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.

Spinal dermal sinus complication in children: case series and literature review / Complicação do seio dérmico espinhalem crianças: séries de casos e revisão de literatura / Complicacióndel seno dérmico espinal en niños: serie de casos y revisión de la literatura

ABSTRACT Introduction: Dermal sinus (DS) is a rare dysraphism. It can be asymptomatic, become infected, and produce severe neurological symptoms. Our objective is to present a series of pediatric cases with spinal DS complicated byinfections (DSCI), describe the findings correlated with the anatomy in a stillbirth, as well as the associated pathologies and their treatment. Method: We analyzeddifferent variables in the clinical histories of 5 children with spinal DSCI. In addition, an anatomical dissection of a stillbirth with lumbar DS was performed. Results: Two males and 3 femaleswith DSCI and a mean age of 2 years and 9 months were included: 2 lumbar (one in the midline and the other in theparamedian region), 1 in the thoracic region, 1 in the upper cervical region,and 1 in the lumbosacral region. The forms of presentation were 3 meningeal profiles (one with pain andlocalized swelling) and 3 neurological deficits (one associated with the meningeal profile and another associated with pain and a tumor). In all cases, the tract of the DS was identified by magnetic resonance imaging (MRI).Associated lesions included 1 dorsal intramedullary dermoid cyst, 1 tethered lumbar spinal cord with syringomyelia, 1 partial cervical medullary disconnection, and 2 spinal dysraphisms. Four were operated on and one died of infectious complications before surgery. In the 12-week-old male fetus with lumbar DS, a permeable tract to the subarachnoid space was verified. Conclusions: DSCIsshould bestudied with MRI to identify their tracts, infectious complications of thecentral nervous system, associated malformations, inclusion tumors, and to enabledifferential diagnosis. Once diagnosed, they should be urgently treated both surgically and with prolonged antibiotictherapy. Level of Evidence IV; Therapeutic Study (Treatment Outcome Investigation)

RESUMO Introdução: O seio dérmico (SD) é uma disrafia rara. Pode ser assintomático, sofrer infecção e produzir quadros graves.Nosso objetivo é apresentar uma série de casos pediátricos com SDs espinhais complicados por infecções (SDCI),descrever os achados correlacionados com a anatomia em um natimorto, as patologias associadas e seu tratamento. Método: Foram analisadasas variáveis da históriaclínica em 5 crianças com SCDI espinhal.Além disso, foi feita uma dissecção anatômica de um natimorto com SD. Resultados: Foram incluídos 2 meninose 3 meninas, com média de idade de 2 anos e 9 meses, com SDCI: 2 lombares(um na linha média e um paramediano), 1 na região torácica, 1 na região cervical superior e 1 lombossacral. As formas de apresentação foram 3 quadros meníngeos, 1 com dor e tumefação lombar local e 3 déficits neurológicos (um associado ao quadro meníngeo e outro associado à dor e tumor). Em todos os casos, o trajeto do SD foi identificado por ressonância magnética (RM).Aslesões associadas foram 1 cisto dermoide intramedular dorsal, 1 medula ancorada lombar com siringomielia, 1 desconexão medular cervical parcial e 2 disrafias espinhais. Quatro participantes foram operados e um foi a óbito decorrente de complicações infecciosas antes da cirurgia.No feto masculino de 12 semanas com SD lombar, foi verificado um trajeto permeável até o espaçosubaracnóideo. Conclusões: Os SDCIs devem ser estudados com RM para identificar o seu trajeto, complicações infecciosas do sistema nervoso, malformações associadas, tumores de inclusão e permitir o diagnóstico diferencial. Eles devem ser tratados cirurgicamente com urgência uma vez diagnosticados e com tratamento prolongado com antibióticos. Nível de evidência IV; EstudosTerapêuticos- Investigação dos Resultados do Tratamento

