cyst, identified on magnetic resonance imaging (MRI) at the C7–D1 level that was successfully excised without any worsening of neurological status. Case Presentation A 23-year-old female patient presented with history of moderate degree intermittent fever followed by gradually progressive right-sided weakness. She had paresthesia below C5 dermatome on the right side. On close inspection of the back, a tiny sinus opening was noted at the D2 level near the midline. MRI of the spine showed an intramedullary lesion at the C6–D1 level with the tract connecting to the subcutaneous lesion. Somatosensory evoked potential (SSEP) monitoring was done during the surgery with near total excision of the lesion. Conclusion Even though the location is rare, a differential diagnosis of the epidermoid should always be considered. A tiny dermal sinus opening helped us in proper diagnosis, along with surgical excision, by providing us a direct path to the intramedullary lesion. An early diagnosis and treatment with neurophysiological monitoring helped us in the maximum safe resection of the lesion without developing any new deficit.

Congenital dermal sinus tract localized in the cervical region in an adult patient – Clinical case. Review of the literature

Dermal sinus tract (DST) is a rare congenital disease, characterized by the presence of a pathological fistulous tract, lined by epithelial cells. This tract begins from the surface of the skin and reaches to a various depth in the subcutaneous tissues, sometimes it even reaches the spinal canal. The incidence is 1 case per 2500 live births. Less than 1% of the cases are localized in the cervical region. Only in 4% of the cases the patients are diagnosed after the age of 20. In 2015 Karatas reported the oldest patient with dermal sinus tract in the cervical region – a 34-year-old woman. We would like to report a 58-year-old male patient with congenital DST at C4-C5 level. Congenital DST could be responsible for tethered cord syndrome. Operative exploration of the DST is recommended as a prophylactic measure against infectious complications or tethered cord syndrome.

Enuresis in children of primary school age and factors affecting enuresis

INTRODUCTION: With this study, it was aimed to reveal the frequency of monosymptomatic enuresis (MSE) and factors affecting MSE in school children aged 7-12 years in Elazığ city. METHODS: In our study, a total of 1888 students, including 945 boys and 943 girls aged between 7-12, were included with the simple random sampling method. In our study, a questionnaire containing factors that might affect enuresis was used. Detailed histories of all cases were taken, physical examinations and laboratory examinations were performed. The findings were evaluated by the chi-square test. RESULTS: MSE was detected in 352 (18.6%) of 1888 students. The frequency of MSE in boys was 20.8% (n=197) and 16.4% (n=155) in girls (P=0.044). The rate of MSE was higher in children with a family history of epilepsy (6.8%) compared to those without (3.4%) (P=0.013). The monthly income level of families of enuretic children is below the minimum wage in 53.1% (n=186), between the minimum wage and twice its amount in 34.9% (n=122), and more than twice the minimum wage in 12.0% (n=42) of the families. (P=0.027). MSE was present in 18.8% of circumcised and 27.2% of non-circumcised children. Cardiac murmur was found in 4.3% of enuretic and 0.3% of nonenuretic patients (P=0.001). Inguinal hernia enuresis was detected in 0.6%, of enuretic, and in 0.2% (P=0.001) of nonenuretic, patients. While undescended testicular was found in 1.7% of enuretic, and in 0.1% of nonenuretic patients (P=0.001). Sacral hair growth, dermal sinus, and hyperpigmentation was encountered in 11.7% of enuretic children (P=0.001). DISCUSSION AND CONCLUSION: It has been understood that the incidence of MSE is higher in Elazig Province, according to the literature. While the frequency of enuresis decreased in circumcised patients, an inverse relationship was detected between income level and enuresis. It was determined that enuresis decreased school success. In addition, enuresis is more common with pathologies such as family history of epilepsy, cardiac murmur, inguinal hernia, undescended testicle, sacral hair growth, dermal sinus and hyperpigmentation.

Cervical Dermal Sinus Associated With Intradural Dermoid Cyst

Cervical Dermal Sinus Associated With Intradural Dermoid Cyst

Progression of spinal dermal sinus tracts to intraspinal abscess: A single center experience

Progression of spinal dermal sinus tracts to intraspinal abscess: A single center experience

Spinal Abscess in a Patient with Undiagnosed Congenital Dermal Sinus Tract.

Spinal Abscess in a Patient with Undiagnosed Congenital Dermal Sinus Tract.

