[size=20px]You are called to see a newborn boy with prune belly syndrome. Which of the following statements regarding the surgical care of patients with prune belly syndrome is true?

 

A malrotation is uncommon in this population

 

B anesthesia risk is normal

 

C abdominal wall reconstruction often improves constipation

 

D mortality rate is five percent

 

E pulmonary function is normal[/size]

A

C

C

Correct

 

Prune belly syndrome is rare - occurring in one in 40,000 patients. Observations regarding the care of these patients come from small single institution reports. The patients appear to fall into 3 groups.

 

group 1 (20%) - poor renal function and pulmonary hypoplasia dying shortly after birth

group 2 (40%) - anatomic and physiologic function issues suffering from urosepsis and obstruction , one-third of this group will die in the first two years of life from renal failure and/or sepsis

group 3 (40%) - normal function, mild urinary abnormalities and has a normal life expectancy

Malrotation occurs in 50% of patients with this syndrome and most patients have difficulties with constipation. Pulmonary problems are common because of the patient’s inability to cough well due to the abdominal wall flaccidity. For this reason, these patients seem to have a higher risk from anesthesia. Abdominal wall reconstruction appears to improve both pulmonary function and constipation.

 

Orchiopexy is a secondary surgical focus in these boys. Open orchiopexy can be done if there is need for abdominal wall reconstruction. This open technique has acceptable results and is conveniently done while the belly is open. However, this often occurs when the child is older and likely renders the testes infertile. A more appropriate goal is to accomplish the orchiopexy during the first year of life if the child can tolerate a laparoscopic procedure. The two stage Fowler Stephens technique seems to have the best results. It can be done with a laparoscopic technique very effectively. A standard groin approach will be difficult due to the high location of these testes.