Decompressive craniectomy for severe traumatic brain injury in children: analysis of long-term neuropsychological impairment and review of the literature.

OBJECTIVE: The effectiveness of decompressive craniectomy (DC) in the context of neurocritical care in adult patients has been recently under debate. The aim of our study was to evaluate the impact of decompressive craniectomy in severe traumatic brain injury (TBI) in children, focusing on short and long-term neurological and neuropsychological outcomes. METHODS: Retrospective review of the medical records of children admitted at a level I trauma center, between January 2012 and December 2015, submitted to DC due to severe TBI. Additionally, an extensive review of literature on this subject was carried out. RESULTS: Sixteen patients underwent DC for TBI at our institution during the evaluated period. 62.5% were males and the mean age was 12 years. Road traffic accident (RTA) was the main mechanism of trauma (62.5%). Average Glasgow Coma Scale (GCS) at admission was 5.2, whereas 75% of the patients presented with pathological pupillary reaction. Initial computed tomography (CT) showed skull fractures in 62.5% and acute subdural hemorrhage (ASH) in 56.3% of the patients. The mean intracranial pressure (ICP) was 27.2 mmHg prior to surgery, and the mean time window between admission and DC was 36.3 h. Unilateral DC was performed in 68.8% of the cases. The average Glasgow Outcome Scale (GOS) at 6-month follow-up was 3.7, whereas 70% of the survivors presented good recovery (GOS 4-5). Abnormal pupillary reaction at hospital admission increased 3-fold the risk of long-term neuropsychological disturbances. Follow-up evaluation revealed cognitive abnormality in 55.6% of the patients. The overall mortality at 6-month follow-up was 37.5%. CONCLUSION: The present study indicates towards a potential benefit of DC in children with severe TBI; nevertheless, our data demonstrated a high incidence of neuropsychological impairment in the long-term follow-up. Psychological and cognitive assessment should be computed in prognosis evaluation in future prospective studies.

Cranial morcellation decompression for refractory idiopathic intracranial hypertension in children.

BACKGROUND: Primary idiopathic intracranial hypertension (PIIH) in children is rare and has a poorly understood pathophysiology. It is characterized by raised intracranial pressure (ICP) in the absence of an identified brain lesion. Diagnosis is usually confirmed by the measurement of a high cerebrospinal fluid (CSF) opening pressure and exclusion of secondary causes of intracranial hypertension. Refractory PIIH may lead to severe visual impairment. The purpose of this study was to evaluate a cranial morcellation decompression (CMD) technique as a new surgical alternative to stabilize intracranial pressure in PIIH. MATERIALS AND METHODS: A literature review was carried out, disclosing only 7 pediatric cases of PIIH treated with surgical skull expansion. In addition, we describe here one case of our own experience treated by CMD. CONCLUSIONS: CMD surgery is a safe and effective option to control refractory PIIH in selected patients.

The supraorbital eyebrow approach for removal of craniopharyngioma in children: a case series.

OBJECTIVE: Craniopharyngiomas can be a surgical challenge for the pediatric neurosurgeon. Ideally, total removal must be achieved. However, the need to reduce surgical morbidity and preserve quality of life has led to a number of neurosurgical approaches in order to attain this goal. The aim of this article is to present an alternative surgical approach to these lesions and to provide the rationale for this technique. MATERIAL AND METHODS: Medical charts and operative records of eight pediatric patients harboring craniopharyngiomas who underwent surgical treatment using a supraorbital eyebrow approach (SOA) were reviewed from 2014 to 2016. Only patients younger than 18 years with a minimum follow-up of 12 months were included in this study. Using pre-operative magnetic resonance (MRI) scans, tumors were classified according to their degree of hypothalamic involvement. The surgical technique is also described in detail. RESULTS: The study group included six males and two females with a mean age of 10 years (range, 2-16 years). The SOA was used successfully in elective surgery of eight craniopharyngiomas. The hypothalamus was displaced by the tumor in three patients and severely involved in five patients. Subtotal resection was undertaken in six patients, whereas gross-total resection was achieved in two. Endoscopic assistance was used after standard microscopic visualization in two out of eight cases. Cosmetic outcomes were excellent, and the complication rate related to the surgical procedure was quite low, apart from diabetes insipidus (which occurred in three out of the eight patients). In one patient, a large subdural collection needed surgery for evacuation. Mean follow-up was 23.2 months (range, 12-36 months). Additionally, no CSF leak or wound infection was identified. CONCLUSIONS: The supraorbital eyebrow approach is an alternative route to operate on craniopharyngiomas in properly selected cases of all pediatric age ranges, from infants to teenagers. There is sufficient working space for the endoscope and all instruments, allowing for endoscopic assistance and bimanual surgical technique. Cosmetic results are excellent, and complications related to the approach are minimal.

