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Objective:The authors evaluate the significance of atlantoaxial instability in the management of idiopathic syringomyelia.Background:We recently observed that atlantoaxial dislocation can be present even when the atlantodental interval was within normal range. Atlantoaxial instability can be identified on the basis of facetal mal-alignment or even by direct observation of status of joint during surgery. Our observations are discussed in nine patients where we identified and treated atlantoaxial instability in cases that would otherwise be considered as having “idiopathic” syrinx.Materials and Methods:The authors report experience with nine cases that were diagnosed to have “idiopathic” syrinx. The main bulk of the syrinx was located in the cervico-dorsal spinal region in all cases. One patient had been treated earlier by syringo-subarachnoid shunt surgery and one patient had undergone foramen magnum decompression.Results:On radiological evaluation, eight patients had posterior atlantoaxial facetal (Type B) dislocation. In one patient there was no facetal mal-alignment and was labeled to have axial or central (Type C) facetal instability. All patients were treated by atlantoaxial fixation. All patients improved symptomatically in the immediate postoperative period and the improvement was progressive and sustained on follow-up. In one case, the size of syrinx reduced in the immediate postoperative imaging. In the period of follow-up (range 6–42 months - average 19 months), reduction in the size of syrinx was demonstrated on imaging in three cases.Conclusions:The positive clinical outcome suggests that atlantoaxial instability may be the defining phenomenon in development of previously considered “idiopathic” syringomyelia.

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We describe two rare cases of frontal cystic lesions presenting with symptoms and signs of raised intracranial pressure. Both had a preoperative diagnosis of an arachnoid cyst and were subjected to a craniotomy with marsupialization of the cyst. However, the histology confirmed them to be an endodermal cyst (EC) on both occasions. Both the patients have been closely followed with no recurrence of symptoms. ECs of the central nervous system are usually reported in the spinal canal, mid-line posterior fossa, and the suprasellar regions. Supratentorial and non-midline ECs are rare, with only about 22 cases previously reported in literature. We discuss both the cases and review the relevant literature.

A 22-year-old female, a known case of neurofibromatosis 1 (NF1), presented with a congenital swelling in the left occipital region. She had developed recent onset dysphagia and localized occipital headache. Neuroradiology revealed a left occipital meningoencephalocele and a left parapharyngeal meningocele. This was associated with ventriculomegaly. She was advised on cranioplasty along with duraplasty which she denied. She agreed to a lumbar-peritoneal shunt. She described a dramatic improvement in her symptoms following the lumbar-peritoneal shunt. Occipital dysplasias, though uncommon, have been reported in the literature. We review this case and its management and discuss relevant literature on occipital dysplasias in NF1.