Spinal Epidermoid Cyst in a Child- An Experience with a Rare Case

Epidermoid cysts are rare benign neoplasm accounting for less than 1% of all intra-spinal tumors. Congenital epidermis cysts are frequently found with syringomyelia, dermal sinus and spina bifida. Most common etiology for an acquired cyst is repeated lumbar puncture. Congenital epidermoid cysts, often associated with other spinal dysraphisms, are caused by the anomalous implantation of ectodermalcells during closure of the neural tube between the third and fifth week of embryonic life. This is a very rare case. With all these characteristics and literature review, we report this case for its rarity and unique characteristics in this part of world (Nepal). These patients commonly present late to the hospital.

Occult Spinal Dysraphism.

1. Lucy C. Holmes, MD, MPH*,‡ 2. Veetai Li, MD†,‡ 1. *Department of Pediatrics, 2. †Department of Neurosurgery, Jacobs School of Medicine and Biomedical Sciences, Buffalo, NY 3. ‡John R. Oishei Children’s Hospital, Buffalo, NY Spinal dysraphism is an incomplete fusion of the spine during embryologic development and encompasses a spectrum of congenital anomalies that can affect the spinal cord, nerve roots, and vertebral column. These anomalies can be categorized into open and closed spinal dysraphism. Open spinal dysraphism is also known as spina bifida aperta. The neural tissue in these lesions is exposed, and examples include meningoceles and myelomeningoceles. Closed spinal dysraphism is also referred to as occult spinal dysraphism (OSD). In these lesions, the neural tissue is covered with skin, making the diagnosis less apparent. Isolated vertebral defects are the most common and least severe form of OSD. Other examples of OSD include neurenteric cysts, split notochord syndrome, split spinal cord malformation, sacral meningeal cysts, spinal lipomas, caudal regression syndrome, dorsal dermal sinus tracts and cysts, and tethered cord syndrome. Among these diagnoses for OSD, evaluations for potential tethered cord syndrome and dermal sinus tracts are the most common reasons for referrals to pediatric neurosurgeons. Because the scope of this In Brief cannot go into detail with all of these diagnoses, we describe tethered cord syndrome and dorsal dermal sinus tracts in more detail and then focus on the epidemiology, associated morbidity, physical findings, challenges with diagnosis, and management of all types of OSD. Tethered cord syndrome can result from several types of spinal dysraphism. A tethered cord arises when the spinal dysraphic malformation creates a situation where the spinal cord is anchored in some way, thereby causing the spinal cord to stretch as the child grows. This leads to ischemic changes in the neurons. Findings on imaging depend on the type of …

Split Cord Malformation—Spectrum of Associated Anomalies

Abstract INTRODUCTION 1. To highlight the spectrum of associated pathological (AP) entities that can occur with (split cord malformations) SCMs, 2. the limitations of imaging techniques to diagnose these entities preoperatively, and 3. the failure of current theories of embryogenesis to explain these complex malformations. METHODS Patients with SCMs and associated pathologies (AP) operated between January 2012 and December 2017 were included. All had neurological evaluation, plain radiographs, CT, and MRI. Postoperative complications were recorded. Follow-up ranged from 6 mo to 3 yr. RESULTS A total of 24 of the 30 patients treated during this period had APs. Age: 1 mo 17 yr. A total of 16 had Type II SCMs and 8 had Type I SCMs. A total of 20 of the 24 patients were neurologically intact. Of the remaining 4, 3 had urinary disturbances and 1 had weakness of 1 lower extremity. All patients had 1 or more APs in addition to SCMs excluding fatty filum. High resolution MRI and/or CT were unable to completely identify the full spectrum of the pathology in these patients in whom the diagnosis was often made intraoperatively.The associated anomalies included in the descending order of frequency included: LDMs (limited dorsal myeloschisis), lipomas; spondylocostal dysostosis, spinal arachnoid cyst, spinal dermal sinus, and neurenteric cyst. Fatty filum was present in 22 of the 24 patients and were sectioned. Intraoperative neurophysiological monitoring was used in the last 6 patients. Complications included cerebrospinal fluid (CSF) leak in 4 patients and wound break down in 2. Of the 4 with neurological deficits, 2 improved. No patient worsened neurologically during the follow-up. CONCLUSION In total 80% of SCMs have APs. Even high resolution imaging cannot completely identify the full spectrum of the pathology in SCMs A high index of suspicion and meticulous search for AP is required while operating patients with SCMs

Acute presentations of intradural lipomas: case reports and a review of the literature

