RESUMO
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.
Assuntos
Espinha Bífida Oculta , Ultrassonografia Pré-Natal , Humanos , Feminino , Gravidez , Espinha Bífida Oculta/diagnóstico por imagem , Espinha Bífida Oculta/cirurgia , Adulto , Recém-Nascido , Imageamento por Ressonância MagnéticaRESUMO
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.
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Neoplasias Meníngeas , Meningioma , Espinha Bífida Oculta , Feminino , Humanos , Idoso , Espinha Bífida Oculta/complicações , Espinha Bífida Oculta/diagnóstico , Espinha Bífida Oculta/cirurgia , Meningioma/complicações , Qualidade de VidaRESUMO
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.
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Lipoma , Espinha Bífida Oculta , Neoplasias da Medula Espinal , Humanos , Espinha Bífida Oculta/complicações , Espinha Bífida Oculta/diagnóstico por imagem , Espinha Bífida Oculta/cirurgia , Lipoma/diagnóstico por imagem , Lipoma/cirurgia , Lipoma/complicações , Procedimentos Neurocirúrgicos/métodos , Radiografia , Medula Espinal/cirurgia , Neoplasias da Medula Espinal/diagnóstico por imagem , Neoplasias da Medula Espinal/cirurgiaRESUMO
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.
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Cisto Dermoide , Neoplasias Nasais , Doenças dos Seios Paranasais , Neoplasias dos Seios Paranasais , Seios Paranasais , Espinha Bífida Oculta , Humanos , Cisto Dermoide/cirurgia , Cisto Dermoide/patologia , Espinha Bífida Oculta/cirurgia , Neoplasias dos Seios Paranasais/cirurgia , Seios Paranasais/patologia , Neoplasias Nasais/cirurgia , Neoplasias Nasais/patologiaRESUMO
Background: Here we aim to report the persistent spinous process in the 'pan sacral type' of spina bifida occulta in an asymptomatic male and discuss its clinical significance. The presence of this type of dorsal wall defect with a bony spur attached to it has never been described in the literature to the best of our knowledge after extensive literature search. Our work presents the first anatomic description where the spinous and paraspinous cleft are seen in a sacrum of a live subject. Case Report: During a morphometric study of the sacra, normal subject computed tomography imaging (CT) was procured from the Department of Radio-diagnosis. A three-dimensional (3D) image of the sacrum was created using Dicom to Print and Geomagic freeform plus software. A complete dorsal wall defect was observed in a 3D reconstructed sacrum of an adult male. The sacral canal was converted into a groove with a bony spur hanging in the centre. The longitudinal bony spur attached to the lamina was the persistent spinous process. Conclusion: Such congenital defects are clinically significant for the anaesthetist during caudal epidural block and for orthopaedic surgeons before any surgical procedure. It may be misdiagnosed as an abnormal bony injury on CT. Thus, it is essential to ensure that patients with congenital anomalies are not treated unnecessarily for spinal fractures.
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Espinha Bífida Oculta , Fraturas da Coluna Vertebral , Adulto , Humanos , Masculino , Sacro/diagnóstico por imagem , Sacro/anormalidades , Sacro/cirurgia , Espinha Bífida Oculta/diagnóstico por imagem , Espinha Bífida Oculta/cirurgia , Tomografia Computadorizada por Raios XRESUMO
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.
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Cistos , Hidrocefalia , Hipertensão Intracraniana , Malformações do Sistema Nervoso , Espinha Bífida Oculta , Gravidez , Recém-Nascido , Humanos , Feminino , Espinha Bífida Oculta/complicações , Espinha Bífida Oculta/diagnóstico por imagem , Espinha Bífida Oculta/cirurgia , Coluna Vertebral , Hidrocefalia/diagnóstico por imagem , Hidrocefalia/etiologia , Hidrocefalia/cirurgia , Hipertensão Intracraniana/complicações , Inflamação/complicações , Malformações do Sistema Nervoso/complicações , Cistos/complicaçõesRESUMO
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.
