Senos dérmicos craneales en pediatría: presentación, diagnóstico, complicaciones y tratamiento. Experiencia de un hospital pediátrico de nivel III / Cranial dermal sinuses in pediatrics: Presentation, diagnosis, complications, and management. Experience at a tertiary care children's hospital

Introducción. El seno dérmico cefálico es un tractotubular formado por una separación incompleta entre el ectodermo neural y el ectodermo epitelial;puede tener complicaciones infecciosas. Existen reportes aislados de esta patología. Objetivo: describir una serie de pacientescon seno dérmico craneal, las formas depresentación, el diagnóstico, el tratamiento y las complicaciones. Población y métodos. Estudio observacionaldescriptivo de una serie de pacientes pediátricos con seno dérmico cefálico atendidos en unhospital pediátrico de nivel III entre 2014 y 2019. Resultados. se incluyeron 18 pacientes. La clínicade presentación fue, en 12 casos, una lesión puntual en el cuero cabelludo, hipertensiónendocraneana en 4 casos, ataxia en 1 caso y lesión puntual con fístula en 1 caso. La mitad presentó síntomas de infección. La localización fue en la línea media sobre el hueso occipital en 13 casos, en la línea media sobre el hueso frontal en 3 casos y en la línea media interparietal en 2 casos. En 5 casos se encontró asociada una imagen extracraneana y, en 11 casos, una complicación intracraneana. Ningún paciente presentó recidiva de la lesión y en todos se realizó un solo procedimiento quirúrgico. Conclusiones. Los senos dérmicos en esta serie se presentaron como lesiones puntuales en cuerocabelludo. La localización más frecuente fue a nivel occipital sobre línea media y, en más de la mitad, atravesaba el hueso. El tratamiento de elección fue la exéresis completa del seno dérmicoy las lesiones asociadas. Ante la presencia de senos dérmicos sintomáticos o asociados alesiones intracraneanas, la cirugía se realizó de urgencia.

Introduction. A cranial dermal sinus is a tubular tract resulting from the incomplete separation of the epithelial ectoderm from the neuroectoderm which may lead to infectious complicationsThere have been isolated reports of this condition. Objective. To describe a series of patients with cranial dermal sinus, its presentation, diagnosis, management, and complications. Population and methods. Observational,descriptive study of a series of pediatric patients with cranial dermal sinus treated at a tertiary care children's hospital between 2014 and 2019. Results. A total of 18 patients were included. Theclinical presentation was a specific lesion on the scalp in 12 cases, intracranial hypertension in 4, ataxia in 1, and a specific lesion with fistula tract in 1. Half of patients had symptoms of infection. The lesion was located in the midline of the occipital bone in 13 cases; in the midline of the frontal bone in 3 cases; and in the interparietal midline in 2 cases. The dermal sinus was associated with anextracranial image in 5 cases and an intracranial complication in 11 cases. No patient hadrecurrence and only one surgery was performed in all of them. Conclusions. In this series, dermal sinusespresented as specific lesions on the scalp. The most common site was the occipital midline, and more than 50% of these extended through the bone. The treatment of choice was complete resection of dermal sinus and associated lesions. An emergency surgery was performed when the dermal sinus was symptomatic or associated with intracranial lesions.

A Case of Lumbar Infantile Hemangioma (SACRAL Syndrome) Accompanied by Spinal Anomaly and Dermal Sinus / 대한피부과학회지

Infantile hemangioma (IH) usually presents solely as a cutaneous manifestation, and rarely accompanies diverse anomalies such as spinal dysraphism. A 2-month-old girl presented with IH on her lumbar skin as a coin-sized red plaque with adjacent depressed skin and a child-palm-sized red plaque on her left ankle since birth. Considering the coexistence of IH and depressed skin on the midline in her lumbosacral area, magnetic resonance imaging of her spine was performed, which showed intraspinal/dermal vascular tumors with spina bifida occulta at the 12th thoracic vertebrae level. Furthermore, no neurologic deficits were observed. She has been taking oral propranolol with topical timolol to prevent neural complications and the lesions clinically improved. However, additional surgery for the intraspinal lesions was considered due to urination/defecation abnormalities since she was 13 months of age. In cases of midline IH, particularly with additional skin lesions, appropriate imaging studies to identify accompanying anomalies should be performed, and referrals to neurosurgical specialists should be considered.

Tubercular Spinal Paravertebral Abscesses Presenting as Multiple Dermal Sinuses in HIV Reactive Patients.

