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.

Normal ventricular-CSF may comfound the diagnosis of tuberculous meningitis hydrocephalus.

BACKGROUND: The standard procedure for the diagnosis of central nervous system (CNS) infections consists of cerebrospinal fluid (CSF) sampling, which is usually accomplished by a lumbar puncture. However, in some patients presenting with acute hydrocephalus submitted to immediate CSF drainage, the fluid is customarily obtained from the placed draining system. In addition, the CSF obtained from the ventricular and lumbar spaces in some cases may show unusual differences, both in physiological and pathological conditions. ILLUSTRATIVE CASES: We report two children who presented with confounding results in the initial studies of their ventricular and lumbar CSF who were subsequently diagnosed with tuberculous meningitis, causing delay in diagnosis and treatment. AIM. By reporting these cases, we wanted to alert the treating physician about the possibility of this discrepancy to avoid the delayed diagnosis and management of the affected patients. DISCUSSION: We comment on the possible pathophysiological mechanisms that may result in this dissociation in ventricular and lumbar CSF composition. CONCLUSIONS; Normal results in CSF studies, especially those of the ventricular fluid, do not always rule out the presence of tuberculous meningitis. We suggest obtaining a CSF sample from the lumbar subarachnoid space in doubtful, or suspicious, cases of CNS infection even in the presence of a normal ventricular CSF.

Normal ventricular-CSF may confound the diagnosis of tuberculous meningitishydrocephalus / LCR ventricular normal: un factor de confusión en el diagnóstico de hidrocefalia por meningitis tuberculosa

Background. The standard procedure for the diagnosisof central nervous system (CNS) infections consistsof cerebrospinal fluid (CSF) sampling, which isusually accomplished by a lumbar puncture. However,in some patients presenting with acute hydrocephalussubmitted to immediate CSF drainage, the fluid is customarilyobtained from the placed draining system.In addition, the CSF obtained from the ventricularand lumbar spaces in some cases may show unusualdifferences, both in physiological and pathologicalconditions.Illustrative cases. We report two children who presentedwith confounding results in the initial studiesof their ventricular and lumbar CSF who were subsequentlydiagnosed with tuberculous meningitis, causingdelay in diagnosis and treatment.Aim. By reporting these cases, we wanted to alert thetreating physician about the possibility of this discrepancyto avoid the delayed diagnosis and management (..) (AU)

Antecedentes. El procedimiento habitual para eldiagnóstico de infección del sistema nervioso central(CNS) consiste en el estudio del liquido cefalorraquídeo(LCR) mediante la realización de una punción lumbar.Sin embargo, en pacientes que debutan con un cuadrode hidrocefalia aguda que son tratados de urgenciamediante la inserción de un sistema de drenaje deLCR, el líquido es rutinariamente obtenido desde elsistema derivativo implantado. Pero, en ciertos casos,el LCR ventricular y lumbar analizados pueden mostrardiferencias significativas, tanto en condicionesfisiológicas como patológicas.Casos ilustrativos. Se describen los casos de dosniños que presentaron resultados dispares en losestudios iniciales del LCR ventricular y lumbar, enlos que se demostró posteriormente que padecíanuna hidrocefalia por meningitis tuberculosa, lo que (..) (AU)

[Coccygeal dermal sinus: Clinical relevance and management]. / Sinus dérmico sacrocoxígeo: importancia clínica y manejo.

INTRODUCTION: The presence of a dermal sinus on a child's back is a common cause for paediatric neurosurgical consultation. The relative frequency of these lesions and fears on their possible clinical consequences enhances the importance of early diagnosis and treatment. With this work we aimed to search for the most relevant clinical features that might indicate an intradural penetration of the lesions and that may advise performing neuroimaging studies and a paediatric neurosurgical consultation. PATIENTS AND METHODS: The records pertaining to children diagnosed with sacro-coccygeal pits seen at our institution during the years 2005-2009 were reviewed. A Pub Med search of the most relevant articles on the subject was also performed. RESULTS: Thirty-two children diagnosed with sacro-coccygeal pits were seen at our institution in the study period. Most cases had been sent for neurosurgical consultation by their paediatricians, paediatric surgeons or paediatricians of the emergency services in our region. Seventeen patients were submitted to some neuroimaging study and 15 were evaluated only by physical examination. In no patient was an intradural penetration of the lesion observed. The lack of reports on coccygeal sinuses probably reflects the unimportance given to these lesions in the absence of neurological involvement. CONCLUSIONS: A detailed physical examination can reduce the number of neuroimaging studies, an intergluteal localization of the sinus being the most important diagnostic clue. Doubtful cases of an intradural penetration of the lesion can be further evaluated by ultrasound as a preliminary screening tool and by magnetic resonance and neurosurgical consultation if deemed necessary.

