19. Cronkhite-Canada Syndrome
Definition
Cronkhite-Canada syndrome is a very rare, sporadically occurring, uninherited disorder. It is characterized by gastrointestinal polyps, cutaneous pigmentation, alopecia, and onychodystrophy.
Incidence
Cronkhite-Canada syndrome is an extremely rare disease. Since first described in 1955, 467 cases have been reported through the year 2002. The vast majority of cases have been documented in Japan, but cases have been reported worldwide. There is a very slight male to female difference in rate of incidence: 1.3:1 to 1.5:1.
The disease may manifest itself at virtually any age; however, fewer than 10 cases in infants have been reported since 1955. Cases have been reported in adults from 31 to 86 years of age.
Etiology
The etiology of Cronkhite-Canada syndrome is not known at present. Currently available evidence and data do not support a familial predisposition to developing the disease. Reports have linked incidences of the disease to arsenic poisoning.
Signs and Symptoms
• Cataracts
• Change in taste sensation
• Cheilosis papillary atrophy of the tongue
• Chronic or recurrent watery diarrhea, sometimes melena
• Difficulty swallowing
• Edema (mild and peripheral to massive anasarca)
• Episodic or constant abdominal pain (upper or lower)
• Hypogeusia (may or may not be present)
• Loss of ability to smell
• Macrocephaly (infants and children)
• Muscle wasting
• Nail dystrophy leading to onycholysis
• Positive Chvostek and Trousseau signs
• Progressive anorexia
• Progressive weight loss
• Rapid alopecia (may include scalp, eyebrows, face, axillae, pubic areas, legs)
• Seizures
• Sensory neuropathy
• Skin hyperpigmentation
• Symmetric, desquamating rash on lower back, buttocks, genital areas, lips, and perioral region (infants and children)
• Syncope
• Vestibular disturbances (gaze-evoked nystagmus, disequilibrium)
• Vitiligo
• Xanthelasma
Medical Management
Therapy consists almost exclusively of interventions to treat symptoms; preventive measures are unavailable because of the lack of knowledge regarding the disease etiology. The ultimate goal is correction of fluid, electrolyte, and protein abnormalities. The patient is prone to frequent, high-volume, watery bowel movements. Regulation of the frequency of bowel movements assists in regulation of the fluid, electrolyte, and protein imbalances. Systemic corticosteroids administered in combination with an antiplasmin have demonstrated effectiveness, as have dietary changes and hyperalimentation. Antibiotics correct intestinal bacterial overgrowth. Severe anemia may necessitate transfusions.
Surgical intervention is typically reserved to treat complications of Cronkhite-Canada syndrome that may arise. These surgically amenable complications include prolapse, bowel obstruction, or malignancy.
Complications
• Cachexia
• Fluid, electrolyte, and protein imbalances
• Gastric polyp-bearing mucosal prolapse
• Gastrointestinal bleeding
• Heart failure
• Intussusception
• Thromboembolic episodes secondary to dehydration
Anesthesia Implications
The patient’s fluid and electrolyte levels should be assessed preoperatively. Electrolyte imbalances should be corrected preoperatively. Protein losses and cachexia may increase the bioavailability of many anesthesia-related medications. The doses of many anesthesia-related drugs may need to be reduced because of low serum proteins and/or cachexia. Fluid level correction must be tempered to avoid heart failure, but will be helped by correction of the low serum protein levels. Chronic corticosteroid administration may require a prophylactic stress response dose of hydrocortisone. The presence of anemia may lower the transfusion threshold, particularly for larger, more complex surgical procedures.
