Towards a pituitary apoplexy classification based on clinical presentation and patient journey

Abstract

Purpose: The condition of pituitary apoplexia contains the clinical spectre from life-threatening emergency to asymptomatic self-limiting course, which partly determines diagnostic delay and management. Outcome evaluation of course and management of pituitary apoplexia is hampered by the diverse presentation of this condition and requires appraisal. This study aimed to describe the patient journey, clinical presentation, and management of various types of pituitary apoplexy in a new classification to facilitate future outcome evaluation and identify unmet needs in the care process.

Methods: A single-center retrospective patient chart study was conducted between 2005-2021 (N = 98). Outcome measures were clinical symptoms at first presentation in hospital, being headache, consciousness, visual acuity, visual field defects (VFD), ophthalmoplegia, nausea, vomiting, fever, and hypopituitarism and care process characteristics.

Results: Mean age was 47.6 ± 16.6 years (51.0% male). We describe their patient journey and identified three different types, differing in clinical presentation, in-hospital route, and final treatment, e.g., Acute (type A, 52%), Subacute (type B, 22.5%), and Non-acute (type C, 25.5%). Type A generally presents with acute onset headaches, VFD, or ophthalmoplegia emergency setting, with lowest mean visual acuity of both eyes and frequent hypocortisolism.

Conclusions: Pituitary apoplexy can be approached as a spectrum of disease with 3 main subtypes, with a different initial presentation, different in-hospital route resulting in different management. Acknowledging subtypes with particular needs for (emergency) referrals to Pituitary Tumors Center of Excellence (PTCOE) will serve patient care improvements, outcome evaluations and address areas for improvement.

Keywords: Acute; Classification system; Pituitary apoplexy; Spectrum of disease; Sub-acute and non-acute.

Fig. 1

Patient journey throughout care process of apoplexy type A, B, and C patients. This figure illustrates the patient journey of apoplexy type A, B, and C patients throughout their care process until they reach treatment start. T1, time period in days between onset of apoplexy symptoms and first presentation in hospital (one way ANOVA between groups yielded p < 0.001); T2, time period in days between first presentation in hospital and moment of diagnosing pituitary apoplexy (one way ANOVA between groups yielded p < 0.001); T3, time period in days between first presentation in hospital and treatment start (one way ANOVA between groups yielded p < 0.001); Proportion of patients that were seen in a regional versus academic hospital at first presentation did not differ significantly between groups (chi-square yielded p = 0.917). Proportion of patients that were admitted to an inpatient ward in the hospital after first presentation did differ significantly between groups (chi-square yielded p < 0.001). The in hospital locations where patients were seen at their first hospital presentation significantly differed between the groups, with type A patients presenting primarily at the emergency department and almost all type C patients at the outpatient clinic (chi-square yielded p < 0.001). Proportion of patients that were treated either surgically or conservatively did not differ significantly between groups (chi-square yielded p = 0.248). Proportion of surgically treated patients that either had emergency (<3 days) or more elective (>3 days) surgery did differ significantly between groups (chi-square yielded p < 0.001)

Fig. 2

Consulting specialism at first hospital entry for apoplexy type A, B, and C patients. The consulting specialism at first presentation in the hospital significantly shifts between the different apoplexy types, from neurology is by far the largest proportion of type A patients to internal medicine and ophthalmology in type C patients (chi-square yielded p < 0.001). FP first presentation in hospital

Fig. 3

Differential diagnosis at first hospital entry for apoplexy type A, B, and C patients. The variation of differential diagnoses at first presentation in the hospital significantly differed between the groups (chi-square yielded p = 0.014). PA pituitary apoplexy; SC Thrombosis, sinus cavernous thrombosis; SAB subarachnoid hemorrhage; RCC Rathke’s cleft cyst

Fig. 4

Proposal for care process improvements for pituitary apoplexy. This figure addresses a proposal for care process improvement with regard to pituitary apoplexy. The suggested possible care paths (red and blue) for each subtype are demonstrated in comparison to the care path of patients with a pituitary tumor without PA (grey). PA pituitary apoplexy; PTCOE Pituitary Tumor Center of Excellence; MDT Multidisciplinary team meeting; ASAP As soon as possible; MRI Magnetic resonance imaging