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Open access

J K Prague, C L Ward, O G Mustafa, B C Whitelaw, A King, N W Thomas and J Gilbert

Summary

Therapeutic shrinkage of prolactinomas with dopamine agonists achieves clinical benefit but can expose fistulae that have arisen as a result of bony erosion of the sella floor and anterior skull base by the invasive tumour, resulting in the potential development of cerebrospinal fluid (CSF) rhinorrhoea, meningitis, and rarely pneumocephalus. Onset of symptoms is typically within 4 months of commencing therapy. The management is typically surgical repair via an endoscopic transnasal transsphenoidal approach. A 23-year-old man presented to the Emergency Department with acute left limb weakness and intermittent headaches. Visual fields were full to confrontation. Immediate computed tomography and subsequent magnetic resonance imaging (MRI), demonstrated a 5 cm lobular/cystic mass invading the right cavernous sinus, displacing and compressing the midbrain, with destruction of the bony sella. He was referred to the regional pituitary multidisciplinary team (MDT). Serum prolactin was 159 455 mIU/l (7514.37 ng/ml) (normal ranges 100–410 mIU/l (4.72–19.34 ng/ml)). Cabergoline was commenced causing dramatic reduction in tumour size and resolution of neurological symptoms. Further dose titrations were required as the prolactin level plateaued and significant residual tumour remained. After 13 months of treatment, he developed continuous daily rhinorrhea, and on presenting to his general practitioner was referred to an otolaryngologist. When next seen in the routine regional pituitary clinic six-months later he was admitted for urgent surgical repair. Histology confirmed a prolactinoma with a low proliferation index of 2% (Ki-67 antibody). In view of partial cabergoline resistance he completed a course of conventional radiotherapy. Nine months after treatment the serum prolactin had fallen to 621 mIU/l, and 12 months after an MRI showed reduced tumour volume.

Learning points

  • CSF rhinorrhoea occurred 13 months after the initiation of cabergoline, suggesting a need for vigilance throughout therapy.

  • Dedicated bony imaging should be reviewed early in the patient pathway to assess the potential risk of CSF rhinorrhoea after initiation of dopamine agonist therapy.

  • There was a significant delay before this complication was brought to the attention of the regional pituitary MDT, with associated risk whilst left untreated. This demonstrates a need for patients and healthcare professionals to be educated about early recognition and management of this complication to facilitate timely and appropriate referral to the MDT for specialist advice and management. We changed our nurse-led patient education programme as a result of this case.

  • Having developed partial cabergoline resistance and CSF rhinorrhoea, an excellent therapeutic response was achieved with conventional radiotherapy after limited surgery.

Open access

Takuma Hara, Hiroyoshi Akutsu, Tetsuya Yamamoto, Eiichi Ishikawa, Masahide Matsuda and Akira Matsumura

Summary

Gastrointestinal perforation is a complication associated with steroid therapy or hypercortisolism, but it is rarely observed in patients with Cushing's disease in clinical practice, and only one case has been reported as a presenting symptom. Herein, we report a rare case of Cushing's disease in which a patient presented with gastrointestinal perforation as a symptom. A 79-year-old man complained of discomfort in the lower abdomen for 6 months. Based on the endocrinological and gastroenterological examinations, he was diagnosed with Cushing's disease with a perforation of the descending colon. After consultation with a gastroenterological surgeon, it was decided that colonic perforation could be conservatively observed without any oral intake and treated with parenteral administration of antibiotics because of the mild systemic inflammation and lack of abdominal guarding. Despite the marked elevated levels of serum cortisol, oral medication was not an option because of colonic perforation. Therefore, the patient was submitted to endonasal adenomectomy to normalize the levels of serum cortisol. Subsequently, a colostomy was successfully performed. Despite its rarity, physicians should be aware that gastrointestinal perforation may be associated with hypercortisolism, especially in elderly patients, and immediate diagnosis and treatment of this life-threatening condition are essential. If a perforation can be conservatively observed, endonasal adenomectomy prior to laparotomy is an alternative treatment option for hypercortisolism.

Learning points

  • Thus far, only one case of gastrointestinal perforation as a presenting clinical symptom of Cushing's disease has been reported.

  • Physicians should be aware that gastrointestinal perforation might be associated with hypercortisolism in elderly patients because elevated levels of serum cortisol may mask the clinical signs of perforation. Because of this masking effect, the diagnosis of the perforation also tends to be delayed.

  • Although parenteral administration of etomidate is a standard treatment option for decreasing the elevated levels of serum cortisol, endonasal adenomectomy prior to laparotomy is an alternative treatment option if etomidate therapy is unavailable.