Pseudoacromegaly Associated with Non-Functioning Pituitary A
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A 16-year-old boy was referred from the neurosurgery clinic to adult endocrinology clinic to rule out acromegaly. His background medical problems included a posterior fossa arachnoid cyst and multiple renal cysts since birth with no clinical consequences. Further questioning revealed that he was born full-term by caesarean section due to transverse lie and possible macrocephaly, his birth weight was 3.7 kg and head circumference was 36 cm. His early developmental milestones were reported to be within normal limits except for speech delay until the age of 3 years, attributed to a large tongue and tongue tie, and delayed sphincter control.

He still had difficulties in articulation. His parents reported behavioural changes early in life in the form of disobedience and being aggressive, which required behavioural therapy with considerable subsequent improvement. He still had learning difficulties with a short attention span. His performance at school was poor. His family noticed different facial features to their other offspring since birth, with rapidly increasing height and frequent changing of clothes and shoe sizes since the age of 6 years.

The patient entered puberty at 15 years old. He still had chronic left-sided headache, excessive sweating and chewing difficulties. Examination revealed height 185 cm (above 97th percentile), weight 91.9 kg (above 97th percentile), BMI 26.8 kg/m2. The patient was normotensive, had coarse facial features, a long face, frontal bossing, orbital hypertelorism, acne, prognathism, macroglossia, tongue tie, sweaty skin, excessive hair and acral overgrowth.

Other systemic examination gave normal findings. The calculated insulin resistance based on HOMA-IR was elevated at 4.69 (N 1–4), indicating early insulin resistance. Pituitary MRI showed a stable, previously seen, posterior fossa arachnoid cyst measuring 24 mm, and a new 3 mm right pituitary gland microadenoma. The patient’s chromosomal analysis at the age of 2 years showed karyotype 46,XY with no numerical or structural chromosome abnormalities. To date, there has been no indication for pituitary surgery in this patient and he has been followed as per recommendations for micro-incidentaloma cases.

Conclusively, clinicians should be aware of the probable aetiologies of pseudoacromegaly and consider biochemical confirmation first in patients presenting with clinical features of acromegaly and pituitary adenoma to avoid unnecessary surgeries.

Source: https://www.ejcrim.com/index.php/EJCRIM/article/view/1950/2376
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