[Spinal cord tethering in myelomeningocele and lipomeningocele patients: the second operation]. / Reanclaje medular en pacientes con mielomeningocele y lipomeningocele: la segunda operación.

BACKGROUND: Spinal cord rethetering can occur after the primary surgical repair of myelomeningoceles (MMC) and lipomeningoceles (LMC) and produce devastating physical and psychological consequences. The inadvertent introduction of skin elements at the time of the initial surgery can lead to the growth of intraspinal epidermoid or dermoid cysts. OBJECTIVES: To review the incidence of spinal cord tethering following surgery for open and occult spinal dysraphism and to analyze factors that might influence the appearance of this complication. We also aimed to search technical measures at the time of the primary operation that might prevent the occurrence of symptomatic cord retethering. MATERIAL AND METHODS: We reviewed the medical records of patients submitted to surgical repair of MMC (n=162) or occult spinal dysraphism (n= 54) during the period 1975-2005 who developed symptomatic tethered cord syndrome. RESULTS: Eleven of 162 (6.79%) patients with MMC and 2 of 54 (3.7%) with LMC developed clinical symptoms and signs of spinal cord tethering after intervals ranging from 2 to 37 years after the initial surgical repair of their back lesions. Indications for surgical re-exploration were based mainly on clinical grounds. Postoperative fibrosis was a constant finding in all instances. Other surgical findings included inclusion tumors of cutaneous origin (n=3), lumbar canal stenosis (n= 2), foreign body reactions (n= 2), residual lipoma (n= 1), and a tight hyalinized filum (n=1). Interestingly, 3 of 162 (or 1.85%) myelomeningoceles were found to harbor an intradural epidermoid tumor at the time of spinal cord dethetering, accounting for an incidence of cutaneous inclusion tumors of 27% in cases of post- MMC repair tethering. After a mean follow-up time of 5.5 years, eight patients were improved, two were unchanged and one was worsened. CONCLUSIONS: Neurological deterioration is not a necessary consequence of the natural history of patients with MMC or LMC. Early or late clinical deterioration can be due to spinal cord re-tethering and deserves timely investigation and surgical exploration. Results of surgical intervention were rewarding as 92% of the patients showed improvement or stabilization in their otherwise deteriorating condition. We also report two infrequent causes of spinal cord deterioration: lumbar canal stenosis and intense foreign-body reactions to implanted materials.

Reanclaje medular en pacientes con mielomeningocele y lipomeningocele: la segunda operación / Spinal cord tethering in myelomeningocele and lipomeningocele patients: the second operation

