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. 2023 Jan 17;13(3):343.
doi: 10.3390/diagnostics13030343.

Absence of a Hernia Sack in Patients Undergoing Prenatal Repair of Spina Bifida Increases the Risk of Developing Shunt-Dependent Hydrocephalus

Agnieszka Pastuszka  1   2 , Tomasz Koszutski  2 , Ewa Horzelska  1 , Sylwia Marciniak  1 , Mateusz Zamłyński  1 , Anita Olejek  1
Affiliations

Absence of a Hernia Sack in Patients Undergoing Prenatal Repair of Spina Bifida Increases the Risk of Developing Shunt-Dependent Hydrocephalus

Agnieszka Pastuszka et al. Diagnostics (Basel). .

Abstract

Spina bifida aperta (SBA), with (myelomeningocele) or without (myeloschisis) a hernia sack, is the most common congenital defect of the central nervous system. Prenatal surgical closure of SBA lowers the risk for developing shunt-dependent hydrocephalus, which offers a chance at improved motor, urinary, and gastrointestinal function. A total of 96 patients who had undergone open surgery prenatal repair for SBA were analyzed. The patients were divided into two groups: Group I-12 patients (12.5%)-without a hernia sack (myeloschisis) and Group II-84 patients (87.5%)-with a hernia sack (myelomeningocele). In this study, we demonstrated that prenatal SBA repair was statistically significantly less often associated with the need for ventriculoperitoneal shunting (p > 0.00001). The shunting was statistically significantly more often required in patients from Group I (p > 0.004). The absence of a hernia sack increases the risk for developing shunt-dependent hydrocephalus in patients after prenatal SBA repair. However, as prenatal SBA repair is associated with better motor, urinary, and gastrointestinal function, increased risk of developing shunt-dependent hydrocephalus in fetuses without a hernia sack should not be treated as a contraindication to prenatal intervention.

Keywords: hydrocephalus; prenatal surgery; spina bifida.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
(A) Fetal NMR at 21 weeks of gestation. The arrow indicates the myeloschisis, with the area of contact between the split and the uterine wall. (B) Fetal NMR at 26 weeks of gestation (4 weeks after intrauterine myeloschisis repair); the arrow indicates the site after a three-layer reconstruction of spina bifida aperta.
Figure 2
Figure 2
(A) Fetal NMR at 22 weeks of gestation. The arrow indicates the myelomeningocele. (B) Fetal NMR at 26 weeks of gestation (4 weeks after intrauterine myelomeningocele repair); the arrow indicates the site after a three-layer reconstruction of spina bifida aperta, with the area of contact between the repair site and the uterine wall.
Figure 3
Figure 3
Normal appearance and size of the ventricles on fontanelle ultrasound.
Figure 4
Figure 4
Ventriculomegaly, which requires V-P shunting on fontanelle ultrasound.
See this image and copyright information in PMC

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