RESUMEN Introducción: El seno dérmico (SD) es una disrafia infrecuente. Puede ser asintomático, infectarse y producircuadros severos. Nuestro objetivo es presentar una serie de casos pediátricos con SD espinalescomplicados porinfecciones (SDCI), describir los hallazgoscorrelacionadoscon la anatomía en un mortinato, las patologías asociadas y su tratamiento. Método: Seanalizaron diferentes variables enlas historias clínicas de 5 niños con SDCI espinales. Además, se realizó unadisección anatómicade un mortinato con SD lumbar. Resultados: Se incluyeron2varones y 3 mujeres,de 2 años y 9 meses de edad promedio, con SDCI:2 lumbares (uno en línea media y otro paramediano), 1 en región torácica, 1 cervical superior y 1 lumbosacro. Las formas de presentación fuerontres cuadros meníngeos, 1 con dolor y tumefacción local lumbar y 3 déficits neurológicos (uno asociado a cuadro meníngeo y otro asociado a dolor y tumor). En todos los casos se identificó el trayecto del SD por resonancia magnética (IRM). Como lesiones asociadas hubo1 quiste dermoide intramedular dorsal,1médula lumbar anclada con siringomielia,1 desconexión medular cervical parcial y 2 disrafias espinales. Cuatro fueron operados y uno falleció por complicaciones infecciosas antes de la cirugía. En el feto masculino de 12 semanas con SD lumbar, se verificóun trayecto permeable hasta el espacio subaracnoideo. Conclusiones: Los SDCI se deben estudiarconIRM, para identificarsu trayecto, las complicaciones infecciosas del sistema nervioso, las malformaciones asociadas,los tumores de inclusión y permitirel diagnóstico diferencial. Se deben tratar quirúrgicamente con urgencia una vez diagnosticados y con antibiótico-terapia prolongada. Nivel de Evidencia IV; Estudio Terapéutico (Investigación de Resultado de Tratamiento).

Smell disorders evolution after chronic rhinosinusitis with polyps surgery

The effects of chronic rhino sinusitis with polyps (CRSWP) surgery on smell symptoms have not been sufficiently studied. The aim of this study was to evaluate the impact of CRSWP surgery on smell symptoms over short and long-term follow-up and to identify the factors that might influence their evolution. Patients and methods: This was a retrospective study about 184 patients operated endoscopically for CRSWP. In post operative period, long-term local steroids were prescribed systematically. The sense of smell was evaluated preand post-operatively according to a subjective score (1: good smell, 2: hyposmia, 3: anosmia). Some factors, related to the patient, the CRSWP and the treatment, were tested in order to identify predictors of smell outcome after surgery.. Results: Before surgery, the anosmia and the hyposmia were noted in 90.8% and in 8.7% of cases, respectively. At six months after surgery, the improvement of olfactory score was significant: 84% of patient had the score 1 compared with 0.5% preoperatively (p < 0.0001). This improvement was maintained during the 2 first years and decreased significantly at 3 years, although an average delay of polyps recurrence was 23.4 months. Among the factors studied in our series, only the observance of postoperative corticosteroids was retained as a predictor of smell recovery after surgery (p = 0,011). Conclusion: CRSWP surgery can significantly improve the smell sense, especially during the two first years. This effect can be sustainable if good post operative compliance for local corticosteroid are achieved.

Senos dérmicos cefálicos en pediatría: Presentación, diagnóstico, complicaciones y tratamiento / Cephalic dermal sinuses in pediatrics: Presentation, diagnosis, complications and treatment

