First report of surgery for anencephaly in twin pregnancies: Technical notes and postoperative management

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TL;DR

This case report describes the first surgical closure of a cranial defect in an anencephalic infant from a dichorionic-diamniotic twin pregnancy, who survived 37 months with multidisciplinary support. The procedure involved duraplasty and scalp reconstruction, with no early or late postoperative complications. This case demonstrates that long-term survival is possible with surgical intervention and intensive care, highlighting the need for ethical and legal frameworks for brain death diagnosis and organ donation in anencephaly.

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**Background:** Anencephaly, the most severe neural tube defect (NTD), involves absence of cerebral hemispheres, skull, and scalp, and is typically fatal before or shortly after birth. No surgical intervention for anencephaly has been previously reported. This study presents the first case of surgical management of anencephaly in a dichorionic-diamniotic twin pregnancy, aiming to share technical details, postoperative challenges, and insights for future management.

**Methods:** A 36-year-old primigravida mother with a dichorionic-diamniotic twin pregnancy after the fifth IVF attempt had one anencephalic fetus detected by ultrasound in the first trimester. The family declined termination. The anencephalic female infant was delivered at 37 weeks (birth weight 2110 g, height 37 cm). Physical examination showed absence of scalp, calvarium, dura, and cerebral hemispheres, with exposed brainstem and multiple cystic sacs. Neurological examination revealed mobile extremities, myoclonus, hypoactive sucking reflex, normal Moro and grasp reflexes, isochoric pupils, no spontaneous eye movement, and absent light, corneal, and oculocephalic reflexes. The patient had spontaneous respiration and moderate spasticity. Central hypothyroidism and diabetes insipidus were treated with L-thyroxine and desmopressin. Surgery was performed at 3 weeks (weight 2500 g) under general anesthesia. Microsurgical dissection removed immature neural tissue and CSF vesicles. Duraplasty used a bovine pericardium xenograft (DuraGen Classic) with watertight closure (4-0 silk, running-locked technique) and fibrin glue. A rotational advancement flap closed the scalp defect; cranioplasty was not performed. No surgical drain was used.

**Key Results:** No early or late postoperative complications (skin healing problems, CSF leak, infection, hematoma, wound dehiscence, meningitis) occurred. The patient was intubated postoperatively, tracheostomy performed at 2 weeks, and discharged at 6 months with a portable ventilator. Hormone replacement included growth hormone, levothyroxine, desmopressin, and low-dose prednisolone. The patient survived 37 months (3 years and 1 week) with intermittent spontaneous breathing and mechanical ventilation. MRI and CT at age 2 years showed absence of cerebrum and cerebellum, with CSF-filled membranous structures and absent major skull portions. The family declined genetic studies and organ donation.

**Clinical Implications:** This first reported surgical closure for anencephaly demonstrates feasibility of long-term survival (37 months) with multidisciplinary care (neurosurgery, plastic surgery, neonatology, endocrinology). The absence of choroid plexus may have contributed to lack of postoperative CSF leak. The case underscores ethical dilemmas: selective feticide (3-16% miscarriage risk) was declined due to precious IVF pregnancy and cultural beliefs. Brain death diagnosis in anencephaly requires brainstem death criteria (positive apnea test, loss of cranial nerve functions for ≥48 hours), but was not applicable here. Organ donation was legally prohibited in Turkey (Law No. 2238 prohibits organ retrieval from minors). The study is limited by single-case generalizability. The authors emphasize the importance of NTD prevention through folic acid supplementation and highlight the need for consensus on ethical and legal approaches to anencephaly management.