Congenital Dermal Sinus Research Articles

Intraspinal abscess associated with dorsal dermal sinus: A rare cause of unexplained hydrocephalus

Intraspinal abscess associated with dorsal dermal sinus: A rare cause of unexplained hydrocephalus

Intramedullary spinal cord abscess: A grave complication of a dermal sinus tract

Congenital dermal sinus tracts are an uncommon form of spinal dysraphism. If identified and treated early, outcomes are favourable. If missed, complications can be significant. Intramedullary spinal cord abscess is a rare but catastrophic complication. We report the case of an infantile patient with an intramedullary spinal cord abscess occurring 2-months post treatment for a coliform meningitis with an unidentified dermal sinus tract. We examined the literature on the investigation and management of dermal sinus tracts and conclude that all cases of unexplained, atypical meningitis in an infant should trigger suspicion for an underlying dermal sinus tract. In such cases, an MRI scan of the neuroaxis should be performed urgently. Keywords: syrinx; dimple; meningitis; dysraphism.

Congenital dermal sinus in the lateral buttock

We report a case of a one-year-old boy with a congenital dermal sinus situated at the lateral buttock. The exact pathway of the sinus was shown by magnetic resonance imaging, contrast medium examination and computed tomographic examination. The sinus was not connected to the spinal canal and the sinus ended near the anterior surface of the sacral bone in a blind manner. Complete surgical resection of the sinus was achieved after these imaging examinations.

Congenital fistula between the lateral buttock and postanal dermal sinus tracts with a low-type anorectal malformation

A congenital fistula between the lateral buttock and postanal dermal sinus tracts with a low-type anorectal malformation has not been previously reported. The aim of this study was to clarify the clinical characteristics and optimal surgical management of this condition. Three female patients were noted to have a pit in the left upper lateral buttock and a postanal orifice. Fistulography and magnetic resonance imaging showed no connection between the pit and orifice in the first case and a connection in the second and third cases. In the first case, surgical exploration showed firm tracts from both the lateral buttock and postanus running deep to the tip of the coccyx, with a thin cord between them. The tracts were excised, but the cord was left. In the second and third cases, the fistula was excised. The center of the sphincter muscle was placed at the postanal orifice in all three cases; thus, anal transplantation was performed. Histopathological examination showed stratified squamous epithelium of the skin in the wall of the fistula. The first case developed a pelvic abscess 22 months after surgery. Therefore, complete excision is crucial to minimize the risk of postoperative pelvic abscess.

P.234 “Inappropriate” spinal ultrasounds for suspected occult spinal dysraphism in northern Alberta

Background: Occult spinal dysraphism (OSD) may be associated with visible cutaneous manifestations. A common non-pathological mimic of a dermal sinus tract is an incidental sacrococcygeal dimple. Choosing Wisely Canada Guidelines recommend these dimples not be imaged. Methods: This study assessed the appropriateness of spinal ultrasounds performed for the investigation of an OSD. We interrogated our local imaging system to analyze spinal ultrasounds being performed in babies less than 6 months of age, from 2017-2018. Results: 429 children had spinal ultrasounds ordered by pediatricians (55%), family doctors (21%), and neonatologists (20%). 183 children (43%) had imaging indications that was deemed “inappropriate”. Some of this cohort had further MRI imaging (5/183) or neurosurgical referral (8/183). None of these children underwent neurosurgery. 231 (54%) children had appropriate indications for imaging. Within this cohort MRI imaging (23/231), neurosurgical referral (24/231) and neurosurgical intervention occurred (4/231). All four “surgical children” harboured either a dorsal appendage or a subcutaneous lipoma. We estimated, declining inappropriate scans would save $22, 500 annually. Conclusions: A significant portion of local spinal ultrasound requests for OSD are inappropriate. Collaboration with other sites is ongoing to investigate if the Choosing Wisely guidelines for imaging this patient population are being practiced more efficiently.

