Total Surgical Reconstruction for Patients With Abdominal Muscular Deficiency (“Prune-Belly”) Syndrome By Judson G. Randolph l In with seen.
the Their
decade,
high
usually
tubeless
has
been
at one stage.
bilateral plantation cystoplasty,
has
shortening, of (3)
the
been
consisted
This ureters,
out,
consists and (2)
of
diver-
Thereafter, carried
tapering
bilateral
children have
urinary
pyelostomy.
reconstruction erably
seven
syndrome
management
immediate sion,
past
the prune-belly
of
excision that
endowed fort
at
of that is
with
total
to gratifying children.
part
most
of the abdominal
redundant
musculature.
mechanical
pref-
and This
reconstruction
progress
in
six
least
early
ef-
has
led
of the
seven
muscular
de&
( 1)
reim-
reduction
orchiopexy,
(4) wall
and
INDEX
WORDS:
ciency;
prune-belly
Abdominal syndrome.
T
HE earliest documented report of a newborn with a wrinkled abdomen devoid of muscle is that of Frolich’ in 1839. Parker,2 in 1895, first correctly defined the syndrome of abdominal muscle deficiency and pointed out that it was associated with obstructive uropathy and failure of testicular decent, The protean interests of Sir William Osler-’ led him to report such a baby at the turn of the century. In 1950, 42 patients with this constellation of anomalies had been documented in the literature.4 By this time, it was apparent that the abdominal wall deficiency comprised a spectrum ranging from complete absence of muscle to the presence of all muscular layers as thin but recognizable structures.5 In the 1950s and 196Os, surgeons began to focus on the urologic difficulties of these children. Plans for management were developed that resulted in more reports of living patients. b 8 Early survivors were treated by permanent nephrostomy or various drainage procedures. In the recent past, efforts have been directed toward reconstruction of the distorted collecting system. In an extensive series, Welch and Kearny’ reported 14 survivors, 4 of whom have functioning bladders with reimplanted ureters. Beginning in 1967, we embarked on a program of immediate high tubeless drainage for all patients who showed abdominal muscle deficiency and distortion of the urinary collecting system. Complete reconstruction of the drainage system, abdominal wall, and malpositioned testicles has subsequently been carried out. This report summarizes the clinical experience with seven patients seen on the Surgical Service of the Children’s Hospital of Washington, D.C., during the past decade.
From the Department of Surgery and Child Health and Development, George Washington University, and the Surgical Service, Children’s Hospital National Medical Center, Washington, D.C. Presented before the 8th Annual Meeting of the American Pediatric Surgical Association, Acapulco. Mexico. April 20-23. 1977. Address reprint requests to Judson G. Randolph, M.D.. Department of Surgery. The Children’s Hospital, Washington, D.C. 20010. 1~I977 by Grune & Stratton, Inr. ISSN 0022-3468. Journal of Pediatric Surgery, Vol. 12, No. 4 (December), 1977
1033
JUDSON
I
G. RANDOLPH
Fig. 1. Newborn with congenital absence of abdominal musculature.
PATHOGENESIS
The basic elements of the syndrome consist of partial or complete deficiency of the abdominal musculature (Fig. 1 and 2) obstructive manifestation of the genito-urinary tract (Fig. 3) and undescended testes.‘“,’ Other congenital anomalies frequently associated with this syndrome are persistent urachus, a pigeon-breast deformity of the chest, and some form of talpies.4 All patients are male, and it remains controversial as to whether females purported to have the syndrome do not present a different form of abdominal wall defect, such as suggested that abdominal muslarge lateroPventral hernias. In 1903, Stumme” cular deficiency occurs because the bladder outlet is obstructed in utero. Urine appears during the fourth fetal month and Stumme theorized that over-distension of the bladder occurred because of outlet obstruction, leading to ureteral dilatation and hydronephrosis. As the bladder enlarged, Stumme reasoned, it pressed against the abdominal wall, causing atrophy of the muscles by direct pressure or by interference with blood supply. Descent of the testes, a later embryologic event, was thought to be interfered with by the large bladder. In support of this theory are the following facts. (I) The missing muscles in order of frequency are the lower recti, then the transversus, the obliques, and finally, the upper recti, in other words, the muscles are destroyed from within outwards, and most completely in the area of the bladder. (2) In no reported case is there good evidence that abdominal muscle deficiency is present in the absence of an enlarged bladder. (3) At birth. the abdominal wall shows obvious evidence of long-standing distension, which could be accounted for only by an
“PRUNE-BELLY”
Fi mw
SURGICAL
RECONSTRUCTION
Typical appearance a I-yr-old child.
of
1035
IOX
enlarged bladder or by intrauterine ascites. (4) The presence of the persistent urachus suggests previous lower urinary obstruction. Dilatation of the upper genito--urinary tract almost always follows long-standing distal obstruction, particularly in the developing fetus; an excellent clinical example is to be found in patients born with posterior uretheral valves. However, this latter observation brings Stumme’s theory into question, since the babies with posterior
Fig. 3. Typical configuration of the urinary collecting system in a patient with absent abdominal IllUSCllS.