Introducción: El seno dérmico cefálico es un tracto tubular, producido por una incompleta separación entre el ectodermo neural y el ectodermo epitelial que puede traer aparejado complicaciones infecciosas1.Hasta el momento, en la bibliografía internacional, existen casos reportados aislados de esta patología.Es por esto que realizamos un estudio descriptivo de una cohorte de 18 pacientes con seno dérmico cefálico que fueron operados en nuestra institución, con el objetivo de describir las formas de presentación, diagnóstico, tratamiento y complicaciones de esta patología poco frecuente.Metodología: Se realizó un estudio observacional descriptivo retrospectivo de una cohorte de pacientes pediátricos con seno dérmico cefálico.Resultados: Se analizaron 18 pacientes con seno dérmico cefálico. La clínica de presentación fue en 13 casos una lesión puntual en el cuero cabelludo, hipertensión endocraneana en 4 casos, ataxia en 1 caso y fístula en 1 caso. La mitad presentó síntomas de infección. Las localización fue en línea media sobre el hueso occipital en 13 casos, en línea media sobre el hueso frontal en 3 casos y en línea media interparietal en 2 casos.En 5 casos encontramos asociada una imagen extracraneana y en 11 casos una complicacion intracraneana. Ningún paciente presentó recidiva de la lesión y todos recibieron un solo procedimiento quirúrgico.Conclusiones: Los senos dérmicos pueden presentarse de diversas formas, desde un defecto puntual en un paciente asintomático, a una meningitis fulminante. El estudio de estas lesiones debe contar con una TC de cerebro y RM con DWI, si se sospecha una infección se debe solicitar contraste.El tratamiento es siempre la exéresis completa del seno dérmico y las lesiones asociadas. Ante el diagnóstico de una lesión asintomática se puede programar la cirugía, sin embargo sí encontramos senos dérmicos sintomáticos o asociadas a lesiones intracraneanas la cirugía debe realizarse sin demasiada demora.

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.

Malformations congénitales visibles à la maternité des Cliniques Universitaires de Kinshasa

Introduction: L'importance des malformations congénitales dans la morbidité et la mortalité périnatales est telle que, cette pathologie constitue un problème de santé publique. Cette étude vise à établir la cartographie des malformations congénitales visibles à la Maternité des Cliniques Universitaires de Kinshasa.Méthodologie:Etude transversaledescriptive, réalisée à la Maternité des Cliniques Universitaires de Kinshasa a été conduitede Janvier 2009 à Décembre 2011, à partir de 1513 dossiers des accouchées. Nous avons analysé les paramètres concernant les nouveau-nés portant des malformations congénitales visibles ainsi que leurs mères. Résultats: 5,1% des nouveau-nésavaient desmalformations congénitales visibles. Les malformations congénitales visibles étaient plus rencontrées chez les nouveau-nés des mères d'âges extrêmes. Le sexe masculin est plus représenté (60,8%). L'hydrocéphalie (24,1%), suivie de l'imperforation anale (17,7%), l'Omphalocele (15,2%) et le Spina bifida (8,9%) sont les malformations les plus rencontrées dans cette série. Conclusion: Cette étude montre que les malformations congénitales visiblessont un fléau à la Maternité des Cliniques Universitaires de Kinshasa et pousse donc àmener d'autres études pour en rechercher les causes

Prevalence of Spina Bifida Occulta and Its Relationship With Overactive Bladder in Middle-Aged and Elderly Chinese People / 대한배뇨장애요실금학회지

PURPOSE: To investigate the prevalence of spina bifida occulta (SBO) and its relationship with the presence of overactive bladder (OAB) in middle-aged and elderly people in China. METHODS: A cross-sectional community-based survey was carried out at 7 communities in Zhengzhou City, China from December 15, 2013 to June 10, 2014, where residents aged over 40 years were randomly selected to participate. All of the participants underwent lumbosacral radiographic analysis and relevant laboratory tests. A questionnaire including basic information, past medical history and present illness, and the OAB symptom score was filled out by all participants. Chi-square tests and logistic regression were used for data analysis with a P-value of 0.05). In men, age, SBO, and constipation were risk factors for OAB (P0.05). In women, age, SBO, and HCI were risk factors for OAB (P0.05). CONCLUSIONS: The prevalence of SBO is high and it is related to OAB in middle-aged and elderly people in China.

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.

Incidencia de espina bífida en una muestra de recién nacidos vivos / Incidence of spina bifida in a sample of live births

Se estudiaron 1505 recién nacidos vivos, tanto clínica como radiológicamente, para conocer la incidencia de Espina Bífida abiertas y cerradas. 416 de ellos presentó Espina bífida en uno o más niveles de la columna vertebral; 398 (26.45%) fueron cerrados y 18 (1.2%) abiertos; no hubo diferencia estadística significativa en cuanto al sexo. Fue más frecuente en el segmento S1, 229/1505 (15.22%). En el sexo masculino prevalecieron las Espinas bífidas a nivel lumbar y en el femenino a nivel sacro. Se ratifica la importancia clínica de las manifestaciones cutáneas(AU)

