Aim: This study aims to report a patient with Chiari type 1 malformation (CM1) occurring in the context of pseudohypoparathyroidism type 1a (PHP-Ia) that we believe represents the first instance of this association in the current literature.
Case Report: The authors describe the case of a 6-year-old girl diagnosed with PHP-Ia who presented an associated tonsillar descent. During the follow-up, the skull vault and the occipital squama became extremely thickened at the same time as the tonsillar herniation showed a marked regression.
Background: Occult spinal dysraphism (OSD) encompasses various conditions. A dermal sinus tract (DST) consists of a duct communicating to the skin with deep structures that carries an important risk of infection. A different lesion consisting of a translucent skin opening and a fibrous tract that lacks a lumen can also be found in OSD.
View Article and Find Full Text PDFBackground: Patients operated on for myelomeningocele (MMC) and lipomeningocele (LMC) can suffer from late functional worsening that, in many cases, is due to spinal cord tethering by the post-repair scarring process.
Illustrative Cases: In this case-based update, we report two patients operated on for MMC and LMC, respectively, who presented the clinical manifestations of spinal cord tethering, which we attributed to severe foreign body reactions to the materials used at their primary back surgery. In the first case, the cause of the tethering was an intense fibrotic scar around the silk suture used at the initial MMC repair, while in the second one, it was due to a fibrotic mesh containing the lyophilized dural graft implanted for LMC surgery.
A 2-month-old girl developed meningitis, ventriculitis and brain abscess in the course of Citrobacter koseri infection. She was successfully treated with the combined use of antibiotics, intra-cavitary urokinase and surgery, thus avoiding the development of hydrocephalus and of ventricular loculation. C.
View Article and Find Full Text PDFCase: A 3-year-old boy underwent emergency external ventricular drainage and excision of a fourth ventricle anaplastic ependymoma. A week later, the child was given a ventriculo-peritoneal shunt. Fourteen days after shunting, the child developed a subphrenic abscess and acute cholecystitis that required surgery.
View Article and Find Full Text PDFBackground: Increased intra-abdominal pressure has been reported to result in raised intracranial pressure in a variety of conditions such as obesity and pregnancy, and it also constitutes an infrequent cause of ventriculoperitoneal (VP) shunt malfunction. Patients with neurological deficits, as those with myelomeningocele or cerebral palsy, are prone to developing a neurogenic bowel and to suffer chronic constipation. Although previously recognized, VP shunt failure attributed to constipation has only recently been described.
View Article and Find Full Text PDFThe authors report the case of a 10-year-old boy who sustained an injury to the cauda equina as a result of the accidental penetration of a wooden pencil into the spinal canal. After neuroimaging evaluation to exclude visceral and vascular lesions, the foreign body was removed and the wound was repaired. This is the first report of a cauda equina injury caused by a pencil.
View Article and Find Full Text PDFObjective: The authors report two unusual cases of extraspinal midline lumbosacral lesions that resembled the usual skin markers of occult spina bifida.
Patients And Methods: The pathological diagnosis of the masses was plexiform neurofibroma and mastocytoma, respectively.
Results: Some lesions, as those we are reporting, although occurring at the lumbosacral midline, do not necessarily belong to occult spinal dysraphism, as was initially suspected.
Case Report: We report the case of a newborn baby who presented with scalp aplasia cutis congenita and an underlying skull defect.
Discussion: 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.