Deformaciones craneales posicionales: estudio clínico-epidemiológico / Positional cranial deformations: a clinical-epidemiological study

Introducción: Las deformidades craneales, que han existido desde siempre, recientemente han suscitado un gran interés. Varios estudios muestran un aumento llamativo de consultas por plagiocefalia occipital. En este trabajo se analizan los factores clínico-epidemiológicos relativos a esta deformación. Pacientes y métodos: Se revisaron las historias de 158 niños atendidos en nuestro hospital para evaluación de deformación craneal. Se recogieron datos clínicos y de neuroimagen, tratamientos y resultados, analizando especialmente los factores de riesgo. Resultados: Durante el estudio se apreció un aumento de visitas por esta causa. La media de edad al consultar fue de 9 meses, existiendo un claro predominio (68%) de varones. El tipo de deformación fue: plagiocefalia occipital unilateral (89,2%), bilateral (7,6%) y dolicocefalia (3,2%). Aparte de la posición para dormir, se encontraron diversos factores de riesgo, como colecciones pericerebrales de líquido cefalorraquídeo (LCR) en el 50% de los niños valorados mediante estudios de neuroimagen. El tratamiento fue conservador (posicional y/o fisioterapia) en la mayoría de niños y con cascos en 12, apreciándose mejoría en 136 pacientes. Discusión y conclusiones: Estos hallazgos concuerdan con los de la bibliografía. Un resultado original del estudio fue la elevada incidencia de colecciones pericerebrales de LCR. El aumento de plagiocefalia posicional coincidió con las indicaciones de la Academia Americana de Pediatría sobre la posición para dormir, con el fin de evitar la muerte súbita del lactante. Se destaca el papel del pediatra en la detección y el manejo inicial de estos pacientes. La instauración de medidas de prevención se considera más importante que las indicadas para su tratamiento (AU)

Introduction: Cranial deformities have always existed, but it is only until recently that they provoked a greater interest. Several reports have demonstrated an increasing tendency for occipital plagiocephaly. This work presents an analysis of the clinical-epidemiological features of these deformities in our area. Patients and methods: The medical records and neuroimaging studies of 158 children seen a tour hospital due to a skull deformation were analyzed, paying special attention to risk factors. Results: During the study period, an increase in the number of consultations for skull deformation of children was noted. The mean age for the patients at consultation was 9 months.There was a predominance of boys (68%) over girls. The deformities were: unilateral- (89.2%) or bilateral-posterior plagiocephaly (7.6%), and dolicochephaly (3.2%). In addition to the supine position for sleeping, there were several risk factors, among them pericerebral collections of cerebrospinal fluid found in 50% of children subjected to neuroimaging studies. Most children were managed conservatively, while 12 patients were given helmets, which led to an improvement in 136 cases. Discussion and conclusions: The present findings agree with those in the current literature. Aunique result of this study was the high rate of pericerebral fluid collections found in children assessed with neuroimaging methods. The increase in positional plagiocephaly seems to parallel the application of the recommendations for supine sleeping position established by the American Academy of Pediatrics for preventing the sudden infant death syndrome. The role played by Pediatricians in the detection, initial management and referral of these children is highlighted. Prevention measures seem to be more important than those regarding treatment (AU)

[Positional cranial deformations: a clinical-epidemiological study]. / Deformaciones craneales posicionales: estudio clínico-epidemiológico.

INTRODUCTION: Cranial deformities have always existed, but it is only until recently that they provoked a greater interest. Several reports have demonstrated an increasing tendency for occipital plagiocephaly. This work presents an analysis of the clinical-epidemiological features of these deformities in our area. PATIENTS AND METHODS: The medical records and neuroimaging studies of 158 children seen at our hospital due to a skull deformation were analyzed, paying special attention to risk factors. RESULTS: During the study period, an increase in the number of consultations for skull deformation of children was noted. The mean age for the patients at consultation was 9 months. There was a predominance of boys (68%) over girls. The deformities were: unilateral- (89.2%) or bilateral-posterior plagiocephaly (7.6%), and dolicochephaly (3.2%). In addition to the supine position for sleeping, there were several risk factors, among them pericerebral collections of cerebrospinal fluid found in 50% of children subjected to neuroimaging studies. Most children were managed conservatively, while 12 patients were given helmets, which led to an improvement in 136 cases. DISCUSSION AND CONCLUSIONS: The present findings agree with those in the current literature. A unique result of this study was the high rate of pericerebral fluid collections found in children assessed with neuroimaging methods. The increase in positional plagiocephaly seems to parallel the application of the recommendations for supine sleeping position established by the American Academy of Pediatrics for preventing the sudden infant death syndrome. The role played by Pediatricians in the detection, initial management and referral of these children is highlighted. Prevention measures seem to be more important than those regarding treatment.

[Reversible primary empty sella. Case report]. / Silla turca vacía primaria reversible. A propósito de un caso.

We report the case of a 13-year-old girl with an increased hypophysis that mimicked an adenoma, who developed a partial empty sella on MRI after an acute episode of hydrocephalus. After replacement of a CSF shunt, the intracranial pressure returned to normal and the hypophysis filled up again all the sellar fossa. We discuss the possibility of the involvement by an ischemic atrophy of the adenohypophysis in the development of a primary empty sella with idiopathic chronic raised intracranial pressure that prevents the recovery of the gland volume after restoring the intracranial pressure to normal values. Restitution of empty sella may be an indicator of normal intracranial pressure in these cases.