Antecedentes. La médula espinal puede fijarse tras la reparación inicial en pacientes operados demielomeningocele (MMC) y lipomeningocele (LMC), produciendo graves lesiones físicas y psicológicas. Asimismo, la introducción accidental de restos cutáneos durante la reparación de estas lesiones puede dar lugar al desarrollo de tumores intraespinales de estirpe cutánea. Objetivos. Averiguar la incidencia del anclaje medular tras la cirugía de MMC y LMC y analizar los factores que puedan explicar su aparición. También, investigamos las maniobras técnicas durante la operación primaria susceptibles de evitar la aparición del síndrome de médula fijada. Pacientes y métodos. Revisión retrospectiva de las historias de los pacientes operados de MMC (n=162) y de espina bífida oculta (n=54) en el período 1975-2005 que desarrollaron cuadros de anclaje medular sintomático. Resultados. Once pacientes con MMC (6,79%) y dos con LMC (3,7%) presentaron manifestaciones de anclaje medular tras intervalos de 2 a 37 años después de la reparación primaria. Las indicaciones de reintervención se basaron fundamentalmente en criterios clínicos. Un hallazgo constante fue la fibrosiscicatricial que estuvo presente en todos los casos. Otros hallazgos operatorios causantes de la fijación medular consistieron en tumores cutáneos de inclusión (n=3),reacciones de cuerpo extraño (n=2), estenosis del canal lumbar (n=2), restos de lipoma (n=1), y filumhialinizado (n=1). En 3 casos de MMC se encontróademás un quiste epidermoide intradural (1,85% delos MMC), lo que supone una tasa de epidermoides en la reintervención de MMC de 27%. El periodo medio de seguimiento fue de 5,5 años y los resultados fueron: mejoría en 8, dos no experimentaron cambios, y uno sufrió empeoramiento. Conclusiones. El deterioro neurológico de los pacientes operados de MMC o LMC no constituye una consecuencia obligada o parte de la historia natural de estos procesos. El deterioro, precoz o tardío, puede estar motivado por el anclaje posquirúrgico de la médula espinal. Ello hace necesario realizar un seguimiento periódico de estos pacientes, acompañado de los oportunos estudios de neuroimagen y, en su caso, de exploración quirúrgica. Los resultados fueron satisfactorios, ya que el 92% de los pacientes reoperados experimentaron mejoría o estabilización de su enfermedad. Se describen además dos causas infrecuentemente descritas de deterioro tardío: la estenosis del canal y las reacciones fibrosas de cuerpo extraño a materiales implantados

Background. Spinal cord rethetering can occur after the primary surgical repair of myelomeningoceles (MMC) and lipomeningoceles (LMC) and produced evastating physical and psychological consequences.The inadvertent introduction of skin elements at the time of the initial surgery can lead to the growth of intraspinal epidermoid or dermoid cysts. Objectives. To review the incidence of spinal cord tethering following surgery for open and occult spinaldys raphism and to analyze factors that might influence the appearance of this complication. We also aimed to search technical measures at the time of the primary operation that might prevent the occurrence of symptomatic cord retethering. Material and methods. We reviewed the medical records of patients submitted to surgical repair of MMC (n=162) or occult spinal dysraphism (n= 54) during the period 1975-2005 who developed symptomatic tethered cord syndrome. Results. Eleven of 162 (6.79%) patients with MMCand 2 of 54 (3.7%) with LMC developed clinical symptoms and signs of spinal cord tethering after intervals ranging from 2 to 37 years after the initial surgical repair of their back lesions. Indications for surgical re-exploration were based mainly on clinical grounds. Postoperative fibrosis was a constant finding in all instances. Other surgical findings included inclusion tumors of cutaneous origin (n=3), lumbar canal stenosis(n= 2), foreign body reactions (n= 2), residual lipoma (n= 1), and a tight hyalinized filum (n=1). Interestingly,3 of 162 (or 1.85%) myelomeningoceles were found to harbor an intradural epidermoid tumor at the time of spinal cord dethetering, accounting for an incidence of cutaneous inclusion tumors of 27% in cases of post-MMC repair tethering. After a mean follow-up time of 5.5 years, eight patients were improved, two were unchanged and one was worsened. Conclusions. Neurological deterioration is not a necessary consequence of the natural history of patients with MMC or LMC. Early or late clinical deterioration can be due to spinal cord re-tethering and deserves timely investigation and surgical exploration. Results of surgical intervention were rewarding as 92% of the patients showed improvement or stabilization in their otherwise deteriorating condition. We also report two infrequent causes of spinal cord deterioration: lumbar canal stenosis and intense foreign-body reactions to implanted materials

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

Retrograde migration of ventriculoperitoneal shunt to the neck. Case report.

We report a patient, given a ventriculoperitoneal shunt at the neonatal period, who presented with a painless subcutaneous mass on his neck. Plain radiographs of the cervical region showed that the swelling was made up by a migrated and coiled peritoneal catheter. We briefly discuss the proposed mechanism for this complication and suggest that this occurrence must be taken into account at the time of evaluating a patient with shunt malfunction.

