Intracranial arteriovenous malformation is rarely presented in newborns or infants. We describe an unusual case of congenital arteriovenous malformation of the brain with multiple sequestered grape-like venous sacs presented with congenital hydrocephalus. This 4-month-old girl born with a large head, presented with progressive hydrocephalus over a period of 4 months. The brain CT showed multiloculated cysts with a high-density mural nodule and thin cerebral mantle. The right lateral ventricle was collapsed by the cystic lesion, and the contralateral ventricle was markedly dilated, which was thought to be due to aqueductal obstruction by the conglomerated nidus of the arteriovenous malformation. Surgical removal of both hemispheric masses, including the overlying thin mantle. The lesion was pathologically confirmed as the arteriovenous malformation which was composed of markedly dilated veins and multiple sequestered aneurysmal sacs. The overlying cerebral tissue was dysplastic and partly infarcted. This case shows that intracerebral arteriovenous malformation is indeed a congenital anomaly and suggests that intrauterine vascular compromise can result in focal or wide maldevelopment of the brain.
Female ; Humans ; Hydrocephalus/*etiology ; Infant ; Intracranial Arteriovenous Malformations/*complications/pathology/radiography ; Tomography, X-Ray Computed

A congenital neoplasm arising in the central nervous system is rarely encountered, and the majority of case reports that have dealt with intracranial tumors have been divided almost equally between teratomas and various gliomas. We experienced a rare case of congenital glioblastoma multiforme encountered in a three day-old male infant who presented with hydrocephalus since birth. Post-mortem examination revealed that the tumor seemed to have originated from the right thalamic region extending centrifugally to the cerebral cortex and through the brain-stem down to the cerebellum.
Autopsy ; Brain Neoplasms/*congenital/pathology ; Glioblastoma/*congenital/pathology ; Humans ; Hydrocephalus/*etiology/pathology ; Infant, Newborn ; Male ; Neoplasm Invasiveness

The causes of congenital hydrocephalus vary widely and have an important effect in determining the future counseling of affected cases. We analyzed the postmortem findings of 49 autopsies diagnosed as hydrocephalus to find the causes and related conditions. The cases were collected during the last 10 years (1981-1990). The cases consisted of 25 cases associated with various congenital malformations of the central nervous system (CNS), 14 cases of posthemorrhagic hydrocephalus, 4 cases of postinfection hydrocephalus, and 2 cases of hydranencephaly. Four cases were associated with supracerebellar arachnoid cyst (1) and unknown causes (3). Twenty-five cases associated with congenital malformation of the CNS consisted of 10 cases of holotelencephaly, 5 cases of stenosis of the Sylvian aqueduct, 4 cases of agenesis of the corpus callosum, and 3 cases each of Dandy-Walker malformation and Arnold-Chiari malformation. Various malformations of other organs were associated with these cases. Cardiovascular malformations were common, consisting of 11 cases of patent ductus arteriosus (PDA), 7 cases of atrial septal defect (ASD), and 6 cases of patent foramen ovale (PFO). Among the anomalies of the respiratory system, abnormal lobation was commonly associated, as well as hypoplasia of the lung. Gastrointestinal malformations included Meckel's diverticulum, diaphragmatic hernia, mobile intestine, and midline small liver. Cryptorchidism was the most common malformation in the genitourinary system. Holotelencephaly cases showed multiple craniofacial anomalies, as well as other malformations in the central nervous system. Skeletal malformations included polydactyly, simian crease, and flexion deformity. There were no specific constellations of malformations in these different groups of CNS malformations associated with hydrocephalus.
*Abnormalities, Multiple/pathology ; Autopsy ; Central Nervous System/abnormalities ; Cerebral Hemorrhage/complications ; Female ; Fetal Diseases ; Herpes Simplex/complications ; Humans ; Hydrocephalus/complications/embryology/*etiology/pathology ; Infant, Newborn ; Male

OBJECTIVE: To study the types, characteristics and common complications as well as disability assessment for the children with craniocerebral injury in the road traffic accidents. METHODS: Data from 204 cases of children with cranio-injury in road traffic accidents were collected and were statistically analyzed according to the location injured, complication, the type of complication and the severity of disability. RESULTS: There were 64 cases of simple diffuse primary craniocerebral injury, 80 cases of simple local primary cranio-injury, 24 cases of diffuse secondary craniocerebral injury and 36 cases of local secondary cranio-injury. The main complications included traumatic epilepsy (14, 6.9%), traumatic cerebral infarction (9, 4.4%), traumatic hydrocephalus (7, 3.4%) and traumatic mental disorder (5, 2.5%). CONCLUSION Among the children with cranio-injury due to road traffic accidents, simple primary cranio-injury was the most common result, whereas the traumatic epilepsy and traumatic cerebral infarction were the major types of complications. The assessment criteria for body impairment of the children with craniocerebral injury in the road traffic accidents should be broadened accordingly, with addition of certain specific items for children.
Accidents, Traffic/statistics & numerical data* ; Adolescent ; Cerebral Infarction/etiology* ; Child ; Child, Preschool ; Craniocerebral Trauma/pathology* ; Disability Evaluation ; Epilepsy, Post-Traumatic/etiology* ; Female ; Forensic Pathology ; Humans ; Hydrocephalus/etiology* ; Male ; Retrospective Studies

