An eye on the future for defeating hydrocephalus, ciliary dyskinesia-related hydrocephalus: review article.

Congenital hydrocephalus affects approximately one in 1000 newborn children and is fatal in approximately 50% of untreated cases. The currently known management protocols usually necessitate multiple interventions and long-term use of healthcare resources due to a relatively high incidence of complications, and many of them mostly provide a treatment of the effect rather than the cause of cerebrospinal fluid flow reduction or outflow obstruction. Future studies discussing etiology specific hydrocephalus alternative treatments are needed. We systematically reviewed the available literature on the effect of ciliary abnormality on congenital hydrocephalus pathogenesis, to open a discussion on the feasibility of factoring ciliary abnormality in future research on hydrocephalus treatment modalities. Although there are different forms of ciliopathies, we focused in this review on primary ciliary dyskinesia. There is growing evidence of association of other ciliary syndromes and hydrocephalus, such as the reduced generation of multiple motile cilia, which is distinct from primary ciliary dyskinesia. Data for this review were identified by searching PubMed using the search terms 'hydrocephalus,' 'Kartagener syndrome,' 'primary ciliary dyskinesia,' and 'immotile cilia syndrome.' Only articles published in English and reporting human patients were included. Seven studies met our inclusion criteria, reporting 12 cases of hydrocephalus associated with primary ciliary dyskinesia. The patients had variable clinical presentations, genetic backgrounds, and ciliary defects. The ependymal water propelling cilia differ in structure and function from the mucus propelling cilia, and there is a possibility of isolated non-syndromic ependymal ciliopathy causing only hydrocephalus with growing evidence in the literature for the association ependymal ciliary abnormality and hydrocephalus. Abdominal and thoracic situs in children with hydrocephalus can be evaluated, and secondary damage of ependymal cilia causing hydrocephalus in cases with generalized ciliary abnormality can be considered.

Migration of the Distal Catheter of Ventriculoperitoneal Shunts in Pediatric Age Group: Case Series.

BACKGROUND: Ventriculoperitoneal (VP) shunting is the most commonly performed procedure in the treatment of hydrocephalus. VP shunt migration can occur at different sites. The aim of the study was to present different sites of abnormal distal shunt location, pathophysiology, and the management in each situation. METHODS: Between 2014 and 2017, all patients with hydrocephalus in the Department of Neurosurgery, Cairo University, were gathered prospectively. All pediatric patients below the age of 12 years with shunt migration of the distal end of the VP shunt were identified. RESULTS: Of 1092 patients operated on by the VP shunt between 2014 and 2017, 15 presented with shunt dysfunction because of distal shunt migration (6 anal, 3 scrotal, 1 colon, 1 peroral, 1 upper lumbar extrusion, 1 paraspinal, 1 penile, and 1 umbilical). Especially upper lumbar extrusion and paraspinal shunt location are extremely rare. All the 15 patients were treated successfully with VP shunts and prospectively followed until they presented with complications on different occasions. CONCLUSIONS: Peritoneal complications are among the most common causes of VP failure. We present a rare complication where the shunt migrates outside the peritoneal cavity elsewhere with ambiguous pathogenesis. Special considerations have to be appointed during the shunt revision surgeries of these cases.