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.

Tratamento cirúrgico de meningomielocele no período neonatal / Urgical trearment of meningomyelocele defects in neonatal period

As meningomieloceles são defeitos congênitos de fechamento do canal medular, com gravidade variável. Os defeitos são encontrados principalmente na região lombossacra e há hidrocefalia em 80-90% dos casos. O objetivo deste trabalho foi estudar uma série de pacientes operados por meningomielocele no período neonatal, no período de janeiro de 2001 a janeiro de 2003. Foram incluídos 22 pacientes no estudo, sendo 12 (54,5%) masculinos e 19 (86%) de etnia caucasiana. A maioria dos pacientes eram a termo (37,5±1,3 semanas) e com peso adequado para idade gestacional (2960,5±609,6 gramas). Foi realizada cesárea em 16 casos (72,7%). O fechamento foi executado da seguinte forma: primário em 5 casos (23%); "skin-over-skin" em 6 casos (27%); bipediculado fasciocutâneo bilateral em 5 casos (23%); bipediculado fasciocutâneo unilateral em 1 caso (5%); fasciocutâneo com pedículo superior em 2 casos (9%); bilobado fasciocutâneo em 1 caso (5%); fasciocutâneo em S em 2 casos (9%). Houve DVP em 18 casos (81,8%). As complicações ocorridas foram: deiscência de sutura (23%); necrose parcial (18%) e fístulas (14%). A técnica "skin-over-skin" e os retalhos fasciocutâneos são alternativas efetivas para o fechamento de meningomieloceles no período neonatal.

Purpose: To study the incidence surgical treatment oflargeth or acolumbar meningomyeloceles duringathree- year period in a Brazilian referral center. Patients and methods: We prospectively evaluated all patients submitted to surgical management of meningomyelocele by both the plastic surgery and neurosurgery teams of Hospital de Clínicas de Porto Alegre between September 2001 and August 2003. Results: Twenty four patients were included in this study, being 13 (54.2%) males and 21 (87.5%) of Caucasian ethnicity. Most patients were born at term (37,5±1,3 weeks) and with weight adjusted for gestacional age (2960,5±609,6 gram). The closing was executed of the following form: direct skin approximation in 5 cases (23%); "skin-over-skin" in 6 cases (27%); bilateral bipedicled fasciocutaneous flaps in 5 cases (23%);unilateral bipedicled fasciocutaneous flap sin 1 case (5%); superior pedicled asciocutaneous in 2 cases (9%); bilobed fasciocutaneous flap in 1 case (5%); bilateral fasciocutaneous flaps (S flap) in 2 cases (9%). V-P shunt was placed in 18 cases (81.8%). Suture dehiscence (23%); partial necrosis (18%) and fistulas (14%) were the main complications. Conclusion: Skin-over-skin and fasciocutaneous flaps are good alternative for reconstruction of meningomielocele sin the neonatalperiod.

Surgical treatment of occult spinal dysraphism with tethered spinal cord

Occult spinal dysraphism [OSD] consists of a subgroup of spinal dysraphism in which the congenital abnormality of the spinal cord is covered by intact skin and is associated with dermatologic, skeletal, urogenital and/or neurological abnormalities. This study was undertaken to evaluate the early prophylactic effect of surgery on OSD on subsequent expression of neurologic and musculoskeletal abnormalities. Thirty six patients with OSD with tethered cord who underwent surgery for treatment from October 2001 to October 2004 in Department of Neurosurgery of Shiraz University of Medical Sciences affiliated hospitals were evaluated 6 and 12 months postoperatively including a complete history and physical examination with special attention on spinal cord and lower limb function, bladder muscular force, and urodynamic studies. The most frequent dermatologic expression of OSD included hairy patches, subcutaneous lipoma, skin dimple and haemangioma. Scoliosis was observed in 13 cases, among them 7 cases had split cord syndrome, 2 cases had hypertrophic filum terminal [HFT] and 4 cases had both of the conditions. Among those with split cord syndrome, 78.6%, 14.3% and 7.6% were bony, fibrocartilagenous and fibrotic respectively. All patients improved significantly after the procedure. It appears that prophylactic surgical intervention in cases of OSD is a safe and recommended procedure prior to the onset of neurologic deterioration

