Juan F. Martínez-Lage

The concurrence of non-traumatic atlanto-axial subluxation with inflammation of the adjacent neck tissues is known as Grisel’s syndrome. We report a 5 year old boy with recurring episodes of head tilt and painful and restricted neck movements that developed after repeated bouts of sinusitis. Radiographs showed a subluxation of the C2–3 joint. Medical treatment, with cervical collar, physiotherapy, and non-steroid anti-inflammatory agents, led to complete cure of the disease. We suggest that Grisel’s syndrome can occur in a location different from the classic atlanto-axial joint. To the best of our knowledge, this is the first report of a symptomatic case of Grisel’s syndrome occurring at the C2–3 segment.

Grisel's syndrome is a uni- or bilateral subluxation of the atlas on the axis associated with an inflammatory condition of the head or neck. This uncommon entity usually affects children. The condition seems to be secondary to hyperemia and laxity of the atlanto-axial joints.

Lumbar disc herniation is a common condition in adults, uncommon in adolescents and exceptional below the age of 10 years. We report on a 6-year-old boy who presented with low-back pain and sciatica after having sustained an injury in a rear-end automobile accident. Due to our limited experience with protruded discs in this age group, we decided to be conservative initially. However, the persistence of incapacitating pain prompted surgical treatment. Operative findings consisted of a torn annulus, attached at one end to the cartilaginous plate, which formed a major portion of the extruded L5-S1 disc. A search of the literature yielded only four cases of lumbar disc (L4-5) protrusions in patients aged 9 years or younger. The child described here is one of the youngest patients with a lumbar disc rupture to be reported in the literature, and the first with an L5-S1 traumatic herniation.

We report the case of a newborn baby who presented with scalp aplasia cutis congenita and an underlying skull defect. We discuss the diverse options for the management of this condition and the feasibility of early surgical repair in the light of the current literature. We also comment on the possible role of benzodiazepines in the genesis of this lesion.

Introduction. Thalamic tumors (TTs) account for approximately 1% of all intracranial neoplasms. TTs are seen predominantly in children and young adults. Most childhood neoplasms in this location are of glial lineage, a large proportion being low-grade tumors. The evolution of symptoms before diagnosis is characteristically shorter in children than in adults. Clinical presentation. Clinical features of TTs reflect the pressure of the mass on the cerebrospinal fluid pathways, the pyramidal tracts, the thalamic nuclei and the optic radiations. In this paper, we review the clinical data of 20 children with TTs seen at our hospital and compare the findings with those in the current literature. An acute presentation is not rare and was encountered in 20% of our patients, although most individuals had a subacute or slow evolution. Symptoms and signs of raised intracranial pressure (65%), motor deficits (40%), and seizures (35%), alone or in combination, were the most frequent manifestations of TTs. Behavioral and mental changes are not uncommon in TTs and were found in 25% of our patients. Involuntary movements (10%) and the classic thalamic syndrome (5%) were quite exceptional. Infants and young children with TTs may present with macrocephaly, psychomotor delay, and failing vision or disorders of ocular movements.

We report the case of a 4-year-old girl who presented spontaneous watery discharge through the left nostril due to a basal encephalocele. Two infrequent operative findings were the presence of an excavation of the cribriform plate, and not of a true osseous defect, and the absence of the left olfactory bulb.

The formation of an epidural hematoma from an eosinophilic granuloma of the skull is an exceptional occurrence. A 9-year-old boy presented with severe headache, somnolence and vomiting following a minor head injury. Cranial computerized tomography scan showed a seemingly depressed skull fracture together with an epidural hematoma in evolution. A neoplasm and an epidural hematoma were removed at operation. Histopathological study of the excised mass confirmed the diagnosis of eosinophilic granuloma.

Ventriculoperitoneal shunting constitutes the standard procedure for draining cerebrospinal fluid (CSF) in children with hydrocephalus. Ventriculoatrial and ventriculopleural shunting are alternative methods of CSF drainage, which have gained less acceptance. Ventriculopleural shunts are seldom used owing to justified fears of pneumothorax and symptomatic effusions of CSF. The addition of an antisiphon device to standard shunt systems seems to have prevented CSF pleural effusion. From 1988 to 1998, we treated each of six hydrocephalic children with a ventriculopleural shunt. In five cases we used new-technology valves designed to prevent the effects of siphoning with current differential pressure valves. Peritoneal adhesions, recent peritonitis, ascites, and obstruction of a previous ventriculoatrial shunt were the indications for pleural shunting. After a mean follow-up period of 2.5 years all shunts were functioning adequately. Only one patient showed transient symptoms of CSF overdrainage, which were corrected by up-grading the valve setting with the magnet. A late death was unrelated to the pleural shunting procedure. The use of valves of a new design designed to prevent overdrainage seems to account for the satisfactory outcomes observed in this series. We suggest that ventriculopleural shunting should be considered as the preferred alternative to peritoneal drainage in children with intra-abdominal adhesions or with a history of recent peritoneal infection.