Extra Pulmonary tuberculosis (EPTB) accounts for more than 50% of the cases of Tuberculosis in HIV-reactive patients and the clinically presentation is often atypical. EPTB sometimes poses diagnostic dilemmas as conventional diagnostic methods have a poor diagnostic yield and definite diagnosis may be delayed. Here we report a case of Spinal TB who uncommonly presented with multiple non healing sinuses in the Thoracolumbar region. Diagnosis was clinched by clinical presentation, +ve Interferon Gamma Release Assay (IGRA) accompanied by suggestive Magnetic resonance imaging findings.

Catastrophic Intramedullary Abscess Caused by a Missed Congenital Dermal Sinus

Congenital dermal sinus (CDS) is a type of occult spinal dysraphism characterized by a midline skin dimple. A 12-month-old girl presented with fever and ascending quadriparesis. She had a midline skin dimple in the upper sacral area that had been discovered in her neonatal period. Imaging studies revealed a holocord intramedullary abscess and CDS. Overlooking CDS or misdiagnosing it as benign sacrococcygeal dimple may lead to catastrophic infection and cause serious neurological deficits. Therefore, further imaging work-up or consultation with a pediatric neurosurgeon is recommended following discovery of any atypical-looking dimples in the midline.

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

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

Lipoma espinhal associado a seio dérmico congênito: relato de caso / Spinal lipoma associated with congenital dermal sinus: a case report

Os lipomas espinhais são raros, respondendo por 1 por cento de todos os tumores espinhais, estando associados ao disrafismo espinhal oculto em mais de 99 por cento dos casos. Estão divididos em três tipos principais: lipomielomeningocele, lipoma intradural e fibrolipoma do filo terminal. Este relato descreve um caso de lipoma lombossacral congênito associado a estigma cutâneo do tipo seio dérmico lombar congênito.

Spinal lipomas are rare, accounting for 1 percent of all spinal tumors and being associated with occult spinal dysraphism in more than 99 percent of cases. Such lesions are divided into three main types, namely, lipomyelomeningoceles, intradural lipomas, and filum terminale fibrolipomas. The present report describes a case of congenital lumbosacral lipoma associated with cutaneous stigmata of the lumbar dermal sinus type.

Intramedullary Entire Spinal Cord Abscess Secondary to a Congenital Dermal Sinus: A Case Report

Intramedullary spinal abscesses are rare and potentially devastating lesions. The first case of spinal abscess was described in 1830 and fewer than 100 cases have been reported subsequently. Only sixteen previous reports have described an intramedullary abscess of the central nervous system secondary to a dermal sinus. Involvement of the entire cord is thus exceedingly rare. We present a case of a 7-month-old girl who had rapidly progressing quadriplegia with urinary and bowel retention and was found to have an intramedullary abscess as a result of a dermal sinus. Spine MRI shows thick irregular ring enhancement appeared within the broadest area of the spinal cord on the thoracic and lumbar area on mid thoracic level to L3 and high cervical area which extended to medulla. Immediate decompressive surgery and antibiotic treatment were performed. Excellent clinical outcome was obtained with a combination of medical and surgical management. Complete neurological assessment and diagnostic study of all patients with a congenital dermal sinus are very important. Prophylatic surgery is indicated in many cases to prevent dangerous and recurrent infections of the central nervous system.

A Case of Intracranial Dermoid Cyst Associated with Scalp Abscess / 대한소아신경학회지

Intracranial dermoid cyst is a rare congenital disease originating in the embryo. Intracranial dermoid cysts are usually detected before five years of age; however, asymptomatic cysts have been discovered after the third decade of life through various complications. Sometimes, there are neurologic symptoms caused by a cystic mass effect and if there is a dermoid sinus, purulent infections can occur. Early diagnosis is important and as such, CT or MR imaging for recognition of the location, size, and characteristics of the cysts are recommended before surgical resection. Extradural dermoid cysts with an intact occipital dermoid sinus are a rare event. We report a case of intracranial dermoid cyst associated with scalp abscess. In this case, a three-year-old boy was presented with scalp abscess and we performed MR imaging, which revealed a cystic region. The prognosis after resection operation was good without any complication.

A Case Report of Congenital Spinal Dermal Sinus Tract

PURPOSE: Congenital spinal dermal sinus tract is a rare lesion connecting skin to deeper structures including neural tissue. It results from the failure of the neuroectoderm to separate from the cutaneous ectoderm in the third to fifth week of gestation. The common locations are the lumbosacral and occipital regions. Sometimes it extends to spinal canal. In this paper we report a case of congenital spinal dermal sinus tract in the coccyx. METHODS: A 21-month-old male child born after an uncomplicated full-term pregnancy was admitted to our institute with a midline dermal sinus and a cartilaginous protrusion in the coccygeal region. There were no signs of infection. Neurologic examination showed no functional deficit in both lower limbs. He was treated with complete excision of the tract and an underlying accessory cartilage. RESULTS: The spinal dermal sinus tract was extended from the skin to the coccyx. The stalk was loosely attached to the accessory cartilage of coccyx. At that point, it was dissected from the accessory cartilage and resected. The accessory cartilage was also resected at the bone and cartilage junction. During the follow-up period of 6 months, the wound healed well without any complication nor recurrence. CONCLUSION: Congenital spinal dermal sinus tract is known as a form of spinal dysraphism. In order to prevent complications, timely surgical intervention including complete resection of sinus tract with correction of associated abnormalities is of utmost importance.

