Chiari Malformation Type III Treated with Primary Closure

Abstract

Chiari malformations represent various clinical and anatomic processes that entail varying degrees of involvement of the rhombencephalon (hindbrain). The type III Chiari malformation is distinctly rare. Chiari described only one case of his type III deformity, which is indicative of its rarity. We report a case of Chiari malformation type III, a female child who presented to us at the age of 1 month with a swelling at the back of the neck since birth. MRI revealed a Chiari type III malformation with a high cervical meningoencephalocele along with herniation of cerebellar tissue through the bony defect in the posterior elements of the C2 vertebra. At surgery, the meningoencephalocele was excised along with the gliotic cerebellar tissue, and a posterior fossa decompression along with augmentation duraplasty was done. The postoperative period was uneventful. The case and relevant literature is reviewed.

Chiari Malformation Type III and Results of Surgery: A Clinical Study

“…Although we could not find any statistical data, we believe that a high number of births with neural tube defects give rise to an increase in CM III in our country. There was also a male predominance, as in the literature [6, 7, 13, 14, 19]. Most of our patients were newborns.…”

“…Aribal et al [11] and Çakırer [18] each reported 2 cases. Other papers describe only 1 case each [7,8,9,10,12,13,14,15,16,17, 19, 20]. The percentage of CM III versus all Chiari malformations (4.4%) in our series is higher than in the literature (0.6–4%) [5,6,7,8].…”

“…There are only 34 cases described in the literature with this malformation [2, 4,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20] (table 3). The largest series, involving 9 cases, was reported by Castillo et al [2] in 1992.…”

“…In the current description, CM III appears with hindbrain herniation into a low occipital (below the inion) and/or high cervical osseous defect as occipitocervical cephalocele (encephalocele) [1, 2,4,5,6,7,8]. In the review of the literature, the encephaloceles were located both at the low occipital bone and high cervical spine in 12 patients [2, 14,16,17,18,19,20]. In 7 patients, there was a low occipital and in another 6 patients, a high cervical encephalocele [2, 7,9,10,11,12,13, 15].…”

“…Owing to its exceptional occurrence, little is known about the natural history, therapeutic approach and postoperative course of this specific type of Chiari malformation. There are some reports on magnetic resonance imaging (MRI) appearances of CM III, but they do not present sufficient clinical data and surgical follow-up results [2, 4, 6, 7,9,10,11,12,13,14,15,16,17,18,19,20]. …”