1036
JUDSON
G. RANDOLPH
uretheral valves, urinary obstruction, and dilated bladders never show abdominal muscular deficiency. An alternative hypothesis suggests that there is a primary somatic defect in the abdominal musculature. The original defect is presumed to be a failure of the myotomes to descend into the ventral wall or failure of their anterior fusion. A concept more consonant with the timing of embryologic events is that failed ventral myotomes permit over-distension of the abdominally positioned fetal bladder. This thesis seems more logical and has the added attraction of explaining the preexisting obstruction of the bladder that is not demonstrated in the newly born infant. DIAGNOSTIC
STUDIES
As soon as it has been established that the newborn infant suffers from congenital absence of the abdominal muscles, assessment of the urinary system should be undertaken. In addition to standard blood counts, urinalysis, and serum electrolytes, laboratory studies should include a BUN, creatinine, creatinine clearance, and urinary electrolytes. The patient should then be studied by intravenous pyelography, cystography, and renal scan. Some have advocated cystoscopy and panendoscopy as part of the evaluation in the first days of life. While this is ultimately of importance in assessing the child’s bladder outlet prior to final reconstruction of the urinary system, it is not a necessary part of the clinical assessment in the newborn period. When the studies are completed, there will be an occasional patient with little or no anatomical derangement of the urinary collecting system. Such patients will be closely monitored, but receive only supportive therapy as needed. However, the majority of infants with abdominal muscular deficiency will show marked distortion of the collecting systems in the form of elongation, tortuosity, dilatation of the ureters, and pelvocaliectasis. In addition, most will show bilateral vesicoureteral rehux, cystomegaly, and a persistent urachus. All patients with dilated collecting systems should have immediate high bilateral tubeless urinary diversion. In most instances, a pyelostomy is easy to accomplish and provides effective drainage. SURGICAL
TREATMENT
Early Management Because the renal pelvis is usually enlarged and the abdominal wall is quite lax, the renal pelvis can readily be brought to the overlying flank skin. A lateral flank incision, with splitting of the oblique muscles, gives excellent exposure. A sizable opening is made in the pelvis to ensure unobstructed flow of urine. Once these drainage sites are established, the patients become easy to treat and easy to study. Renal function and bacteriologic studies are readily obtained from each side. The infant is maintained in this condition throughout the first year of life. During this time, baseline studies are repeated at appropriate intervals so that the end of the year brings a dynamic picture of ongoing renal function. If, at this time, there is no measurable renal function on one side, nephrectomy may be indicated. In most instances, renal function will be im-
“PRUNE-BELLY”
SURGICAL
1037
RECONSTRUCTION
proved. At a year of age, if the condition of the patient satisfactory, total reconstruction is undertaken.
and his kidneys
are
Reconstruction The abdomen is opened through a generous incision that begins in the right flank, passes downward across the suprapubic region, and continues to the left flank. The pyelostomies are not disturbed. The peritoneum is opened, and the huge apron of abdominal wall is easily retracted. Exploratory laparotomy is carried out. Both kidneys are inspected, and the ureters are identified. The testicles are found in their abdominal position. The testicles are dissected free on each side, fully freeing the vas deferens to the bladder and the vessels well up toward the kidney. The testicle will then reach the scrotum in most instances. The ureters are disconnected from the bladder, and the bladder is closed from the outside with interrupted sutures in the muscle layer. The ureters are always found to be tortuous, elongated, and enlarged in diameter. The length and distortion of the ureters is usually astonishing. In the last three of our patients, the disconnected ureter reached well below length, and the antithe knee (Fig. 4). The ureter is cut to the appropriate vascular side of the ureter (which is that part of the ureter away from the entering blood supply) is inspected and cut open for most of its length from the bladder junction to the renal pelvis. Approximately two-thirds of the circumference of the ureter is trimmed away. The remaining posterior wall of the ureter is folded around a No. 8 catheter, using a running locked suture for the upper two-thirds of the repair. Interrupted sutures are then used in the distal portion so that the ureter can be trimmed to length without disrupting a
Fig. tient
4.
Operative
undergoing
photograph
total
repair.
testicle
has
been
scrotum
and
wilt be replaced
pouch.
The
an abdominal length large
left
to the knee.
testicle,
position,
of vessels bladder
brought
and
is open;
in
right
through
the
in a Dartos
which shows
vas the
pa-
The
was
in
adequate
deferens. ureters
The reach
JUDSON G. RANDOLPH
1038
Fig. 5. Postoperativeappearance after total correction. Note that the redundancy of the abdominal wall has been substantially reduced.
running suture line. Each of the two ureters is reimplanted in the bladder using a standard submucosal tunnel after the manner of Politano and Leadbetter. ln most patients, the suprapubic area of abdominal wall musculature is completely absent. It has seemed reasonable, therefore, to remove a rather generous slice of the lower abdominal segment, beginning at the upper end of the incision in the flank and taking away l-2 inches in the centeral suprapublic area. Standard closure is then carried out using interrupted sutures through the existing fascia and peritoneal layers. A suprapubic cystostomy tube is established, and the two catheters from the ureteral reconstructions are led out through a central stab wound with the suprapubic tube. The subcutaneous tissue and skin are closed appropriately. By removing some of the least developed portion of the redundant abdominal wall, significant tightening is achieved (Fig. 5). Thus, there is a real improvement in the redundancy of the abdominal wall without sacrificing significant recoverable muscle. CLINICAL
MATERIAL
Since the summer of 1967, seven male patients with the full blown syndrome have been encountered. Classifying them in the categories developed by Welch’ reveals that six patients were either 11 (moderate) or 111 (severe), and one proved to be in category IV (hopeless). We have seen no patients with normal or near normal upper tracts during the period of study. All had major derangement of the collecting systems, and all seven patients showed major loss of abdominal musculature with distorted, wrinkled, prune-like bellies. Ail had undescended testicles. No females have been seen who could be considered within the definition of the syndrome, although one female patient was encountered who had enormous lateral herniae with prominent coils of intestine beneath the subcutaneous tissue. Although she was originally incorrectly labelled a prune-belly. her rectus muscles and urinary system were intact. In all patients, the defect was noted immediately after birth, and urinary assessment began shortly thereafter. All patients underwent high diversion: one had bilateral loop ureterostomy no and six, bilateral pyelostomy. One patient died 4 wk after birth, having evinced essentially urinary function from birth. The remaining six patients thrived and showed improving function after drainage, as evidenced by scan, pyelogram, and urinary studies. While kidneys on one side or the other were clearly stronger than the mate in four of the six patients, in no instance was a kidney deemed unsalvageable, except in the one death. In Table 1, the main clinical data for each patient is listed.