Assuntos
Hidrocefalia , Hipertensão Intracraniana , Defeitos do Tubo Neural , Espinha Bífida Oculta , Humanos , Adulto , Feminino , Defeitos do Tubo Neural/cirurgia , Hidrocefalia/complicações , Coluna Vertebral , Diagnóstico por Imagem , Hipertensão Intracraniana/complicações , Espinha Bífida Oculta/cirurgia , Medula Espinal/anormalidadesRESUMO
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.
Assuntos
Cauda Equina , Defeitos do Tubo Neural , Espinha Bífida Oculta , Masculino , Feminino , Humanos , Adulto , Espinha Bífida Oculta/complicações , Espinha Bífida Oculta/diagnóstico , Espinha Bífida Oculta/cirurgia , Estudos Retrospectivos , Defeitos do Tubo Neural/complicações , Defeitos do Tubo Neural/diagnóstico , Defeitos do Tubo Neural/cirurgia , Cauda Equina/cirurgia , Medula Espinal/diagnóstico por imagem , Medula Espinal/cirurgia , Medula Espinal/anormalidades , Imageamento por Ressonância Magnética/métodos , DorRESUMO
Objective:To review the clinical and radiological presentation and management of infected nasal dermal sinus cystsï¼NDSCï¼ 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 ï¼33.9%ï¼, nasal bridge in 34 cases ï¼57.6%ï¼, nasal tip in 4 cases ï¼6.8%ï¼, and nasal tip and nasal root in 1 case ï¼1.7%ï¼. The depth of lesions included 6 cases ï¼10.2%ï¼ of superficial type of nasal frontal bone, 33 cases ï¼55.9%ï¼ of nasal frontal bone, 19 cases ï¼32.2%ï¼ of intracranial epidural type, and 1 case ï¼1.7%ï¼ of intracranial epidural type. The main sites of infection included inner canthus in 15 cases ï¼25.4%ï¼, nasal dorsum in 22 cases ï¼37.3%ï¼, nasal root in 16 cases ï¼27.1%ï¼, and forehead in 6 cases ï¼10.2%ï¼. Among 59 cases, 7 cases ï¼11.9%ï¼ were complicated with other diseases, and 4 casesï¼6.8%ï¼ had external nasal deformities. Surgical approaches included transverse incision in 12 casesï¼20.3%ï¼, minimal midline vertical incision in 41 cases ï¼69.5%ï¼, external rhinoplasty in 4 cases ï¼6.8%ï¼ and bicoronal incision with vertical incision in 2 cases ï¼3.4%ï¼. 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 monthsï¼average 52.2 monthsï¼. 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.
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Cisto Dermoide , Neoplasias Nasais , Doenças dos Seios Paranasais , Rinoplastia , Espinha Bífida Oculta , Criança , Humanos , Cisto Dermoide/diagnóstico , Cisto Dermoide/patologia , Cisto Dermoide/cirurgia , Neoplasias Nasais/cirurgia , Espinha Bífida Oculta/cirurgia , Doenças dos Seios Paranasais/cirurgiaRESUMO
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.
Assuntos
Humanos , Lactente , Pré-Escolar , Criança , Adolescente , Pediatria , Espinha Bífida Oculta/cirurgia , Espinha Bífida Oculta/complicações , Espinha Bífida Oculta/diagnóstico , Atenção Terciária à Saúde , Tomografia Computadorizada por Raios X , HospitaisRESUMO
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.
Assuntos
Pediatria , Espinha Bífida Oculta , Criança , Hospitais , Humanos , Espinha Bífida Oculta/complicações , Espinha Bífida Oculta/diagnóstico , Espinha Bífida Oculta/cirurgia , Atenção Terciária à Saúde , Tomografia Computadorizada por Raios XRESUMO
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.
