In utero Diagnosis of Spinal Dermal Sinus.

INTRODUCTION: Congenital dermal sinus (CDS) is an open neural tube defect (NTD) that occurs in 1 in 2,500 births a year and often goes undetected until patients present with complications like infection and neurological deficits. Early diagnosis and repair of CDS may prevent formation of these complications. In utero diagnosis of these lesions may improve long-term outcomes by enabling referral to specialty services and planned postnatal repair; however, only 2 such cases have been reported in the literature. We present a third case of in utero diagnosis of CDS with a description and discussion of findings from surgical exploration and pathology. CASE PRESENTATION: Routine prenatal ultrasound scan detected a tethered cystic structure arising from the back of the fetus at 20 weeks of gestation. Dedicated fetal ultrasound confirmed the presence of a cystic lesion protruding through a lamina defect, while fetal magnetic resonance imaging showed an intact spinal cord and meninges, suggesting a diagnosis of CDS. Neurosurgery followed along closely and took the child for surgical exploration on day 2 of life. A fibrous stalk with an intradural component and associated cord tethering was excised. Histology showed fibrous tissue without an epithelial-lined lumen. CONCLUSION: CDS is a form of NTD that occurs from nondisjunction of the cutaneous ectoderm and neuroectoderm during formation of the neural tube. Slight differences in how this error occurs can explain variations seen in this spectrum of disease, including CDS without an epithelial-lined lumen as seen in this case. Newborns with CDS can go undiagnosed for years and present with long-term complications. Fetal imaging can assist in early recognition and surgical excision of CDS in newborns.

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

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

Surgery for spina bifida occulta: spinal lipoma and tethered spinal cord.

The technical evolution of the surgery for spina bifida occulta (SBO) over the course of a half-century was reviewed with special foci placed on the spinal lipoma and tethered spinal cord. Looking back through history, SBO had been included in spina bifida (SB). Since the first surgery for spinal lipoma in the mid-nineteenth century, SBO has come to be recognized as an independent pathology in the early twentieth century. A half-century ago, the only option available for SB diagnosis was the plain X-ray, and pioneers of the time persevered in the field of surgery. The classification of spinal lipoma was first described in the early 1970s, and the concept of tethered spinal cord (TSC) was proposed in 1976. Surgical management of spinal lipoma with partial resection was the most widely practiced approach and was indicated only for symptomatic patients. After understanding TSC and tethered cord syndrome (TCS), more aggressive approaches became preferred. A PubMed search suggested that there was a dramatic increase of publications on the topic beginning around 1980. There have been immense academic achievements and technical evolutions since then. From the authors' viewpoint, landmark achievements in this field are listed as follows: (1) establishment of the concept of TSC and the understanding of TCS; (2) unraveling the process of secondary and junctional neurulation; (3) introduction of modern intraoperative neurophysiological mapping and monitoring (IONM) for surgery of spinal lipomas, especially the introduction of bulbocavernosus reflex (BCR) monitoring; (4) introduction of radical resection as a surgical technique; and (5) proposal of a new classification system of spinal lipomas based on embryonic stage. Understanding the embryonic background seems critical because different embryonic stages bring different clinical features and of course different spinal lipomas. Surgical indications and selection of surgical technique should be judged based on the background embryonic stage of the spinal lipoma. As time flows forward, technology continues to advance. Further accumulation of clinical experience and research will open the new horizon in the management of spinal lipomas and other SBO in the next half-century.

The Necessity of Dural Resection for Nasal Dermal Sinus Cyst With Intracranial Extension.

Nasal dermal sinus cysts are characterized by an intracranial-extradural extension. Complete extirpation of nasofrontal dermoid sinus cysts is essential for effective treatment to minimize recurrence. The authors revealed the pathologic findings of the cranial end connected to the dura. In our case, the cranial end and dura were fibrous connective tissues that were difficult to separate. For complete extirpation of the nasal dermal sinus cyst with intracranial extension, the cranial ends of the nasofrontal dermoid sinus cyst and dura should be resected en bloc.

Case report: double dermal sinus tracts of the cervical and thoracic spine associated with congenital intracranial pathology (hydrocephalus and an interhemispheric cyst) in a newborn patient.

