Inguinal
Pathology and Its Association Classical Bladder Exstrophy
With
By D.A. Husmann, G.A. McLorie, B.M. Churchill, and S.H. Ein Toronto,
Ontario and Dallas,
0 One hundred thirty-four cases of classical bladder exstrophy. managed at our institution, were reviewed. Fifty-six percent of the boys and 15% of the girls developed inguinal hernias over an average follow-up time-span of 10 years. Thirty-one percent of the patients with hernias underwent repair at the time of initial bladder closure. Forty-six percent of the patients who developed a hernia were diagnosed during the first year following their initial procedure. More than 50% of the individuals in the latter category presented with an incarcerated hernia, and required emergent management. Boys managed by staged reconstruction had a statistically significant risk of developing an inguinal hernia (P < .Wl) compared with boys undergoing primary cystectomy and diversion. We believe the increased incidence of herniation with this congenital anomaly is secondary to a lack of obliquity of the inguinal canal, due to pubic diastasis along with an increased elevation of intraabdominal pressure following initial closure of the abdominal wall and bladder plate. To decrease the attendent morbidity of incarcerated hernias in this population, we stress the need for careful physical examination of the inguinal region and spermatic cord prior to surgery, along with repair of the patent processus vaginalis at the time of initial repair. 0 1990 by W.B. Saunders Company. INDEX WORDS: Bladder exstrophy: inguinal hernia.
D
URING THE CONTINUED long-term followup evaluation of patients with classical bladder exstrophy, a significant morbidity associated with inguinal hernias and cryptorchidism in children having this congenital anomaly has been noted. In order to assess the prevalence of these disorders in this patient population, a large series of individuals with classical bladder exstrophy presenting for routine follow-up care at our institution were reviewed. MATERIALS AND METHODS One hundred thirty-four patients having classical bladder exstro-
phy, followed at our institution, were evaluated. One hundred twelve patients were managed by staged bladder reconstruction, and 22 patients underwent primary cystectomy and urinary diversion with no attempt at staged reconstruction. It should be noted that during the review period used for this paper, only a limited number of girls
From The Hospital for Sick Children, Toronto. Ontario and the University of Texas Southwestern, Dallas, TX. Date accepted: January 9.1989. Address reprint requests to D.A. Husmann. MD. Division of Urology. University of Texas Southwestern, 5323 Harry Hines Blvd. Dallas, TX 75235. o 1990 by W.B. Saunders Company. 00.?2-3468/90/2503UO15$03.00/0 332
Texas
(2). treated by primary cystectomy and diversion, presented for follow-up. Due to this limitation, both girls in this category were excluded from the study. All patients were evaluated by reviewing their medical records along with current history and physical examinations. For comparison purposes, an age- and sex-matched control population was chosen at random from individuals attending our pediatric clinics for routine physical examinations. We defined the diagnosis of bilateral inguinal hernias based upon the preoperative clinical evaluation of the patient. Routine contralatera1 exploration of the inguinal region was not carried out in our patient population. All statistical evaluations were performed using the Fisher’s exact two-tailed test. Statistical significance was attended to P values less than .05. RESULTS
Inguinal hernias were noted in 53 patients (40%) with classical bladder exstrophy, compared with three patients (2%) of the control population, during an average follow-up of 10.5 years (P < .OOl). In the girls, eight of 53 exstrophy patients (15%) developed inguinal hernias, compared with none of the controls (P < .006). Forty-five of 8 1 boys (56%) compared with three (4%) of the boy control patients, developed hernias (P < .OOl). Table 1 depicts the comparison between the male patients managed by staged reconstruction versus primary cystectomy and diversion. Although both patient groups had a statistically significant risk of developing inguinal hernias when compared with the control population (P < .OOl and P -c.04, respectively), a greater risk of developing an inguinal hernia occurred in patients managed with staged reconstruction compared with primary cystectomy and diversion (P < .OOl). It is