Pituitary Duplication with Nasal Hamartoma and Palatal Hairy Polyp

Background:
- This diagnosis is also known as duplication of the pituitary gland-plus syndrome (DPG-plus).
- It is a rare developmental anomaly with ~40 cases reported through 2012.
- It most often occurs in women.
- There are many theories as to the etiology, though the leading theory posits the splitting of the rostral notochord and the prechordal plate during blastogenesis.
Clinical Presentation:
- DPG-plus can have either precocious puberty or delayed puberty.
Key Diagnostic Features:
- More frequently associated anomalies include:
  - Hypothalamic mass (58%)
  - Agenesis of the corpus callosum (39%)
  - Hypertelorism (65%)
  - Cleft palate (61%)
  - Broadening or thickening of the optic chiasm (23%)
  - Oropharyngeal masses (teratomas, 23%; epignathus, 16%; dermoids, 10%; polypoid masses, 6%)
  - Clefts/fusion of vertebral bodies (35%)
Treatment:
- Management strategies include the resection of nasopharyngeal and oropharyngeal masses, cleft palate repair/airway management, and endocrine testing and monitoring.

January 2018

A 2-day-old neonate with multiple orofacial abnormalities and difficulty passing NG tube

Main menu