BackgroundLumbosacral lipomas (LLs) may remain asymptomatic or lead to progressive neurological deterioration. However, sudden neurological deterioration is a rare and severe event. Herein, we report rare occurrences of sudden clinical deterioration in two previously asymptomatic children harbouring intradural LLs without dermal sinus tracts or signs of occult dysraphism. A review of the pertinent literature is also included.Case presentationOne child exhibited acute deterioration because of an epidural abscess associated with a filar lipoma without a sinus tract (probably caused by haematogenous spreading from a respiratory tract multiple infection), and the other child exhibited acute deterioration because of a very large, holocord syringomyelia-like cyst associated with a small conus lipoma. Both patients were 4 years old. In case #2, a previously undetected, severe tethered cord (conus at the S3-S4 level) was also present. A complete recovery was attained after an urgent surgical operation in both cases (in addition to targeted antibiotic therapy in case #1). All cases of deterioration in the literature were caused by abscess formation in dermal sinus tracts.ConclusionsProphylactic surgery may be indicated even in asymptomatic children that have tethered cord and surgically favourable LLs (small dorsal and filar LLs), especially if the conditions are associated with progressive syringomyelia. Similarly, intradural dermal sinus tracts should be regarded as surgery-indicated, even if the conus is in its normal position and the patient is asymptomatic because there is a consistent risk of severe, infection-related complications. Finally, asymptomatic patients with filar LLs and a normally located conus can be candidates for surgery or an accurate clinical and radiological follow-up.

Congenital Dermal Sinus and Limited Dorsal Myeloschisis: "Spectrum Disorders" of Incomplete Dysjuction Between Cutaneous and Neural Ectoderms.

The existence of tethering tracts in spinal dysraphism, other than congenital dermal sinus (CDS), has been recognized and has been summated into an integrated concept of limited dorsal myeloschisis (LDM). To elucidate the underlying embryology of LDM in relation to CDS by focusing on the pathological features of special cases of tethering tracts. Out of 389 spinal dysraphism patients who were operated on from 2010 to 2016, 5 patients who had tethering tracts composed of both CDS and LDM (or "probable LDM" if only fibrous tissue was found) were identified. Their clinical presentation, radiological images, operative findings, and pathology were thoroughly reviewed. Three nonsaccular-type patients harbored stalks in which the squamous epithelial lined sinus (CDS) was found in the distal portion, and fibroneural (LDM) or fibrous (probable LDM) tissue in the proximal part. Two patients had saccular lesions, and a stalk was found inside the sac, connecting a small pit on the skin to the spinal cord. The tracts were pathologically identical to a CDS. This study reports the coexistence of CDS and LDM (or probable LDM) components. These unique cases support the hypothesis that the CDS and LDM are among a spectrum of an anomaly that is caused by failure of complete dysjunction between cutaneous and neural ectoderms. Neurosurgeons should be aware of the possibility of coexisting "CDS" components in cases suggestive of LDM. In such cases, not only untethering but also meticulous removal of the squamous epithelium is critical.

Recurrent cerebellar abscess secondary to cranial dermal sinus associated with dermoid cyst in children

Background: Posterior fossa dermoid cysts are rare, benign lesions whose diagnosis can be quite challenging because of their slow growth and subsequent paucity of symptoms. We present herein an unusual case of recurrent cerebellar abscesses induced by an adjacent extradural dermoid cyst with a complete occipital dermal sinus. Methods: The authors report the case of a 20-month-old girl who presented with signs of acutely raised intracranial pressure and whose head scans showed a left cerebellar hemisphere abscess associated with obstructive hydrocephalus. The patient was treated initially with an external ventricular drain, followed by burr-hole aspiration of the abscess and long-term antibiotics. Since the cerebellar abscess recurred, a posterior fossa craniotomy was performed and gross total resection of the lesion along with the dermal sinus tract and abscess contents was achieved. Histopathological analysis confirmed a dermoid tumor. Conclusions: The occurrence of recurrent cerebellar abscesses must always rise up the suspicion of an associated dermoid cyst. Neuroradiological scans should be carefully evaluated in search for this lesion. Once the diagnosis is established, radical resection of the cyst, sinus tract and infectious components is the treatment of choice.

