Spontaneous acute subdural hematoma contralateral to an arachnoid cyst.

Arachnoid cysts (AC) are extra-cerebral cerebrospinal fluid collections of unknown origin. They correspond to 1% of all intracranial nontraumatic space-occupying lesions and appear more frequently in the middle fossa (50%). More than 25% of these cysts are incidental findings and the majority of patients are asymptomatic. Seizures, intracranial hypertension signs, neurological deficits, macrocrania, developmental delay and bulging of the skull are the main signs and symptoms of the lesion. AC rupture and bleeding are rare, usually occurring in young adults and associated with trauma. The risk of hemorrhage does not exceed 0.04% / year. We describe the case of a ten-year-old boy who presented with acute signs of intracranial hypertension secondary to a spontaneous acute subdural hematoma, contralateral to an AC of the middle fossa. Three factors were significant in this case: signs and symptoms occurred spontaneously; the presence of an acute subdural hematoma exclusively contralateral to the AC; successful outcome of the conservative treatment.

Spontaneous acute subdural hematoma contralateral to an arachnoid cyst

Arachnoid cysts (AC) are extra-cerebral cerebrospinal fluid collections of unknown origin. They correspond to 1 percent of all intracranial nontraumatic space-occupying lesions and appear more frequently in the middle fossa (50 percent). More than 25 percent of these cysts are incidental findings and the majority of patients are asymptomatic. Seizures, intracranial hypertension signs, neurological deficits, macrocrania, developmental delay and bulging of the skull are the main signs and symptoms of the lesion. AC rupture and bleeding are rare, usually occurring in young adults and associated with trauma. The risk of hemorrhage does not exceed 0.04 percent / year. We describe the case of a ten-year-old boy who presented with acute signs of intracranial hypertension secondary to a spontaneous acute subdural hematoma, contralateral to an AC of the middle fossa. Three factors were significant in this case: signs and symptoms occurred spontaneously; the presence of an acute subdural hematoma exclusively contralateral to the AC; successful outcome of the conservative treatment.

Os cistos aracnóideos (CA) são coleções liquóricas extra-cerebrais e intra-aracnóideas de origem desconhecida. Correspondem a 1 por cento de todas as lesões expansivas intracranianas não traumáticas e têm nítido predomínio na fossa média (50 por cento). Até 25 por cento destes cistos são achados incidentais sendo que a maioria dos pacientes é assintomática. Crises epilépticas, sinais de hipertensão intracraniana, déficits neurológicos focais, macrocrania, atraso no desenvolvimento e abaulamento da calota craniana são os principais sinais e sintomas da lesão. A ruptura dos CA, assim como seu sangramento, são situações raras, geralmente associadas a traumas e acometem adultos jovens. O risco de hemorragia em pacientes com CA não excede 0,04 por cento ao ano. É descrito caso de paciente de dez anos de idade que subitamente apresentou sinais de hipertensão intracraniana secundários a hematoma subdural agudo espontâneo, contralateral a volumoso CA de fossa média. Três fatores foram de relevância neste paciente: a ocorrência de sinais e sintomas espontaneamente, especialmente em um menino; presença de hematoma subdural agudo exclusivamente contralateral ao CA; e a boa evolução com o tratamento conservador.

Bobble-head doll syndrome associated with Dandy-Walker syndrome. Case report.

Bobble-head doll syndrome (BHDS) presents in childhood and is usually associated with lesions of the third ventricle. This disorder is characterized by stereotypical head movements of the type "yes-yes" (up and down) at a frequency of 2 to 3 Hz. Rarely, movements of the type "no-no" (side-to-side) are described. There are a few hypotheses to explain the mechanism responsible for BHDS, but its real pathophysiological characteristics are still unknown. The authors describe the case of a child born with hydrocephalus and Dandy-Walker syndrome. A ventriculoperitoneal shunt was implanted in the child because of progressive head enlargement. One year after shunt placement, she began making frequent horizontal head movements of the type "no-no". There were no other signs or symptoms. Imaging studies demonstrated small ventricles and a posterior fossa cyst with no signs of hypertension. The child's growth, development, and head circumference (within the 5th percentile) remained satisfactory. Three aspects of this case were of interest: the association of BHDS with Dandy-Walker syndrome, the rare occurrence of BHDS of the "no-no" type, and the absence of third ventricle dilation. The authors' findings support the hypothesis that cerebellar malformations themselves can

Intraoperative acute tissue expansion for the closure of large myelomeningoceles.