A Case of Recurrent Bacterial Meningitis Associated with Lumbosacral Dermal Sinus during Early Infancy / 대한소아신경학회지

Acute bacterial meningitis is an uncommon, potentially life-threatening infection and recurrent episodes of bacterial meningitis are rarely seen. But when they occur, an exhaustive search for the mechanisms underlying the recurrent episodes must be pursued, especially some possible routes of migration of bacteria to the cerebrospinal fluid. Dermal sinus tracts are common skin manifestations seen with spinal dysraphism and may be associated with recurrent meningitis. Here, we present a case of a 2-month-old girl who had a small dimple on the lower lumbosacral area. She was attacked twice by purulent bacterial meningitis due to a dermal sinus tract and a tethered cord. She was treated with the excision of the tract, the removal of the dermoid and the detethering of the cord. Staphylococcus and Klebsiella were cultured separately and assumed to be causative agents. The lesion was suspected on the physical examination and demonstrated by lumbosacral magnetic resonance imaging.

Congenital Dermal Sinuses: An Clinical Analysis of 20 Cases

OBJECTIVE: Congenital dermal sinus is a rare congenital disease that results from the failure of the neuroectoderm to separate from the surface ectoderm during the process of neurulation, where there is communication between the skin and the deeper structures. Their pathogenesis, clinical course and treatment strategy are well known. We analyze our series and compare our results with other series. METHODS: Twenty patients were diagnosed as congenital dermal sinus and confirmed pathologically from October 1986 to July 2003 at our hospital. We studied the patients' clinical manifestations, radiological findings and pathological profiles. RESULTS: Seven cases were located in the suboccipital area and 13 cases were located in the spinal area. Interestingly, 4 of 13 spinal lesion cutaneous openings were located lower than the 3rd sacral body level. 8 of 20 lesions were terminated at neural structures, 4 of 20 lesions were terminated at the intradural portion and others terminated at the extradural portion. Nine anomalies were combined with the dermal sinus, including 4 lipomas, 2 Currarino's triad, 1 encephalocele, 1 myelomeningocele and 1 diastematomyelia. Eleven patients had dermoid tumors. CONCLUSION: Congenital Dermal Sinus must be surgically removed immediately if they are diagnosed. The surgical procedure of congenital dermal sinus is complete removal, but in some cases, complete removal is impossible. In those cases, we removed all epithelial tissues. We consider sacrococcygeal dimple almost invariably have no connection with intraspinal structures. But, if other cutaneous manifestations are combined with cutaneous pits, it can communicate with the sacrococcygeal dimple.

Case of Infant with Congenital Dermal Sinus Complicating Multiple Intramedullary Spinal Abscess / 实用儿科临床杂志

Objective To improve the recognition of intramedullary spinal abscess by a case of congenital dermal sinus with intramedullary spinal abscess and reduco the incidence of congenital dermal sinus with intramedullary spinal abscess.Methods Clinical,laboratory data and image of a confirmed case about one infant of congenital dermal sinus with multiple intramedullary spinal abscess were investigated,the related literature was reviewed.Results In this case,when the infant with congential dermal sinus had infection,he failed to gain antibiotic therapy, timely surgical treatment,his infection had diffused, and multiple intramedullary spinal abscess flared up.Conclusions Intramedullary spinal abscess is a rare disease.If treatment is delayed, the prognosis is poor and the mortality rate is high.MRI is the ideal investigation for diagnosis.Intramedullary spinal abscess can happen subsequent to congenital dermal sinus with infection, and cause neurological sequela. So an infant with congenital dermal sinus should be offered to avoid complication caused by infection.

Meningocele with Cervical Dermoid Sinus Tract Presenting with Congenital Mirror Movement and Recurrent Meningitis

Dermoid sinuses and meningoceles are seldom encountered in the cervical region. Besides, to the best of our knowledge, the coexistence of these types of congenital abnormalities with recurrent meningitis, as well as with mirror movement, has never been reported before. A 14-year-old female with the diagnosis of recurrent meningitis was referred to our clinic from the Department of Infectious Diseases. She had a cervical meningocele mass that was leaking cerebro-spinal fluid (CSF) and an associated mirror movement symptom. Spina bifida, dermoid sinus and meningocele lesions were demonstrated at the C2 level on computed tomography (CT) and magnetic resonance imaging (MRI). She underwent an operation to remove the sinus tract together with the sac, and at the same time the tethered cord between the sac base and the distal end of the spinal cord was detached. The diagnosis of dermoid sinus and meningocele was confirmed histopathologically. These kinds of congenital pathologies in the cervical region may also predispose the patient to other diseases or symptoms. Herein, a case of meningocele associated with cervical dermoid sinus tract which presented with recurrent meningitis and a rare manifestation of mirror movement is discussed. Neurosurgeons should consider the possible coexistence of mirror movement and recurrent meningitis in the treatment of these types of congenital abnormalities.

