Hydranencephaly corresponds (in its major and bilateral form) to the total is-chemic destruction (most often occurring between 3 and 6 months of preg-nancy) of the normally developed cerebral hemispheres which are replaced by a vast liquid cavity surrounded by the meninges. However, there are the basal ganglia, the brainstem and the cerebellum. We report and describe a case of an infant, aged 9 months received in Medical Imaging for All for a brain scan on macrocephaly. In the CT-scan, we note the absence of the cerebral hemi-spheres replaced by LCS, without peripheral cortical plate except at the medial part of the temporal lobes, constituting a cerebral water pocket. The thalamus, cerebellum, brain stem and falx cerebri are intact. The residual temporal and occipital lobes. Exaggerated opening of the fontanelles. We concluded with hydranencephaly and we completed by transcranial ultrasound. We find an anechoic aspect of the cranial cavity, the presence of the falx of the brain; the persistence of cerebral remnants in the occipital region, medial of the temporal lobes vascularized by the anterior and posterior cerebral arteries and their anastomoses, the posterior fossa, the tent of the cerebellum, the basal nuclei and the brainstem which are preserved; the cranial vault is deformed with macrocephaly by continuous production of LCS.
Cite this paper
Diallo, B. I. , Ndudi, P. , Mazoba, T. K. , Mbongo, A. T. , Mvila, G. L. , Tshibola, J. M. , Kuzoma, L. , Minouche, C. , Anelia, P. L. , Kola, R. S. , Tshikwela, M. L. and Molua, A. A. (2024). Hydranencephaly in an Infant: About a Case at Medical Imaging for All (IMT) in Kinshasa/The Democratic Republic of Congo. Open Access Library Journal, 11, e941. doi: http://dx.doi.org/10.4236/oalib.1110941.
Khalid, M., Khalid, S., Zaheer, S., Redhu, N. and Ekramullah. (2012) Hydranencephaly: A Rare Cause of an Enlargement Head Size in an Infant. North American Journal of Medicine and Science, 4, 520-522.
Sandberg, D.I., Chamiraju, P., Zoeller, G., Bhatia, S. and Ragheb, J. (2012) Endoscopic Choroid Plexus Coagulation in Infants with Hydranencephaly or Hydrocephalus with a Minimal Cortical Coat. Pediatrician Neurosurg, 48, 6-12. https://doi.org/10.1159/000339850
Malheiros, J.A., Trivelato, F.P., Oliveira, M.M., Gusmao, S., Cochrane, D.D. and Steinbok, P. (2010) Endoscopic Choroid Plexus Cauterization versus Ventriculoperitoneal Shunt for Hydranencephaly and near Hydranencephaly: A Prospective Study. Neurosurgery, 66, 459-464. https://doi.org/10.1227/01.NEU.0000365264.99133.CA
Pavone, P., Praticò, A.D., Vitaliti, G., Ruggieri, M., Rizzo, R., Parano, E., Pavone, L., Pero, G. and Falsaperla, R. (2014) Hydranencephaly: Cerebral Spinal Fluid Instead of Cerebral Mantles. Italian Journal of Pediatrics, 40, Article No. 79. https://doi.org/10.1186/s13052-014-0079-1
Merker, B. (2008) Life Expectancy in Hydranencephaly. Clinical Neurology and Neurosurgery, 110, 213-214. https://doi.org/10.1016/j.clineuro.2007.11.011
Ray, C., Mobley, J., Thompson, M. and Nagy, L. (2015) Hydranencephaly: Considering Extended Survival and Treatment by Endoscopic Choroid Plexus Coagulation. Turkish Neurosurgery, 25, 788-792. https://doi.org/10.5137/1019-5149.JTN.10453-14.1
Mbaye, M., Sodjinou, N., SY El-Hadj, C.N., Wagué, D., Thiam, A.B., Ndoye, N., et al. (2022) Hydranencephaly: A pathology with No Way out! African Journal of Neurological Sciences, 41, 59-63. http://ajns.paans.org
Mcabee G.N., Chan, A. and Erde, E.L. (2000) Prolonged Survival with Hydrancephaly: Report of Two Patients and Literature Review. Pediatric Neurology, 23, 80-84. https://doi.org/10.1016/S0887-8994(00)00154-5
Hino-Fukuyo, N., Togashi, N., Takahashi, R., Saito, J., Inui, T., Endo, W., Sato, R., Okubo, Y., Saitsu, H. and Haginoya, K. (2016) Neuroepidemiology of Porencephaly, Schizencephaly, and Hydranencephaly in Miyagi Prefecture, Japan. Pediatric Neurology, 54, 39-42.E1. https://doi.org/10.1016/j.pediatrneurol.2015.08.016
Husain, T., Langlois, P.H., Sever, L.E. and Gambello, M.J. (2008) Descriptive Epidemiologic Features Shared by Birth Defects Thought to Be Related to Vascular Disruption in Texas, 1996-2002. Birth Defects Research Part A: Clinical and Molecular Teratology, 82, 435-440. https://doi.org/10.1002/bdra.20449