Intramedullary spinal abscess associated with intramedullary dermoid and infected dermal sinus:case report and review of the literature

Intramedullary spinal abscesses are extremely rare. Most occur secondary to cardiopulmonary spread;contiguous origin is less frequent. Few intramedullary spinal abscesses have been reported secondary to dermal sinus tracts. A dermoid sinus is a congenital lesion (closed simple dysraphism) with rare incidence.Dermoid sinuses can ease local invasion and spinal infections (meningitis, intradural extramedullary and intramedullary abscesses), usually with thoracolumbar involvement. Here we describe acase of a 20-month-old boy who presented with fever and refusal to walk 2 days prior to admission. On examination of the lumbar spine, a small sinus with skin stigmata was noted.Neurological evaluation revealed paraparesis more prominent on the left side, reduced tone and reflexes, left leg hypoesthesia,reduced anal tone, and urinary retention.MRI scan demonstrated intramedullary abscess extending from L2 to S1 level. The patient was urgently admitted for drainage of intramedullary abscess and excision of the dermal sinus tract. Proper antibiotic treatment was completed for 6 weeks with gradual improvement and ultimately full ambulation ability.

Isolated extramedullary intradural thoracic epidermoid cyst: A rare case report

Spinal epidermoid cyst is a rare entity. It may be congenital with associated spinal dysraphism such asspina bifida ,dermal sinus tract and other neural tube defects or acquired due to repeated lumbar puncture and trauma. Congenital spinal epidermoid cysts without spinal dysraphism are rare. Here we are reporting a rare and interesting case of thoracic extramedullary intradural epidermoid cyst with no associated features of spinal dysraphism or history of iatrogenic trauma in a young male patient.

Intramedullary spinal cord abscess—rare complication of a congenital dermal sinus: Report of a case in a toddler

Abstract Lumbosacral region is the commonest location for a dermal sinus (DS). The DS provides a natural pathway for infections of the deeper tissues such as the meninges or spinal cord, depending on its termination. One of the rarest and serious complications of a DS is an intramedullary spinal cord abscess. One such case has been reported here. A 2-year boy presented with high-grade fever, acute onset flaccid paraplegia and urinary retention. A L5 level DS was noted. Imaging showed a D12-L5 heterogeneously enhancing intradural lesion suggestive of an abscess. Multi-level laminectomy and drainage of abscess with removal of an inclusion epidermoid was performed. Complete recovery of all neurological functions was achieved that remained stable at 60 months follow up.

Surgical Treatment of Congenital Dermal Sinus: An Experience of 56 Cases

Objective: This study aims to assess the impact of early diagnosis and surgery on children with congenital dermal sinus, investigate the relationship between MRI findings and extent of surgical exploration, and summarize our clinical experience with the surgical management in cases with central nervous system (CNS) infection. Methods: The skin features, preoperative MRI images, intraoperative findings, postoperative pathological characteristics, and prognoses of 56 children with congenital dermal sinus were analyzed retrospectively. Results: All the children had a pinpoint ostium in the skin, and 52 out of the 56 children (92.9%) had intraspinal dermoid cysts or epidermoid cysts. Before surgery, MRI did not show intraspinal lesions in 13 children, and surgery revealed intradural lesions in 9 of these children (69.2%). Among 46 children without CNS infection, 16 children had neurological impairment before surgery. After surgery, recovery was complete in 36 children, partial in 9 children, and absent in 3 children. All children with CNS infection had neurological impairment before surgery. After surgery, the condition improved in 8 children and exacerbated in 2 children. Children without CNS infection had statistically significantly better prognosis than children with CNS infection (p = 0.03). Conclusion: A pinpoint ostium in the dorsal midline is the characteristic feature of congenital dermal sinus. In cases without intraspinal lesions on MRI, the spinal canal should be explored intraoperatively to ensure complete removal of the lesion and prevent recurrences. In cases without CNS infection, early diagnosis and timely surgery are beneficial to the recovery of nerve function.

A critical analysis of surgery for occult tethered cord syndrome.