“PRUNE-BELLY”
SURGICAL
RECONSTRUCTION
1039
Table
1. Clinical
Data
Original
RWlOl Function Date Birth
Right
Left
6/15/67
1+
2+
Patient
J. S.
Tested
of Right
Abdomen
Left
Abdomen
Original
Operation
Loop
Subseauent
Bilateral
ureterostomies
Suraerv
reimplantation,
6/26/60 Bilateral
closure
ureterosto-
1/14/69
mies,
Abdominopexy,
orchiopexy,
1973 R. T.
l/17/71
2+
3+
Abdomen
Groin
Bilateral
pyelostomy
Bilateral
reimplantation
tapering, Closure
and
7/73
left pyelostomy,
1 o/73 Repeat
right
taper, Closure
reimplant
and
2/74 right
pyelostomy,
4/74 T. 8.
B/l
l/73
2+
2+
Abdomen
Groin
Left pyeloplosty Right
BB. S
7/l/74
0
0
Abdomen
J. T.
11/30/74
1+
3+
Abdomen
Abdomen
nephrostomy
Biloterol
Groin
Right
reimplantation,
ob-
dominopexy,
left
orchiectomy,
4/76
pyelostomy
Death
ot 4 wk
-
Bilateral
tapering
and re-
implantation orchiopexy
with and ab-
dominopexy,
4/24/75
Lysis of adhesions, Lodd
procedure
rotation, Right
right
lostomy, 12/0/74
1+
3+
Abdomen
Groin
Bilateral
pyelostomy
Bilateral plant,
5/19/75
abdominopexy
closure
K. H.
5/7/75
pyelostomy,
Second
5/4/75
for mot-
6/76
tailoring,
reim-
orchiopexy,
and
abdominopexy, Left ureterostomy
1 Z/75 and right
reimplantation, Closure M. H.
7/27/75
1+
l+
Abdomen
Abdomen
Bilateral
pyelostomy
Bilateral plant,
6/76
pyelostomies, tailoring, orchiopexy,
obdominopexy, Closure
and
pye-
pyelostomies,
lo/76 reimand 6/76 l/77
.RESlJLTS
There was one death in this group of seven patients. That patient was seen promptly and underwent immediately bilateral pyelostomy without ill effect. This drainage functioned well, and there was no infection. However, the kidneys were unable to concentrate, and all parameters showed essentially no renal function; this situation did not improve after the institution of high drainage, and the patient died in renal failure at 30 days of age. The 6 children have been followed for 10, 6,4, 3, 3, 2&, and 2 yr, respectively.
JUDSON
1040
G.
RANDOLPH
Tnble 2. Results
Testes
______
Patient (Age)
Abdominal Wall
J. S. (10)
Excellent
R. T. (6)
Needs
T. 6. (4)
Good
66.T. s.(3) J.
Dead Good
K. H. (24)
Excellent
M.
Good
Ii. (2) t
, Testicle
up;
repair
Kidney Left
Creotinine
BUN
(mg/ 100 ml)
(mg/ 100 ml)
Right
Left
Right
1
1
2+
3+
0.8
1
t
i
0
2+ 2+
2t 3+
0.6 0.8
1
2+
3+
1
2+
3+
0.7
772+4+
i 4
, testicle
down;
0, testicle
Bladder
Growth
9
Excellent
Normal
a
Excellent
Normal
Hypertension
14
Incontinent
Normal
Satisfactory
z
1
Excellent
Normal
Excellent
0.8
31
Incontinent
Normal
Excellent
34
Excellent
Normal
Good
Overall Excellent
sacrificed.
Fig. 6. (A) IVP in a patient at 9 mo of age. (g) Same patient after repair, now 7 yr old. (C) Some patient at 8; yr. Note measured kidney growth.
1041
“PRUNE-BELLY” SURGICAL RECONSTRUCTION
Fig. 7. Patient at 9 yr of crge. lateral muscle can be seen. He can do sit ups and does not wear a corset.