INTRODUCTION: A dermal sinus tract (DST) is an uncommon type of spinal dysraphisms characterized by a tract lined with stratified squamous epithelium that extends from the subcutaneous tissue to the underlying thecal sac or neural tube. These developmental anomalies can present asymptomatically with cutaneous abnormalities or with devastating complications. Usually, it is presented as a unique lesion, and there are only a few reports that show multiple sinuses, and none of them associated with midline brain malformations. METHODS: We present the case of a 3-day-old girl with an antenatal diagnosis of hydrocephalus who was diagnosed with double dermal sinus tracts of the cervical and thoracic regions at admission. The patient presented signs of elevated intracranial pressure (ICP), which imposed a challenge in the management of the case. RESULTS: Our patient was successfully treated initially with a lumbar puncture in order to discard a cerebrospinal fluid (CSF) infection. With negative CSF cultures, a ventriculoperitoneal shunt (VPS) was placed. Nine days after the VPS surgery and without signs of infection, the DST was excised in a single procedure, without follow-up complications. CONCLUSION: To our knowledge, this is the first description of a patient with multiple midline neural tube defects (NTDs) associated with congenital intracranial pathology. Although there are no guidelines regarding the best treatment for this complex associated pathology, the patient was treated, without follow-up complications.

Chronic Hydrocephalus and Tethered Cord Masquerading as Postconcussive Syndrome.

We present a rare case of chronic hydrocephalus and tethered cord syndrome in a high-functioning asymptomatic college student. Her presentation was triggered by a fall, at which time, she developed subacute symptoms consistent with progressive increased intracranial pressure. After endoscopic third ventriculostomy for hydrocephalus, the patient presented with progressive bilateral lower extremity and sphincter symptoms, leading to spinal imaging and diagnosis of tethered cord syndrome with fatty filum. The patient was treated with tethered cord release and had a favorable long-term outcome. This case highlights the innocuous onset of 2 congenital diagnoses in adulthood seemingly associated with a mild traumatic injury. Furthermore, it shows classic imaging findings associated with chronic increased intracranial pressure, aqueductal stenosis, and tethered cord syndrome (preoperative and postoperative appearances), which serve an important educational role given the relate rarity of these coexisting diseases in adults.

[Diagnosis and surgical treatment of tethered cord syndrome accompanied by congenital dermal sinus tract in adults].

OBJECTIVE: To summarize the clinical manifestations, imaging characteristics and experience of surgical treatment of tethered cord syndrome (TCS) accompanied by dermal sinus tract (DST) in adulthoods. METHODS: The authors retrospectively analyzed a series of 25 adult patients with TCS due to DST that were surgically treated under microscope from September 2010 to October 2019. There were 10 males and 15 females with an average age of 29.7 years (rang, 18-48 years). Characterized cutaneous malformation and dermal sinus were found in the lumbosacral region in all the 25 patients. Clinically, all the patients presented with chronic back and lower-extremity pain, numbness and weakness of lower limbs, and bowel and bladder dysfunction. Two cases were admitted to the emergency room with acute infectious cerebral spinal fluid (CSF) leakage complicated with meningitis. According to magnetic resonance imaging (MRI) images, the subdural course of DST whose traction of the spinal cord, the location of the conus medullaris, the presence of subdural lesions, and the diameter of the internal filum terminale were evaluated. The surgical procedure included separating and excising of the DST, section of the internal filum terminale, detethering of the TCS, and reconstruction of the dural sac under microscopy. The patients remained in prone position in 7 days postoperation. RESULTS: MRI showed that the position of the conus medullaris was lower than the level of lumbar 2 vertebrae, and the distal part of the DST entered the subdural stretched part of the spinal cord, to constitute one of the factors of TCS in all the 25 patients. Twenty patients had fatty infiltration of internal filum terminale and another patient had thickened (approximately 5 mm in diameter) internal filum terminale resulting in tightening the conus medullaris. A total of 25 operations were performed including completely dissection and resection of the DST through the skin down to the subdural space, section of the internal filum terminale, detethering of the TCS, and the subdural dermoid cysts were removed in two patients. There were no postoperative complications. The postoperative pathology was consistent with the structure of the DST and internal filum terminale. The local pain was relieved, and the lower-extremity weakness and bowel and bladder dysfunction were gradually relieved postoperatively. The period of follow-up ranged from 3 months to 9 years (mean, 3.9 years). The neurological function of all the patients was intact, and MRI showed that the physiological curvature of the thoracolumbar spine remained normal. There was no recurrence of TCS observed during the follow-up. CONCLUSION: The adult TCS accompanied with DST is characterized by typical cutaneous malformation in the lumbosacral region and tethering of the spinal cord. The patients are usually combined with internal filum terminale enlargement tightening of conus medullaris as well. The surgical treatment including totally resection of the DST and section of the internal filum terminale to detethering the TCS at the same time under microscopy. The outcome of surgical treatment is satisfactory.

