Hydranencephaly treatments: retrospective case series and review of the literature

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  • 1 Division of Neurosurgery, Department of Surgery, Kijabe Hospital, Kijabe, Kenya; and
  • 2 University of Toronto, CIGITI, Hospital for Sick Children, Toronto, Ontario, Canada
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The objective of this study was to review treatment options for infants with hydranencephaly and to consider the pros and cons of each treatment modality.


This paper is a review of hydranencephaly as well as a retrospective analysis evaluating the outcomes of 52 infants with hydranencephaly who were treated at the Kijabe Hospital, Kijabe, Kenya, in one of four ways: ventriculoperitoneal shunt (VPS) insertion, endoscopic choroid plexus coagulation (CPC), open choroid plexectomy (CPlx), and palliative care. The primary outcome measure was control of head size, with the aim of improving patient care. One-year mortality was a secondary outcome.


Of the 52 patients analyzed, 11 underwent VPS insertion, 17 CPC, 14 CPlx, and 10 were treated palliatively. Head size was controlled at the 3-month evaluation interval in 5 of 7 infants treated with VPS, 10 of 16 of those treated with CPC, 6 of 9 of those treated with CPlx, and 1 of 4 treated palliatively. The number of infants in each category with complete follow-up data that were needed to analyze change in head size was lower than the total number of patients included in each category. Mortality at 1 year of age was 9 of 11 in the VPS group, 14 of 17 in the CPC group, 6 of 14 in the CPlx group, and 7 of 10 in the palliative group.


Head size decreased by 1 cm or more in similar proportions (62%–71%) of infants with hydranencephaly who were treated by VPS insertion, CPC, and CPlx, and progressed in those who received palliative care. Mortality at 1 year of age was similar in infants treated by a VPS, CPC, and palliative care (70%–82%), but lower (43%) in those treated with CPlx.

ABBREVIATIONS CPC = choroid plexus coagulation; CPlx = choroid plexectomy; OFC = occipitofrontal circumference; VPS = ventriculoperitoneal shunt.

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Contributor Notes

Correspondence Grace Thiong’o: University of Toronto, CIGITI/Hospital for Sick Children, Toronto, ON, Canada. grace.muthonithiongo@sickkids.ca.

INCLUDE WHEN CITING Published online May 15, 2020; DOI: 10.3171/2020.3.PEDS19596.

Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

  • 1

    Pavone P, Praticò AD, Vitaliti G, Hydranencephaly: cerebral spinal fluid instead of cerebral mantles. Ital J Pediatr. 2014;40:79.

  • 2

    Sherer DM, Anyaegbunam A, Onyeije C. Antepartum fetal intracranial hemorrhage, predisposing factors and prenatal sonography: a review. Am J Perinatol. 1998;15(7):431441.

    • Search Google Scholar
    • Export Citation
  • 3

    Kurtz AB, Johnson PT. Diagnosis please. Case 7: Hydranencephaly. Radiology. 1999;210(2):419422.

  • 4

    Adeloye A. Hydranencephaly in Malawian children. East Afr Med J. 2000;77(6):316318.

  • 5

    Mansouri A, Chan V, Njaramba V, Sources of delayed provision of neurosurgical care in a rural Kenyan setting. Surg Neurol Int. 2015;6:32.

    • Search Google Scholar
    • Export Citation
  • 6

    Shitsama S, Wittayanakorn N, Okechi H, Albright AL. Choroid plexus coagulation in infants with extreme hydrocephalus or hydranencephaly. J Neurosurg Pediatr. 2014;14(1):5557.

    • Search Google Scholar
    • Export Citation
  • 7

    Moher D, Liberati A, Tetzlaff J, Altman DG. Preferred reporting items for systematic reviews and meta-analyses: the PRISMA statement. PLoS Med. 2009;6(7):e1000097.

    • Search Google Scholar
    • Export Citation
  • 8

    Albright L. Percutaneous choroid plexus coagulation in hydranencephaly. Childs Brain. 1981;8(2):134137.

  • 9

    Kim SY, Cho JH, Kim KH. Endoscopic coagulation of choroid plexus in hydranencephaly. J Korean Neurosurg Soc. 2014;55(6):375378.

  • 10

    Ogiwara H, Uematsu K, Morota N. Obliteration of the choroid plexus after endoscopic coagulation. J Neurosurg Pediatr. 2014;14(3):230233.

    • Search Google Scholar
    • Export Citation
  • 11

    Sandberg DI, Chamiraju P, Zoeller G, Endoscopic choroid plexus coagulation in infants with hydranencephaly or hydrocephalus with a minimal cortical mantle. Pediatr Neurosurg. 2012;48(1):612.

    • Search Google Scholar
    • Export Citation
  • 12

    Malheiros JA, Trivelato FP, Oliveira MM, Endoscopic choroid plexus cauterization versus ventriculoperitoneal shunt for hydranencephaly and near hydranencephaly: a prospective study. Neurosurgery. 2010;66(3):459464.

    • Search Google Scholar
    • Export Citation
  • 13

    Dandy WE. Extirpation of the choroid plexus of the lateral ventricles in communicating hydrocephalus. Ann Surg. 1918;68(6):569579.


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