important to note that 11 (53%) of the patients presenting with inguinal hernias less than 1 year after initial closure of their bladder plate, presented with an incarceration and required emergent management. The clinical findings noted in exstrophy patients at the time of their diagnosis are found in Table 2. Thirty of the 53 patients (57%) were noted to have synchronous bilateral inguinal hernias, and seven ( 13%) had metachronous bilateral inguinal hernias. Solitary right inguinal hernias were noted in 12 (23%), and left inguinal hernias were noted in four (7%) patients. Twenty-three children underwent repair of a clinically diagnosed unilateral hernia. Of the 13 children undergoing unilateral herniorrhaphy when less than 1 year old, seven (54%) developed a contralateral hernia during an average 9.5 year follow-up following Journal of Pediatric Surgery, Vol 25, No 3 (March), 1990: pp 332-334
333
BLADDER EXSTROPHY: INGUINAL PATHOLOGY
Table 1. Comparing the Incidence of lnguinal Hernias in Male Patients Managed Primary Cyateotomy
by Staged Reconstruction
Veraus
and Diversion Diagnosisof lnguinalHernia
TYpeof surgery
No. of Patients
Average Follow-Up (years)
Staged reconstruction
59
10.5
5d
22
13
3mo
MedianAge at SurgBly
Priorto SWgenl (%I
LessThan 1 Year Postsurgsq (%I
Greaterthan 1 Year POstsWgeq (%f
PatientsWith lnguinalHernias (%) 68%
11 (19%)
20 (34%)
9 (15%)
3 (13%)
1 (5%) ,017
1 (5%)
23%
.27
< ,001
Primary cystectomy and diversion
.5
P value
surgery. Four (57%) of the children developing a contralateral hernia presented with incarceration requiring emergent management. Ten children underwent unilateral repair between 18 months and 10 years of age with no contralateral hernias developing during an average follow-up of 6 years following herniorrhaphy. The significance of a contralateral hernia developing in individuals undergoing unilateral repair when less than 1 year of age compared with those individuals undergoing unilateral repair at greater than 1 year of age is P c.003. Ten of 81 boys with exstrophy had cryptorchidism (12%). In eight individuals this was bilateral, and in two it was unilateral. In all cases, a patent processus vaginalis was diagnosed at the time of orchiopexy and required repair. One (1%) of the 8 1 boys in the control population required an orchiopexy for an undescended testicle (P -c .012). Complications of surgery occurred in eight (15%) patients undergoing repair of their inguinal pathology. Recurrent hernias, all of which were indirect in nature, occurred in six (9%) patients. Testicular atrophy following orchiopexy occurred in one, or IO%, of the patients undergoing orchiopexy. Cellulitis of the wound occurred in one patient (2%). In no cases, did the pathological material examined show vasal remnants, nor did iatrogenic cryptorchidism result. DISCUSSION
The pathologic relationship between classical bladder exstrophy and inguinal pathology seems to be based upon an inherent regional musculoskeletal weakTable 2. Clinical Findings in Exstrophy
Patients at the Time of
Diagnosis of lnguinal Pathology ClinicalFindings Asymptomatic inguinal bulge
Numberof Patients(%) 32 (60%)
Incarcerated inguinal hernia
9 (17%)
Patent processus with cryptorchidism
6 (15%)
Incarcerated inguinal hernia with bowel necrosis Incarcerated hernia with cryptwchidism
2 14%) 1 (2%)
Patent processus with testicular torsion and necrosis of a cryptorchid testicle
1 (2%)
ness due to failure of mesodermal ingrowth in the lower abdominal wall.‘” This results in pubic diastasis with loss of the obliquity of the inguinal canallm3 along with an alteration in the normal intraabdominal pressure.4V5 Normal testicular descent is brought about by an interaction with three major components: hormonal influences,6 distal gubernacular attachments,4 and intraabdominal pressure.‘*’ Kaplan et al’ in 1986 documented that an increased frequency of cryptorchidism occurred in individuals with abdominal wall defects of omphalocele, gastroschisis, and umbilical hernias. These clinical findings helped to confirm the experimental evidence that intraabdominal pressure plays a major role in cryptorchidism. In individuals with classical bladder exstrophy, we believe that the fascial wall defect produced by the exstrophic bladder leads to a diminished intraabdominal pressure at the time of testicular descent, resulting in a statistically significant increased incidence of cryptorchidism in these individuals. Because the primary defect causing classical bladder exstrophy results in a lack of obliquity of