Tethered cord syndrome (TCS) is an amalgamation of neurological, urological, orthopedic, and dermatologic signs and symptoms with radiographic evidence of a thickened filum and low-lying conus. Surgical sectioning of the filum and disconnection of any tethering entities such as dermal sinus tracts or lipomas has been shown to improve outcomes. The manifestation of TCS symptoms in the absence of a low-lying conus has been referred to as occult tethered cord syndrome (OTCS) and is much less well reviewed in the literature. To date, there has only been one randomized controlled trial examining the effect of intervention in OTCS; therefore, contemporary data is often elicited from limited cohorts. To perform a comprehensive literature review of management in OTCS and evaluate treatment response rates to sectioning of the filum terminale. Seventeen papers met inclusion criteria for our review. Sample sizes ranged from 8 to 60 children, and results were mixed, often dependent on study design, definition of typical OTCS symptoms, and follow-up intervals. Symptomatic improvement was observed in > 50% of patients for all but one study; however, the recurrence rates were highly variable. The data regarding the efficacy of surgical treatment in OTCS is mixed and merits more rigorous scientific examination with strict and clear parameters regarding symptomatic operationalization and follow-up time points to monitor for TCS recurrence.

Pushing the Limits of Prenatal Ultrasound: A Case of Dorsal Dermal Sinus Associated with an Overt Arnold–Chiari Malformation and a 3q Duplication

We present a case of a fetus with cranial abnormalities typical of open spina bifida but with an intact spine shown on both ultrasound and fetal MRI. Expert ultrasound examination revealed a very small tract between the spine and the skin, and a postmortem examination confirmed the diagnosis of a dorsal dermal sinus. Genetic analysis found a mosaic 3q23q27 duplication in the form of a marker chromosome. This case emphasizes that meticulous prenatal ultrasound examination has the potential to diagnose even closed subtypes of neural tube defects. Furthermore, with cerebral anomalies suggesting a spina bifida, other imaging techniques together with genetic tests and measurement of alpha-fetoprotein in the amniotic fluid should be performed.

Disorders of Secondary Neurulation : Mainly Focused on Pathoembryogenesis.

Recent advancements in basic research on the process of secondary neurulation and increased clinical experience with caudal spinal anomalies with associated abnormalities in the surrounding and distal structures shed light on further understanding of the pathoembryogenesis of the lesions and led to the new classification of these dysraphic entities. We summarized the changing concepts of lesions developed from the disordered secondary neurulation shown during the last decade. In addition, we suggested our new pathoembryogenetic explanations for a few entities based on the literature and the data from our previous animal research. Disordered secondary neurulation at each phase of development may cause corresponding lesions, such as failed junction with the primary neural tube (junctional neural tube defect and segmental spinal dysgenesis), dysgenesis or duplication of the caudal cell mass associated with disturbed activity of caudal mesenchymal tissue (caudal agenesis and caudal duplication syndrome), failed ingression of the primitive streak to the caudal cell mass (myelomeningocele), focal limited dorsal neuro-cutaneous nondisjunction (limited dorsal myeloschisis and congenital dermal sinus), neuro-mesenchymal adhesion (lumbosacral lipomatous malformation), and regression failure spectrum of the medullary cord (thickened filum and filar cyst, low-lying conus, retained medullary cord, terminal myelocele and terminal myelocystocele). It seems that almost every anomalous entity of the primary neural tube may occur in the area of secondary neurulation. Furthermore, the close association with the activity of caudal mesenchymal tissue in secondary neurulation involves a wider range of surrounding structures than in primary neurulation. Although the majority of the data are from animals, not from humans and many theories are still conjectural, these changing concepts of normal and disordered secondary neurulation will provoke further advancements in our management strategies as well as in the pathoembryogenetic understanding of anomalous lesions in this area.