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Fístula , Monitorização Neurofisiológica Intraoperatória , Espinha Bífida Oculta , Criança , Pré-Escolar , Potencial Evocado Motor/fisiologia , Humanos , Lactente , Recém-Nascido , Monitorização Neurofisiológica Intraoperatória/métodos , Procedimentos Neurocirúrgicos/métodos , Estudos Retrospectivos , Espinha Bífida Oculta/diagnóstico por imagem , Espinha Bífida Oculta/cirurgia , Coluna VertebralRESUMO
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.
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Infecções do Sistema Nervoso Central , Espinha Bífida Oculta , Disrafismo Espinal , Antibacterianos/uso terapêutico , Infecções do Sistema Nervoso Central/tratamento farmacológico , Infecções do Sistema Nervoso Central/cirurgia , Humanos , Estudos Retrospectivos , Espinha Bífida Oculta/complicações , Espinha Bífida Oculta/cirurgia , Disrafismo Espinal/complicaçõesAssuntos
Articulação do Ombro , Espinha Bífida Oculta , Anormalidades Congênitas , Humanos , Costelas/diagnóstico por imagem , Costelas/cirurgia , Escápula/anormalidades , Escápula/diagnóstico por imagem , Escápula/cirurgia , Articulação do Ombro/anormalidades , Espinha Bífida Oculta/complicações , Espinha Bífida Oculta/diagnóstico por imagem , Espinha Bífida Oculta/cirurgiaRESUMO
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.
Assuntos
Cisto Dermoide , Cisto Epidérmico , Espinha Bífida Oculta , Criança , Cisto Dermoide/diagnóstico por imagem , Cisto Dermoide/cirurgia , Humanos , Imageamento por Ressonância Magnética , Recidiva Local de Neoplasia , Estudos Retrospectivos , Espinha Bífida Oculta/diagnóstico por imagem , Espinha Bífida Oculta/cirurgiaRESUMO
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.
Assuntos
Cisto Dermoide , Espinha Bífida Oculta , Neoplasias da Medula Espinal , Criança , Cisto Dermoide/diagnóstico por imagem , Cisto Dermoide/cirurgia , Humanos , Recém-Nascido , Masculino , Espinha Bífida Oculta/diagnóstico por imagem , Espinha Bífida Oculta/cirurgia , Neoplasias da Medula Espinal/diagnóstico por imagem , Neoplasias da Medula Espinal/cirurgia , UltrassonografiaRESUMO
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.
Assuntos
Cisto Dermoide , Doenças do Cão , Espinha Bífida Oculta , Disrafismo Espinal , Animais , Bengala , Cisto Dermoide/cirurgia , Cisto Dermoide/veterinária , Doenças do Cão/diagnóstico por imagem , Doenças do Cão/cirurgia , Cães , Masculino , Espinha Bífida Oculta/cirurgia , Espinha Bífida Oculta/veterinária , Disrafismo Espinal/cirurgia , Disrafismo Espinal/veterináriaRESUMO
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.
Assuntos
Espinha Bífida Oculta , Doenças da Medula Espinal , Abscesso/complicações , Abscesso/diagnóstico por imagem , Criança , Humanos , Imageamento por Ressonância Magnética , Espinha Bífida Oculta/complicações , Espinha Bífida Oculta/diagnóstico por imagem , Espinha Bífida Oculta/cirurgia , Doenças da Medula Espinal/complicações , Doenças da Medula Espinal/diagnóstico por imagemRESUMO
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.
Assuntos
Caramujo Conus , Cisto Dermoide , Espinha Bífida Oculta , Abscesso , Animais , Feminino , Humanos , Lactente , Imageamento por Ressonância Magnética , Recidiva Local de Neoplasia , Espinha Bífida Oculta/complicações , Espinha Bífida Oculta/diagnóstico por imagem , Espinha Bífida Oculta/cirurgia , Coluna VertebralRESUMO
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.