[Clinical analysis of 59 cases of pediatric nasal dermal sinus cysts with midfacial infection as the first symptom].

Objective:To review the clinical and radiological presentation and management of infected nasal dermal sinus cysts（NDSC） in children. Methods:Clinical data were collected from 59 NDSC children with secondary fronto-orbital area infection who presented to Beijing Children's Hospital from January 2007 to December 2021. All patients underwent preoperative imaging workup, including MRI and CT. All patients underwent endoscopic excision of a NDSC under general anesthesia. Results:A total of 59 patients were included in the study,while 58 presented with a sinus, 1 presented with a cyst.The main lesions of NDSC included nasal root in 20 cases （33.9%）, nasal bridge in 34 cases （57.6%）, nasal tip in 4 cases （6.8%）, and nasal tip and nasal root in 1 case （1.7%）. The depth of lesions included 6 cases （10.2%） of superficial type of nasal frontal bone, 33 cases （55.9%） of nasal frontal bone, 19 cases （32.2%） of intracranial epidural type, and 1 case （1.7%） of intracranial epidural type. The main sites of infection included inner canthus in 15 cases （25.4%）, nasal dorsum in 22 cases （37.3%）, nasal root in 16 cases （27.1%）, and forehead in 6 cases （10.2%）. Among 59 cases, 7 cases （11.9%） were complicated with other diseases, and 4 cases（6.8%） had external nasal deformities. Surgical approaches included transverse incision in 12 cases（20.3%）, minimal midline vertical incision in 41 cases （69.5%）, external rhinoplasty in 4 cases （6.8%） and bicoronal incision with vertical incision in 2 cases （3.4%）. The range of lesions was completely consistent with MRI results.All cases were successfully operated without one-stage nasal dorsum reconstruction. All patients were followed up from 7 to 173 months（average 52.2 months）. There were 6 cases of recurrence, all of which were in situ recurrence. The operation was performed again, and no recurrence has occurred since the follow-up, No nasal deformity was noted, and cosmetic outcome were favorable for all patients. Conclusion:NDSC infection in children with midfacial infection as the first symptom is rare in clinical practice, and its manifestations are diverse. Early diagnosis and rational treatment are very important to improve the cure rate.Preoperative high resolution MRI combined with CT scanning is of great significance in judging the course of NDSC, especially the intracranial extension. The treatment goal is to achieve minimally invasive and individualized treatment under the premise of complete excision of the lesion, and take into account the cosmetic needs.

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.

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

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 complications. There have been isolated reports of this condition. OBJECTIVE: 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 complications. There have been isolated reports of this condition. 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: 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. CONCLUSIONS: In this series, dermal sinuses presented 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.

Introducción. El seno dérmico cefálico es un tracto tubular 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 pacientes con seno dérmico craneal, las formas de presentación, el diagnóstico, el tratamiento y las complicaciones. Población y métodos. Estudio observacional descriptivo de una serie de pacientes pediátricos con seno dérmico cefálico atendidos en un hospital pediátrico de nivel III entre 2014 y 2019. RESULTADOS: se incluyeron 18 pacientes. La clínica de presentación fue, en 12 casos, una lesión puntual en el cuero cabelludo, hipertensión endocraneana 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 cuero cabelludo. 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 dérmico y las lesiones asociadas. Ante la presencia de senos dérmicos sintomáticos o asociados a lesiones intracraneanas, la cirugía se realizó de urgencia.

Successful Neonatal, Intraoperative Neuromonitoring in the Surgical Correction of a Thoracic Dermal Sinus Tract: Technical Note.

INTRODUCTION: Intraoperative neuromonitoring (IONM) is commonly used during surgery of the spine and spinal cord for early surveillance of iatrogenic injury to the central and peripheral nervous system. However, for infants and young children under 3 years of age, the use of IONM is challenging due to incomplete central and peripheral myelination. CASE PRESENTATION: We report a case of a T4-T6 dermal sinus tract (DST) that was resected on day of life 23, with the successful use of IONM. CONCLUSION: To our knowledge, this is the youngest reported case of the use of IONM in the surgical correction of a DST in a neonatal patient. This case demonstrates the potential efficacy of IONM in neonatal spine surgery and the techniques used to adapt the technology to an immature nervous system.