Quiste neuroentérico: diagnóstico y tratamiento / Neuroenteric cyst: diagnosis and treatment

Se presenta un caso de diagnóstico prenatal de quiste neuroentérico. Caso clínico: paciente de 27 años embarazada de 38 semanas, la ecografía muestra masa quística intratorácica de paredes lisas y un tabique fino. Con diagnóstico probable de hernia diafragmática se resuelve el parto. En el estudio postnatal la TAC encuentra hemivértebras y se somete a cirugía que resulta exitosa, la biopsia confirma quiste neuroentérico

Quistes ependimarios coexistentes con espina bífida oculta y escoliosis congénita. Informe de un caso / Ependimal cysts coexisting with congenital scoliosis and spina bifida oculta

Se presenta el caso de una niña de 5 años de edad en quien se encontraron 2 zonas de ectasia ependimaria en los niveles T1 y T4 mediante RMN cuando se intentaba investigar la profundidad del defecto congénito espinal que producía escoliosis congénita lumbar izquierda de L2 a L4 y amplia espina bífida oculta asintomática de L5 y de todo el sacro. Debe considerarse que difícilmente se podría diagnosticar los defectos ependimarios sin resonancia magnética o tomografía computada

Cisto enterogênico intra-espinhal: relato de caso / Intraspinal enterogenous cyst: report of case

Os autores apresentam um caso de Cisto Enterogênico intradural cervical operado, com ótimo resultado. Discutem a embriogénese, quadro clínico, diagnóstico neurorradiológico e terapêutica desta patologia a partir de revisäo da literatura

Compressão medular alta por cisto neuroentérico / High medular compression caused by neurenteric cyst

The authors report the case of a child with a neuroenteric cyst compressing the high cervical spinal cord at the level of C1 to C3. The tumor was completely excised through a laminotomy with good results. The literature is revised, and the embryology, symptomatology, treatment and results are discussed.

Diastematomielia: análisis de 14 casos / Diastematomyelia: analysis of 14 cases

Se presentan catorce pacientes operados de diastematomielia. Se indican los signos y síntomas cutáneos, neurológicos y radiológicos y se describe la técnica quirúrgica empleada. En 10 casos el espolón era de naturaleza ósea y en cuatro cartilaginosa. En nueve pacientes la lesión se ubicaba en la columna lumbar y en cinco en la columna dorsal. Los pacientes con déficit neurológico progresivo estabilizaron su cuadro clínico después de la cirugía. El resultado fue excelente en todos ellos. Se recomienda el tratamiento quirúrgico en todos los pacientes con diastematomielia

Observations on spinal dysraphism.

An attempt has been made to look into the age at presentation, incidence and clinical presentation of 40 patients with spinal dysraphism. Thirteen cases of myelomeningocele and 11 cases of meningocele constituted the 'aperta' group and there were 16 'occulta' cases. Though among the 'aperta' cases male is to female ratio was 1:1, a marked male preponderance of 2.2:1 was seen among 'occulta' cases. First born child was found to be most frequently (44.50%) affected in the present series. Myelography remained a mainstay in deciding operation in 'occulta' cases. All 'aperta' cases and 11 out of 16 'occulta' cases were treated by operation. Regarding results of treatment, all cases with meningocele were fully cured, cases with myelomeningocele showed no improvement but further deterioration of the neurological status could be arrested. Hydrocephalus was associated in 8 out of 13 cases with myelomeningocele and ventriculoperitoneal (VP) shunt were instituted in all of them. Prognostic significance of presence of hydrocephalus was profoundly grave as was evident from the mortality rate (10%) in this series.