In five of these children, there was measured improvement in renal function following early pyelostomy drainage. The sixth child had reconstruction without pyelostomy drainage, but we now consider this unwise. Reconstruction has taken place at various ages but, ideally, it should be performed as a total operation at a year of age, as in the last three patients. There have been a total of 19 operations in the 6 living patients or an average of 3 per child. One of the children had two abdominal procedures to relieve intestinal obstruction following the major intraabdominal reconstruction. In another child, it was impossible to bring down the left testicle, and it was sacrificed. As shown in Table 2, the patients are generally doing well. While no kidney shows perfect function, at present, all are capable of providing renal function adequate for health, development, and normal growth of the children (Fig. 6). Infection has not been a major problem but has necessitated revision of an implanted ureter in two patients. The testes are normal in position and size. Bladder function is good, but two patients are being watched because of incontinence. One patient, age 6 yr, has hypertension and requires Apresoline for control. All others are normotensive. The abdominal muscularture has been followed clinically and found to strengthen in a very pleasing way. This is particularly true of the lateral and upper abdominal muscles, which appear less deficient than the lower central area. The IO-yr-old (Fig. 7) can now do sit-ups repetitively and is working hard on an exercise program. We have specifically avoided the use of girdles or abdominal slings, preferring to encourage the patients to develop the muscle available. Four of the patients have been studied by electromyography. This technique allows us to plot existing muscle function and also permits some evaluation of potential muscle that can be strengthened. ACKNOWLEDGMENT Dr. Fred Frensilli primary
surgeon.
has permitted
Dr.
boys’ muscular function
Gloria
the inclusion
of his patient
Eng has enthusiastically
by electromyography.
(case 2) for whom
accepted
the challenge
he has been the
of mapping
these
JUDSON
1042
G. RANDOLPH
REFERENCES 1. Frohlich F: Der Mangel der Muskeln lnsbesondere der Seitebauchmuskeln. Dissertation. Wurzburg, C.A. Zurn, 1839 2. Parker RW: Absence of abdominal muscles in an infant. Lancet 1:1252, 1895 3. Osler W: Congenital absence of the abdominal muscles, with distended and hypertrophied urinary bladder. Bull Johns Hopkins Hosp 12:331, 1901 4. Eagle JF, Barret GS: Congenital deficiency of abdominal musculature with associated genitourinary abnormalities: A Syndrome. Pediatrics 6:721, 1950 5. Silverman FN, Huang N: Congenital absence of the abdominal muscles, associated with malformation of the genitourinary and alimentary tracts: Report of cases and review of literature. Am J Dis Child 88:91, 1950 6. Lattimer JK: Congenital deficiency of the
the abdominal musculature and associated genitourinary anomalies: A report of 22 cases. J Urol79:343, 1958 7. Waldbaum RS, Marshall VF: The prune belly syndrome: A diagnostic therapeutic plan. J Ural 103:668, 1970 8. Williams DI. Burkholder GV: The prune belly syndrome. J Urol98:244, 1967 9. Welch KJ, Kearney GP: Abdominal musculature deficiency syndrome: Prune belly. J Urol 1 I1:693, 1974 IO. Stumme EG: Ueber die symmetrischen kongenitalen bauchmuskeldefekte und uber die kombination derselben mit anderen bildungsanomalien des rumpfes (hochstand, hypertrophie und dilatation der blase, ureterendilation, kryptorchismus, furchennabel, thoraxdeformitat, etc.) Mitt Grenzgeb Med U Chir I l:S48, 1903
Discussion S.Kim
(Boston): In the past 9 yr, we have seen I6 children suffering from this syndrome; were newborns, and I I were older (up to 16 yr of age). Our preferred treatment has been primary reconstruction without pyelostomy. We have done this in five cases. The other 11 had had previous surgery elsewhere. Nine of the 16 had typical posterior urethral valves. Our primary treatment is a transurethral resection of valves, partial cystectomy, and lower megaureter repair. Nephrectomy is done if function is absent. We avoid nephrostomies. Several children have required pyeloplasties at a later date. We have had 2 newborn deaths, an immediate postoperative death associated with anesthesia complications, and another who died from respiratory problems 31 mo after discharge. When doing orchidopexy in the older child, we have chosen to divide the spermatic vessels, preserve the vessels associated with the vas, and have brought the testes down successfully. The abdominal wall reconstruction has been done in several cases, but it is of interest that in the older children, their abdominal support has improved with time alone. Surgery is difficult, but these cases are not hopeless. With aggressive surgical management, these children can enjoy an excellent quality of life. R. Izant (Cleveland)c 1, too, would like to echo Dr. Kim’s note of encouragement for this bad problem. Some patients with prune-belly do not have as much in the way of obstructive uropathy as Eagle originally said they all did. There are also varying degrees of abdominal wall musculature and fascia, closing the abdominal wall with skin and dividing all the spinal nerves from about T-6 to L-3. We put them back with the control litter. At the end of 6 and 9 mo, we performed IVPs on them to see if the ureters, bladder, and kidneys would enlarge with this greatly enlarged abdominal space, now unprotected by fascia or muscle. The fact was that they had no urinary tract problems, including clearances. At the conclusion of the study, these dogs had perfectly normal kidneys and ureters, and the bladder did not enlarge. This does not mean that Eagle’s theory is right or wrong. It is not an intrauterine experiment, which probably should be done, and possibly the dog is not the ideal animal. J. Randolph (Closing); 1 know what cardiac surgeons “from the bush,” (as Bill Morgan said yesterday) feel like when they get up to present a few cases and then the group from Houston gets up to tell about their 800 cases. But we do appreciate Dr. Kim bringing us the experience
“PRUNE-BELLY”
SURGICAL
from Drs. Hendren.
RECONSTRUCTION
Kim, and Donahoe’s
1043
service.
