Abdominal Wall Reconstruction in the Prune Belly Syndrome By Judson Randolph, Clint Cavett, and Gloria Eng Washington, D.C. e ln our institution, 12 patients have been encountered with prune belly syndrome. Eight children have undergone evaluation of their abdominal musculature by electromyography. Results of their studies show that major functioning or recoverable muscle exists in the lateral and upper sector of the abdomen, but that little or no muscle exists in the lower central abdomen. Based in part on these findings, an operation has been devised which spares all potentially functioning musculature and corresponding m o t o r nerves, and disposes of nonfunctioning and nonrecoverable muscle. In terms of cosmetic appearance and gross motor testing, these growing boys show significant improvement. INDEX W O R D S : Prune belly syndrome.
have described an overall management plan for patients born with this syndrome. 5'1~The purpose of this report is to describe in detail our experience with a procedure devised to reconstruct the flaccid abdominal wall, preserve that portion of the parietes that contains functioning muscles, and to spare all motor nerves to the retained segments. Surface electromyography has been used to help delineate potential function and predicted recoverability of various abdominal segments. The present information is derived from experience with the abdominal wall tailoring procedure in eight children. CLINICAL MATERIAL
ALE I N F A N T S born with congenital deficiency of the abdominal musculature, bilateral undescended testicles, and malformation of the urinary collection system have been the subject of much published controversy. In the first place, a number of interesting theories have been advanced about the etiology of this constellation of anomalies.! '2 Additionally, arguments have centered on the operative versus the nonoperative management o f : t h e urinary collection system. 3 5 Surgeons have also debated the timing and the techniques for orchiopexy) 8 Finally, suggested management of the lax abdominal wall has ranged from exercises and corsetry to expectant observation. 9 Ten years ago in our institution, a project was begun that included generous excision of the redundant lower abdominal wall. Previous studies from this surgical service
M
From the Departments of General Surgery and Physical Medicine, at Children's Hospital National Medical Center, and the Departments of Surgery and Child Health, of George Washington University, Washington, D.C. Presented before the Twelfth Annual Meeting of the American Pediatric Surgical Association together with the British Association of Paediatric Surgeons, Tarpon Springs. Florida, April 29-May 2, 1981. Address reprint requests to Dr. Randolph, c/o Children's Hospital, I l l Michigan Avenue, N.W., Washington, D.C. 20010. 9 1981 by Grune & Stratton, Inc. 0022-3468/81/1606~037501.00/0 960
Twelve patients with the prune belly syndrome (PBS) have been seen in our hospital. Eight of these patients have undergone a surgical effort to improve abdominal wall function and appearance (Table 1). The first operation was performed on a 6-yr-old boy Who was undergoing ureteral reimplantation and reduction Cystoplasty. His abdominal muscle reconstruction was te,tative, with excision of only a small amount of abdominal wall in the suprapubic region. Thereafter four patients were repaired by using essentially the same type of incision described below, under Operative Technique. Beginning 3 yr ago, postoperative abdominal wall status was assessed by surface electromyography. Three patients had such marginal improvement by clinical appraisal and by EMG that a second procedure, i.e., more extensive resection, was Carried out. In each instance, this was Coupled with a planned intraabdominal procedure on the urinary system or testicles. The last three patients seen have undergone preoperative mapping r their abdominal muscle components and have also been stud~ed after surgical correction.
Electromyography The electromyographic study is accomplished by using surface electrodes to recover over what' would normally be the rectus and oblique muscles of the abdomen. Contraction of the muscles is facilitated by assisting the child in flexion sit-ups. The recorded potentials are visualized on the TECA-4 cathode ray oscilloscope (Model TE4EMG, TECA Corp., White Plains, N.Y.) and documented on the high speed direct recorder (Fig. 1). The recruitment pattern of voluntary motor units are graded from 0 to 5, the last being a normal, full contraction. Delineation of recorded potentials suggest a pattern of involvement in PBS. The upper rectus muscles and upper oblique muscles at the costoehondral margin as well as the flank usually have fairly good musculature. The lower abdominal wall including the lower recti and lower external obliques are frequently nonexistent (Table 2). Journal of Pediatric Surgery, Vol. 16, No. 6 (December),1981
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Table 1. Abdominal Wall R e c o n s t r u c t i o n in PBS Patient
Age
(yr)
Pre Op
EMG
Post Op
Post Op
EMG-1
EMGg-2
Assessment
JS
14
41413
41413
Excellent*
KH TR JT'
7 7 6 5 3 2 1 ~/2
312/2 41312 3/3/1 31211 3/2/1 31211
31212 41312
Excellent*
MA RM AH JT 2
3/211 2/111 31111
Good Good
31311
3/1/0
Excellent Good Good Recently Operated
31211= upper/middle/lower abdomen *Following second operation
Occasionally, one side of the abdominal musculature may be more intact than the other, which portends the development of a rotational scoliosis of the spine.
