| IHS | Diagnosis | ICD-10 |
|---|---|---|
| 10.3.1 | Headache attributed to phaeochromocytoma [D53.0 (benign) or C74.1 (malignant)] | G44.813 |
Diagnostic criteria:
- Intermittent discrete attacks of headache accompanied by at least one of the following and fulfilling criteria C and D:
- sweating
- palpitations
- anxiety
- pallor
- Phaeochromocytoma demonstrated by biochemical investigations, imaging and/or surgery
- Headache develops concomitantly with abrupt rise in blood pressure
- Headache resolves or markedly improves within 1 hour of normalisation of blood pressure
Comments:
Paroxysmal headache occurs in 51-80% of patients with phaeochromocytoma. It is often severe, frontal or occipital and is generally described as either pulsating or steady in quality. An important feature of the headache is its short duration: <15 minutes in 50% and <1 hour in 70% of patients. Other features include apprehension and/or anxiety, often with a sense of impending death, tremor, visual disturbances, abdominal or chest pain, nausea, vomiting and occasionally paraesthesia. The face can blanch or flush during the attack.
The diagnosis is established by the demonstration of increased excretion of catecholamines or catecholamine metabolites, and can usually be secured by analysis of a single 24-hour urine sample collected when the patient is hypertensive or symptomatic.
When hypertensive encephalopathy is present, headache is coded as 10.3.3 Headache attributed to hypertensive encephalopathy. When the diagnosis of phaeochromocytoma has not yet been made, and hypertensive encephalopathy is not present, patients may meet the diagnostic criteria for 10.3.2 Headache attributed to hypertensive crisis without hypertensive encephalopathy.