FRONTONASAL EPIDERMOID CYST WITH PATENT DERMAL SINUS TRACT OPENING ON THE DORSUM OF NOSE AND INTRACRANIAL EXTENSION THROUGH CRIBRIFORM PLATE DEFECT: A RARE CASE REPORT WITH REVIEW OF LITERATURE

Congenital midface anomalies are rare. Multiple congenital midface anomalies occur in children. Imaging techniques like computed tomography (CT) and magnetic resonance imaging (MRI) help in characterising the lesions, making definite diagnosis and knowing about intracranial extension. We present a case report of 8 year old female child with Frontonasal Epidermoid Cyst with patent dermal sinus tract opening on the dorsum of nose and intracranial extension through cribriform plate defect.

Focal Spinal Nondisjunction in Primary Neurulation : Limited Dorsal Myeloschisis and Congenital Spinal Dermal Sinus Tract.

Spinal dysraphic lesions due to focal nondisjunction in primary neurulation are commonly encountered in paediatric neurosurgery, but the “fog-of-war” on these conditions was only gradually dispersed in the past 10 years by the works of the groups led by the senior author and Prof. Kyu-Chang Wang. It is now clear that limited dorsal myeloschisis and congenital spinal dermal sinus tract are conditions at the two ends of a spectrum; and mixed lesions of them with various configurations exist. This review article summarizes the current understanding of these conditions’ embryogenetic mechanisms, pathological anatomy and clinical manifestations, and their management strategy and surgical techniques.

Characteristics of Pediatric Recurrent Bacterial Meningitis in Beijing Children's Hospital, 2006-2019.

Few data on recurrent bacterial meningitis (RBM) in children are available. Here, we estimated the frequency of RBM in children and investigated the predisposing conditions, etiology, and clinical characteristics of RBM in children. Cases of RBM in the Beijing Children's Hospital medical record database between January 2006 and December 2019 were collected. In total, 1905 children with bacterial meningitis (BM) were documented in the Beijing Children's Hospital medical record database. A total of 43 patients had RBM. The rate of RBM in children was 2.3% (43/1905). Forty (93.0%) patients had predisposing conditions, including 15 (34.9%) cases of inner ear malformations, 5 (11.6%) cases of dermal sinus tracts, 9 (20.9%) cases of head injury, 5 (11.6%) cases of congenital cranial meningocele, 3 (7.0%) cases of congenital skull base defects, 3 (7.0%) cases of immunodeficiency, and other 3 (7.0%) cases of unknown reason. Among all the 121 BM episodes, a total of 64 episodes were etiologically confirmed BM and the other 57 episodes were probable BM. Streptococcus pneumoniae (n = 52) was accounted for 81.3% of confirmed BM episodes. Thirty-four of the 37 patients with congenital or acquired anatomical defects were available to follow up after surgeries, and all of them had no BM after surgeries. Three patients with antibody deficiencies got intravenous immunoglobulin therapy and they did not suffer BM anymore. RBM is rare in children. The majority of children with RBM had predisposing conditions including congenital/acquired anatomical defects and immunodeficiency. Interventions should be implemented to solve the underlying conditions to avoid RBM.

A surgical case of coccyx removal because of a pre-sacral abscess through congenital dermal sinus tract infection

A surgical case of coccyx removal because of a pre-sacral abscess through congenital dermal sinus tract infection

A 2-year-old child with dorsal dermal sinus presenting with sudden onset of inability to stand

A 2-year-old child presented to the paediatric emergency department with sudden onset of inability to stand unsupported for 1 week. The parents noticed a discharging sinus in posterior aspect of...

Congenital Dermal Sinus: case series and the consequences of late diagnosis and treatment