the inguinal canal,lM3 it is not surprising that an increased frequency of inguinal hernias is noted in individuals at birth. The increased incidence of inguinal hernias noted in patients with staged reconstruction versus individuals managed by primary cystectomy and diversion (P -c .OOl), leads one to conjecture that interventional management by staged reconstruction is leading to an increased incidence in inguinal herniation. The increased incidence of hernias occurring in these individuals may be due to an elevation in intraabdominal pressure resulting from the placement of the exstrophic bladder within the pelvis, a slowly increasing bladder capacity, and closure of the abdominal wall fascial defect. The increased abdominal pressure resulting from the previously mentioned factors results in an increased frequency of indirect herniation through the patent processus vaginalis of these young children. A similar finding may be seen in children with ventriculoperitoneal shunts or those undergoing peritoneal dialysis. The increased frequency of herniation found in these latter children is believed to be secondary to
334
HUSMANN
either an increase in intraabdominal fluid or an elevation in intraabdominal pressure.7 The surgical management of the contralateral groin in children with a unilateral inguinal hernia has been an issue of controversy since Rothenberg and Barrett’s’ original article in 1955. In children with unilateral inguinal hernia, Rowe et a19S’onoted that patency of the contralateral processus vaginalis occurred in 63% of children during the first 2 months of life, with a decrease in the patency rate to 41% at 16 years of age. Approximately half of these children (20%) will develop an apparent contralateral hernia. In infants subjected to an elevation of intraabdominal pressure or fluid, a contralateral hernia may rapidly develop from a patent processus vaginalis following a unilateral repair. ” In this study, the high incidence of contralatera1 hernia formation (53%) in individuals undergoing repair when less than 1 year old, would almost match the estimated patency rate of the contralateral proces-
ET AL
vaginalis. Because of the added morbidity of a second operation, plus the fact that over 50% of the individuals developing a contralateral hernia presented with incarceration requiring emergent management, we strongly urge that all infants less than 1 year of age, with a history of bladder exstrophy, undergo bilateral inguinal exploration in the face of a unilateral hernia. In order to decrease the attendent morbidity observed in this patient population due to incarcerated inguinal hernias, careful physical examination of the inguinal region and spermatic cord is mandatory prior to the initial bladder closure. In addition, careful intraoperative inspection of the spermatic cord at the time of peritoneal mobilization will allow one to diagnose a patent processus vaginalis if it exists. If a hernia or patent processus vaginalis is diagnosed in these individuals, primary repair of this defect at the time of the initial surgery will aid in decreasing the morbidity of inguinal hernias in this population. sus
REFERENCES 1. Muecke EC: The role of the cloacal membrane in exstrophy: The successful experimental study. J Urol92:659-667,1964 2. Thomalia V, Rudolph RA, Rink RC, et al: Induction of cloaca1 exstrophy in the chick embryo using the CO, laser. J Urol 134:991995,198s 3. Jeffs RD, Guire SL, Gesch I: The factors in successful exstrophy closure. J Urol 127:974-976,1982 4. Frey HL, Rajfer J: Role of the gubemaculum and intraabdominal pressure in the process of testicular descent. J Urol 132574579,1984 5. Kaplan LM, Koyle M, Kaplan G, et al: Association between abdominal wall defects and cryptorchidism. J Urol 136645647, 1986 6. Frey HL, Peng S. Rajfer J: Synergy of abdominal pressure and
androgens in testicular descent. Biol Reprod 29:1233-1239,1983 7. Rowe MI, Lloyd DA: Inguinal hernia, in Welch KJ, Randolph J, Ravitch M, et al &is): Pediatric Surgery. Chicago, IL, Year Book, 1986, p 779-793 8. Rothenberg RE, Barrett T: Bilateral hemiotomy in infants and children. Surgery 37:947-950,1955 9. Rowe MI. Chatworthy HW: The other side of the pediatric inguinal hernia. Surg Clin North Am 51:1371-1376, 1971 10. Rowe MI, Copelson LW, Chatworthy HW: The patent processus vaginalis and inguinal hernia. J Pediatr Surg 4:102-107, 1969 11. Alexander SR, Tank ES Surgical aspects of continuous ambulatory peritoneal dialysis in infants, children and adolescents. J Urol 127:501-504,198l