Spinal dorsal dermal spinus tract:Case report and review of the literature

A 5 year old male child was brought with a history of fever headache and intermittent discharge from a sinus in the mid-dorsal area in the back. He had recurrent episodes 0f similar complaints since birth and the symptoms used to subside with antibiotic treatment each time.nbsp On examination he was febrile. There was no neurological abnormality.nbsp Posterior aspect of mid-dorsal spine D7 showed a dimple in the skin with pigmentation and there was minimal active discharge.nbsp USG showed dermal sinus tract with suspected communication with subarachnoid space. CT scan showed dorsal dermal sinus tract with a bifid 7th thoracic vertebra. The spinal dermal sinus tract was excised and the tract disconnected from dural space and dura was repaired. All patients with spinal dorsal dermal sinus tract should undergo aggressive surgical treatment in the form of total excision of the sinus tract and correction of spinal malformation.

Corpus Callosum Lipomas in Children.

To determine the clinical and radiographical aspects of six patients diagnosed with pericallosal lipomas (PCL). A retrospective analysis of patients who presented to the neurosurgery outpatient clinics of Selcuk Faculty of Medicine between 2009 and 2019, revealed that six patients were diagnosed with PCL. The clinical and magnetic resonance imaging (MRI) data were obtained by reviewing patients? records. A total of six patients (two girls and four boys), with a mean age of 53.8 months (38?72 months), were included in this study. They were followed up for a mean period of 36.5 months (32?41 months). PCL were detected on MRIs, which were obtained to investigate headache in two patients, epilepsy in one patient, frontal dermal sinus tract and left frontal epidermoid tumor in one patient, and subcutaneous lipoma associated with PCL in one patient. Five patients displayed tubulonodular lipomas and one patient displayed curvilinear lipomas. Agenesis or dysgenesis of the corpus callosum (CC) was observed in four (66%) patients. Two patients received surgical treatment for cosmetic skin problem. Because of the benign course of PCL, i.e. no growth or very slow growth, and close proximity to the surrounding neurovascular structures, surgical removal should be considered only in symptomatic PCL. Furthermore, other malformations and anomalies may accompany PCL.

Occipital Dermal Sinus Tract Causing Craniospinal Infection: Case Report and Review of Literature

Background: Congenital dermal sinus tract (DST) is a rare entity of cranial or spinal dysraphism that may occur anywhere from nasion and along the midline neuraxis from occipital to sacral regions. Craniospinal infection caused by occipital DST is even rarer. Because of their scarcity, these lesions are not well documented in the literature, often mixed with dermal sinuses in other location or other dysmorphic features. This paper reports a unique case of an infant presented with craniospinal abscesses resulting from occipital dermal sinus tract. Methods and materials: In this paper, we report a case of a 16-month-old girl presented with high grade fever, vomiting and lethargy. She had a discharging occipital skin lesion. Her diagnosis was DST with Cerebellospinal Abscess, which was treated successfully by excision of the DST and cerebellar abscess. Histopathological examination revealed a dermoid cyst. She received 8 weeks of parenteral antibiotic treatment with a good outcome. Results: Occipital DST is a rare condition. Its clinical presentation varies and clinical suspicion is required. Early neurosurgical intervention is important to prevent the risk of potential complications such as abscess and bacterial meningitis. Conclusion: This case highlights the importance of early recognition and evaluation of midline craniospinal cutaneous stigmata in infant. Further neurosurgical assessment with radiological investigations are recommended for early detection and management. Once diagnosed is made, surgical intervention and appropriate antibiotic therapy are the mainstay of treatment.

Spinal Intramedullary Abscess Secondary to Dermal Sinus in Children.

Congenital dermal sinuses (CDS) are uncommon lesions. They are most often noted in lumbosacral region and may lead to meningitis or spinal abscess. Intramedullary spinal cord abscess (IMSCA) due to CDS is rare and often co-exists with an inclusion tumor such as dermoid/epidermoid cyst. Literature review was done to analyze all cases of pediatric IMSCA secondary to CDS by searching online databases starting from the oldest case reported. Only 50 cases have been reported and were analyzed. Mean age was 22.6 months (range 1 month-15 years). Fever, acute flaccid lower limb weakness, and urinary disturbances were the most common presenting features. Dermal sinus was commonest in lumbosacral region. Inclusion cysts were observed in 50% of cases. Staphylococcus aureus was the most the common organism. Mean follow-up duration was 18.2 months (range 1 week-156 months). Majority of the cases underwent multilevel laminectomy with myelotomy and drainage of abscess. Outcome was good-to-excellent in around 60% cases with four deaths. Presence of fever and limb weakness was significantly associated with poor outcomes. Intramedullary abscess secondary to CDS is very rare. Complete sinus tract excision, myelotomy and drainage of abscess, and decompression of co-existent inclusion cysts with prolonged antibiotic therapy remain the standard treatment. Approximately 60% cases achieve good outcomes. Fever and limb weakness portend poorer outcomes than those without.

