Congenital Dermal Sinus Research Articles

Infected congenital cervical dermal sinuses leading to spinal cord abscess: two case reports and a review of the literature

PurposeCongenital dermal sinuses are a rare form of spinal dysraphism. The developmental defects are located along the midline neuroaxis, with sinuses in the cervical region being the least common. Congenital dermal sinuses can be associated with intraspinal infection as they act as a direct route from the skin and subcutaneous tissues into the spinal cord.MethodsThe authors present two cases of cervical dermal sinuses complicated by intramedullary abscess. Both children presented with neurological decline and febrile illness. MRI showed intraspinal abscess. Both underwent prompt surgical excision of the sinus tract, exploration of the cord and intravenous antibiotics.ResultsBoth patients demonstrated excellent neurological recovery.ConclusionsComplete surgical excision of the sinus and tract in addition to long-term antimicrobials can yield excellent neurological outcomes. At surgery, do not expect to find pus when exploring the intramedullary component. Long-term follow-up is advocated due to potential late recurrence.

Perspectives on Spinal Dysraphism : Past, Present, and Future.

This article recounts the author’s personal views and recollections of the history, conception of embryogenetic theories, application of intraoperative electrophysiology, and development of prospective treatment recommendations of several important spinal dysraphic malformations, including limited dorsal myeloschisis, dermal sinus tract, retained medullary cord, terminal myelocystocoele, and complex spinal cord lipomas.

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.

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.

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

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

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

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 …

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.

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.

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.

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.

Prevotella melaninogenica Meningitis and Abscess: A Complication of the Removal of a Midline Dermoid Cyst

Abstract Prevotella species are gram-negative anaerobic commensal bacteria of the oropharynx, which frequently cause periodontal disease but are otherwise rarely implicated in serious bacterial infections. Cranial dermoid cysts are benign neoplasms that grow along the planes of the embryonic neural tube closure. In infants, they most commonly present in frontal locations, including periorbital, nasal, and within the anterior fontanelle. Although dermoid cysts are slow growing, usually uncomplicated, and easily treated definitively with surgical excision, cranial cysts located on the midline are associated with a higher risk for persistent dermal sinus tract with intracranial extension of the tumor. We describe a case of a 10-month-old male patient with an occipital midline dermoid cyst with intracranial extension, infected with Prevotella melaninogenica, and complicated by intracranial abscess formation and meningitis.This case highlights two unusual disease entities: the uncommon occipital location of a dermoid cyst, and complications of that cyst caused by a serious bacterial infection with a normal oral flora. We discuss the recommendation for neuroimaging prior to surgical excision of a midline dermoid cyst, given the risk for dermal sinus tract with intracranial communication. We also discuss potential mechanisms for bacterial inoculation of this cyst with Prevotella melaninogenica. This pathogen has not previously been reported as a complication of dermoid cysts.

One-stage posterior debridement and fusion combined with irrigation and drainage for the treatment of postoperative lumbar spondylodiscitis

ObjectiveThe aim of this study was to evaluate the clinical outcomes of one-stage posterior debridement, interbody fusion, and instrumentation, combined with irrigation and drainage, for treating lumbar spondylodiscitis. MethodsThe study included 23 patients (13 male and 10 female, mean age: 45 years) who had posterior debridement, interbody fusion, and instrumentation, followed by continuous closed irrigation and drainage for lumbar postoperative spondylodiscitis. The visual analog scale, Oswestry disability index, and lumbar lordosis angle were assessed before and after surgery to evaluate the clinical outcome. ResultsThe mean follow-up time was 27 (24–36) months. All patients tolerated the procedure well, and there were no instances of spondylodiscitis recurrence, though a dorsal dermal sinus developed in one patient after surgery. Infection was eliminated, as evidenced by the normalization of the erythrocyte sedimentation rates and C-reactive protein levels. The mean visual analog scale scores were significantly decreased after the operation. The mean lumbar lordosis angle before surgery was 21.61 ± 6.88° and the angle at the final follow-up was 31.61 ± 4.24°. The mean Oswestry disability index scores improved significantly both after the operation and at the follow-up visits (p < 0.05). Bone union was confirmed in all patients at a mean of 8.6 months post-operation, though this was not achieved until 2 years post-operation in one patient. All 3 patients who had neurological deficits showed great improvement at the last follow-up. ConclusionSurgical management using one-stage posterior debridement, interbody fusion, and instrumentation, followed by continuous closed irrigation and drainage, might be an effective treatment option for lumbar postoperative spondylodiscitis. Level of evidenceLevel IV, Therapeutic study.

Photodynamic therapy for Hidradenitis Suppurativa/acne inversa: Case report

Hidradenitis suppurativa/acne inversa (HS/AI) is a type of chronic suppurative inflammatory reaction of the hair follicles characterized by recurrent dermal abscesses, sinus tracts and scars. So far, there has not been any prospective study proving the efficacy and safety of photodynamic therapy for the disease. In this report, one case of HS/AI achieved resolution of skin lesions, ulcer healing and disappearance of symptoms after nine treatments with 5-aminolevulinic acid photodynamic therapy (ALA-PDT).

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.

Intramedullary Dermoid Cyst of the Cervical Spinal Cord – C5–C7 Level

Introduction Inclusion cysts of the spinal cord are rarely intramedullary. Such cysts are commonly located in the lumbar and thoracic regions and are usually associated with congenital spinal dysraphism and dermal sinus. Intramedullary dermoid cysts in the cervical region without spinal dysraphism are extremely rare. To our knowledge, only seven such cases are reported in the literature to date. Materials and Methods An 18-year-old female patient presented with weakness in all four limbs, more distal than proximal muscle weakness, that had been progressing for 3 years. The magnetic resonance imaging (MRI) showed an intramedullary lesion from C5– C7 with peripheral ring enhancement. “Whorls” were observed within the lesion on T2 weighted image, with associated excavation of vertebral bodies C5– C7. Operative procedure and findings: partial laminectomy of C5– D1was performed. The dura was opened. A small myelotomy was made in the root entry zone. About 1.5 ml of yellowish colored fluid was drained. White shiny debris with hair, whitish pultaceous content and teeth were removed. Complete excision of cyst and its wall was performed. Results The histopathological examination revealed that the cyst wall was lined by stratified squamous epithelium with underlying dermis showing hair follicles, sebaceous glands, adipose tissue and cyst filled with keratin debris suggestive of dermoid cyst. Conclusion The intramedullary location of the dermoid cyst in the cervical cord and the absence of any congenital spinal dysraphism make this case a very unique and rare entity and warrants its inclusion in the reported cases of rare intramedullary space occupying lesions.

Congenital dermal sinus with extensive intramedullary expansion and an infected spinal epidermoid cyst in an infant

An 11-month-old female child presented with progressive weakness involving both lower limbs and left upper limb. There was hypertonia and hyperreflexia in the affected limbs. This was associated with multiple episodes of urinary tract infection and overflow incontinence of the urinary bladder. Magnetic resonance imaging of the spine revealed extensive intramedullary expansion of a congenital dermal sinus. There was a cystic lesion along the lower end of the sinus tract. Surgical exploration had confirmed the presence of an infected epidermoid cyst along with the dermal sinus tract. The entire tract as well as the abscessed epidermoid cyst was removed. Following this, the child showed gradual improvement of neurological function and bladder dysfunction. This is perhaps the first report in current literature of a congenital dermal sinus tract with such an extensive intramedullary extension (almost the entire spine) and an infected epidermoid tumor in an infant.