Prevalence of subdural collections in children with macrocrania.

BACKGROUND AND PURPOSE: The relationship between enlarged subarachnoid spaces and subdural collections is poorly understood and creates challenges for clinicians investigating the etiology of subdural collections. The purpose of this study was to determine the prevalence of subdural collections on cross sectional imaging in children with macrocephaly correlating with subarachnoid space enlargement. MATERIALS AND METHODS: The radiology information system of a large pediatric medical center was reviewed for "macrocrania" and "macrocephaly" on reports of cranial MRI/CT examinations in children <24 months of age, over a 24-month period. Head circumference was obtained from the clinical record. Studies were reviewed blindly for subdural collection presence and subarachnoid space size. Children with prior cranial surgery, parenchymal abnormalities, hydrocephalus, or conditions predisposing to parenchymal volume loss were excluded. Chart review was performed on those with subdural collections. RESULTS: Imaging from 177 children with enlarged head circumference was reviewed. Nine were excluded, for a final cohort of 168 subjects (108 with enlarged subarachnoid space). Subdural collections were identified in 6 (3.6%), all with enlarged subarachnoid space (6/108, 5.6%). In 4, subdural collections were small, homogeneous, and nonhemorrhagic. In 2, the collections were complex (septations or hemorrhage). Two children were reported as victims of child abuse (both with complex collections). No definitive etiology was established in the other cases. CONCLUSIONS: The prevalence of subdural collections in imaged children with macrocrania was 3.6%, all occurring in children with enlarged subarachnoid space. Our results suggest that enlarged subarachnoid space can be associated with some subdural collections in this cohort. Despite this, we believe that unexpected subdural collections in children should receive close clinical evaluation for underlying causes, including abusive head trauma.

Implantation of the common cavity malformation may prevent meningitis.

UNLABELLED: OBJECTIVES AND IMPORTANCE: Children with certain congenital malformations of the inner ear, including those with a common cavity defect, have a higher incidence of spontaneous cerebrospinal fluid (CSF) leak and resulting meningitis. However, they may also benefit from cochlear implantation. We suggest that surgical management may be possible that both prevents meningitis and provides hearing rehabilitation during the same procedure. CLINICAL PRESENTATION: A 2-year-old girl with bilateral common cavity defects who had previously undergone cochlear implantation developed contralateral CSF leak resulting in meningitis. INTERVENTION: After resolution of the infection, cochlear implantation was performed at the same time as definitive CSF leak repair. Simultaneous cochlear implantation and repair of the CSF leak successfully decreased the chance of recurrent meningitis in this case. She has been deriving hearing benefit from the bilateral implants. CONCLUSION: This case suggests a role for cochlear implantation to be combined with simultaneous CSF leak repair in children with a cochlear malformation. Furthermore, bilateral cochlear implantation at an early age may be warranted in these patients before CSF leaks and meningitis have occurred.

Developmental outcome of children with enlargement of the cisterna magna identified in utero.

An enlarged cisterna magna can be identified during routine ultrasound screening in the second half of pregnancy. It is important to be able to give an accurate prognosis. We evaluated the developmental outcome of these children. A total of 29 fetuses with a large cisterna magna identified in utero were compared to 35 children with a normal fetal ultrasound. The children were evaluated by the Gesell Developmental Schedules and the Peabody Developmental Motor Scale. The study group showed a significantly worse performance in the Gesell test. However, the overall performance for both groups was within normal limits. Four children in the study group had a borderline developmental quotient. Both groups performed similarly in the Peabody test. Walking age was significantly delayed in the study group. Children with an enlarged cisterna magna may be at risk for mild developmental delay. In cases of nonisolated enlargement of the cisterna magna, the outcome may be guarded.

Expansión del espacio subaracnoideo en el niño / Enlargement of subarachnoid space in child

Se presentan dos casos de niños referidos a consulta de neurocirugía por macrocranea y dilatación de los espacios subaracnoideos en la convexidad y cisternas basales. Ambos evolucionan con resolución espontánea de esta anomalía, demostrada y seguida con tomografía computarizada (TC) y resonancia magnética (RM). Sin alteraciones significativas en su desarrollo psicosomático

Detection of nerve rootlet avulsion on CT myelography in patients with birth palsy and brachial plexus injury after trauma.