Operative Technique An incision was originally planned with four objectives in mind: (1) to excise the least recoverable and most redundant parts of the abdominal wall; (2) to gain adequate exposure for surgical correction of the urinary collecting system and the undescended testes; (3) to preserve all motor nerves to the retained abdominal musculature; and (4) to reattach the retained muscular units to the bony pelvis. With the patient anesthetized and supine on the operating table, a general assessment of the redundancy of the abdominal wall is made. Sterility is best provided by having an attendant lift the patient's legs leaving only the shoulders and head resting on the operating table. The skin is then prepared circumferentially around the trunk and the patient positioned on sterile sheets. Draping should permit exposure of the lower chest and all of the abdomen including the groin and pubis. The area of the abdominal wall to be excised is now carefully marked out, taking into account the EMG studies, and the amount of redundancy that has been demonstrated by traction of the abdominal wall (Fig. 2). A mark is made in the midline at the pubis. A second mark is made also in the midline, at that point above the pubis where the estimated upper semicircle of incision will be made. Identical
Fig. 1. T r a c i n g s f r o m e l e c t r o m y o g r a p h y . S t r o n g activity is seen in u p p e r abdominal samples. Essentially no recorded a c t i v i t y is f o u n d in lower abdomen.
Table 2. Depiction of EMG Assessment in Individual Patients First
Pre Operative EMG
Post Operative EMG
X
MA Born 7175
X
3222 10
~ 10
p
P
p
7178
6179
x
x
3343 23
23
4444 33
P
P
p
4177
4178
7179
EMG Muscle Grading 5 = Normal 4 = Good
X 3332 22
4/77
x TB Born 8173
Second Post Operative EMG
3 = Fair 2 = Poor
1 = Trace 0 = None
X= xiphoid P = pubis
Fig. 2. Diagram of proposed lines of incisions. Modest recordings of muscle activity are depicted in u p p e r h a l f of the abdomen, commensurate w i t h l o c a t i o n o f recognizeable muscle at operation.
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Fig. 3.
RANDOLPH, CAVE'I-I-, AND ENG
Midline incision A to B connects the lower and proposed upper incision. Full thickness of abdominal wall is excised
by halves. marks are now made laterally in each costovertebral angle. From these lateral marks, lines are drawn in a curve to the anterior iliac crest, thence, along the groins to the mark at the pubis. Beginning again at the mark in the costovertebral area, a second line is drawn in a gentle curve encompassing an ever-widening portion of lower abdominal wall until the upper central mark is reached. This line is continued in a matching fashion to the opposite costovertebral angle and the sketch now resembles a giant smile as might be put on a pumpkin to make a jack-o-lantern. The incision is begun in the lower line through skin, subcutaneous tissue, the thin fascia planes and into the peritoneal cavity. The line is followed as a full thickness cut to the lateral-most point in the each costovertebral angle. All vessels are clamped and ligated. At this time, additional intraabdominal operative maneuvers, such as revision of the urinary system, orchiopexy, or appendectomy can be performed. Excision of the full thickness of redundant abdominal wall is best b e g u n by making an incision in the midline, connecting the lower incision and the proposed upper one. The redundant abdominal tissue is next excised in halves, along the upper line (Fig. 3). Closure is commenced by placing key sutures of 2-0 silk at the anterior iliac spine and at each pubic tubercle (Fig. 4). These sutures should catch the periosteum of the bony structures and include such fascia and muscle of the abdominal wall as is available in the opposing tissue edge. A final assessment of tension and remaining redundancy is made at this point. Occasionally excision of a little more of the abdominal wail, especially in the lateral portion is required. Each section of the incision between the key sutures is closed with permanent sutures which pierce all the abdominal layers except skin and subcutaneous tissue. The subcutaneous tissue is closed with running sutures of 4-0 nylon or with staples. Careful management of nasogastric decompression is imperative to minimize postoperative abdominal distention.
