Prune belly syndrome
Prune belly syndrome
Prune belly syndrome (pronunciation: /pruːn bɛli sɪndroʊm/), also known as Eagle-Barrett syndrome or Triad syndrome, is a rare, genetic disorder characterized by the partial or complete absence of abdominal muscles, urinary tract malformation, and undescended testes in males.
Etymology
The term "prune belly" refers to the distinctive wrinkled appearance of the abdomen, similar to a dried plum or prune. The syndrome was first described by Dr. John M. Barrett in the 19th century, hence the alternative name Eagle-Barrett syndrome.
Symptoms
The primary symptoms of Prune belly syndrome include:
- Abdominal muscle deficiency: The absence or underdevelopment of abdominal muscles, giving the belly a prune-like appearance.
- Urinary tract abnormalities: These can range from mild dilation to severe kidney damage.
- Cryptorchidism: This is the medical term for undescended testes in males.
Causes
The exact cause of Prune belly syndrome is unknown, but it is believed to be genetic in nature. Some researchers suggest it may be due to a disruption in the development of the mesoderm (middle layer of an embryo) during gestation.
Diagnosis
Diagnosis of Prune belly syndrome is typically made prenatally through an ultrasound. Postnatal diagnosis is based on the characteristic physical features.
Treatment
Treatment for Prune belly syndrome is symptomatic and supportive. It may include surgery to correct urinary tract abnormalities and cryptorchidism, and physiotherapy to improve muscle tone.
Prognosis
The prognosis for individuals with Prune belly syndrome varies depending on the severity of the urinary tract abnormalities and the presence of associated conditions.
See also
References
External links
- Medical encyclopedia article on Prune belly syndrome
- Wikipedia's article - Prune belly syndrome
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