Management and Outcome of a Neurologically Intact Infant with Pierre Robin Sequence and Dandy-Walker Variant Diagnosed with Large Hemispheric Brain Abscess ­ Case Report

Pierre Robin sequence (PRS) is a condition consisting of three essential components: micrognathia or retrognathia, cleft palate, and glossoptosis. It can be part of multiple congenital anomalies. We present the case and outcome of a 3-month-old clinically stable patient who has PRS with Dandy-Walker variant ­ which is a rare presentation in the literature ­ with a large right hemispheric brain abscess, treated with multiple minimally-invasive surgical drainage procedures with adjuvant antibiotics.

Variante de Dandy Walker: reporte de un caso / Dandy-Walker variant: Case report

Introducción: La variante de Dandy Walker se define como una hipoplasia variable del vermis cerebeloso, con o sin aumento de la fosa posterior y sin elevación del tentorio. Objetivo: Describir el caso de una enfermedad poco frecuente y hacer énfasis en la necesidad de precisar la etiología de malformaciones prenatales en niños que son clasificados erróneamente como parálisis cerebral secundaria a asfixia, así como su manejo multidisciplinario. Caso clínico: Paciente varón, de 8 años de edad, con antecedentes de parálisis cerebral infantil, epilepsia y retraso del desarrollo, que fue ingresado por historia de convulsiones tónico-clónicas. Durante su hospitalización presentó múltiples episodios convulsivos, controlados con anticonvulsivantes. Se realizó tomografía computarizada, observándose comunicación entre la cisterna magna y el cuarto ventrículo; este último aumentado de tamaño. Además, el vermis del cerebelo presentaba hipoplasia parcial, siendo estos hallazgos compatibles con una variante del síndrome Dandy Walker. Conclusión: La variante de Dandy Walker puede ser sintomática o asintomática, y las imágenes encontradas no necesariamente se relacionan con las alteraciones del desarrollo, debido a los múltiples síndromes y alteraciones cromosómicas vinculadas a este cuadro. La presentación clínica y el pronóstico dependen de las alteraciones presentes. Por ello, es importante un manejo multidisciplinario considerando que el tratamiento depende de los síntomas presentados.

Introduction: Dandy Walker variant is defined by a variable hypoplasia of the cerebellar vermix with or without posterior fossa increase and without tentorium elevation. Objective: describe the case of a rare disease and emphasise the need to clarify the aetiology of prenatal malformations, as well as its multidisciplinary management. Case report: A male patient, 8 years of age, with a history of Infantile Cerebral Palsy and epilepsy, who was admitted with a history of tonic-clonic seizures. He was admitted due to psycho-motor developmental delay. During his hospitalisation, he had multiple seizure episodes, controlled with anticonvulsants. A computerized tomography was performed, in which communication was observed between the cisterna magna and fourth ventricle (the latter increased in size). In addition, the cerebellar vermix showed a partial hypoplasia. All these findings were compatible with a variant of the Dandy Walker syndrome. Conclusion: Dandy Walker variant may be asymptomatic and the images found may not indicate them as the cause of developmental disorders, due to its association with multiple syndromes and chromosomal abnormalities. Clinical presentation and prognosis depends on the related disorders, and a multidisciplinary approach is important, because the treatment depends on the symptoms presented.

Midline cerebellar cystic schwannoma : a case report.

An extremely unusual case of a cystic schwannoma in the region of the inferior vermis and posterior to the fourth ventricle in a fifteen year old boy is reported. The cystic tumour caused partial obstruction to the outflow of cerebrospinal fluid from fourth ventricle and resulted in development of supratentorial hydrocephalus. On investigations, the schwannoma simulated a Dandy-Walker cyst. The boy presented with symptoms of increased intracranial pressure. On surgery, the lesion was not arising from any cranial nerve, nor was it attached to brain parenchyma, blood vessel or to the dura. The possible histogenesis of the cystic schwannoma in a rare location is discussed.

Dandy-walker malformation associated with syringomyelia: an unusual presentation

We are reporting a patients with a Dandy-Walker malformation who underwent insertion of a cysto-peritoneal shunt as a neonate. At the age of 17, she presented with difficulty in walking and black-out attacks for 6 months before she was rushed to the hospital in a coma. Following an urgent CT scan, the shunt was revised and was found to be partially obstructed by omentum. After recovery, the patients demonstrated newly developed cerebellar and pyramidal signs. MRI showed evidence of cervical syringomyelia with no brain stem involvement. A second MRI carried out 2 weeks after shunt revision, showed collapse of the syringomyelia that correlated well with the patients clinical improvement. In addition to the rare association of Dandy-Walker malformation and syringomyelia, this case also demonstracts an unusual presentation of shunt malfunction. Revision of the shunt resulted in both clinical and radiological improvement. We believe that cerebrospinal fluid shunting should be the initial step in the management of those cases