1505 new born were studied, both clinically and radiologically, to determine the incidence of open and closed spina bifida. 416 of these patients had spina bifida in one or more levels of the spine. 398 (26.45%) were closed and 18 (1.2%) open; there was no statistically significant difference in sex. Was more frequenting the segment S1, 229/1505 (15.22%). In males prevailed spina bifida in the lumbar segment, and sacral level in female sex. Its confirms the clinical relevance of cutaneous manifestations(AU)

Prevalence des anomalies transitionnelles lombosacrees dans une population de sujets camerounais souffrant de lombalgies chroniques

INTRODUCTION :Les vertebres transitionnelles lombosacrees (VTLS) et le spina bifida occulta (SBO) peuvent entrainer des lombalgies en particulier chez des sujets jeunes. Du fait de leur origine genetique; leur distribution pourrait varier en fonction des races ; pourtant leur prevalence demeure largement inconnue en Afrique. OBJECTIFS : Determiner la prevalence des VTLS et du SBO chez des noirs africains souffrant de lombalgie chronique. PATIENTS ET MeTHODES 575 radiographies du rachis lombaire (incidences de face et de profil) de patients noirs africains souffrant de lombalgie chronique ont ete revues a la recherche de VTLS de SBO. Les VTLS ont ete classees selon le systeme de Castellvi. Les proportions d'anomalies par sexe ont ete comparees par le test de Fisher. ReSULTATS L'age median des sujets etait de 44 ans [15 a 80 ans] et le sex ratio femme/homme de 1.03. La prevalence globale des VTLS et du SBO etait de 13;6 et 2;1 respectivement. Les VTLS etaient plus frequentes dans la population masculine avec une predominance du type IIa. La frequence du SBO n'etait pas influencee par le sexe. CONCLUSION :La prevalence de VTLS et de SBO chez le sujet noir africain souffrant de lombalgies est similaire a celles retrouvees dans les series caucasiennes et asiatiques

Sacral Cutaneous Clues to Underlying Spinal Abnormalities

PURPOSE: Sacral cutaneous lesions in newborns are associated with numerous spinal abnormalities. Early detection is important, because spinal abnormalities may cause neurological symptoms. Radiologic screening tests have been performed on newborns with sacral cutaneous lesions. This study aimed to substantiate the associations between sacral cutaneous lesions and spinal abnormalities. METHODS: From January 2007 until November 2013, we retrospectively reviewed the charts of 743 newborns with sacral cutaneous lesions that included sacral dimples, which were deeper than 5 mm and situated further than 2.5 cm from the anus, deviated gluteal furrow, hairy patch, hemangioma, dyspigmentaion, and the presence of mass, and skin tag. RESULTS: 743 newborns with sacral cutaneous lesions were examined, including 24 newborns with abnormal ultrasonographic images. Tethered cord which affected 18 (2.4%) of the newborns, was the most commonly found spinal abnormaility. Of these 9 newborns had other spinal abnormalities in addition to tethered cord including lipoma, cyst, spina bifida occulta, lipomyelomeningocele, and dermal sinus tract, and 9 newborns had isolated tethered cord only. Other spinal abnormalities found included isolated lipoma (3 newborns, 0.4%), and subarachnoid cyst (2 newborns, 0.3%), and of the 2 newborns (0.3%) who had dermal sinus tract, 1 also had a lipoma and the other also had a tethered cord. Normal variants included coccygeal pit (43 newborns, 5.8%), and ventriculus terminalis (10 newborns, 1.4%). Of the 646 newborns with isolated sacral cutaneous lesion, 11 (1.7%) had abnormal ultrasonographic images, and of the 97 newborns with combined sacral cutaneous lesions, 13 (13.4%) had abnormal ultrasonograpic images. CONCLUSION: Sacral cutaneous lesions in newborns can be associated with spinal abnormalities, and the strongest marker of spinal abnormality is a combined lesion. Therefore, ultrasonography should be performed on newborns who present with sacral cutaneous lesions to detect and investigate any underlying spinal abnormalities.