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

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

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.

Growth of Intramedullary Spinal Cord Dermoid Cyst from a Congenital Thoracic Dermal Sinus Tract after Negative Screening Ultrasound Imaging.

INTRODUCTION: Intramedullary thoracic dermoid cysts are rare lesions that are associated with dermal sinus tracts (DSTs). Current recommendations advocate for imaging-based screening of suspected DSTs shortly after birth to exclude associated inclusion lesions. CASE PRESENTATION: A 6-year-old male child presented with a 2-week history of progressive ataxia, lower limb weakness, and hyperreflexia. He was suspected to have a thoracic DST at birth, though initial screening ultrasound was negative for an inclusion lesion or intradural tract. On representation, MRI demonstrated a 3.9-cm intramedullary thoracic dermoid cyst causing significant spinal cord compression. Intraoperatively, a DST extending intradurally was found. The associated dermoid cyst was removed via intracapsular resection. CONCLUSIONS: Whilst dermoid cysts are presumed to progressively develop from DSTs, to our knowledge, this is the first case in English literature documenting a thoracic spinal cord intramedullary dermoid cyst following a negative screening ultrasound for a suspected DST. We use this case to highlight the false-negative rates associated with postnatal screening and advocate for early neurosurgical referral of suspected DSTs, regardless of imaging findings.

Infected congenital lumbosacral dermal sinus tract with conus epidermoid abscess: a rare entity.

PURPOSE: Dermal sinus is more commonly associated with intradural dermoid than an epidermoid cyst. Conus epidermoid cyst with dermal sinus is a rare entity. We are presenting a rare case of infected conus epidermoid cyst along with the dermal sinus in an 18-month-old girl presented with flaccid paraparesis with sphincter dysfunction and timely intervention leads to complete recovery. We had searched PubMed for previously reported similar cases and did a case-based review of the literature. CASE REPORT: This 18-month-old girl with discharging lumbosacral sinus with fever since 3 days presented with flaccid paraparesis with sphincter dysfunction. Preoperative magnetic resonance imaging (MRI) showed a large enhancing lesion from L1-S1 along with the dermal sinus tract. Complete excision of the cyst along with the sinus tract, followed by long-term antibiotic therapy. The excision of the infected cyst was done through myelotomy under neuromonitoring, while some part of the capsule densely adherent to the neural tissue was left behind. The patient gradually improved following surgery and motor power of the lower limbs were [Formula: see text] while going home. Histopathology revealed epidermoid cyst with secondary inflammatory tissue. Follow-up MRI of the spine showed excision of the dermal sinus tract and cyst with postoperative changes. At 1-year follow-up, the patient was asymptomatic without any focal deficits. CONCLUSION: Early surgical intervention followed by long-term antibiotic therapy is a must for good functional recovery in patients of an infected dermal sinus tract with associated cyst. While excising cyst through myelotomy, some part of the capsule densely adherent to neural tissue may be left behind. Regular follow-up in the first year of surgery is essential to look for the recurrence of the lesion.

Type IV dermoid sinus, intramedullary dermoid cyst and spina bifida in a Cane Corso.

A 1-year-old male Cane Corso was presented for progressive hindlimb neurologic deficits, with a history of suspected recurrent meningitis. Physical examination and advanced diagnostic imaging findings were suggestive of a thoracic dermoid sinus extending into the dura mater, and spina bifida. After initial medical treatment, the lesion was surgically excised, and the presence of an intramedullary cyst in continuation with the dermoid sinus was confirmed, requiring partial durectomy and myelotomy for removal. Histopathological examination of the excised tissue confirmed the diagnosis of a dermoid sinus associated with an intramedullary dermoid cyst. After surgery, the dog temporarily declined neurologically but then showed progressive neurological improvement. This report describes presentation and successful surgical treatment of a type IVa dermoid sinus associated with an intramedullary dermoid cyst and spina bifida in a dog.

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

PURPOSE: Congenital 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. METHODS: The 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. RESULTS: Both patients demonstrated excellent neurological recovery. CONCLUSIONS: Complete 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.

Congenital Dermal Sinus Elements in Each Tethering Stalk of Coexisting Thoracic Limited Dorsal Myeloschisis and Retained Medullary Cord.

