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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OBJECTIVE

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.

METHODS

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.

RESULTS

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.

CONCLUSIONS

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.

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