Marcadores cutáneos de disrafia espinal oculta: reporte de 5 casos / Skin markers of occult spinal dysraphism: report of 5 cases

Las disrafias espinales ocultas son patologías caracterizadas por la fusión incompleta del tubo neural, en las que la lesión se encuentra cubierta por piel y no hay exposición del tejido nervioso. El diagnóstico precoz es de gran importancia, ya que la corrección quirúrgica oportuna puede prevenir daño neurológico irreversible. Existen alteraciones cutáneas que se asocian a la presencia de disrafias espinales ocultas. Debido a que en muchos casos estos marcadores cutáneos son la única manifestación inicial de estas alteraciones, es fundamental que el pediatra esté familiarizado con ellos, para poder realizar un estudio diagnóstico y terapéutico adecuado. Dentro de los marcadores cutáneos descritos en la literatura, una combinación de dos o más alteraciones cutáneas congénitas de la línea media constituye probablemente el predictor más importante de disrafia espinal oculta. Lesiones como el lipoma de la línea media y la cola de fauno son los marcadores aislados de mayor riesgo. Actualmente las disrafias espinales ocultas cuentan con cobertura GES, por lo que todo paciente en el que se sospeche el diagnóstico, debe ser derivado a neurocirujano, quien deberá evaluarlo en un plazo máximo de tres meses.

Occult spinal dysraphism are diseases characterized by the incomplete fusion of the neural tube, where the lesion is covered by skin and there is no exposure of nerve tissue. Early diagnosis is very important, because prompt surgical correction can prevent irreversible neurological damage. There are usually skin changes associated with the presence of occult spinal dysraphism. Because in many cases these skin lesions are the only initial manifestation of the disease, it’s essential for the pediatrician to know them well, for achieving a timely and appropriate diagnosis. Among the skin markers described in the literature, a combination of two or more skin lesions of the midline is probably the best predictor of occult spinal dysraphism. Lesions such as lipoma of the midline and faun tail have the highest risk for these disorders. Occult spinal dysraphism have GES financing, so every patient in whom the diagnosis is suspected should be referred to a neurosurgeon, who will perform an evaluation within three months.

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.

Rehabilitación de un paciente operado de síndrome de medula anclada / Rehabilitation of an operated on tethered spinal cord patient

El concepto de médula anclada se refiere a una posición anormalmente baja de la médula espinal, que puede acompañar a varios tipos de disrafia espinal y clínicamente se caracteriza por afectación de los sistemas músculo esquelético, cutáneo, neurológico y urológico. Nos propusimos mostrar que la aplicación del Programa de Neurorrehabilitación Multifactorial Intensiva de la Clínica de Lesiones Raquimedulares y Enfermedades Neuromusculares del CIREN, puede mejorar la calidad de vida de los pacientes portadores de trastornos neurológicos operados de médula anclada. Los resultados demostraron incremento de la fuerza muscular y en los índices de marcha. Concluyendo que el programaaplicado puede contribuir a mejorar la fuerza, las capacidades físicas y motoras en pacientes con estas secuelas neurológicas

Estudo retrospectivo de casos de disrafismo espinhal no Hospital de Clínicas da Universidade Federal de Uberlândia (HCUFU), no período 1992-2002 / Retrospective study of spine dysraphism at the clinical hospital of the Federal University of Uberlândia, from 1992 to 2002