INTRODUCTION: The embryogenesis of limited dorsal myeloschisis (LDM) likely involves impaired disjunction between the cutaneous and neural ectoderms during primary neurulation. Because LDM and congenital dermal sinus (CDS) have a shared origin in this regard, CDS elements can be found in the LDM stalk. Retained medullary cord (RMC) is a closed spinal dysraphism involving a robust, elongated, cord-like structure extending from the conus medullaris to the dural cul-de-sac. Because the RMC is assumed to be caused by impaired secondary neurulation, concurrent RMC and CDS cannot be explained embryologically. In the present article, we report a case in which CDS elements were noted in each tethering stalk of a coexisting LDM and RMC. CASE PRESENTATION: A 2.5-month-old boy with left clubfoot and frequent urinary and fecal leakage had 2 tethering tracts. The upper tract, which ran from the thoracic tail-like cutaneous appendage, had CDS elements in the extradural stalk and a tiny dermoid cyst in the intradural stalk immediately after the dural entry. In the lower tract, which ran from the lumbosacral dimple, the CDS as an extradural stalk continued to the RMC at the dural cul-de-sac. Both stalks were entirely resected through skip laminotomy/laminectomy at 1 stage to untether the cord and resect the CDS elements. CONCLUSION: Surgeons should be aware that CDS elements, in addition to LDM, may coexist with RMC that extends out to the extradural space.

Complete resection and untethering of the cervical and thoracic spinal dermal sinus tracts in adult patients.

Dermal sinus tracts (DSTs) of the cervical and thoracic spine are extremely rare, particularly in adult patients because diagnosis is typically made in the early stage after birth by pediatricians. These cases should be treated surgically as soon as possible to prevent neurological sequelae. This report describes two rare adult cases with cervical and thoracic spine DSTs. The first patient presented with back pain and headache, whose skin lesion had been long known, but disregarded since birth. The second patient had long suffered from residual cervical myelopathy from the prior incomplete surgical treatment. Both cases had these sinus tracts excised completely and had spinal cord untethered successfully without any neurological deterioration. There has been a trend toward earlier diagnosis of these entities, but still some cases that were diagnosed in a delayed fashion or underwent incomplete treatment are reported. Improper management during childhood could lead to irreversible neurological deficit caused by spinal cord tethering and/or direct compression due to DSTs-associated tumors. The early detection and prompt surgical intervention improve the chance of a good surgical outcome. Furthermore, complete excision of the sinus tracts and associated tumors could help prevent future bacterial contamination and recurrence.

Neurosurgical Pathology and Management of Limited Dorsal Myeloschisis Associated with Congenital Dermal Sinus in Infancy.

BACKGROUND: Because of the shared origin of limited dorsal myeloschisis (LDM) and congenital dermal sinus (CDS), CDS elements may be found within the fibroneural LDM stalk. When part of the CDS invested in the intradural stalk is left during untethering surgery, inclusion tumors such as dermoid cysts may develop. However, the most appropriate surgical strategy for LDM with CDS is still under debate. METHODS: Of 19 patients with LDM, 3 (15.8%) had histologically verified CDS elements. We retrospectively analyzed the clinicopathological findings of these patients. RESULTS: In patient 1, the entire stalk including a tiny dermoid cyst at the intradural stalk could be resected through two-level laminectomy during untethering at 6 months of age. In patients 2 and 3, the stalk appeared to be a typical LDM stalk during the initial surgery at 18 and 7 days, respectively; however, CDS was histologically diagnosed in the proximal severed end of the stalk. Postoperative three-dimensional heavily T2-weighted imaging demonstrated spherical enlargement of the remnant stalk, and the entire length of the remnant stalk including newly developed dermoid was resected during the second surgery at 3 years 11 months and 11 months, respectively. Histopathologically, glial fibrillary acidic protein-immunopositive neuroglial tissues and CDS elements were mainly located at the proximal and distal sites of the stalk, respectively, supporting the "dragging down and pulling up" theory. In patients 2 and 3, however, the proximal head of the dermoid cyst passed the distal head of the neuroglial tissues and located at the stalk-cord attachment. CONCLUSION: Surgeons should be aware of the approximately 10% possibility of the coexistence of CDS when managing infant LDM. However, the recommendation for excision of the entire length of the LDM stalk in all patients should be more carefully made because such a strategy may result in an unnecessary extent of laminotomy/laminectomy for most patients with pure LDM. However, once the postoperative histological examination reveals coexistence of CDS in the resected proximal part of the stalk, the entire length of the remnant stalk should be excised as soon as possible.