OBJECTIVE: Recent advances in neurosurgical treatment of traumatic and birth-related brachial plexus injuries require differentiation of preganglionic nerve rootlet avulsion from postganglionic lesions. The purpose of this study was to evaluate the efficacy of thin-section high-resolution CT myelography for revealing cervicothoracic nerve rootlet avulsion in patients with brachial plexus injuries before surgery. MATERIALS AND METHODS: We evaluated eight patients with posttraumatic or birth-related brachial plexus injury on cervical plain film myelography and high-resolution CT myelography before surgical exploration and repair. CT myelograms were retrospectively evaluated for nerve rootlet avulsion, traumatic pseudomeningocele, and deformity of the subarachnoid space. Results were correlated with surgical exploration and intraoperative somatosensory evoked potentials. RESULTS: Seventy-two (95%) of 76 imaged cervicothoracic levels were adequately shown on CT myelography. Nerve rootlet avulsion, or preganglionic disruption, was shown at 21 levels. Associated pseudomeningocele, or deformity of the subarachnoid space, was seen at 12 (57%) of the 21 avulsion levels. Surgical exploration and intraoperative somatosensory evoked potentials showed complete preganglionic nerve rootlet avulsion at 22 levels. One of the complete avulsions revealed by surgery was not included on the patient's CT myelogram. Of the 21 imaged levels, 20 were correctly revealed on CT myelography (95% sensitivity, 98% specificity). At surgery, partial nerve rootlet avulsion was found at three other levels. None of the partial avulsions was correctly identified on the CT myelograms. CONCLUSIONS: High-resolution CT myelography with thin contiguous axial section is sensitive for revealing complete nerve rootlet avulsion in patients with brachial plexus birth palsies and brachial plexus injuries after trauma. Preoperative CT myelography in these patients allows a more complete injury evaluation for accurate prognosis and surgical planning.

Hipertrofia ou alargamento benigno do espaço subaracnóide / Hyphertrophy or benign lenghtening of subaracnoidal space

Relata-se caso de macrocefalia relacionada ao alargamento benigno do espaço subaracnóide. Discutem-se os achados clínicos, etiopatogenia e manuseio dessa condiçäo. Salienta-se a importância do diagnóstico radiológico (tomografia computadorizada) e evoluçäo caracteristicamente favorável dos pacientes

Brain MR in Fukuyama congenital muscular dystrophy.

PURPOSE: To determine the MR characteristics of brain abnormalities in Fukuyama congenital muscular dystrophy (FCMD). METHODS: We reviewed 30 MR examinations of 21 patients with FCMD to assess cerebral and cerebellar cortical dysplasia, white matter changes, and miscellaneous abnormalities. RESULTS: On MR images, all patients had thick and bumpy cortices with shallow sulci corresponding to polymicrogyria, and 12 patients had pachygyric cortices with smooth surfaces, corresponding to type II lissencephaly. Both types of cortical dysplasia had characteristic distributions: the first type involved the frontal lobe in all 21 patients and also the parietotemporal lobe in 6 patients; the second type involved the temporooccipital lobes. Eighteen patients had prolonged T1 and T2 signal in the white matter, which was indistinct in neonates and seen infrequently in adolescents. In four patients, abnormal vessels were seen within the pachygyric cortices. CONCLUSION: MR studies of the brain show findings consistent with the known characteristics of FCMD. The MR detection of the two types of cerebral cortical dysplasia with characteristic distribution and cerebellar abnormalities is helpful in the differential and early diagnosis of FCMD.

X-linked progressive mixed deafness: a new microdeletion that involves a more proximal region in Xq21.

