Berdon syndrome
Berdon Syndrome
Berdon Syndrome (pronunciation: bur-don sin-drohm), also known as Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS), is a rare congenital disorder characterized by a largely distended urinary bladder (megacystis), very small colon (microcolon), and decreased or absent intestinal movements (intestinal hypoperistalsis). The syndrome was first described by Walter Berdon, an American radiologist, in 1976.
Etymology
The syndrome is named after Walter Berdon, who first described the condition in 1976. The term "megacystis" is derived from the Greek words "mega" meaning large, and "kystis" meaning bladder. "Microcolon" is derived from the Greek words "micros" meaning small, and "kolon" which is the Greek term for the large intestine. "Hypoperistalsis" is derived from the Greek words "hypo" meaning under, and "peristellein" which means to wrap around, referring to the decreased intestinal movements.
Symptoms
The main symptoms of Berdon Syndrome include a largely distended urinary bladder, a very small colon, and decreased or absent intestinal movements. Other symptoms may include abdominal distension, vomiting, constipation, and urinary tract infections.
Diagnosis
Diagnosis of Berdon Syndrome is typically made based on the characteristic clinical and radiological findings. Prenatal ultrasound may show a large bladder and a small colon. After birth, a series of tests including abdominal ultrasound, abdominal X-ray, and a barium enema may be performed to confirm the diagnosis.
Treatment
There is currently no cure for Berdon Syndrome. Treatment is supportive and may include management of urinary tract infections, nutritional support, and in some cases, surgical intervention to manage the bladder and bowel problems.
Prognosis
The prognosis for individuals with Berdon Syndrome is generally poor, with most infants not surviving beyond the first year of life due to complications such as sepsis and malnutrition.
See Also
External links
- Medical encyclopedia article on Berdon syndrome
- Wikipedia's article - Berdon syndrome
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