Langerhans Cell Histiocytosis; Managing an Uncommon Condition in Pregnancy and Labor: A Case Report

Introduction: Langerhans cell histiocytosis (LCH) is a rare heterogeneous disease that normally occurs in children under 15 years but can rarely be diagnosed in adulthood. It can affect any organ of the body, and a less favorable prognosis is expected when either liver, lung, spleen, or bone marrow are affected. Diabetes insipidus, from pituitary dysfunction, is one of the most common consequences that can develop anytime during the disease, and symptoms normally worsen during pregnancy. Pregnancy with this disease is uncommon, and when it occurs, a cesarean section is normally performed.

Case presentation: We report a 34-year-old pregnant woman (37 weeks and two days gestation) with the diagnosis of a disseminated LCH at the age of 30 who presented herself at the obstetrics emergency department due to respiratory distress. During pregnancy, her pulmonary function remained stable, and she was asymptomatic until the last two days before she was admitted. Labor was induced, with vaginal misoprostol, to achieve a vaginal birth with epidural analgesia. The child was born without complications, and the patient had an immediate clinical improvement. In the following three months, she remained asymptomatic without any worsening of her disease.

Conclusions: In LCH, a cesarean section is the first choice in many cases due to pulmonary impairment. We concluded that early placement of an epidural catheter allows labor analgesia while safeguarding the possibility of an epidural anesthesia in case of urgent/emergent cesarean section. If pulmonary function remains stable, vaginal delivery is possible, thereby being less invasive and allowing for a faster recovery.