What Is Eagle-Barrett Syndrome?
Eagle-Barrett Syndrome which is also known by the name of Prune Belly Syndrome is an extremely rare pathological condition in which the affected individual has partial or complete absence of the abdominal muscles and the stomach is underdeveloped.
The affected individual also has undescended testes along with multiple abnormalities of the urinary tract. Some of the urinary abnormalities include abnormal widening of the tubes which carries urine to the ureters. There is also abnormal accumulation of the urine in the ureters or the kidneys or a backflow of the urine from the bladder back into the ureters.
Some of the other abnormalities associated with Eagle-Barrett Syndrome include underdeveloped lungs and dysfunctional kidney. The reason as to why this condition occurs is not yet known but research is ongoing so as to identify a cause for the development of Eagle-Barrett Syndrome.
What Are The Causes Of Eagle-Barrett Syndrome?
The root cause for Eagle-Barrett Syndrome is not yet known but there have been many suggestions put forward by various researchers that could potentially be the cause of Eagle-Barrett Syndrome.
One theory suggests that Eagle-Barrett Syndrome may result from bladder abnormality during fetal development causing this condition. The reason behind this is stated as when there is abnormal accumulation of urine within the ureter and the bladder there is enlargement of the bladder leading to atrophy of the abdominal muscles.
This urinary retention is also believed to be the cause of undescended testicles. The second theory for the development of Eagle-Barrett Syndrome suggests that underdeveloped abdominal muscles are the cause of Eagle-Barrett Syndrome.
The third and final theory suggests that a nervous system defect is a possible cause for the abnormalities in the abdominal muscles resulting in the development of Eagle-Barrett Syndrome.
These theories put together various observations made by researchers which may point towards a cause of this condition but there is no actual proof that any one of these above mentioned theories are actually the cause of Eagle-Barrett Syndrome.
What Are The Symptoms Of Eagle-Barrett Syndrome?
The primary presenting feature of Eagle-Barrett Syndrome is complete or partial absence of abdominal muscles or underdeveloped abdominal muscles which can be detected at the time of birth which gives the stomach a prune like appearance and hence this condition is also called as Prune Belly Syndrome.
The thickness of the muscles above the bladder is at a minimum in this condition. The abdomen appears abnormally large and is lax. The bladder is also enlarged in cases of Eagle-Barrett Syndrome.
The bladder neck is usually obstructed which causes bladder and urinary retention. The bladder and ureter opening may also be quite narrow or in some cases completely closed.
There is also kidney and ureteral obstruction. In some cases, the affected individual may also have renal cysts as a result of Eagle-Barrett Syndrome. The affected individual may also have hematuria or pyuria signaling to the possibility of an infection. Undescended testicles are also one of the features of Eagle-Barrett Syndrome.
How Is Eagle-Barrett Syndrome Diagnosed?
The diagnosis of Eagle-Barrett Syndrome is usually made shortly after the birth of a child. The characteristic features of the condition with an abnormally large abdomen may be a pointer towards this condition.
Once a diagnosis of Eagle-Barrett Syndrome is suspected radiographic studies in the form of MRI or an ultrasound of the abdomen will clearly show abnormalities of the muscles of the abdomen confirming the diagnosis of Eagle-Barrett Syndrome.
How Is Eagle-Barrett Syndrome Treated?
The treatment for Eagle-Barrett Syndrome is mostly symptomatic and depends on the severity of the condition. In some severe cases, surgical treatment will have to be done to correct the abnormalities that are present as a result of Eagle-Barrett Syndrome.
Some of the procedures that may be done is creating an opening in the bladder through the abdomen thus getting rid of the urinary obstruction and help in voiding. Surgical procedure may also be required to correct the undescended testicles and correct the abnormality.
In some cases more complicated surgical procedures such as bladder reconstruction, widening of the urethra, and detrusor augmentation have been quite successful in treating the symptoms of Eagle-Barrett Syndrome. In extremely rare cases, kidney transplantation has been required to treat Eagle-Barrett Syndrome.