I think Bob Izant is on to an important
area
of study. There should be some embryologic studies that can be done in the fetus that would bear on this problem. In the McKay-Vaughan Textbook of Pediatrics (1975). there is only one paragraph on prune-belly in which it is stated that essentially nothing can be done for these unfortunate children. So, we all must go home and be missionaries.
the Their
decade,
high
usually
tubeless
has
been
at one stage.
bilateral plantation cystoplasty,
has
shortening, of (3)
the
been
consisted
This ureters,
out,
consists and (2)
of
diver-
Thereafter, carried
tapering
bilateral
children have
urinary
pyelostomy.
reconstruction erably
seven
syndrome
management
immediate sion,
past
the prune-belly
of
excision that
endowed fort
at
of that is
with
total
to gratifying children.
part
most
of the abdominal
redundant
musculature.
mechanical
pref-
and This
reconstruction
progress
in
six
least
early
ef-
has
led
of the
seven
muscular
de&
( 1)
reim-
reduction
orchiopexy,
(4) wall
and
INDEX
WORDS:
ciency;
prune-belly
Abdominal syndrome.
T
HE earliest documented report of a newborn with a wrinkled abdomen devoid of muscle is that of Frolich’ in 1839. Parker,2 in 1895, first correctly defined the syndrome of abdominal muscle deficiency and pointed out that it was associated with obstructive uropathy and failure of testicular decent, The protean interests of Sir William Osler-’ led him to report such a baby at the turn of the century. In 1950, 42 patients with this constellation of anomalies had been documented in the literature.4 By this time, it was apparent that the abdominal wall deficiency comprised a spectrum ranging from complete absence of muscle to the presence of all muscular layers as thin but recognizable structures.5 In the 1950s and 196Os, surgeons began to focus on the urologic difficulties of these children. Plans for management were developed that resulted in more reports of living patients. b 8 Early survivors were treated by permanent nephrostomy or various drainage procedures. In the recent past, efforts have been directed toward reconstruction of the distorted collecting system. In an extensive series, Welch and Kearny’ reported 14 survivors, 4 of whom have functioning bladders with reimplanted ureters. Beginning in 1967, we embarked on a program of immediate high tubeless drainage for all patients who showed abdominal muscle deficiency and distortion of the urinary collecting system. Complete reconstruction of the drainage system, abdominal wall, and malpositioned testicles has subsequently been carried out. This report summarizes the clinical experience with seven patients seen on the Surgical Service of the Children’s Hospital of Washington, D.C., during the past decade.
From the Department of Surgery and Child Health and Development, George Washington University, and the Surgical Service, Children’s Hospital National Medical Center, Washington, D.C. Presented before the 8th Annual Meeting of the American Pediatric Surgical Association, Acapulco. Mexico. April 20-23. 1977. Address reprint requests to Judson G. Randolph, M.D.. Department of Surgery. The Children’s Hospital, Washington, D.C. 20010. 1~I977 by Grune & Stratton, Inr. ISSN 0022-3468. Journal of Pediatric Surgery, Vol. 12, No. 4 (December), 1977
1033
JUDSON
I
G. RANDOLPH
Fig. 1. Newborn with congenital absence of abdominal musculature.
PATHOGENESIS
The basic elements of the syndrome consist of partial or complete deficiency of the abdominal musculature (Fig. 1 and 2) obstructive manifestation of the genito-urinary tract (Fig. 3) and undescended testes.‘“,’ Other congenital anomalies frequently associated with this syndrome are persistent urachus, a pigeon-breast deformity of the chest, and some form of talpies.4 All patients are male, and it remains controversial as to whether females purported to have the syndrome do not present a different form of abdominal wall defect, such as suggested that abdominal muslarge lateroPventral hernias. In 1903, Stumme” cular deficiency occurs because the bladder outlet is obstructed in utero. Urine appears during the fourth fetal month and Stumme theorized that over-distension of the bladder occurred because of outlet obstruction, leading to ureteral dilatation and hydronephrosis. As the bladder enlarged, Stumme reasoned, it pressed against the abdominal wall, causing atrophy of the muscles by direct pressure or by interference with blood supply. Descent of the testes, a later embryologic event, was thought to be interfered with by the large bladder. In support of this theory are the following facts. (I) The missing muscles in order of frequency are the lower recti, then the transversus, the obliques, and finally, the upper recti, in other words, the muscles are destroyed from within outwards, and most completely in the area of the bladder. (2) In no reported case is there good evidence that abdominal muscle deficiency is present in the absence of an enlarged bladder. (3) At birth. the abdominal wall shows obvious evidence of long-standing distension, which could be accounted for only by an
“PRUNE-BELLY”
Fi mw
SURGICAL
RECONSTRUCTION
Typical appearance a I-yr-old child.
of
1035
IOX
enlarged bladder or by intrauterine ascites. (4) The presence of the persistent urachus suggests previous lower urinary obstruction. Dilatation of the upper genito--urinary tract almost always follows long-standing distal obstruction, particularly in the developing fetus; an excellent clinical example is to be found in patients born with posterior uretheral valves. However, this latter observation brings Stumme’s theory into question, since the babies with posterior
Fig. 3. Typical configuration of the urinary collecting system in a patient with absent abdominal IllUSCllS.