Silla turca vacía primaria reversible. A propósito de un caso / Reversible primary empty sella. Case report

Se presenta el caso de un niña de 13 años con un aumento del tamaño de la hipófisis sugerente de adenoma que, tras un episodio de hidrocefalia aguda, desarrolló en la RM una silla turca vacía parcial.Tras la colocación de una derivación de líquido cefalorraquídeo, se normalizó la presión intracraneal y la hipófisis volvió a ocupar todo el espacio de la fosa sellar. Se discute la posibilidad de que en la silla turca vacía primaria con hipertensión intracraneal crónica idiopática, esté implicada una atrofia isquémica de la adenohipófisis que impide la recuperación del volumen glandular tras la restauración de la PIC a los niveles normales. La reversión de la silla turca vacía es un indicador de la normalización de la PIC (AU)

We report the case of a 13-year-old girl with an increased hypophysis that mimicked an adenoma, who developed a partial empty sella on MRI after an acute episode of hydrocephalus. After replacement of a CSFshunt, the intracranial pressure returned to normal and the hypophysis filled up again all the sellar fossa. We discuss the possibility of the involvement by an ischemic atrophy of the adenohypophysis in the development of a primary empty sella with idiopathic chronic raised intracranial pressure that prevents the recovery of the gland volume after restoring the intracranial pressure to normal values. Restitution of empty sell a may bean indicator of normal intracranial pressure in these cases (AU)

Regression of syringomyelia and tonsillar herniation after posterior fossa arachnoid cyst excision. Case report and literature review.

BACKGROUND: Some reports have documented posterior fossa cysts resulting in syringomyelic obstruction of cerebrospinal fluid (CSF) flow caused by cyst displacement within the foramen magnum. Rarely the syringomyelia is caused by acquired Chiari malformation due to a retrocerebellar arachnoid cyst. OBJECTIVE: To report the case of a 38-year-old man with hydrocephalus and syringomyelia, who was found to have a Chiari malformation secondary to a posterior fossa arachnoid cyst. After endoscopic third ventriculostomy, the patient was submitted to foramen magnum decompression and arachnoid cyst removal that were followed by resolution of both the Chiari malformation and the syringomyelia. DISCUSSION: In most published cases the syringomyelia has been attributed to obstruction of CSF flow at the foramen magnum by the arachnoid cyst itself. There is only one previous report of a posterior fossa arachnoid cyst producing tonsillar descent and syringomyelia. CONCLUSIONS: Posterior fossa arachnoid cysts can result in acquired Chiari malformation and syringomyelia. In our view, the management of these patients should be directed at decompressing the foramen magnum and include the removal of the walls of the coexistent arachnoid cyst as it seems to be the crucial factor that accounts for the development of the syringomyelia that these patients present.

Regression of syringomyelia and tonsillar herniation after posterior fossa arachnoid cyst excision. Case report and literature review

Antecedentes. Algunos trabajos han descrito laexistencia de quistes aracnoideos de fosa posterior queproducían siringomielia al obstruir la circulación deliquido cefalorraquídeo (LCR) por bloqueo del agujeromagno ocasionado por el propio quiste. Rara vezla siringomielia asociada a quiste aracnoideo de fosaposterior es producida por malformación de Chiariadquirida.Objetivos. Publicar el caso de un hombre de 38años con hidrocefalia y siringomielia, en el que seencontró descenso de las amígdalas cerebelosas por lapresión ejercida por un quiste aracnoideo retrocerebeloso.Tras tratar primero la hidrocefalia medianteventriculostomía neuroendoscópica, el paciente fueoperado de descompresión del agujero magno yextirpación de las membranas del quiste que condujerona la resolución tanto del Chiari como de lasiringomielia.Discusión. En la mayoría de los casos publicados lasiringomielia se debía a la obstrucción de LCR en el agujeromagno ocasionada por el propio quiste aracnoideo.Hemos encontrado solamente un caso previo en el queel quiste de fosa posterior era causante del descenso delas amígdalas cerebelosas y éste, a su vez, producía lasiringomielia.Conclusiones. Los quistes aracnoideos de fosa posteriorpueden producir descenso de las amígdalas ysiringomielia. En nuestra opinión, el manejo de estospacientes debe de encaminarse no sólo a descomprimirel agujero magno, sino también a comunicar las paredesdel quiste ya que éste parece ser el principal responsabledel complejo cuadro de siringomielia en estos casos

Background. Some reports have documented posteriorfossa cysts resulting in syringomyelic obstruction ofcerebrospinal fluid (CSF) flow caused by cyst displacementwithin the foramen magnum. Rarely the syringomyeliais caused by acquired Chiari malformationdue to a retrocerebellar arachnoid cyst.Objective. To report the case of a 38-year-old manwith hydrocephalus and syringomyelia, who was foundto have a Chiari malformation secondary to a posteriorfossa arachnoid cyst. After endoscopic third ventriculostomy,the patient was submitted to foramen magnumdecompression and arachnoid cyst removal that werefollowed by resolution of both the Chiari malformationand the syringomyelia.Discussion. In most published cases the syringomyeliahas been attributed to obstruction of CSF flow at theforamen magnum by the arachnoid cyst itself. There isonly one previous report of a posterior fossa arachnoidcyst producing tonsillar descent and syringomyelia.Conclusions. Posterior fossa arachnoid cysts canresult in acquired Chiari malformation and syringomyelia.In our view, the management of these patientsshould be directed at decompressing the foramenmagnum and include the removal of the walls of thecoexistent arachnoid cyst as it seems to be the crucialfactor that accounts for the development of the syringomyeliathat these patients present