Prune-belly syndrome is a rare disease whose incidence is estimated in 1 case every 35.000/50.000 live births. The syndrome affects almost exclusively males and is characterized by congenital absence (aplasia) of the muscles of the abdominal wall, cryptorchidism (undescended testes in the abdominal cavity) and abnormalities of the urinary tract.
Several attempts have been made to surgically reconstruct the abdominal wall (abdominoplasty).
Randolph and colleagues (1977) had tried a U-shaped transverse incision with the following excision of the exciding lower abdominal flap and advancement of the band and the muscle. This technique, however, besides being difficult to perform, has proved ineffective, requiring numerous revisions over time.
Ehrlich and colleagues (1986), instead, had described a technique characterized by overlapping fasciomuscular flaps in a "double-breasted" way. This technique was subsequently amended by Monfort (Monfort intervention) with results considered to be aesthetically better: the action involves bilateral vertical incision of the fascia with removal of the wall in excess and subsequent progress and reunion of longitudinal strips (the navel is preserved). Another operation is the one described by Ger and Coryllos (2000), who proposed the reconstruction of the muscles through the transposition of the rectus femoris.
Another, newer technique has however been described by Firlit. It consists in recasting the abdominal wall in an extraperitoneal way, namely not to affect the band and/or enter the peritoneal cavity, thereby reducing the possibility of complications and adhesions. According to the authors, this technique seems to give noticeable and lasting results from the aesthetic point of view. Since 2005, Firlit technique has also been performed laparoscopically (i.e. opening the abdomen) with even fewer complications and further improvement of aesthetic results. Dr. Israel Franco is one of authors describing this technique.
References
Levine E, Taub PJ, Franco I. Laparoscopic-assisted abdominal wall reconstruction in prune-belly syndrome. Ann Plast Surg. 2007 Feb;58(2):162-5. PMID: 17245142.
Franco I. Laparoscopic assisted modification of the firlit abdominal wall plication. J Urol. 2005 Jul;174(1):280-3. PMID: 15947667.
Furness PD 3rd, Cheng EY, Franco I, Firlit CF. The prune-belly syndrome: a new and simplified technique of abdominal wall reconstruction. J Urol. 1998 Sep;160(3 Pt 2):1195-7; discussion 1216. PMID: 9719308.
Woodard JR. Prune-belly syndrome: a personal learning experience. BJU Int. 2003 Oct;92 Suppl 1:10-1. PMID: 12969002
Bukowski TP, Smith CA. Monfort abdominoplasty with neoumbilical modification. J Urol. 2000 Nov;164(5):1711-3. PMID: 11025757