1036
JUDSON
G. RANDOLPH
uretheral valves, urinary obstruction, and dilated bladders never show abdominal muscular deficiency. An alternative hypothesis suggests that there is a primary somatic defect in the abdominal musculature. The original defect is presumed to be a failure of the myotomes to descend into the ventral wall or failure of their anterior fusion. A concept more consonant with the timing of embryologic events is that failed ventral myotomes permit over-distension of the abdominally positioned fetal bladder. This thesis seems more logical and has the added attraction of explaining the preexisting obstruction of the bladder that is not demonstrated in the newly born infant. DIAGNOSTIC
STUDIES
As soon as it has been established that the newborn infant suffers from congenital absence of the abdominal muscles, assessment of the urinary system should be undertaken. In addition to standard blood counts, urinalysis, and serum electrolytes, laboratory studies should include a BUN, creatinine, creatinine clearance, and urinary electrolytes. The patient should then be studied by intravenous pyelography, cystography, and renal scan. Some have advocated cystoscopy and panendoscopy as part of the evaluation in the first days of life. While this is ultimately of importance in assessing the child’s bladder outlet prior to final reconstruction of the urinary system, it is not a necessary part of the clinical assessment in the newborn period. When the studies are completed, there will be an occasional patient with little or no anatomical derangement of the urinary collecting system. Such patients will be closely monitored, but receive only supportive therapy as needed. However, the majority of infants with abdominal muscular deficiency will show marked distortion of the collecting systems in the form of elongation, tortuosity, dilatation of the ureters, and pelvocaliectasis. In addition, most will show bilateral vesicoureteral rehux, cystomegaly, and a persistent urachus. All patients with dilated collecting systems should have immediate high bilateral tubeless urinary diversion. In most instances, a pyelostomy is easy to accomplish and provides effective drainage. SURGICAL
TREATMENT
Early Management Because the renal pelvis is usually enlarged and the abdominal wall is quite lax, the renal pelvis can readily be brought to the overlying flank skin. A lateral flank incision, with splitting of the oblique muscles, gives excellent exposure. A sizable opening is made in the pelvis to ensure unobstructed flow of urine. Once these drainage sites are established, the patients become easy to treat and easy to study. Renal function and bacteriologic studies are readily obtained from each side. The infant is maintained in this condition throughout the first year of life. During this time, baseline studies are repeated at appropriate intervals so that the end of the year brings a dynamic picture of ongoing renal function. If, at this time, there is no measurable renal function on one side, nephrectomy may be indicated. In most instances, renal function will be im-
“PRUNE-BELLY”
SURGICAL
1037
RECONSTRUCTION
proved. At a year of age, if the condition of the patient satisfactory, total reconstruction is undertaken.
and his kidneys
are
Reconstruction The abdomen is opened through a generous incision that begins in the right flank, passes downward across the suprapubic region, and continues to the left flank. The pyelostomies are not disturbed. The peritoneum is opened, and the huge apron of abdominal wall is easily retracted. Exploratory laparotomy is carried out. Both kidneys are inspected, and the ureters are identified. The testicles are found in their abdominal position. The testicles are dissected free on each side, fully freeing the vas deferens to the bladder and the vessels well up toward the kidney. The testicle will then reach the scrotum in most instances. The ureters are disconnected from the bladder, and the bladder is closed from the outside with interrupted sutures in the muscle layer. The ureters are always found to be tortuous, elongated, and enlarged in diameter. The length and distortion of the ureters is usually astonishing. In the last three of our patients, the disconnected ureter reached well below length, and the antithe knee (Fig. 4). The ureter is cut to the appropriate vascular side of the ureter (which is that part of the ureter away from the entering blood supply) is inspected and cut open for most of its length from the bladder junction to the renal pelvis. Approximately two-thirds of the circumference of the ureter is trimmed away. The remaining posterior wall of the ureter is folded around a No. 8 catheter, using a running locked suture for the upper two-thirds of the repair. Interrupted sutures are then used in the distal portion so that the ureter can be trimmed to length without disrupting a
Fig. tient
4.
Operative
undergoing
photograph
total
repair.
testicle
has
been
scrotum
and
wilt be replaced
pouch.
The
an abdominal length large
left
to the knee.
testicle,
position,
of vessels bladder
brought
and
is open;
in
right
through
the
in a Dartos
which shows
vas the
pa-
The
was
in
adequate
deferens. ureters
The reach
JUDSON G. RANDOLPH
1038
Fig. 5. Postoperativeappearance after total correction. Note that the redundancy of the abdominal wall has been substantially reduced.
running suture line. Each of the two ureters is reimplanted in the bladder using a standard submucosal tunnel after the manner of Politano and Leadbetter. ln most patients, the suprapubic area of abdominal wall musculature is completely absent. It has seemed reasonable, therefore, to remove a rather generous slice of the lower abdominal segment, beginning at the upper end of the incision in the flank and taking away l-2 inches in the centeral suprapublic area. Standard closure is then carried out using interrupted sutures through the existing fascia and peritoneal layers. A suprapubic cystostomy tube is established, and the two catheters from the ureteral reconstructions are led out through a central stab wound with the suprapubic tube. The subcutaneous tissue and skin are closed appropriately. By removing some of the least developed portion of the redundant abdominal wall, significant tightening is achieved (Fig. 5). Thus, there is a real improvement in the redundancy of the abdominal wall without sacrificing significant recoverable muscle. CLINICAL
MATERIAL
Since the summer of 1967, seven male patients with the full blown syndrome have been encountered. Classifying them in the categories developed by Welch’ reveals that six patients were either 11 (moderate) or 111 (severe), and one proved to be in category IV (hopeless). We have seen no patients with normal or near normal upper tracts during the period of study. All had major derangement of the collecting systems, and all seven patients showed major loss of abdominal musculature with distorted, wrinkled, prune-like bellies. Ail had undescended testicles. No females have been seen who could be considered within the definition of the syndrome, although one female patient was encountered who had enormous lateral herniae with prominent coils of intestine beneath the subcutaneous tissue. Although she was originally incorrectly labelled a prune-belly. her rectus muscles and urinary system were intact. In all patients, the defect was noted immediately after birth, and urinary assessment began shortly thereafter. All patients underwent high diversion: one had bilateral loop ureterostomy no and six, bilateral pyelostomy. One patient died 4 wk after birth, having evinced essentially urinary function from birth. The remaining six patients thrived and showed improving function after drainage, as evidenced by scan, pyelogram, and urinary studies. While kidneys on one side or the other were clearly stronger than the mate in four of the six patients, in no instance was a kidney deemed unsalvageable, except in the one death. In Table 1, the main clinical data for each patient is listed.