OBJECT: Skin closure has always been the main challenge in treating myelomeningoceles (MMCs). Most cutaneous defects can be treated with the simple undermining and primary suture of the wound edges. This is the ideal treatment, but it is not adequate in cases in which the lesions are greater than 5 cm in diameter. Numerous reconstructive procedures have been described and the results have been satisfactory. The need to mobilize large skin areas and the fact of excessive blood loss, however, are major problems in newborns. Moreover, the tissue undermining destroys most of the skin's vascularization and can harm the adaptation of the skin flaps. The authors describe a technique for primary closure of large MMC skin defects in which they use acute skin expansion during the surgical procedure. METHODS: Skin expansion was achieved by traction of the wound edges with U-shaped sutures and without the need of skin flaps. Sixteen patients with MMCs greater than 5 cm in diameter were evaluated. RESULTS: The MMC areas ranged from 30 to 64 cm2 (mean 45 cm2). Two suture systems were developed based on the quality of the skin edge and the size of the skin defect. Wound edge traction was performed in 10-minute periods. The edges were gradually approximated and this allowed the primary closure of the wound without undermining the tissue in all patients. In one patient skin necrosis developed, which was associated with compression of the malformed underlying vertebrae. CONCLUSIONS: Simplicity, low cost, and satisfactory results were the main advantages of the method and an increase in operative time was a disadvantage. The goal of this technique is not to replace the other methods, but the technique constitutes an effective option in the treatment of large MMCs.

Minor skin lesions as markers of occult spinal dysraphisms--prospective study.

BACKGROUND: Neonates with occult spinal dysraphisms (OSDs) may not present any clinical manifestations, but may be associated to cutaneous stigmas that indicate dysraphism. Association of minor cutaneous stigmas (discreet skin lesions, most of which are not assessed) with OSD was investigated in this study, as well as the use of ultrasonography (US) as a screening method for those patients. METHODS: Two thousand ten neonates were evaluated trough active search. Both the pediatrician and the neurosurgeon performed the search for the presence of cutaneous stigmas on the midline of the dorsal region. For all of them, the gestational age, type of delivery, gender, ethnicity, age of mother, and whether it was a high-risk gestation were recorded. Patients with skin lesions comprised the case group. For each case, another neonate of the same gender, gestational age, and ethnicity was selected as control. Both groups were evaluated with respect to personal, social, and medical information. Spinal US was performed in all case and control patients-if altered or inconclusive, it was complemented with magnetic resonance imaging (MRI). RESULTS: Of the 2010 patients, 144 presented cutaneous stigmas. Of these, 8 had alterations to US (5.5%) and 6 to MRI. There were no alterations to US in the control group. The most frequent lesions were tufts of hair and dimples; through US, the most frequent findings were dermal sinuses. CONCLUSIONS: The so-called minor skin lesions were not markers of OSD in the evaluated population. However, in 4 patients, US was decisive for the surgical decision. From the statistical point of view, there is no indication for complementary examinations in patients with minor cutaneous stigmas. However, because of the feasibility, simplicity, and low cost of the spinal US, the examination is justified in the benefits of early diagnosis, regardless of the need of immediate surgical treatment.

[Large scalp and skull defect in patient with aplasia cutis congenita]. / Extensa falha cutânea e craniana em paciente com aplasia cutis congenita.