Background: Decision-making concerning the treatment of choroid plexus tumor (CPT) in pediatric patients remains a topic of considerable debate. The aim of this work was to describe clinical features and prognostic risk factors of CPT in the pediatric population and to provide theoretical opinions regarding clinical decisions for CPT. Methods: The data of 96 patients with CPT and younger than 14 years were retrospectively analyzed. Clinical characteristics such as pathological type of CPTs, rate and severity of hydrocephalus, treatment and outcome, and recurrence were investigated. For categorical variables, the Pearson's Chi-square test was performed. The Mann-Whitney U-test was used for comparisons between nonnormally distributed parameters. Log-rank test was used for progression-free survival (PFS). Results: The study included 70 choroid plexus papilloma (CPP) cases, 17 atypical choroid plexus papilloma (aCPP) cases, and 9 choroid plexus carcinoma (CPC) cases. Compared with patients with CPP or aCPP, patients with CPC had a shorter disease course (median: CPP, 4 months; aCPP, 2 months; CPC, 1 month; H: 23.5, P < 0.001), higher rate of acute hydrocephalus (CPP, 27.1%; aCPP, 52.9%; CPC, 77.8%; χ = 10.9, P < 0.05), and lower incidence of cure rate (CPP, 85.7%; aCPP, 70.5%; CPC, 33.3%; χ = 13.5, P < 0.05). The severity of hydrocephalus with tumor in the lateral or third ventricle was significantly higher than that with tumors in the fourth ventricle (severe hydrocephalus: lateral ventricle, 51.7%; third ventricle, 47.0%; fourth ventricle, 11.1%; χ = 26.0, P < 0.001). Patients with gross total surgical resection had no better PFS than those with partial resection because of the use of adjuvant therapy in the latter (χ = 4.0, P > 0.05). Patients with CPC experienced shorter time for recurrence than those with CPP or aCPP (χ = 40.1, P < 0.0001). Conclusions Our results indicated that CPP in the fourth ventricle could trigger serious clinical symptoms at an early stage, requiring early intervention. Adjuvant treatment might be necessary for patients with partially resected CPP, aCPP, and CPC to achieve a favorable outcome.
Child ; Child, Preschool ; Choroid Plexus Neoplasms ; mortality ; pathology ; surgery ; Female ; Humans ; Hydrocephalus ; etiology ; Infant ; Male ; Neoplasm Metastasis ; Neoplasm Recurrence, Local ; Prognosis ; Risk Factors

OBJECTIVETo review the clinical manifestations, imaging, tumor markers, treatment methods, pathology results and clinical curative effects of pineal region tumors and to evaluate the characteristics and intervention strategies for those tumors.

METHODSThe clinicopathological data of 132 patients with pineal region tumor treated in our department between January 2000 and May 2008 were retrospectively studied.

RESULTSA moderate predominance in males was presented. The clinical manifestations of the disease included increased intracranial pressure and ocular movement impairment. There were some features but no regularity and specific appearance on imaging including CT and MRI. 88.6% of patients associated with hydrocephalus. A high serum level of alpha-fetoprotein (AFP) was presented in 14 cases and high HCG in 9 cases. Eighteen cases received direct radiation therapy and 7 had radiotherapy post biopsy. 107 cases were treated surgically and 63 cases received postoperative adjuvant treatment. 114 cases had pathology results including 56 germ cell tumors. The patients were followed up for 12 approximately 132 months. Recurrence developed in 23 cases and 12 cases died. The 5-year survival rate was 89.3%.

CONCLUSIONPineal region tumors are often associated with hydrocephalus and this makes preoperative diagnosis difficult. Imaging examination may help diagnosis but less specific. Germ cell tumors may diagnosed by some tumor markers. Radiation therapy is the choice of treatment for pure germinomas. Other types of pineal region tumors should receive surgical treatment. Postoperative adjuvant treatment based on pathology can provide a good prognosis in pineal region tumor.

Adolescent ; Adult ; Aged ; Brain Neoplasms ; blood ; diagnosis ; therapy ; Child ; Child, Preschool ; Chorionic Gonadotropin ; blood ; Combined Modality Therapy ; Female ; Follow-Up Studies ; Humans ; Hydrocephalus ; etiology ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; Pineal Gland ; pathology ; surgery ; Pinealoma ; blood ; diagnosis ; therapy ; Retrospective Studies ; Sex Factors ; Survival Rate ; Tomography, X-Ray Computed ; Young Adult ; alpha-Fetoproteins ; metabolism