“PRUNE-BELLY”
SURGICAL
RECONSTRUCTION
1039
Table
1. Clinical
Data
Original
RWlOl Function Date Birth
Right
Left
6/15/67
1+
2+
Patient
J. S.
Tested
of Right
Abdomen
Left
Abdomen
Original
Operation
Loop
Subseauent
Bilateral
ureterostomies
Suraerv
reimplantation,
6/26/60 Bilateral
closure
ureterosto-
1/14/69
mies,
Abdominopexy,
orchiopexy,
1973 R. T.
l/17/71
2+
3+
Abdomen
Groin
Bilateral
pyelostomy
Bilateral
reimplantation
tapering, Closure
and
7/73
left pyelostomy,
1 o/73 Repeat
right
taper, Closure
reimplant
and
2/74 right
pyelostomy,
4/74 T. 8.
B/l
l/73
2+
2+
Abdomen
Groin
Left pyeloplosty Right
BB. S
7/l/74
0
0
Abdomen
J. T.
11/30/74
1+
3+
Abdomen
Abdomen
nephrostomy
Biloterol
Groin
Right
reimplantation,
ob-
dominopexy,
left
orchiectomy,
4/76
pyelostomy
Death
ot 4 wk
-
Bilateral
tapering
and re-
implantation orchiopexy
with and ab-
dominopexy,
4/24/75
Lysis of adhesions, Lodd
procedure
rotation, Right
right
lostomy, 12/0/74
1+
3+
Abdomen
Groin
Bilateral
pyelostomy
Bilateral plant,
5/19/75
abdominopexy
closure
K. H.
5/7/75
pyelostomy,
Second
5/4/75
for mot-
6/76
tailoring,
reim-
orchiopexy,
and
abdominopexy, Left ureterostomy
1 Z/75 and right
reimplantation, Closure M. H.
7/27/75
1+
l+
Abdomen
Abdomen
Bilateral
pyelostomy
Bilateral plant,
6/76
pyelostomies, tailoring, orchiopexy,
obdominopexy, Closure
and
pye-
pyelostomies,
lo/76 reimand 6/76 l/77
.RESlJLTS
There was one death in this group of seven patients. That patient was seen promptly and underwent immediately bilateral pyelostomy without ill effect. This drainage functioned well, and there was no infection. However, the kidneys were unable to concentrate, and all parameters showed essentially no renal function; this situation did not improve after the institution of high drainage, and the patient died in renal failure at 30 days of age. The 6 children have been followed for 10, 6,4, 3, 3, 2&, and 2 yr, respectively.
JUDSON
1040
G.
RANDOLPH
Tnble 2. Results
Testes
______
Patient (Age)
Abdominal Wall
J. S. (10)
Excellent
R. T. (6)
Needs
T. 6. (4)
Good
66.T. s.(3) J.
Dead Good
K. H. (24)
Excellent
M.
Good
Ii. (2) t
, Testicle
up;
repair
Kidney Left
Creotinine
BUN
(mg/ 100 ml)
(mg/ 100 ml)
Right
Left
Right
1
1
2+
3+
0.8
1
t
i
0
2+ 2+
2t 3+
0.6 0.8
1
2+
3+
1
2+
3+
0.7
772+4+
i 4
, testicle
down;
0, testicle
Bladder
Growth
9
Excellent
Normal
a
Excellent
Normal
Hypertension
14
Incontinent
Normal
Satisfactory
z
1
Excellent
Normal
Excellent
0.8
31
Incontinent
Normal
Excellent
34
Excellent
Normal
Good
Overall Excellent
sacrificed.
Fig. 6. (A) IVP in a patient at 9 mo of age. (g) Same patient after repair, now 7 yr old. (C) Some patient at 8; yr. Note measured kidney growth.
1041
“PRUNE-BELLY” SURGICAL RECONSTRUCTION
Fig. 7. Patient at 9 yr of crge. lateral muscle can be seen. He can do sit ups and does not wear a corset.
