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Endgames Case Review

A 61 year old man with pancreatitis, pituitary dysfunction, and painful exophthalmos

BMJ 2019; 364 doi: https://doi.org/10.1136/bmj.l93 (Published 21 February 2019) Cite this as: BMJ 2019;364:l93
  1. Christopher Smith, medical student1,
  2. Saira Hameed, consultant endocrinologist, honorary clinical senior lecturer2 3,
  3. Geoffrey E Rose, consultant ophthalmic surgeon4,
  4. Florian Wernig, consultant endocrinologist, honorary clinical senior lecturer23
  1. 1Imperial College School of Medicine, Imperial College London, London, UK
  2. 2Imperial Centre for Endocrinology, Imperial College Healthcare NHS Trust, London, UK
  3. 3Section of Endocrinology and Investigative Medicine, Division of Diabetes, Endocrinology and Metabolism, Imperial College London, London, UK
  4. 4Moorfields Eye Hospital NHS Foundation Trust, London, UK
  1. Correspondence to F Wernig Christopher.Smith13{at}imperial.ac.uk

A 61 year old man with a several years’ history of chronic pancreatitis and complex partial seizures was referred to endocrinology with low serum thyroid stimulating hormone (TSH).

At the time, examination was normal, and pituitary function tests revealed secondary hypothyroidism and secondary hypogonadism with normal prolactin and normal 9am cortisol.

Magnetic resonance imaging (MRI) of the pituitary gland showed an 8 mm infundibular mass with elevation, but not compression of the optic chiasm. Thyroxine and testosterone replacement were commenced.

His condition was stable for three years, after which he presented with an acute flare of pancreatitis, associated with painful, bilateral exophthalmos (fig 1). He was clinically and biochemically euthyroid on thyroxine replacement. Repeat MRI showed an increase in the size of the infundibular mass, and bilateral extra-ocular muscle expansion with lacrimal gland swelling.

Fig 1

Bilateral inflammation of orbital tissue with yellow exudate.

Questions

  1. What is the most likely unifying diagnosis?

  2. What is the definitive investigation to diagnose this condition?

  3. How would you manage this condition?

Answers

1. What is the most likely unifying diagnosis?

This patient’s multiple pathologies (pituitary gland mass, exophthalmos, dacryoadenitis, chronic pancreatitis, and …

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