Introduction:Congenital Dermal Sinuses (CDS) are rare closed dysraphisms that can present throughout the extent of the neuroaxis. They occur due to a failure of the disjunction of the neuroectoderm and cutaneous ectoderm in a focal point during 3-4 week of embryogenic development. The prevalence of CDS of all types has been estimated to be 1 in 2,500 live births, most commonly localized in the lumbar region. More than half of the cases are associated with dermoid or epidermoid tumors. Clinical presentation of CDS usually consists in cutaneous stigmas like dimples, which has the potential to be diagnosed at birth. However, the majority of patients are diagnosed older and after complications such as meningitis, abscess, osteomyelitis, rupture of an associated epi/dermoid cyst. Once suspected the patient should be submitted to an image study with CT scan and/or MRI, and surgical consultation. Complete exeresis is the definitive treatment. Case report: we present 3 cases of CDS, including an extremely rare case of frontonasal location, to illustrate the extent of the disease and the importance of early diagnosis and treatment. All of the 3 cases presented with complications, requiring surgical treatment and long term antibiotic therapy. Conclusion: Although well reported in the literature, CDS are usually diagnosed after complications. The knowledge of clinical presentation, early diagnosis and treatment are essential to prevent its life threatening complications.

Multidisciplinary Management of Tethered Spinal Cord Syndrome in Children: Operationalizing an Outpatient Patient-Centered Workflow.

IntroductionMultidisciplinary care for patients with tethered spinal cord syndrome (TCS) is valuable in ensuring comprehensive evaluation, timely follow-up, optimal functional outcome, and patient-centered care. The family-centered focus aims to minimize patient and parental burdens associated with care coordination. We present our first-year institutional experience in operationalizing a multidisciplinary, patient-centered, pediatric tethered cord clinic (TCC) to manage routine, long-term surgical follow-up for children with non-myelomeningocele-related tethered spinal cords.MethodsTCC is composed of three surgical services: orthopedic surgery, neurosurgery, urology. A retrospective chart review of patients seen in the TCC from January 2019 to January 2020 was conducted. Patients enrolled in the clinic were intended for long-term follow-up. Demographic and outcome variables were collected.ResultsFifty-nine patients were seen in TCC. Types of tethered spinal cords amongst these patients were the following: fatty filum (62.7%), dermal sinus tract (15.2%), meningocele manqué (8.4%), lipomyelomeningocele (6.7%), low lying conus medullaris (5.1%), and sacral arachnoid cyst (1.7%). Age at diagnosis was 1.31 ± 2.21 (median: 0.25 years) and at follow-up was 9.0 ± 5.18 years (median: 8 years). A total of 50.9% of patients were female, and 93.2% had a prior untethering procedure. Of all patients, 6.8% have no surgical intervention and continue to be monitored conservatively for evidence of decline. All three services evaluated 84.8% of patients during the same clinic session, while 15.3% of patients were seen by two of the services, and 20.3% of patients were able to schedule related imaging or diagnostic testing during the same visit.ConclusionWe describe successful implementation of a multidisciplinary pediatric TCC and document the first year of experience. The TCC streamlines care, decreases burden on families, and reduces those lost to follow-up. Complex disease pathologies, even when clinically stable, require long-term follow-up with multiple subspecialties and benefit from multidisciplinary clinics.

Utility of CISS imaging in the management of tethered cord syndrome

To compare the utility of constructive interference in steady-state (CISS) sequence over T2-weighted (T2W) sequence in the accurate delineation of the tethering element on magnetic resonance (MR) imaging in patients of tethered cord syndrome (TCS) and correlation with intraoperative findings. MR findings of fifty-six consecutive patients with operative findings of TCS were studied. The intraoperative findings of all patients were studied and compared with the preoperative imaging findings on T2W imaging and CISS images. CISS images provided more information than T2W imaging. The overall sensitivity of CISS in MR evaluation of patients with TCS was higher (99.17%) as compared with the T2W sequence (71.48%) especially in the detection of precise position/extent of tethered cord, assessment of filum terminale thickening, detection of a fibrous spur in cases of split cord malformation, detection of dorsal dermal sinus, and evaluation of its precise extent and ramifications. The area under the ROC curve was higher with CISS (0.99) than with T2W sequence (0.85) which reflects its good predictive value as a screening test. This information was useful to the operating surgeon. CISS sequence for imaging TCS can help enhance the overall surgical outcome of the patients ensuring completeness of the surgery. CISS should be routinely performed in the work-up of patients with tethered cord syndrome.