Aplasia cutis congenita is a rare condition characterized by the absence of skin layers. It is most common on the scalp, middle line, and it can be seen as a congenital ulcer involving periosteum, skull and dura. We present the case of a female newborn infant with a dysmorphic facies, a large scalp and skull defect exposing the dura. There was no cerebrospinal fluid leakage. The rarity of cases with large defects and small series reported make difficult to determinate the ideal treatment for aplasia cutis congenita. More studies are necessary to define the etiology and best management of this patients.

Extensa falha cutânea e craniana em paciente com aplasia cutis congenita / Large scalp and skull defect in patient with aplasia cutis congenita

Aplasia cutis congenita (ACC) é doença rara, caracterizada pela ausência de formação completa da pele. Geralmente ocorre no couro cabeludo, na linha mediana, e apresenta-se ao nascimento como uma ferida ulcerada que pode atingir diferentes profundidades e envolver o periósteo, crânio e dura-máter. Descrevemos o caso de menina recem-nascida que apresentava fácies dismórfica com defeito no couro cabeludo que abrangia a quase totalidade da abóbada craniana e apresentava falha óssea desde a base dos ossos frontais até os occipitais e mastóides. A dura-máter estava em sua maior parte exposta, sem sinais de fissuras. Devido à raridade da ACC e ao pequeno número de pacientes nas séries publicadas na literatura a padronização do tratamento ainda é incipiente. O que existem são recomendações. Ainda são necessários estudos que abordem desde a etiologia da doença até a avaliação dos métodos de tratamento e evolução de grupos maiores de pacientes.

Uso da ultra-sonografia na triagem de disrafismos espinhais ocultos / Screening of occult spinal dysraphism by ultrasonography

O uso da ultra-sonografia para diagnóstico de lesões da coluna vertebral é difundido desde a década de oitenta. Suas aplicações envolvem: diagnóstico de lesões medulares traumáticas; uso intra-operatório em cirurgias do trauma e tumores medulares; uso pré-operatório e evolutivo em pacientes com disrafismos espinhais; diagnóstico pré-natal dos disrafismos; diagnóstico dos disrafismos espinhais ocultos ao nascimento. Pacientes com suspeita de disrafismo espinhal oculto (estigmas cutâneos em linha média ou história familiar de disrafismos) representaram a principal indicação do exame que foi realizado em 292 pacientes do Hospital das Clínicas da Universidade Federal de Minas Gerais. Alterações como lipomas do cone medular, medula baixa, filo terminal espesso, cistos da ponta do cone medular e seios dérmicos foram identificados com fidelidade à ultra-sonografia. A interpretação do exame não apresentou dificuldades técnicas. Sua realização é rápida, não é necessária sedação, apresenta baixo custo e idealmente deve ser realizado pelo próprio neurocirurgião devido ao grande número de informações possíveis durante o exame. O exame entretanto não substitui a ressonância magnética mas pode ser um bom método de triagem para os pacientes.

[Screening of occult spinal dysraphism by ultrasonography]. / Uso da ultra-sonografia na triagem de disrafismos espinhais ocultos.

Ultrasonography for diagnosis of spinal cord diseases has been used since the eighties. There are different fields for its use: traumatic spine lesions, intra-operative use in trauma and spine tumors surgery, pre-operative and follow up study for spinal dysraphism, occult spinal dysraphism of the newborn. Patients with suspected occult spinal dysraphism are the most important indication for the method. Ultrasonography was performed in 292 patients of Hospital das Clínicas, Universidade Federal de Minas Gerais. Lesions such as conus medullaris lipomas, low level of conus medullaris, filum terminale tethering, cauda equina cysts and dermal sinuses can be well demonstrated. There is no difficulty in interpreting the image. The exam does not need sedation; it is safe, fast and cheap. Ideally it should be performed by the neurosurgeon as it may provide many information during the examination. The ultrasonography does not replace magnetic resonance imaging but it is an excellent method for screening.