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Lethal Pulmonary Hypoplasia After In-Utero Myelomeningocele Repair

From the Department of Obstetrics and Gynecology and Department of Pathology, University of Alabama at Birmingham, Birmingham, Alabama.

Address reprint requests to: Ona Faye-Petersen, MD, Department of Pathology, Anatomic Division, University of Alabama at Birmingham, Kracke 729, 619 19th Street South, Birmingham, AL 35249-7333; E-mail: onamarie{at}path.uab.edu.

BACKGROUND: In-utero surgical repair of fetal myelomeningocele has been performed as a means to improve the postnatal condition of affected infants.

CASE: A nulliparous woman underwent in-utero surgical repair of a fetal lumbosacral myelomeningocele at 24 weeks’ gestation. Her postoperative convalescence was complicated by pulmonary edema, abdominal pain, chronic oligohydramnios, and preterm labor. The infant was delivered by cesarean at 33 weeks’ gestation, but expired from respiratory distress caused by pulmonary hypoplasia at 9 hours of age.

CONCLUSION: Until the benefits of in-utero repair of fetal myelomeningoceles are determined by well-controlled clinical trials, this technique remains investigational. Physicians and their patients who are considering this procedure must be fully aware of the potential risks that can occur.