Open Fetal Surgery for Ventricular-Amniotic Valve Implantation in Aqueductal Stenosis-Dependent Severe Fetal Hydrocephalus: A Case Report with 7-Year Follow-Up.
Autor: | Zamłyński M; Department of Gynecology, Obstetrics and Oncological Gynecology, Faculty of Medical Sciences in Zabrze, Medical University of Silesia, Katowice, Poland., Olejek A; Department of Gynecology, Obstetrics and Oncological Gynecology, Faculty of Medical Sciences in Zabrze, Medical University of Silesia, Katowice, Poland., Koszutski T; Department of Pediatric Surgery and Urology, Faculty of Medical Sciences in Katowice, Katowice, Poland., Bohosiewicz J; Department of Pediatric Surgery and Urology, Faculty of Medical Sciences in Katowice, Katowice, Poland., Mandera M; Department of Pediatric Neurosurgery, Faculty of Medical Sciences in Katowice, Medical University of Silesia, Katowice, Poland., Zamłyński J; Department of Gynecology, Obstetrics and Oncological Gynecology, Faculty of Medical Sciences in Zabrze, Medical University of Silesia, Katowice, Poland., Maruniak-Chudek I; Department of Neonatology and Neonatal Intensive Care, Faculty of Medical Sciences in Katowice, Medical University of Silesia, Katowice, Poland., Herman-Sucharska I; Department of Radiology, Faculty of Health Sciences, Jagiellonian University Medical College, Kraków, Poland., Pastuszka A; Department of Descriptive and Topographic Anatomy, School of Medicine with Division of Dentistry Zabrze, Medical University of Silesia in Katowice, Katowice, Poland. |
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Jazyk: | angličtina |
Zdroj: | Fetal diagnosis and therapy [Fetal Diagn Ther] 2024; Vol. 51 (3), pp. 278-284. Date of Electronic Publication: 2024 Feb 17. |
DOI: | 10.1159/000536121 |
Abstrakt: | Introduction: Fetal ventriculomegaly is one of the most commonly diagnosed central nervous system pathologies of the second trimester, occurring with a frequency of 0.3-0.5/1,000 births. Severe fetal ventriculomegaly (SVM) may necessitate intrauterine intervention. Most such interventions have been made percutaneously with ultrasound guidance insertion of a pigtail catheter, which sadly often became obstructed or migrated. Case Presentation: Our case report presents the possibility of ventriculo-amniotic valve implantation (VAVI) by classic hysterotomy in isolated severe fetal hydrocephalus (IVSM) due to aqueductal stenosis. The patient was operated on similarly to open fetal surgery MOMS criteria at 24+4/7 GA, with an initial lateral ventricular dimension of 22.5 mm. A female newborn was delivered by elective cesarean section at 31+1/7 GA due to PPROM (Apgar 10' 8 points, birth weight 1,600 g), required CPAP, and removal of the drainage system due to infection and narrow lateral ventricles. Evans index (EI) gradual increase and clinical symptoms of high-pressure hydrocephalus after 10 days required a ventricle-peritoneal shunt (VPS) implantation. The newborn was discharged home after 28 days with stabile hydrocephalus (EI: 0.59-0.6), in good clinical condition. The 7-year follow-up was complicated by epilepsy, VPS shunt infections, delay in motor and intellectual functions (mild to moderate), and symptoms of atypical autism, the phenotype possibly related to a variant in ZEB2 gene. Conclusion: Intrauterine VAVI is a one-step procedure that is effective in draining CFS. The limitations of the method remain complications due to preterm labor and infection of the drainage system. (© 2024 S. Karger AG, Basel.) |
Databáze: | MEDLINE |
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