Perinatal management for fetal oral epignathus with duplication of the pituitary gland (DPG)‐plus syndrome: A case report and literature review

Author:

Khwankaew Noppasin1ORCID,Sawaddisan Rapphon1,Siripruekpong Sirikarn2,Leelasawatsuk Peesit3,Praditaukrit Manapat4,Tanaanantarak Pattama5

Affiliation:

1. Department of Obstetrics and Gynecology, Faculty of Medicine Prince of Songkla University Hat Yai Thailand

2. Department of Anesthesiology, Faculty of Medicine Prince of Songkla University Hat Yai Thailand

3. Department of Otolaryngology Head and Neck Surgery, Faculty of Medicine Prince of Songkla University Hat Yai Thailand

4. Department of Pediatrics, Faculty of Medicine Prince of Songkla University Hat Yai Thailand

5. Department of Radiology, Faculty of Medicine Prince of Songkla University Hat Yai Thailand

Abstract

AbstractThe prenatal diagnosis of epignathus presents a unique challenge for physicians. Differential diagnosis is usually based on the anatomic location of the tumor. Typical prenatal ultrasound characteristics of epignathus include a mixed solid and cystic lesion with vascularity in the solid component, originating from the hard or soft palate, and it is often associated with other anomalies such as craniofacial clefts or trans‐sphenoidal intracranial extension. Herein, we present a case of prenatal diagnosis of epignathus with rare ultrasonographic findings, prenatal management requiring collaborative efforts of a multidisciplinary team, and a well‐planned innovative ex utero intrapartum treatment procedure. In addition, this report highlights the evolving postnatal diagnosis of the rare developmental anomaly, duplication of the pituitary gland‐plus syndrome, which includes various midline craniofacial, central nervous system, spinal, and endocrine abnormalities.

Publisher

Wiley

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