Adult Versus Pediatric Tethered Cord Syndrome: Clinicoradiological Differences and its Management.

Background:Dysraphic lesions in adults, presenting clinically as tethered cord syndrome (TCS), are relatively rare, and their optimal management remains controversial.Patients and Methods:We performed a retrospective analysis of our pediatric database over a period of last 7 years to focus on the adult TCS. Our aim was to determine the clinicoradiological and etiopathological differences between adult and pediatric patients as well as to determine the results of surgery in adult TCS.Results:Adult spinal dysraphisms constituted 15.4% of our patients (20 out of 130). Motor weakness, sphincteric dysfunction, and backache (n = 13, 65.0% each) predominated in adults unlike children who presented with subcutaneous swellings (n = 74, 67.6%) followed by motor weakness (n = 40, 46.4%), backache being reported by only three patients. The different pathologic substrates underlying adult dysraphisms were lipomeningocele (n = 8), split cord malformation (total = 7; Type 1: n = 5; Type 2: n = 2), dermal sinus (n = 2), and fatty filum (n = 3). On the other hand, meningomyelocele/meningocele (61, 54.9%) followed by split cord malformation Type 1 and 2 (n = 29, 26.1%) predominated in children. The radiological differences between the two groups were a higher incidence of vertebral body defects (hemivertebrae and butterfly vertebrae) and lack of intracranial anomalies in adults. At a mean follow-up of 20.5 months, the most common symptoms to improve following detethering were pain (11 out of 13, 84.6%) followed by motor weakness (six out of 13, 56.2%) and sphincteric control (7 out of 13, 53.8%).Conclusion:Most common symptoms to improve following detethering in adult TCS were pain followed by motor weakness. The major radiological differences between these two groups were a higher incidence of vertebral body defects (hemivertebrae and butterfly vertebrae) and lack of intracranial anomalies in adults.

Lipomes du filum terminal : à propos de 17 cas et revue de littérature

Introduction Filum terminal lipomas are being identified with increasing frequency due to the use of modern imaging (MRI). It is a benign pathology when asymptomatic. Surgical untethering is the treatment. Its incidence is unknown, but it had been estimated to be approximately 0.1%. Surgical intervention in pediatric neurosurgery has increased during the last two decades. Tethered cord syndrome may be due to thickened and fatty structural elements within the filum terminal that render it appropriately tense preventing normal stretching and movement. Material and methods We study the clinical and imaging for 17 patients; this filum was associated to other pathologies in 41.17% of cases: diastematomyelia, lipomyelomeningocele, dermal sinus, myelomeningocele. Clinical findings were mainly the cutaneous stigmatas and the orthopedic deformities and less frequently urinary disorders 29.41% of patients were asymptomatic. All patients were explored with MRI. A medullary CT scan was performed in 58.82% of cases. Results All patients were operated between 2010 to 2016. Evolution was favorable in 8 cases, stabilisation in 7 and aggravation in 2 patients. Conclusion Filum terminal lipomas are a common incidental finding on lumber spinal. MRI surgery is the best option. Evolution after surgery is generally good.

Double paramedian occipital dermal sinus revealing an infected intra-dural dermoid cyst of the posterior cerebral fossa: Case report

Abstract Background Intracranial dermoid cysts (DC) are rare benign embryologic tumors. They represent approximately 0.3% of all intracranial tumors. The association of intracranial DC and multiple dermal sinuses is exceptional. Methods A 3 year-old girl presented with headache and vomiting. Physical examination showed nothing abnormal except for a small subcutaneous nodule above the occipital protuberance with a small skin opening. She had no neurological deficits. MRI and CT scan showed a cyst in the posterior fossa with occipital sinuses. The child was treated by total resection of the intra-dural occipital cyst and the abscess though a suboccipital approach and antibiotics after surgery with good outcome. Results Histopathologic examination confirmed a diagnosis of DC, and bacterial investigation of the abscess did not yield any microbes. Conclusion Infected Intradural DC with complete dermal sinus is a rare type. Radical neurosurgical treatment of DC and dermal sinus associated with antibiotics should be planned early to prevent infections such as abscess and meningitis.