Fig. 4. Remaining abdominal wall is closed beginning with key sutures in the periosteum of the pubis and of the anterior iliac spine.
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RESULTS
In four patients an immediate and gratifying improvement of abdominal contour has been realized. In the remaining four boys, improvement was achieved, but major abdominal protruberence remained. In three of these, a second abdominal tailoring has been carried out; all three of these children now have a satisfactory abdominal wall. All seven of these youngsters are classified as having good cosmetic outcome and improved function. One boy at age 18 mo has recently been operated upon and although his early postoperative result is pleasing, it is too early to make a final appraisal. As shown in Tables 1 and 2, function as judged by E M G ranges from poor to satisfactory in the sectors of the abdominal wall that remain after surgical reconstruction. In two, the muscles are classed as 3 or 4 indicating substantial muscle function. Three children have scoliosis and are being closely monitored. It is our hope that children may be less prone to develop scoliosis after repair of the abdominal wall. Older 9patients who are able to form an opinion and all
parents of the children are pleased with the improvement offered by the procedure. CONCLUSIONS
1. An operation has been described that eliminates a major portion of the abdominal redundancy in children with the p r u n e belly syndrome. 2. Improvement in function is achieved by discarding the nonfunctioning abdominal wall and anchoring the remaining potentially recoverable muscle elements to the bony pelvis. 3. The incision is planned so that none of the motor nerves to retained muscles are sacrificed. 4. Exposure of the peritoneal cavity is such that corrective surgery of the urinary drainage system or of testicular position can be readily carried out. 5. Surface electromyography delineates with precision functioning muscle units in the abdominal wall before surgery, and plots improvement in certain areas when gains are made by operation.
REFERENCES
1. Eagle JF, Barrett GS: Congenital deficiency of abdominal musculature with associated genito-urinary abnormalities: A syndrome. Pediatrics 6:721-726, 1950 2. Gray SW, Skandalakis JE: The anterior body wall, in: Embrology for Surgeons: The Embryological Basis for Treatment of Congenital Defects. Philadelphia, Saunders, 1972. 3. Duckett JW: Prune belly syndrome. Dialog Pediatr Urol 3:1-8, 1980 4. Perlmutter AD: Reduction cystoplasty in prune belly syndrome. J Urol 116:356-360, 1976 5~ Randolph JIG: Total surgica! reconstruction for patients with abdominal muscular deficiency (prune belly) syndrome. J Pediatr Surg 12:1033-1043, 1977
6. Fowler R, Stephen FD: The role of testicular vascular anatomy in the salvage of the high undescended testicle. Aust NZ J Surg 29:92-97, 1959 7. Gibbons MD, Cromie W J, Duckett JW Jr: Management of the abdominal undescended testice. J Urol 122:7679, 1979 8. Woodward JR, Parrott TS: Orchiopexy in the prune belly syndrome. Br J Urol 50:348--351, 1978 9. Welch K J: Abdominal muscular deficiency syndrome (prune belly), in Ravitch MM et al (eds): Pediatric Surgery, vol 2. Chicago, Year Book, 1979, pp 1191-1203 10. Randolph JG, Cavett C, Eng G: Surgical correction and rehabilitation for children with "prune belly" syndrome, Ann Surg 193:757-762, 1981
Discussion David Trump (Phoenix): We have operated upon four or five of these children through a midline abdominal incision, freeing up the fascia and then overlapping them. We were impressed how bad our results were. I was intrigued with Dr. Randolph's paper because it illustrates what my problem has been. The postoperative problem with our patients has been in the lower abdomen, just as Dr. Randolph has said. His
operative procedure would seem to help with that particular area of the abdominal wall reconstruction. Judson Randolph (Closure): Dr. Kleinhaus, the equipment is cumbersome; the studies are done in our Physical Medicine department and I don't know if the process could be adapted to sterile technique. I believe that the correlation with measured tension in the operating room is
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accurate enough. I appreciate Dr. Trump, with his customary honesty, sharing his own experience, which emphasizes that the approach described today is a logical one. Dr. Welch has carefully analyzed the large series in Boston; he mentioned that respiratory problems can occur in these children because of ineffectual abdominal muscle function, another indication for
RANDOLPH, CAVEI-r, AND ENG
repair. I would agree with him that these patients present a spectrum. There are good "prunes" and there are bad "prunes." Certainly, the picture of that 31-yr-old man lets us know that some patients will require an operation to improve their abdominal appearance and function.