Pathology and Its Association Classical Bladder Exstrophy
With
By D.A. Husmann, G.A. McLorie, B.M. Churchill, and S.H. Ein Toronto,
Ontario and Dallas,
0 One hundred thirty-four cases of classical bladder exstrophy. managed at our institution, were reviewed. Fifty-six percent of the boys and 15% of the girls developed inguinal hernias over an average follow-up time-span of 10 years. Thirty-one percent of the patients with hernias underwent repair at the time of initial bladder closure. Forty-six percent of the patients who developed a hernia were diagnosed during the first year following their initial procedure. More than 50% of the individuals in the latter category presented with an incarcerated hernia, and required emergent management. Boys managed by staged reconstruction had a statistically significant risk of developing an inguinal hernia (P < .Wl) compared with boys undergoing primary cystectomy and diversion. We believe the increased incidence of herniation with this congenital anomaly is secondary to a lack of obliquity of the inguinal canal, due to pubic diastasis along with an increased elevation of intraabdominal pressure following initial closure of the abdominal wall and bladder plate. To decrease the attendent morbidity of incarcerated hernias in this population, we stress the need for careful physical examination of the inguinal region and spermatic cord prior to surgery, along with repair of the patent processus vaginalis at the time of initial repair. 0 1990 by W.B. Saunders Company. INDEX WORDS: Bladder exstrophy: inguinal hernia.
D
URING THE CONTINUED long-term followup evaluation of patients with classical bladder exstrophy, a significant morbidity associated with inguinal hernias and cryptorchidism in children having this congenital anomaly has been noted. In order to assess the prevalence of these disorders in this patient population, a large series of individuals with classical bladder exstrophy presenting for routine follow-up care at our institution were reviewed. MATERIALS AND METHODS One hundred thirty-four patients having classical bladder exstro-
phy, followed at our institution, were evaluated. One hundred twelve patients were managed by staged bladder reconstruction, and 22 patients underwent primary cystectomy and urinary diversion with no attempt at staged reconstruction. It should be noted that during the review period used for this paper, only a limited number of girls
From The Hospital for Sick Children, Toronto. Ontario and the University of Texas Southwestern, Dallas, TX. Date accepted: January 9.1989. Address reprint requests to D.A. Husmann. MD. Division of Urology. University of Texas Southwestern, 5323 Harry Hines Blvd. Dallas, TX 75235. o 1990 by W.B. Saunders Company. 00.?2-3468/90/2503UO15$03.00/0 332
Texas
(2). treated by primary cystectomy and diversion, presented for follow-up. Due to this limitation, both girls in this category were excluded from the study. All patients were evaluated by reviewing their medical records along with current history and physical examinations. For comparison purposes, an age- and sex-matched control population was chosen at random from individuals attending our pediatric clinics for routine physical examinations. We defined the diagnosis of bilateral inguinal hernias based upon the preoperative clinical evaluation of the patient. Routine contralatera1 exploration of the inguinal region was not carried out in our patient population. All statistical evaluations were performed using the Fisher’s exact two-tailed test. Statistical significance was attended to P values less than .05. RESULTS
Inguinal hernias were noted in 53 patients (40%) with classical bladder exstrophy, compared with three patients (2%) of the control population, during an average follow-up of 10.5 years (P < .OOl). In the girls, eight of 53 exstrophy patients (15%) developed inguinal hernias, compared with none of the controls (P < .006). Forty-five of 8 1 boys (56%) compared with three (4%) of the boy control patients, developed hernias (P < .OOl). Table 1 depicts the comparison between the male patients managed by staged reconstruction versus primary cystectomy and diversion. Although both patient groups had a statistically significant risk of developing inguinal hernias when compared with the control population (P < .OOl and P -c.04, respectively), a greater risk of developing an inguinal hernia occurred in patients managed with staged reconstruction compared with primary cystectomy and diversion (P < .OOl). It is important