High risk of cerebrospinal fluid leakage in surgery of a rare primary intraosseous cavernous hemangioma of the clivus showing meningeal infiltration: A case report and review of the literature.

BACKGROUND: Primary intraosseous cavernous hemangiomas (PICH) of the skull represent an infrequent bone tumor. Although some rare cases of PICHs located in the skull base have been published, to our concern only three cases have been reported in the English literature of PICHs arising within the clivus. CASE DESCRIPTION: We present the case of a patient presenting an isolated abducens paresis due to a rare PICH of the clivus showing also an unusual destruction of the inner table as well as infiltration of the dura mater. Due to this uncommon infiltrative pattern of an otherwise expected intraosseous tumor, a cerebrospinal fluid (CSF)-fistula occurred while performing a transnasal biopsy. The patient recovered successfully without need of lumbar drainage or re-surgery. Additionally, intratumoral decompression was sufficient to relief the abducens paresis. CONCLUSIONS: Our case provides new and meaningful information about clinical features as well as growth pattern of these rare clival tumors. We also discuss the importance of knowing these peculiarities before surgery in order to plan the optimal operative management as well as to avoid complications while approaching PICHs localized in such a delicate cranial region.

Revascularisation surgery and long-term follow-up in juvenile Moyamoya syndrome: a retrospective analysis.

Due to its low incidence in Western countries, physician awareness of juvenile Moyamoya disease should be improved. The benefits of revascularisation surgery have only been proven in the juvenile version of the disease. Therefore, early revascularisation may prevent irreversible ischaemic deficits and rapidly progressive mental retardation in young patients.From 1984 to 2009, a total of 19 children (mean age 8 years, range 1-18 years, female predominance 2:1, 17/19 European white patients, 2/19 Asian origin of at least one parent) were treated for juvenile Moyamoya disease by surgical revascularisation. The leading symptoms were epilepsy (17/19), followed by transient ischaemic attacks (TIA) or prolonged reversible ischaemic neurologic deficits (PRIND) (15/19) and mental retardation (11/19). Angiography showed a clear neovascularisation in the majority of patients after indirect bypasses after 6 months. The mean follow-up was 17 years and 3 months (maximum 25 years, minimum 2 years). Two patients were lost to follow-up. In accordance with the literature, ischaemic symptoms were eliminated by the revascularisation operation in 94% of our patients with a very low rate of complications, and no lasting morbidity and mortality in any of the patients.Early diagnosis and surgical treatment seem to potentiate the benefits independently of the type of revascularisation procedure.

Posthemorrhagic hydrocephalus in very low birth weight infants--a new gentle surgical technique for external ventricular drainage.

OBJECTIVE: The germinal matrix hemorrhage in premature infants is related to the immaturity of the subependymal vascular bed. Posthemorrhagic ventricular dilation (PVD) in extremely low birth weight infants (ELBI) is a medical challenge and is associated with a high risk of long-term disability. Our aim was to find a safe and gentle surgical technique for external ventricular drainage implantation for this patient population. PATIENTS AND METHODS: We implanted eight external ventricular drainages in ELBI with birth weights ranging from 479 to 884 g (24 to 27 weeks gestational age) and a grade III to IV hemorrhage, who developed a PVD soon after birth. A replacement of the drainage was required three times in one infant with a birth weight of 479 g. Due to the subcutaneous tunneling and drawing of the catheter through the sleeve, the skin contact remains slight, and thus, infections are avoided. RESULTS: There were no complications during the procedures which can also be carried out in the neonatal intensive care unit. Only in one case (479 g birth weight and severe concomitant diseases) was an infection observed, though the origin was most likely abdominal. In the same case, a temporary leakage was treated by suture. This patient died of non-neurological complications related to the patient's prematurity. Three of the five preterms were shunted over time. CONCLUSIONS: Although there is only a small number of extremely low birth weight infants with posthemorrhagic hydrocephalus who were treated with this technique, our findings suggest that this method is very safe and useful. It reduces the risk of infection and cerebrospinal fluid leaks and might be a better alternative to serial lumbar or transfontanel punctures in extremely low birth weight infants for bridging the first weeks of life.

Acute epidural hematoma with infarction of the right hemisphere in a 5-month-old child: case report with a long-term follow-up and a review of the literature.

Two hours after a fall, a 5-month-old girl was admitted to our hospital because of an extended galea hematoma and restlessness. Five hours after the trauma, a left hemiparesis developed. The child became drowsy. The hematocrit had fallen to 7.1 g/dL. Cranial computerized tomography disclosed a huge frontoparietal epidural hematoma on the right side and a hypodensity in the territories of the middle and posterior cerebral arteries. Immediately before surgery, the right pupil dilated. After evacuation of the epidural hematoma and ligation of the middle meningeal artery, the girl recovered satisfactorily. Nevertheless, magnetic resonance imaging showed a vast defect zone in the territories of the right middle and posterior cerebral arteries. Six weeks after the injury, the visual-evoked potentials were unavailable on both sides. Four years after the accident, visual assessment revealed normal acuity and stereopsis. Cognitive and neuromotor development were undisturbed and appropriate to the age.