We report a large two-generation pedigree with seven affected males segregating for an X-linked mixed conductive sensorineural deafness. The patients present with atypical Mondini-like dysplasia, dilated petrous facial canal, dilatation of the internal auditory meatus fully connected with enlarged cochlear canals, and, in one patient, a wide bulbous posterior labyrinth. Obligatory carrier females are mildly affected. Molecular characterization of this family revealed a deletion of locus DXS169, in Xq21.1. Loci DXS72 and DXS26, which, respectively, flank DXS169 proximally and distally, were intact. Since a gene responsible for X-linked progressive mixed deafness with perilymphatic gusher (DFN3) has previously been assigned by deletion mapping to a slightly more distal interval between DXS26 and DXS121, this study indicates either two different deafness genes or the involvement of a very large region in Xq21.

Follow-up study of macrocephalic children with enlargement of the subarachnoid space

Trata-se de estudo prospectivo de 18 crianças com macrocefalia por aumento do espaço sub-aracnóideo, com ou sem dilataçäo ventricular, seguidas até idade em média de 56 meses. Todas nasceram a termo, sem intercorrências perinatais e com testes negativos para TORCH. O tempo médio de seguimento foi 46 meses. Havia 17 meninos e apenas uma menina no grupo estudado. A porcentagem de anormalidades neurológicas no seguimento foi de 11%. Durante o seguimento, o perímetro cefálico retornou aos níveis da normalidade em 45% das crianças. Nenhum caso desenvolveu hipertensäo intracraniana durante o estudo. Todas as crianças realizaram TAC de crânio como parte da avaliaçäo inicial e, além do aumento do espaço sub-encefálico, 77% delas apresentavam discreta dilataçäo ventricular. No seguimento, 11 realizaram TAC de controle que revelaram resoluçäo completa do processo em 3 casos, melhora em 2 e permaneceram inalteradas em 6. Concluimos que o aumento do espaço sub-aracnóideo em crianças macrocefálicas é entidade que apresenta bom prognóstico neurológico na maioria dos casos e que a macrocefalia e o aumento do sub-aracnódeo continuaräo presentes, na maioria das crianças, no seguimento a longo prazo

Perfil neuroevolutivo del lactante con macrocefalia y ensanchamiento benigno del espacio subaracnoideo / Neuroevolutionary profile of nursing with macrocephalia and benign widening of the subarachnoid space

Con la finalidad de estudiar el pronóstico neuroevolutivo de los niños con macrocefalia (percentil>95) y ensanchamiento del espacio subaacnoideo identificados por TAC del cráneo, se seleccionaron cinco casos atendidos en el consultorio de Neurología Pediátrica del Hospital Regional Docente de Trujillo, Perú, entre enero de 1983 a enero de 1988 y cuyo seguimiento alcanzó entre 1.5 a 3 años. Todos los lactantes mostraron en la TAC, separación anormal del espacio subaracnoideo frontal, fisura interhemisférica y cisura de Silvio sin ventriculomegalia o mínima dilatación del sistema ventricular. Mediante la evaluación neurológica y la prueba de Desarrollo de Denver se observó en todos los casos, excepto uno, un patrón caracterizado por hipotonía y retardo motor grueso durante el primer año de vida, el cual fue modificándose hacia la normalidad los controles secuenciales hasta la edad de 25 a 40 meses. Estos hallazgos sugieren el buen pronóstico y la benignidad de esta condición que se resuelve espontáneamente y relacionada al parecer con macrocefalia familiar benigna

Megalocephaly and hypodense extracerebral fluid collections.

Computed tomographic (CT) scans were identified and reviewed for 25 pediatric patients with enlarged heads and extracerebral fluid collections that showed cerebrospinal fluid-like attenuation. Mild to moderate enlargement of the subarachnoid space over the convexity, interhemispheric fissure, and the lateral ventricles was observed in most of the cases. The CT findings are not specific, and it is not always possible to determine the nature of the extracerebral fluid, whether it lies in the subarachnoid space, the subdural space, or both. The differential diagnosis included subdural effusions, hygromas, preclinical or external hydrocephalus, hypodense subdural hematomas, megalocephaly, and possibly a normal variant. CT remains the method of choice for the initial study of these patients and for subsequent follow-up in selected cases.