to note that 11 (53%) of the patients presenting with inguinal hernias less than 1 year after initial closure of their bladder plate, presented with an incarceration and required emergent management. The clinical findings noted in exstrophy patients at the time of their diagnosis are found in Table 2. Thirty of the 53 patients (57%) were noted to have synchronous bilateral inguinal hernias, and seven ( 13%) had metachronous bilateral inguinal hernias. Solitary right inguinal hernias were noted in 12 (23%), and left inguinal hernias were noted in four (7%) patients. Twenty-three children underwent repair of a clinically diagnosed unilateral hernia. Of the 13 children undergoing unilateral herniorrhaphy when less than 1 year old, seven (54%) developed a contralateral hernia during an average 9.5 year follow-up following Journal of Pediatric Surgery, Vol 25, No 3 (March), 1990: pp 332-334
333
BLADDER EXSTROPHY: INGUINAL PATHOLOGY
Table 1. Comparing the Incidence of lnguinal Hernias in Male Patients Managed Primary Cyateotomy
by Staged Reconstruction
Veraus
and Diversion Diagnosisof lnguinalHernia
TYpeof surgery
No. of Patients
Average Follow-Up (years)
Staged reconstruction
59
10.5
5d
22
13
3mo
MedianAge at SurgBly
Priorto SWgenl (%I
LessThan 1 Year Postsurgsq (%I
Greaterthan 1 Year POstsWgeq (%f
PatientsWith lnguinalHernias (%) 68%
11 (19%)
20 (34%)
9 (15%)
3 (13%)
1 (5%) ,017
1 (5%)
23%
.27
< ,001
Primary cystectomy and diversion
.5
P value
surgery. Four (57%) of the children developing a contralateral hernia presented with incarceration requiring emergent management. Ten children underwent unilateral repair between 18 months and 10 years of age with no contralateral hernias developing during an average follow-up of 6 years following herniorrhaphy. The significance of a contralateral hernia developing in individuals undergoing unilateral repair when less than 1 year of age compared with those individuals undergoing unilateral repair at greater than 1 year of age is P c.003. Ten of 81 boys with exstrophy had cryptorchidism (12%). In eight individuals this was bilateral, and in two it was unilateral. In all cases, a patent processus vaginalis was diagnosed at the time of orchiopexy and required repair. One (1%) of the 8 1 boys in the control population required an orchiopexy for an undescended testicle (P -c .012). Complications of surgery occurred in eight (15%) patients undergoing repair of their inguinal pathology. Recurrent hernias, all of which were indirect in nature, occurred in six (9%) patients. Testicular atrophy following orchiopexy occurred in one, or IO%, of the patients undergoing orchiopexy. Cellulitis of the wound occurred in one patient (2%). In no cases, did the pathological material examined show vasal remnants, nor did iatrogenic cryptorchidism result. DISCUSSION
The pathologic relationship between classical bladder exstrophy and inguinal pathology seems to be based upon an inherent regional musculoskeletal weakTable 2. Clinical Findings in Exstrophy
Patients at the Time of
Diagnosis of lnguinal Pathology ClinicalFindings Asymptomatic inguinal bulge
Numberof Patients(%) 32 (60%)
Incarcerated inguinal hernia
9 (17%)
Patent processus with cryptorchidism
6 (15%)
Incarcerated inguinal hernia with bowel necrosis Incarcerated hernia with cryptwchidism
2 14%) 1 (2%)
Patent processus with testicular torsion and necrosis of a cryptorchid testicle
1 (2%)
ness due to failure of mesodermal ingrowth in the lower abdominal wall.‘” This results in pubic diastasis with loss of the obliquity of the inguinal canallm3 along with an alteration in the normal intraabdominal pressure.4V5 Normal testicular descent is brought about by an interaction with three major components: hormonal influences,6 distal gubernacular attachments,4 and intraabdominal pressure.‘*’ Kaplan et al’ in 1986 documented that an increased frequency of cryptorchidism occurred in individuals with abdominal wall defects of omphalocele, gastroschisis, and umbilical hernias. These clinical findings helped to confirm the experimental evidence that intraabdominal pressure plays a major role in cryptorchidism. In individuals with classical bladder exstrophy, we believe that the fascial wall defect produced by the exstrophic bladder leads to a diminished intraabdominal pressure at the time of testicular descent, resulting in a statistically significant increased incidence of cryptorchidism in these individuals. Because the primary defect causing classical bladder exstrophy results in a lack of obliquity of the