CAMA A.

Purpose Describe presentation and management of Chiari type 1 malformation. We report our surgical case series proposing a decision making scheme for helping surgeons decide which surgical procedure to perform and when. Methods We retrospectively examined a series of surgically treated patients with Chiari type 1 malformation. Treatment of...

Purpose: Arachnoid cysts may present with symptoms deriving from cyst rupture, usually causing intracystic hemorrhage and subdural hematoma or hygroma. Rupture is usually caused by minor trauma, spontaneous rupture is an exceptional event, and 57 cases have been described in literature. We here present and discuss the largest series of...

Objective: Many neonatal intensive care units (NICUs) have adopted the practice of performing routine brain MRI in very low birth weight (VLBW) infants at term-equivalent age in order to better evaluate prematurity-related acquired lesions. A number of unexpected brain abnormalities of potential clinical significance can be visualised on...

Background/Purpose: The long-term urologic outcome in a large series of patients with neural tube defects was evaluated. Methods: The following clinical parameters in 398 patients ranging from 1 to 37 years of age-69 with caudal regression syndrome (CRS), 244 with meningomyelocele (MMC), and 85 with spinal lipoma (SL)-were studied: congenital...

BACKGROUND: Bilateral lambdoid and posterior sagittal synostosis is a rarely encountered multisutural craniosynostosis accounting for 0.3%-0.7% of all craniosynostoses. It has been named "Mercedes- Benz Syndrome" (MBS) because of the bone ridges that resemble the Mercedes-Benz logo. The typical head shape is usually described as anterior...

Objective: We describe a rare case of functioning gonadotropins-producing pediatric adenoma immunostaining positively for FSH and focally for TSH causing central precocious puberty associated to central hypothyroidism in a 6 year-old girl. Materials and Methods: Clinical evaluation revealed precocious puberty, as confirmed by hormonal...

OBJECTIVE: Given the lack of significant responses to many queries regarding behavior, treatment options, and possible prevention of iatrogenic intraspinal inclusion tumors (IITs), we think that further clinical, radiologic, and follow-up data are mandatory to better characterize this entity. We retrospectively reviewed a series of 14...

OBJECTIVE: The aim of this article is to introduce a new skin and fascia opening for intrathecal baclofen pump implantation in the abdomen, with the purpose of reducing complications related to wound breakdown.METHODS: We introduce a novel way of cutaneous and fascial opening that leads two opposed "L shaped" incisions.RESULTS: This method...

Cerebral cavernous malformations (CCMs) are vascular malformations mostly located within the central nervous system. Heterozygous loss of function mutations in CCM1/KRIT1, CCM2/MGC4607, and CCM3/PDCD10 genes are identified in about 90 % of familial cases of CCMs and two thirds of sporadic cases with multiple lesions. In this study, we performed...

Purpose Chiari malformation type I (CMI), a rare disorder of the craniocerebral junction with an estimated incidence of 1 in 1280, is characterized by the downward herniation of the cerebellar tonsils of at least 5 mm through the foramen magnum, resulting in significant neurologic morbidity. Classical CMI is thought to be caused by an...

Neural tube defects (NTDs) are severe malformations of the central nervous system, affecting 1 of 1,000 live births. Mouse models were instrumental in defining the signaling pathways defective inNTDs, including the planar cell polarity (PCP), also called noncanonical Frizzled/Disheveled pathway. Based on the highly penetrant occurrence of NTDs...

BACKGROUND: Dermoid and epidermoid cysts are among the most frequent lesions of the scalp in the pediatric population. Optimal management of a suspected dermoid or epidermoid cyst in children is debated: Some authors advocate conservative management on the basis of the benign histology of these entities, while others prefer surgical...

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Cavernous malformations (CM) are cerebral irradiation-related late complications. Little is known about their natural history and the pathogenetic role of concomitant chemotherapy. We present a retrospective, single-institution study of 108 children affected with medulloblastoma, ependymoma, or germinoma treated with radio- and chemotherapy....

BACKGROUND: Nontraumatic atlantoaxial subluxation, also known as Grisel syndrome, is a rare disease that usually affects children. The typical presentation is torticollis in patients with a history of surgical operations or airway infections.METHODS: We describe 5 patients with Grisel syndrome, referring to medical care for a torticollis, a few...

Infiltrative astrocytomas (IAs) represent a group of astrocytic gliomas ranging from low-grade to highly malignant, characterized by diffuse invasion of the brain parenchyma. When compared with their adult counterpart, pediatric IAs may be considered biologically distinct entities; nevertheless, similarly to those in adults they represent a...