Objetivou-se avaliar as características clínicas, formas de tratamento e evolução dos pacientesportadores de disrafismo espinhal, atendidos no Hospital de Clínicas da Universidade Federal de Uberlândia (HC-UFU) em um intervalo de onze anos (janeiro de 1992 a dezembro de 2002). Realizou-se um estudo descritivo, identificando os casos analisados conforme sua apresentação clinica (espinha bífida oculta, meningocele e mielomeningocele). As seguintes variáveis foram analisadas: gênero, raça, idade materna, peso de nascimento, época do diagnostico, presença de malformações associadas, tratamento cirúrgico, ocorrência e causa do óbito. Dentre os pacientes analisados, 50,9% eram do sexo feminino e 73,1% da etnia branca. Em relação a idade materna, a maioria (68,5%) encontrava-se entre 20 e 29anos. A media dos pesos dos pacientes ao nascimento foi de 2.938 gramas. Em 81,5% dos casos, o diagnostico dodisrafismo foi feito ao nascimento. Considerando a apresentação clinica, observou-se a ocorrência de mielomeningocele em 94,4% dos casos, de espinha bífida oculta em 3,8% e de meningocele em 2,8%. A localização do disrafismo preponderante foi a lombossacral, correspondendo a 84% dos casos. Dentre as lesões associadas, observou-se maior freqüência de hidrocefalia, Síndrome de Arnold Chiari tipo II e estrabismo. A cirurgia foi realizada em 94,4% dos pacientes. Houve óbito em 9,26% dos casos. O índice de diagnostico intra-utero dos disrafismos espinhais foi baixo,refletindo, possivelmente, a deficiente disponibilidade de equipamentos apropriados e/ou inexperiência dos profissionais nos serviços públicos. Os demais achados foram semelhantes aos da literatura.

The objective was to evaluate the clinical aspects, treatment and evolution of the carriers of spinedysraphism. The charts of the carriers of spine dysraphism (occult spina bifida, meningocele and myelomeningocele),admitted in the hospital at the period were analysed and the following were studied: gender, race, age of the mother,weight at birth, age of diagnosis, associated malformations, surgical treatment, death. 50,9% of the patients were female and 73,1% white; 68,5% of the mother were between 20 and 29 years old. The average weight of the neonates were 2938 g. At 81,5% the diagnosis of dysraphism were made at birth. Myelomeningolcle were present in 94,4% of the patients, occult spina bifida in 3,8% and meningocele in 2,8%. At lombosacral area were found 84% of the malfomation. Amongthe associated lesions were found the Hydrocefalia-, Arnold Chiari II and strabism. Surgical treatment were realized in 94,4% of the children and occurred 9,26% of death. The rate of intra-uterine diagnosis were very low expressing, probably, the deficiency of equipment and specialized assistance at the public health system.

Evaluación de la frecuencia de espina bífida oculta y su posible relación con el arsénico ambiental en una muestra prehispánica de la Quebrada de Camarones, norte de Chile / Spina bifida occulta associated with environmental arsenic exposure in a prehispanic sample from northern Chile

Background: The Camarones River Valley, located in the extreme north of Chile, is characterized by high environmental arsenic levels and an arid desert. It has been inhabited by humans for the past 7,000 years. Evidence exists for chronic arsenic poisoning in both prehispanic and present populations residing in the area. Chronic arsenic exposure causes multi-systemic problems and can induce congenital malformations, in particular neural tube development defects such as spina bifda. Aim: To study the prevalence of spina bifda among prehispanic mummies of the area. Material and Methods: Onehundred and twenty prehistoric adult individuals were analyzed for evidence of spina bifda occulta of the sacrum in skeletal samples from the sites of Camarones 8, Camarones 9, Azapa 140 and Lluta 54, held in repository at the Museo Universidad de Tarapacá de Arica- San Miguel de Azapa. A diagnosis was considered positive when at least S1, S2 or S3 were affected. As controls, mummies of individuals that resided in Lluta and Azapa valley, with a low arsenic exposure, were analyzed. Results: The frequency of spina bifda occulta among samples from the Camarones coast and Lluta and Azapa Valley were 13.5 and 2.4 percent respectively. Conclusions: Considering these were contemporaneous samples, and are believed to have had no other differences in diet or other factors, the differential exposures to arsenic could have produced the observed differences in spina bifda frequencies.

Cisto dermóide com sinus dérmico da região occipital: relato de caso e revisão de literatura / Occipital dermoid cyst with dermal sinus: report of a case and literature review

An 18 months-old baby presented with a midline occipitaldimple with thickening of scalp since birth. Skull X-rays showed an oval bony defect in the mid occipital region. CT and MRI shown extension of the lesion to the posterior fossasubarachnoid space. The lesion was a congenital dermoid cyst, completely removed by surgery with good outcome1,2,3,6.

A Case of Intracranial Dermoid Cyst Associated with Scalp Abscess

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.