BSPED2021 Poster Presentations Pituitary and Growth (8 abstracts)
Duplication of pituitary gland-plus syndrome presenting with a transcranial nasal dermoid cyst
Manju Chandwani 1 , Diamantina-Xanthi Spilioti 2 , Sabah Alvi 3 , Paul Chumas 3 , Mark Liddington 3 , John Russell 3 , Daniel Warren 3 , Martin Elliott 3 , Paul Nix 3 & Alistair Smyth 3
1Harrogate District Hospital, Harrogate, United Kingdom; 2The Hull Teaching Hospitals NHS Trust, Hull, United Kingdom; 3Leeds Children’s Hospital, Leeds, United Kingdom
Duplication of pituitary gland in association with other midline craniofacial anomalies -DPG-plus syndrome - is extremely rare. So far the only described endocrine associations are precocious or delayed puberty. We describe the multifaceted management of a female infant with DPG-plus syndrome. Interestingly, the patient also presented with trans-cranial nasal dermoid cyst and a nasal dimple with protruding hair, which hasn’t been described in previously reported cases. Our patient was diagnosed with a cleft palate and an unusual tongue polyp after birth. She was hospitalised twice with respiratory distress, during feeding and in flat position, while awaiting assessment by a cleft surgeon. She was examined by the cleft surgeon at 11 weeks of age, who identified that her palatal cleft was unusually wide and that a large nasopharyngeal mass was partly filling the defect. He also noted that she had a nasal dermoid cyst, a nasal dimple with protruding hair, hypertelorism and low-set ears. The patient’s MRI brain scan revealed duplicated pituitary gland, thickening of the floor of the third ventricle (hypothalamic hamartoma), basilar artery duplication, odontoid peg cleft, large right nasopharyngeal teratoma and trans-cranial nasal dermoid cyst extending through the nasal bones to just above the cribriform plate. A skull-base CT scan confirmed the midline cranial osseous defect. The patient was diagnosed with DPG-plus syndrome. Resection of nasopharyngeal mass and tongue polyp was performed at 8 months of age, which remarkably reduced the patient’s breathing and feeding difficulties. Histopathology revealed the nasopharyngeal mass to be a benign teratoma and the tongue polyp, a hamartoma. The cleft palate was repaired at 15 and the trans-cranial dermoid cyst was resected at 27 months of age. Histologically, the dermoid cyst was lined by keratinised squamous epithelium and contained adnexal structures with no evidence of malignancy. At 3 years of age, the patient shows normal growth, development, neurological examination and baseline endocrine tests. She will need long-term neuroendocrine surveillance, specifically for precocious puberty and any recurrence of nasopharyngeal/intracranial pathology. This case report adds significantly to the growing body of literature on the clinical presentation and complex management of children with DPG-plus syndrome.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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