Pickwickian syndrome

Published on 02/04/2015 by admin

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Last modified 22/04/2025

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110 Pickwickian syndrome

Instruction

Look at this patient.

Salient features

History

• Daytime somnolence

• Unrefreshing sleep

• Daytime fatigue

• Snoring

• Shortness of breath

• Headache particularly in the morning

• Decreased libido

• Swelling of feet

• Poor concentration

• Systemic hypertension

• Family history of obesity

• Gastroesophageal reflux

• Poor quality of life

• History of stroke: obstructive sleep apnoea significantly increases the risk of stroke or mortality from any cause, and the increase is independent of other risk factors, including hypertension.

Examination

• Obese patient who is plethoric and cyanosed

• Maxillary or mandibular hypoplasia:

• Short of breath at rest
• May be nodding off to sleep.

• Systemic hypertenion

• Nocturnal angina

• Look for signs of pulmonary hypertension and right heart failure

• Tell the examiner that you would like to measure the neck circumference

Remember: Nearly 50% of patients with sleep apnoea syndrome are not obese.

Diagnosis

This patient has marked obesity and hypersomnolence with signs of pulmonary hypertension (lesion), which indicate she has Pickwickian syndrome. The patient is in cardiac failure (functional status).

Advanced-level questions

What is the cause of cyanosis in such a patient?

A mixture of obstructive apnoea and sleep-induced hypoventilation (Fig. 110.1). The blood gas picture is hypoxia and carbon dioxide retention.

image

Fig. 110.1 Patterns of sleep-disordered breathing.

Where is the obstruction?

It is caused by the apposition of the tongue and the palate of the posterior pharyngeal wall.

What is the significance of neck circumference?

A prediction rule based on neck circumference is used to estimate a patient’s probability of having a sleep test result that is diagnostic of sleep apnoea. Neck circumference (measured in centimeters) is adjusted if the patient has hypertension (4 cm is added), is a habitual snorer (3 cm is added) or is reported to choke or gasp most nights (3 cm is added). Adjusted neck circumference of:

<43 cm: low clinical probability

43–48 cm: intermediate probability (4–8 times as probable as a low probability)

>48 cm: high probability (20 times as probable).

How would you treat such a patient?

• Weight reduction

• Avoidance of smoking and alcohol

• Continuous nasal positive airway pressure (CPAP) delivered by a nasal mask (Lancet 1999;353:2100–5). CPAP is recommended even if the apnoea–hypopnoea index is in the mild range (5 to 15), although evidence for the efficacy of CPAP is strong for patients with an apnoea–hypopnoea index >15. CPAP acts like a pneumatic splint (resulting in patency of the upper airway during inspiration and expiration). The resultant reductions in gas-exchange perturbations, respiratory effort, abrupt arousals and BP surges all probably ameliorate symptoms

• Home oxygen

• Surgery. Tracheostomy, uvulopalatopharyngoplasty, linguoplasty, mandibular advancement, plastic remodelling of the uvula (laser-assisted or radiofrequency ablation), tonsillectomy, reconstruction of maxillae

• Drugs: serotonin, receptor blockade, acetazolamide, methylxanthines, weight loss medications

• Atrial overdrive pacing may benefit selected patients (the vagal tone that accompanies bradycardia also causes the sleep apnoea, so prevention of the enhanced vagal tone prevents the apnoea) (N Engl J Med 2002;346:404–12).

William Howard Taft, US President from 1909 to 1913, had a minimum body mass index of 42 kg/m2, wore size 54 pajamas, had a neck size of 19 inches (47.5 cm), snored and had hypersomnolence. He appears to have become symptomatic at approximately 300 lb (140 kg). Within 12 months of leaving office, Taft permanently lost over 60 lb (27 kg). His somnolence resolved. As Chief Justice of the United States from 1921 to 1930, he was not somnolent.

Mr Pickwick is a character in the novel ‘Pickwick papers’, written by Charles Dickens, and this term was applied by Sir William Osler. In Charles Dickens’ The Posthumous Papers of the Pickwick Club’, Dickens describes the first meeting of Mr Wardle with his servant:

‘Damn that boy,’ said the old gentleman, ‘he’s gone to sleep again.’

‘Very extraordinary boy, that,’ said Mr. Pickwick, ‘does he always sleep in this way?’

‘Sleep!’ said the old gentleman, ‘he’s always asleep. Goes on errands fast asleep, and snores as he waits at the table.’

‘How very odd!’ said Mr. Pickwick

‘Ah! Odd indeed,’ returned the old gentleman; ‘I’m proud of that boy—wouldn’t part with him on any account—damme, he’s a natural curiosity!’

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