inguinal canal,lM3 it is not surprising that an increased frequency of inguinal hernias is noted in individuals at birth. The increased incidence of inguinal hernias noted in patients with staged reconstruction versus individuals managed by primary cystectomy and diversion (P -c .OOl), leads one to conjecture that interventional management by staged reconstruction is leading to an increased incidence in inguinal herniation. The increased incidence of hernias occurring in these individuals may be due to an elevation in intraabdominal pressure resulting from the placement of the exstrophic bladder within the pelvis, a slowly increasing bladder capacity, and closure of the abdominal wall fascial defect. The increased abdominal pressure resulting from the previously mentioned factors results in an increased frequency of indirect herniation through the patent processus vaginalis of these young children. A similar finding may be seen in children with ventriculoperitoneal shunts or those undergoing peritoneal dialysis. The increased frequency of herniation found in these latter children is believed to be secondary to
334
HUSMANN
either an increase in intraabdominal fluid or an elevation in intraabdominal pressure.7 The surgical management of the contralateral groin in children with a unilateral inguinal hernia has been an issue of controversy since Rothenberg and Barrett’s’ original article in 1955. In children with unilateral inguinal hernia, Rowe et a19S’onoted that patency of the contralateral processus vaginalis occurred in 63% of children during the first 2 months of life, with a decrease in the patency rate to 41% at 16 years of age. Approximately half of these children (20%) will develop an apparent contralateral hernia. In infants subjected to an elevation of intraabdominal pressure or fluid, a contralateral hernia may rapidly develop from a patent processus vaginalis following a unilateral repair. ” In this study, the high incidence of contralatera1 hernia formation (53%) in individuals undergoing repair when less than 1 year old, would almost match the estimated patency rate of the contralateral proces-
ET AL
vaginalis. Because of the added morbidity of a second operation, plus the fact that over 50% of the individuals developing a contralateral hernia presented with incarceration requiring emergent management, we strongly urge that all infants less than 1 year of age, with a history of bladder exstrophy, undergo bilateral inguinal exploration in the face of a unilateral hernia. In order to decrease the attendent morbidity observed in this patient population due to incarcerated inguinal hernias, careful physical examination of the inguinal region and spermatic cord is mandatory prior to the initial bladder closure. In addition, careful intraoperative inspection of the spermatic cord at the time of peritoneal mobilization will allow one to diagnose a patent processus vaginalis if it exists. If a hernia or patent processus vaginalis is diagnosed in these individuals, primary repair of this defect at the time of the initial surgery will aid in decreasing the morbidity of inguinal hernias in this population. sus
REFERENCES 1. Muecke EC: The role of the cloacal membrane in exstrophy: The successful experimental study. J Urol92:659-667,1964 2. Thomalia V, Rudolph RA, Rink RC, et al: Induction of cloaca1 exstrophy in the chick embryo using the CO, laser. J Urol 134:991995,198s 3. Jeffs RD, Guire SL, Gesch I: The factors in successful exstrophy closure. J Urol 127:974-976,1982 4. Frey HL, Rajfer J: Role of the gubemaculum and intraabdominal pressure in the process of testicular descent. J Urol 132574579,1984 5. Kaplan LM, Koyle M, Kaplan G, et al: Association between abdominal wall defects and cryptorchidism. J Urol 136645647, 1986 6. Frey HL, Peng S. Rajfer J: Synergy of abdominal pressure and
androgens in testicular descent. Biol Reprod 29:1233-1239,1983 7. Rowe MI, Lloyd DA: Inguinal hernia, in Welch KJ, Randolph J, Ravitch M, et al &is): Pediatric Surgery. Chicago, IL, Year Book, 1986, p 779-793 8. Rothenberg RE, Barrett T: Bilateral hemiotomy in infants and children. Surgery 37:947-950,1955 9. Rowe MI. Chatworthy HW: The other side of the pediatric inguinal hernia. Surg Clin North Am 51:1371-1376, 1971 10. Rowe MI, Copelson LW, Chatworthy HW: The patent processus vaginalis and inguinal hernia. J Pediatr Surg 4:102-107, 1969 11. Alexander SR, Tank ES Surgical aspects of continuous ambulatory peritoneal dialysis in infants, children and adolescents. J Urol 127:501-504,198l
