Researchers believe that this disease is the result of a defect in the brain’s control over breathing.
Obesity hypoventilation syndrome was classically described as “Pickwick syndrome” in a 1956 report.
It was named for the resemblance of a patient’s symptoms to the character Fat Joe with the Red Face, portrayed by Charles Dickens, in his story “The Posthumous Papers of the Pickwick Club.”
A similarity was observed in obesity accompanied by excessive hypersomnolence. In addition to the fact that the patient had hypoventilation during wakefulness, with erythrocytosis induced by hypoxemia , pulmonary hypertension.
Pickwick syndrome is a respiratory disorder that generally occurs in obese people and leads to low oxygen levels and too much carbon dioxide in the blood.
These low levels of oxygen and high levels of carbon dioxide can develop due to a condition called hypoventilation during the day (daytime hypoventilation).
Hypoventilation means that you are not moving enough air in and out of your lungs.
With Pickwick syndrome, you can also have trouble sleeping due to obstruction, known as sleep apnea .
This syndrome is a combination of obstructive apnea in moderately to severely obese people, most of whom try to sleep on their backs.
Many people suffer from sleep apnea (episodes of cessation of breathing during sleep) with a higher percentage of men with the condition outnumbering women.
Obesity hypoventilation syndrome affects breathing, sleep, and heart and lung health.
Causes of Pickwick syndrome
Malignant obesity hypoventilation syndrome (Pickwick syndrome) is a severe multisystem disease due to the effects of obesity.
Pickwick syndrome is a disorder in which poor breathing reduces oxygen and increases carbon dioxide levels in the blood.
The various conditions often associated with obstructive sleep apnea include obesity and a thick, short neck, and reduced muscle tone of the soft palate, the uvula (the small, conical, fleshy tissue that hangs from the center of the soft palate), and the pharynx. .
The upper airway may be narrowed by enlarged tonsils or adenoids, a deviated nasal septum, nasal polyps, or congenital abnormalities. Even at high altitudes, sleep disruption can occur due to low oxygen concentration.
This condition is related to obesity, but studies show that the syndrome can also exist in non-obese patients, so the exact cause is unknown.
The details of the origin and development of this obesity hypoventilation syndrome, as well as its true incidence, are unknown.
The explanations for inadequate ventilation and reduced functional lung volume are unclear, although there appears to be a decrease in the efficiency of the respiratory muscles, a reduction in respiratory capacity, a decreased ventilatory response to CO2, and an increase in lung dead space. and atelectasis.
The syndrome may be due to a defect in the brain’s ability to control breathing. The extra weight against the chest wall can also make it difficult for the muscles to breathe deeply. This results in too much carbon dioxide and not enough oxygen.
If left untreated, obesity hypoventilation syndrome can lead to heart and blood vessel disease, severe disability, or death.
Pickwick syndrome symptoms
Hypoventilation due to obesity or Pickwickian syndrome is characterized by:
- Marked degrees of obesity.
- Prolonged drowsiness with tiredness during the day.
- Periodic apnea, especially at night, with poor quality of sleep, loud and frequent snoring and / or slow breathing.
- Chronic hypoxemia, hypercapnia (CO2 retention), and secondary polycythemia (excessive red blood cells caused by low oxygen levels) causing cyanosis, a bluish or violet discoloration of the feet, lips and fingers.
Subjects with this syndrome have obesity-related symptoms such as systemic hypertension, diabetes and metabolic syndrome, left ventricular hypertrophy with diastolic dysfunction, pulmonary hypertension, and liver dysfunction.
Complications associated with Pickwick syndrome
Pickwick syndrome is associated with increased use of medical care and a high risk of mortality. This syndrome is largely unrecognized because it is not associated with the multiple medical problems and obesity of patients.
Pickwick syndrome should be suspected in any obese patient with acute respiratory failure.
Complications associated with untreated sleep apnea include increased risk of stroke, blood pressure, osteoarthritis, liver dysfunction, hyperlipidemia, arrhythmias.
Complications include pulmonary artery constriction resulting in pulmonary hypertension and right heart failure, abnormal levels of oxygen and carbon dioxide in the blood, and peripheral edema.
Other complications include sleepwalking, blackouts, automated robot behavior, intellectual impairment, hallucinations, anxiety, irritability, aggressiveness, jealousy, distrust, and irrational behavior.
Loss of interest in sex, morning headaches, and bed-wetting can also occur over time.
Chronic hypoventilation can be associated with congestive heart failure and angina.
Future research is likely to focus on the links between the syndrome and cardiovascular morbidity.
Early epidemiological data suggest a link to coronary artery disease and stroke.
Pickwick syndrome diagnosis
A physical exam and a complete history of your symptoms,
including sleeping habits, is done.
Body mass index is assessed. Height and weight are used to calculate the body mass index which must be less than or equal to 30 kg / m2.
Oxygen levels and carbon dioxide levels are measured. These levels are measured by taking a blood sample from the artery, usually an artery in the wrist.
A pulse oximeter (a sensor loosely attached to the finger) is also used that can be used to obtain an estimate of the amount of oxygen (but not carbon dioxide) in the blood.
So pulse oximetry is not as accurate as an artery blood sample.
There are tests such as X-rays, where a chest X-ray can be taken to rule out any other causes of your shortness of breath.
The sleep study to find out if there is a sleep-related breathing disorder (sleep apnea-hypopnea syndrome or sleep hypoventilation).
Finally, other known causes of hypoventilation are ruled out.
A differential diagnosis of other conditions such as:
- Sleep apnea and other sleep-related breathing disorders (although these can coexist).
- S Prader-Willi syndrome .
- Beckwith-Wiedemann syndrome.
- Narcolepsy .
- Use and / or abuse of sedatives and antihistamines .
- Sleep deprivation.
Pickwick syndrome treatment
Treatment of this condition includes short-term measures (correction of acute hypercapnia and treatment of triggers for the acute decompensation episode) and long-term measures (aimed at weight loss, maintenance treatment with non-invasive ventilation, and management of comorbidities).
The therapy consists mainly of weight loss, which reduces episodes of sleep apnea and improves blood gases and drowsiness during the day.
Due to its proven benefits in reversing the multiple metabolic and organic dysfunctions associated with Pickwick syndrome, bariatric surgery should always be considered in this condition.
Periodic apnea can be caused by intermittent upper airway obstruction other than Pickwick syndrome.
Such obstruction often responds to appropriate treatment, including surgical therapy, continuous positive airway pressure, and oral appliances designed to maintain airway patency during sleep.
These breathing devices include continuous positive airway pressure or positive airway pressure through a mask that is worn over the nose and mouth.
These are usually used only at night to help the patient sleep better.
Nighttime positive pressure airflow can be dramatically effective.
Treatment may include, in addition to respiratory assistance therapies, tracheostomy.
A tracheostomy is an opening in the windpipe. During this procedure, surgeons create a small opening in your neck to help you breathe through a breathing device.
The prognosis is improved with early recognition, weight loss.
The syndrome is reversible if treated, even a moderate reduction in weight can reverse the hypoventilation syndrome. It is recommended to continue the treatment with diet and exercise.