PM2013: A Case Report of Body Stalk Anomaly and Integrated Perinatal Palliative Care Management

Introduction: Body stalk anomalies are the rarest and most severe fetal abdominal wall defects with an incidence of 0.32 per 100,000 births (1). This anomaly is characterized by absence of the anterior abdominal wall, severe kyphosis, and limb malformations (1). Diagnosis usually occurs by first trimester ultrasound, many result in miscarriage or stillbirth, and with only three reports of survival, it is considered life-limiting (2). Due to its fatal prognosis and complex presentation, early perinatal palliative care consultation is essential, and the goal of prioritizing comfort measures only is appropriate.

Case Description: A pregnant patient of the Amish community presented at 29 weeks gestation with moderate vaginal bleeding. Ultrasound revealed placenta previa, possible placenta accreta, and suspected fetal body stalk anomaly. Fetal MRI showed concern for placenta percreta and confirmation of multiple fetal anomalies with bowel loops and liver abutting, and possibly adhered to, the placenta. Extensive counseling was provided regarding the grim fetal prognosis, and through consultation with their religious community, the parents made the decision for comfort care at delivery. Multiple teams were involved in the counseling, planning, and preparation of this complex delivery including: Maternal Fetal Medicine, Obstetrics, Neonatology, Perinatal Palliative Care, Gynecology Oncology, Interventional Radiology, Pediatric Genetics, and Prenatal Genetics. Delivery was via scheduled cesarean section, and the infant was born alive. Infant was noted to have significant kyphosis with bowel and liver externalized. The infant was placed in a plastic bowel bag up to the neck and diapers were used over the bag to create a support for the abdominal organs. He was wrapped in a blanket and given to parents to hold until his death.

Discussion: This case illustrates the need for a multidisciplinary and interdisciplinary approach when preparing for delivery of a fetus with a body stalk anomaly. Due to its rare incidence, most providers at our institution had never seen an infant with a body stalk anomaly, so education of neonatal providers about the potential appearance of the infant and the special management approach was important. The uncertain and potentially emergent timing of delivery, appearance of the infant’s externalized organs, post-birth fluid seepage, prolonged bleeding, potential adherence of the infant to the placenta, and elevated risk of maternal hemorrhage presented a complex situation requiring all stakeholders be well-informed and well-prepared. Special considerations that the perinatal palliative care team had to appraise included: staff preparation, ongoing communication, scenario planning, pain control, attention to dressing of the infant to allow for prolonged holding, family integration, cultural humility, and specific and sensitive bereavement care.

Conclusion: Integrated perinatal palliative care as part of a multidisciplinary team is essential in providing a holistic approach to medically and emotionally complex management of the mother/baby dyad in rare and impactful fetal anomalies.