Staged Neuroendoscopic Management of a Dandy Walker Malformation Case Presenting with Complex Hydrocephalus
Keywords:Ventriculoperitoneal shunt valves, Hydrocephalus, endoscopic, Lushka and Magendie foraminoplasty, tentorium fenestration
Backgroud: Dandy-Walker malformation is a rare congenital condition characterized by alteration of posterior fossa anatomy. Hydrocephalus is a common finding in these patients. VP-shunt placement remains the treatment of choice. Endoscopic Third Ventriculostomy (ETV) is an acceptable alternative for older children. Other techniques have also been proposed.
Case presentation: A full-term infant presented with signs of hydrocephalus. An endoscopic foraminoplasty of the posterior fossa cyst was conducted as a first stage. The cyst’s volume was reduced, without any changes of the supratentorial ventricular dimensions. A month post-surgery signs of hydrocephalus reoccurred. An ETV was performed alongside a fenestration of the tentorium. The toddler was discharged in good condition with amelioration of her symptoms. One month later she presented with recurrent hydrocephalus and subdural hygromas. A shunt was placed in the posterior fossa under endoscopic guidance, and an additional one was introduced in the subdural space. The patient was followed-up for 18 months without any further need for treatment. VP-shunt placement is the treatment of choice.
Discussion: Lushka and Magendie foraminoplasty seems to be ineffective. ETV should be reserved for older children. The ventriculoscope can be employed for positioning the VP-shunt in a specific anatomical region. Subdural hygromas may present after neuroendoscopic treatment. A staged approach may be mandatory for complex hydrocephalus treatment.
Spennato P, Mirone G, Nastro A, Buonocore MC, Ruggiero C, Trischitta V, et al. Hydrocephalus in Dandy-Walker malformation. Childs Nerv Syst. 2011;27(10):1665-81.
Jaspan T. New concepts on posterior fossa malformations. Pediatr Radiol. 2008;38 Suppl 3:S409-14.
Imataka G, Yamanouchi H, Arisaka O. Dandy-Walker syndrome and chromosomal abnormalities. Congenit Anom (Kyoto). 2007;47(4):113-8.
Spennato P, del Basso de Caro ML, Buonocore MC, Cascone D, Cinalli G. Hydrocephalus and the Dandy-Walker Malformation. In: Cinalli G, Özek M, Sainte-Rose C, editors. Pediatric Hydrocephalus: Springer; 2019. p. 685-721.
Achiron R, Achiron A. Transvaginal ultrasonic assessment of the early fetal brain. Ultrasound Obstet Gynecol. 1991;1(5):336-44.
Hirsch JF, Pierre-Kahn A, Renier D, Sainte-Rose C, Hoppe-Hirsch E. The Dandy-Walker malformation. A review of 40 cases. J Neurosurg. 1984;61(3):515-22.
Fischer EG. Dandy-Walker syndrome: an evaluation of surgical treatment. J Neurosurg. 1973;39(5):615-21.
Takami H, Shin M, Kuroiwa M, Isoo A, Takahashi K, Saito N. Hydrocephalus associated with cystic dilation of the foramina of Magendie and Luschka. J Neurosurg Pediatr. 2010;5(4):415-8.
Theologou M, Natsis K, Kouskouras K, Chatzinikolaou F, Varoutis P, Skoulios N, et al. Cerebrospinal Fluid Homeostasis and Hydrodynamics: A Review of Facts and Theories. Eur Neurol. 2022;85(4):313-25.
Kumar R, Jain MK, Chhabra DK. Dandy-Walker syndrome: different modalities of treatment and outcome in 42 cases. Childs Nerv Syst. 2001;17(6):348-52.
Yengo-Kahn AM, Wellons JC, Hankinson TC, Hauptman JS, Jackson EM, Jensen H, et al. Treatment strategies for hydrocephalus related to Dandy-Walker syndrome: evaluating procedure selection and success within the Hydrocephalus Clinical Research Network. J Neurosurg Pediatr. 2021:1-9.
Yuceer N, Mertol T, Arda N. Surgical treatment of 13 pediatric patients with Dandy-Walker syndrome. Pediatr Neurosurg. 2007;43(5):358-63.
Habashy K, El Houshiemy MN, Alok K, Kawtharani S, Shehab H, Darwish H. Membranous obstruction of the foramen of magendie: A case report, literature review and recommendations. Interdisciplinary Neurosurgery. 2022;27.
Longatti P, Fiorindi A, Feletti A, Baratto V. Endoscopic opening of the foramen of magendie using transaqueductal navigation for membrane obstruction of the fourth ventricle outlets. Technical note. J Neurosurg. 2006;105(6):924-7.
Theologou M, Kouskouras K, Natsis K, Varoutis P, Zaggelidou E, Tsonidis C. Microanatomic Morphometric Characteristics of the Third Ventricle Floor. Brain Sci. 2023;13(4).
Warf BC, Dewan M, Mugamba J. Management of Dandy-Walker complex-associated infant hydrocephalus by combined endoscopic third ventriculostomy and choroid plexus cauterization. J Neurosurg Pediatr. 2011;8(4):377-83.
Mohanty A. Endoscopic third ventriculostomy with cystoventricular stent placement in the management of dandy-walker malformation: technical case report of three patients. Neurosurgery. 2003;53(5):1223-8; discussion 8-9.
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