A Case of Intradural Dermoid Cyst in the Cervical Region Associated with Congenital Dermal Sinus: Case Report

We report a rare case of a 10-month-old male with a cervical intradural dermoid cyst associated with a congenital dermal sinus. He was presented motor weakness of right upper extremity 1 month before admission. The cervical spinal MRI demonstrated an intradural extramedullary mass from third cervical vertebra to fourth cervical vertebra, which was connected with a skin through a sinus tract. With extensive laminectomy, the mass and the sinus tract were completely removed and the mass was histopathologically diagnosed as a dermoid cyst. six months after surgery, the neurological status return to normal and MR study showed no evidence of recurrence or any deformity. In case of the intradural dermoid cyst associated with a congenital dermal sinus, early diagnosis and total excision of the cyst, including the entire tract may provide an excellent outcome.

Congenital Dermal Sinus at Thoracic Region Associated

Congenital dermal sinus (CDS) is a rare entity widely known to occur as a result of the of the failure of the neuroectoderm to separate from the cutaneous ectoderm during the process of neurulation at the third to fifth week of gestation. The lesion can occur at any level of the craniospinal axis, but are located predominantly at the lumbosacral and occipital region. CDS of thoracic region and cervical region are rare. The patient with CDS presents with meningitis and/or mass effect from the associated inclusion tumor. They are usually dermoid or epidermoid cyst. Teratoma is rarely associated with CDS. We report our experience of CDS an 11-month old boy associated with an intramedullary dermoid tumor at the thoracic region, and together we report a review of the literature.

Spinal congenital dermal sinus--experience of 5 cases over a period of 10 years

Spinal congenital dermal sinus (CDS) is a rare entity which supposedly results from the failure of the neuroectoderm to separate from the cutaneous ectoderm during the process of neurulation. The lesions are most frequent at the lumbosacral followed by the occipital region. CDS of the thoracic region is very rare. The patients with spinal CDS present with meningitis and/or mass effect from the associated inclusion tumor. They are usually dermoid or epidermoid cysts. Teratoma is rarely associated. The authors experienced 5 cases of spinal CDS over a 10 year period. Of the 5 cases, 2 were at thoracic and 3 were at lumbosacral levels. Dermoid cyst, epidermoid cyst and teratoma were associated in one case each. Two cases presented with neurological deficit and meningitis while an additional case presented with neurological deficit and a history of probable meningitis. Pain was present in 2 cases. Magnetic resonance imaging played an important role in the diagnosis of the lesion and planning of surgery. All the cases showed a good response to surgery even though one patient had persistent neurological deficit.

A Case of Congeital Occipital Dermoid Cyst

A 18-year-old girl, who presented with painful scalp mass on occipital region, was found in have a congential dermal sinus. At surgery, upon incision of the occipital muscle fascia, pus poket was encountered and it was connected with thickened occipito-cervical dura through the small foramen on midline occiptial bone. The pathogenesis and principles of management are briefly reviewed.

A Case of Congenital Lumbosacral Dermal Sinus Associated with Recurrent Meningitis(Case Report)

Recurrent episode of meningitis in infants and children frequently constitute a frustrating and distressing, both in determination of course and treatment. The authors are reporting a infected case of congenital dermal sinus with dermoid cyst in the subarachnoid space of cauda equina and conus medullaris which was cured after complete removal of cyst and sinus tract.

Congenital Cranial Dermal Sinus: Case Report

Congenital cranial dermal sinus is one of the causes of meningitis in pediatric patients. In spite of its rarity, this entity should be considered when a child has meningitis of unexplained origin. Authors report a case of congenital cranial dermal sinus in a 15 month old girl who presented with recurrent meningitis.

A Case of Dermoid in the Thoracolumbar Region

Dermoid and epidermoid of the spinal cord are rare and their percentage was 2.6% of spinal cord tumors. In dermoid average age of occurrence is 28.3 years. They are dysembryonic malformations. Dermoid consists of desquamated material, sebaceous material and hairs. The region of preference is lumbosacral spinal cords, especially cauda equina and conus medullaris. We are reporting a case of dermoid which has taken intracapsular enucleation and discuss about the epidemiology and pathogenesis.