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Sonographic and clinical features of 26 fetal cephaloceles were reviewed retrospectively. The most frequent reason for referral was elevated maternal serum α-fetoprotein levels. The smallest lesion identified was 0.4 × 0.5 cm (frontal, at 20 weeks); the largest was 9.0 × 10.0 cm (frontal, at 27 weeks). Twenty-four of 26 cephaloceles were detected on prenatal ultrasound examination; in 13 of these, more than 50% of the intracranial contents were exteriorized. Fifteen of 24 cephaloceles detected prenatally had a sulcal pattern (identified between 16 and 36 weeks' gestation); five were solid without a sulcal pattern (identified between 13 and 21 weeks' gestation), three were cystic, and one underwent a change in appearance from solid at 21 weeks to cystic at 26 weeks. Other cranial features were evaluated and included visible skull defect, seen in 23/24 (96%), ventriculomegaly, in 6/26 (23%); microcephaly, in 12/24 (50%); beaked tectal plate, in 6/16 (38%); and flattened basiocciput, in 9/24 (38%). Of the 26 cases, 14 had normal amniotic fluid volume, five had oligohydramnios and seven had polyhydramnios. Fetuses with oligohydramnios had the highest incidence of concurrent fetal abnormalities; four of five fetuses (80%) with oligohydramnios had additional structural abnormalities. In the overall population, a very high incidence of other abnormalities was found; 17/26 (65%) cases showed additional abnormalities, some of which were not detected by ultrasound. Five fetuses had Meckel-Gruber syndrome and three had amniotic band syndrome. Only one of the 18 karyotypes obtained was abnormal (trisomy 18). Survival was very poor; only two of the eight who survived until birth are currently living. Copyright © 1995 International Society of Ultrasound in Obstetrics and Gynecology

We report an infant with a craniopharyngioma which was detected in utero. Maternal uterine ultrasonography, done at 27 weeks because of polyhydramnios, revealed a 4 cm midline mass near the base of the fetal skull. At 31 weeks, magnetic resonance imaging of the maternal abdomen confirmed the presence of a mass in the region of the third ventricle and revealed hydrocephalus. Two days post-partum a computed tomography (CT)-guided needle biopsy of the mass was performed and recovered tissue which was histologically consistent with a craniopharyngioma. The infant' s postnatal period was complicated by seizures, which were treated with phenobarbital, and by progressive hydrocephalus, necessitating placement of a ventriculo-peritoneal shunt. He also received therapy for central hypothyroidism and diabetes insipidus. The infant' s parents refused permission for attempted resection of the tumour and he died at 8 weeks of age. This represents the second reported case of an antenatally detected craniopharyngioma. Four other cases of different intracranial tumours have been detected in utero using ultrasound, with no reported survivors past 3 days of age. There is a uniformly poor prognosis of such infants, but earlier diagnosis and intervention may change this result.

We present a newborn infant with paraplegia and contractures of the lower limbs, consistent with neurologic injury rather than malformation. The mother was involved in a severe motor vehicle accident during the sixth month of pregnancy. We propose that this infant's injuries are a result of that accident. Language: en

Recently, we evaluated three sibs with Carpenter syndrome, permitting further clinical, orthopedic, radiographic, and psychometric delineation of this disorder. All three patients were operated on for craniostenosis at or before 2 months. Although all had gross motor delay in early infancy, two had normal intelligence at 12 months and 10 years, respectively. Bony abnormalities contributed to functional impairment especially in the older children. Preaxial polydactyly of feet was present in all three affected sibs and in all other reported individuals with this condition, allowing differentiation of Carpenter syndrome from the other autosomal-recessive acrocephalopolysyndactyly syndromes.

A staging system has been proposed for the following tumors: craniopharyngiomas, germinomas, and intracranial teratomas. Various aspects of the specific pathologic and growth characteristics of the tumor determines such categorization and subclassifiation of the tumors. A brief analysis of the clinical aspects in reference to classification is presented. The suggested systems will need to be tested in clinical studies to determine their validity. Cancer 56: 1800-1803, 1985.

A staging system is needed for midline intra-axial (nuclear) tumors so that treatment results can be compared, insights about tumor biology can be obtained, and prognosis can be estimated. The TNM staging classification is of limited value because nuclear tumor margins cannot be defined by an operation, there are no nodes to sample, and the tumors rarely metastasize. Nuclear tumors can be staged best now by computed tomography (CT) and pathology findings. Diagnostic staging by CT scans will demonstrate tumor features that may be of prognostic importance: tumor size, density, volume, enhancement, laterality, and presence of cysts and calcifications. In the current series, prognosis was better for tumors with cysts or calcifications, and for tumors less than 5 cm, but correlated poorly with laterality and site. Biopsy and often partial resection can be performed on nuclear tumors with low risks, but operative staging is not helpful because operations are necessarily limited. Most diencephalic tumors are astrocytomas or mixed gliomas, but approximately 15% are malignant astrocytomas or malignant mixed gliomas that need more intensive therapy, and 5% to 10% are neoplasms such as hamartomas that require no additional therapy. Two individual histopathologic characteristics, high cell density and mitoses, indicate a worse prognosis. Pediatric brain tumor study groups need to accumulate additional radiographic and pathologic information to stage nuclear tumors more accurately.