In five of these children, there was measured improvement in renal function following early pyelostomy drainage. The sixth child had reconstruction without pyelostomy drainage, but we now consider this unwise. Reconstruction has taken place at various ages but, ideally, it should be performed as a total operation at a year of age, as in the last three patients. There have been a total of 19 operations in the 6 living patients or an average of 3 per child. One of the children had two abdominal procedures to relieve intestinal obstruction following the major intraabdominal reconstruction. In another child, it was impossible to bring down the left testicle, and it was sacrificed. As shown in Table 2, the patients are generally doing well. While no kidney shows perfect function, at present, all are capable of providing renal function adequate for health, development, and normal growth of the children (Fig. 6). Infection has not been a major problem but has necessitated revision of an implanted ureter in two patients. The testes are normal in position and size. Bladder function is good, but two patients are being watched because of incontinence. One patient, age 6 yr, has hypertension and requires Apresoline for control. All others are normotensive. The abdominal muscularture has been followed clinically and found to strengthen in a very pleasing way. This is particularly true of the lateral and upper abdominal muscles, which appear less deficient than the lower central area. The IO-yr-old (Fig. 7) can now do sit-ups repetitively and is working hard on an exercise program. We have specifically avoided the use of girdles or abdominal slings, preferring to encourage the patients to develop the muscle available. Four of the patients have been studied by electromyography. This technique allows us to plot existing muscle function and also permits some evaluation of potential muscle that can be strengthened. ACKNOWLEDGMENT Dr. Fred Frensilli primary
surgeon.
has permitted
Dr.
boys’ muscular function
Gloria
the inclusion
of his patient
Eng has enthusiastically
by electromyography.
(case 2) for whom
accepted
the challenge
he has been the
of mapping
these
JUDSON
1042
G. RANDOLPH
REFERENCES 1. Frohlich F: Der Mangel der Muskeln lnsbesondere der Seitebauchmuskeln. Dissertation. Wurzburg, C.A. Zurn, 1839 2. Parker RW: Absence of abdominal muscles in an infant. Lancet 1:1252, 1895 3. Osler W: Congenital absence of the abdominal muscles, with distended and hypertrophied urinary bladder. Bull Johns Hopkins Hosp 12:331, 1901 4. Eagle JF, Barret GS: Congenital deficiency of abdominal musculature with associated genitourinary abnormalities: A Syndrome. Pediatrics 6:721, 1950 5. Silverman FN, Huang N: Congenital absence of the abdominal muscles, associated with malformation of the genitourinary and alimentary tracts: Report of cases and review of literature. Am J Dis Child 88:91, 1950 6. Lattimer JK: Congenital deficiency of the
the abdominal musculature and associated genitourinary anomalies: A report of 22 cases. J Urol79:343, 1958 7. Waldbaum RS, Marshall VF: The prune belly syndrome: A diagnostic therapeutic plan. J Ural 103:668, 1970 8. Williams DI. Burkholder GV: The prune belly syndrome. J Urol98:244, 1967 9. Welch KJ, Kearney GP: Abdominal musculature deficiency syndrome: Prune belly. J Urol 1 I1:693, 1974 IO. Stumme EG: Ueber die symmetrischen kongenitalen bauchmuskeldefekte und uber die kombination derselben mit anderen bildungsanomalien des rumpfes (hochstand, hypertrophie und dilatation der blase, ureterendilation, kryptorchismus, furchennabel, thoraxdeformitat, etc.) Mitt Grenzgeb Med U Chir I l:S48, 1903
Discussion S.Kim
(Boston): In the past 9 yr, we have seen I6 children suffering from this syndrome; were newborns, and I I were older (up to 16 yr of age). Our preferred treatment has been primary reconstruction without pyelostomy. We have done this in five cases. The other 11 had had previous surgery elsewhere. Nine of the 16 had typical posterior urethral valves. Our primary treatment is a transurethral resection of valves, partial cystectomy, and lower megaureter repair. Nephrectomy is done if function is absent. We avoid nephrostomies. Several children have required pyeloplasties at a later date. We have had 2 newborn deaths, an immediate postoperative death associated with anesthesia complications, and another who died from respiratory problems 31 mo after discharge. When doing orchidopexy in the older child, we have chosen to divide the spermatic vessels, preserve the vessels associated with the vas, and have brought the testes down successfully. The abdominal wall reconstruction has been done in several cases, but it is of interest that in the older children, their abdominal support has improved with time alone. Surgery is difficult, but these cases are not hopeless. With aggressive surgical management, these children can enjoy an excellent quality of life. R. Izant (Cleveland)c 1, too, would like to echo Dr. Kim’s note of encouragement for this bad problem. Some patients with prune-belly do not have as much in the way of obstructive uropathy as Eagle originally said they all did. There are also varying degrees of abdominal wall musculature and fascia, closing the abdominal wall with skin and dividing all the spinal nerves from about T-6 to L-3. We put them back with the control litter. At the end of 6 and 9 mo, we performed IVPs on them to see if the ureters, bladder, and kidneys would enlarge with this greatly enlarged abdominal space, now unprotected by fascia or muscle. The fact was that they had no urinary tract problems, including clearances. At the conclusion of the study, these dogs had perfectly normal kidneys and ureters, and the bladder did not enlarge. This does not mean that Eagle’s theory is right or wrong. It is not an intrauterine experiment, which probably should be done, and possibly the dog is not the ideal animal. J. Randolph (Closing); 1 know what cardiac surgeons “from the bush,” (as Bill Morgan said yesterday) feel like when they get up to present a few cases and then the group from Houston gets up to tell about their 800 cases. But we do appreciate Dr. Kim bringing us the experience
“PRUNE-BELLY”
SURGICAL
from Drs. Hendren.
RECONSTRUCTION
Kim, and Donahoe’s
1043
service.
I think Bob Izant is on to an important
area
of study. There should be some embryologic studies that can be done in the fetus that would bear on this problem. In the McKay-Vaughan Textbook of Pediatrics (1975). there is only one paragraph on prune-belly in which it is stated that essentially nothing can be done for these unfortunate children. So, we all must go home and be missionaries.