Retrograde migration of ventriculoperitoneal shunt to the neck. Case report

Describimos el caso de un adulto que había sido tratado de recién nacido mediante la colocación de una válvula ventrículo peritoneal que se presentó con una tumoración indolora en el cuello. La radiografía simple demostró que la masa estaba formada por el catéter peritoneal que había emigrado cranealmente y se había enrollado en el subcutáneo. Discutimos brevemente los posibles mecanismos para explicar esta complicación y sugerimos que esta posibilidad sea tenida en cuenta a la hora de valorar un paciente con mal función de una derivación de LCR

We report a patient, given a ventriculoperitoneal shunt at the neonatal period, who presented with a pain less subcutaneous mass on his neck. Plain radiographs of the cervical region showed that the swelling was made up by a migrated and coiled peritoneal catheter. We briefly discuss the proposed mechanism for this complication and suggest that this occurrence must be taken into account at the time of evaluating a patient with shunt malfunction

[Syndromes of overdrainage of ventricular shunting in childhood hydrocephalus]. / Síndromes de hiperdrenaje de las válvulas en hidrocefalia infantil.

Overdrainage in ventricular shunting constitutes a difficult to prevent and to treat complication. The authors reviewed a retrospective series of 512 children submitted to a ventricular shunting procedure aimed at analysing factors influencing this type of complication. The causes for the hydrocephalus were congenital (n=172), post-myelomeningocele (n=123), posthemorrhagic (n=103), tumoral (n=64), postmeningitis (n=40) and posttraumatic (n=10). Eighty-eight children (17.8%) evolved with a complication related to the excessive function of the valve. The authors investigated the relationship between hydrocephalus' etiology and type of overdrainage syndromes. The most frequent complication was ventricular catheter block (n=50), followed by symptomatic slit ventricle syndrome (SVS) (n=19), subdural hematoma (n=10) and trapped fourth ventricle (n=9). There were no statistical differences regarding complications for each etiologic subset of hydrocephalus. SVS occurred in 19 children (3.71%), a low rate according to the current literature. Posthemorrhagic and postinfectious hydrocephalus grouped together showed a higher rate of SVS (p=0.005), a feature that we attributed to the cerebral destruction caused by these two conditions. Treatment of SVS was complex and required diverse procedures, applied in an escalated way, which included five decompressive craniectomies. The authors suggest avoiding, as much as possible, the use of ventricular shunts, and recommend the alternative use of new technology valves and neuroendoscopic procedures.

Occipital encephalocele associated with a dermoid cyst.

The case of a neonate with a dermoid cyst originating within an occipital encephalocele is reported. The finding of the tumor was made during the routine repair of the meningoencephalocele. To our knowledge this is the first time that this association has been described.

Atretic cephalocele: the tip of the iceberg.

Atretic cephalocele appears as an unimportant and benign lesion. This malformation consists of meningeal and vestigial tissues (arachnoid, glial, or central nervous system rests). The authors report the findings in 16 cases (seven parietal and nine occipital) of rudimentary cephaloceles. Twelve patients presented with associated brain abnormalities detected by either computerized tomography (CT) or magnetic resonance imaging (MR). Nine lesions also exhibited an anomalous vascular component demonstrated by CT or MR imaging or at surgery. The existence of this tiny malformation in five cases was the main diagnostic clue to a severe complex of cerebral anomalies, namely cerebro-oculomuscular (Walker-Warburg) syndrome. An occipital location of the atretic cephalocele was associated with the worst prognosis, with only two children developing normally. However, a parietal location carried a better prognosis, which is contrary to the outcome reported in the current literature. The authors classify atretic cephaloceles into two types based on histological examination of the surgical specimens, and suggest that these types represent different stages in the development of this malformation. It is concluded that, in the evaluation of the atretic cephalocele, the neurosurgeon is obliged to proceed to a detailed neuroradiological study of the patient and that the prognosis does not depend on the existence of the cephalocele itself, but rather on associated "occult" brain anomalies.