Myasthenia gravis and thymus: long-term follow-up screening of thymectomized and non-thymectomized patients.

Thymoma screening is recommended at the onset of myasthenia gravis (MG) or when patients with MG present with clinical deterioration or a progressive increase of anti-acetylcholine receptor antibody. However, it is unknown if it is necessary to repeat the screening of thymoma at fixed intervals, even in the absence of MG deterioration, when the initial screening is negative. We analyzed the recurrence rate and incidence of new thymoma in a series of patients with well-controlled MG. The sample consisted of 53 patients, aged 17 to 72 years, and the follow-up varied between 75 and 472 months. The chest computerized tomography detected thymus abnormalities in eight patients at the initial screening and no abnormalities in all patients at a second screening after five years. The findings of this study support the classical opinion that screening for thymoma should be recommended only if there is clinical deterioration due to the disease.

Myasthenia gravis and thymus: long-term follow-up screening of thymectomized and non-thymectomized patients / Miastenia gravis e timo: investigacao no seguimento de longo prazo em pacientes timectomizados e nao timectomizados

Thymoma screening is recommended at the onset of myasthenia gravis (MG) or when patients with MG present with clinical deterioration or a progressive increase of anti-acetylcholine receptor antibody. However, it is unknown if it is necessary to repeat the screening of thymoma at fixed intervals, even in the absence of MG deterioration, when the initial screening is negative. We analyzed the recurrence rate and incidence of new thymoma in a series of patients with well-controlled MG. The sample consisted of 53 patients, aged 17 to 72 years, and the follow-up varied between 75 and 472 months. The chest computerized tomography detected thymus abnormalities in eight patients at the initial screening and no abnormalities in all patients at a second screening after five years. The findings of this study support the classical opinion that screening for thymoma should be recommended only if there is clinical deterioration due to the disease.

A investigação de timoma é recomendada em pacientes com miastenia gravis (MG) no início da doença, em caso de haver piora clínica ou aumento dos níveis do anticorpo antirreceptor de acetilcolina. Contudo, não foi estabelecido se é necessário repetir a investigação de timoma em intervalos fixos, na ausência de piora clínica, quando a investigação inicial foi negativa. A taxa de recorrência e a incidência de novo timoma foram analisadas em uma série de pacientes com MG bem controlada. A amostra consiste de 53 pacientes, idade entre 17 e 72 anos, com tempo de acompanhamento variando entre 75 e 472 meses. A primeira tomografia computadorizada de tórax detectou anormalidades no timo em oito pacientes durante a investigação inicial da doença e nenhuma anormalidade no segundo exame, após cinco anos de doença, em todos os pacientes. Os achados desse estudo corroboram a clássica opinião de que a investigação de timoma deveria ser recomendada somente se houver piora clínica da doença.

Acrania e outras falhas na formação dos ossos do crânio: uma revisão da literatura / Acrania and other failures in the formation of the skull bones: a literature review

A acrania é uma malformação congênita rara que cursa com ausência parcial ou total do crânio de fetos humanos. Está associada frequentemente com anencefalia e é, via de regra, fatal em curtíssimo prazo. Existem alguns marcadores pré-natais, sendo a ultrassonografia o exame diagnóstico padrão-ouro. Os autores revisam aspectos relacionados a epidemiologia, fisiopatologia, diagnóstico ultrassonográfico, diagnóstico diferencial entre malformações cefálicas e manejo obstétrico da doença.

Acrania is a rare congenital malformation with partial ortotal absence of skull of human fetuses. It is often associatedwith anencephaly and is usually fatal in very short time.There are some prenatal markers and the ultrasound is thegold-standard diagnostic method. The authors review aspectsof the epidemiology, pathophysiology, ultrasound diagnosis,differential diagnosis of cephalic malformations and obstetricmanagement of the disease.