Pediatr Surg Int (2015) 31:305–309 DOI 10.1007/s00383-015-3671-6
ORIGINAL ARTICLE
Epidydimo-orchitis and anorectal malformations: when and in whom? A. Zaccara • S. Ragozzino • B. D. Iacobelli • F. Rivosecchi • M. L. Capitanucci • G. Mosiello M. Silveri • M. De Gennaro • P. Bagolan
•
Accepted: 27 January 2015 / Published online: 5 February 2015 Ó Springer-Verlag Berlin Heidelberg 2015
Abstract Purpose Epididymo-orchitis (EO) is infrequently reported in anorectal malformation (ARM) cases. Therefore, it is difficult to assess its risk factors. Methods A total of 110 male patients who were operated on for ARM at the same Institution over a period of 13 years were contacted. Association was assessed between EO and the following: spinal dysraphism (SD), symptomatic VUR (VUR), and bowel management (BM) requiring enemas. The data were analyzed with the Chi-square test. Results A total of 89 patients were contacted. Ten cases of EO were found, and all occurred in patients with rectourethral (RU) fistula after reconstruction. The patients’ age at first episode ranged between 4 and 11 years. RU fistula patients experiencing EO (Group A, 10 patients) were compared with those without EO (Group B, 33 patients). VUR occurred in 9/10 cases in Group A and in 13/33 cases A. Zaccara (&) M. L. Capitanucci G. Mosiello M. De Gennaro Department of Urology and Nephrology, Bambino Gesu’ Children’s Hospital, Rome, Italy e-mail: [email protected] S. Ragozzino Department of Surgery, Meyer Children’s Hospital, Florence, Italy B. D. Iacobelli P. Bagolan Newborn Surgery Unit, Bambino Gesu’ Children’s Hospital, Rome, Italy
in group B (Chi-square 7.8658, p = 0.005038). SD was present in 4/10 cases in group A and in 13/33 cases in Group B (Chi-square 0.0434, p = 0.83491). A total of 8/10 cases in Group A and 12/33 cases in Group B were on BM (Chi-square 5.87, p = 0.0015). Conclusions EO occurs in approximately in 20 % of male cases with ARM, and recto-urinary communication and should be considered the primary diagnosis in the presence of testicular pain. This could avoid unnecessary surgical exploration, and the family should be counseled about this subject Keywords Anorectal malformations Epididymoorchitis Bowel management
Introduction Among the different sequelae of surgery for treating imperforate anus, epididymo-orchitis (EO) is infrequently reported in the literature. To date, most of the cases are either anecdotal or collected from different series, and it has an estimated incidence of 1.2–6.1 % [1, 2]. Because of its rarity, clinical findings of EO are often unexpected and worrisome for patients and families. However, even though imperforate anus patients usually suffer from many associated conditions, which often affect them for their entire lifetime (e.g., spinal dysraphism, constipation, and recurrent UTIs), attempts to identify risk factors have only been made on the basis of the sparse data available in the literature.
F. Rivosecchi Department of Pediatrics, Bambino Gesu’ Children’s Hospital, Rome, Italy
Materials and methods
M. Silveri Department of Surgery, Bambino Gesu’ Children’s Hospital, Rome, Italy
In the period ranging from 2000 to 2013, 110 male patients with imperforate anus were operated upon at our
123
306
Institution. In order to investigate the occurrence of signs and/or symptoms of EO, an appropriately designed questionnaire was administered over the telephone to patients and/or families. EO was defined as at least two recurrent episodes of acute scrotum (scrotal pain and swelling), with or without concomitant dysuria. All the patients were treated by the same surgical team with the same surgical techniques, specifically, posterior sagittal anorectoplasty (PSARP) for intermediate and high conditions and limited PSARP for low forms. The former were treated with a three-step approach (colostomy-PSARP-colostomy closure), whereas the latter were operated upon with a single-stage procedure. In the three-step approach, PSARP was carried out at 3 months of age, and colostomy was closed shortly thereafter. In all cases requiring colostomy prior to reconstruction, a totally diverting (divided) colostomy was carried out. The level of communication between the gastrointestinal and urinary tract was assessed by a distal colostogram prior to surgical reconstruction. The association was assessed between EO and the following: Symptomatic Vesico Ureteral Reflux (VUR), Spinal Dysraphism (SD), and the necessity of bowel management (BM). Symptomatic VUR was defined as VUR leading to at least to an episode of acute Pyelonephritis (APN). VCUG was not performed in asymptomatic cases. The films of VUR patients were further reviewed for associated anomalies (ureteral ectopia, reflux of urine into the vas, and/or seminal vesicles). SD was defined as spinal cord malformations and was assessed by screening MRI (with or without neurological signs and/or symptoms). According to our follow-up policy, all patients with ARM were screened with MRI. BM was defined as the necessity for regular colonic washouts, along with dietary adjustments, for complete cleanliness. Enemas were administered daily or every other day and mostly consisted of 40 ml/kg warm saline with the addition of petroleum jelly products. The data were analyzed with the Chi-square test. Informed consent was obtained from all the individual participants included in the study.
Results Eighty-nine patients could be traced and were included in the study. Of these, 42 had no communication between the gastrointestinal and urinary tract, while in 47, a communication was present (recto-bladderneck fistula in 4 cases and Recto-Urethral (RU) fistula in 43 cases). Ten cases of
123
Pediatr Surg Int (2015) 31:305–309
EO were found, all of which occurred in patients with RU fistula. The patients with RU fistula were thus divided into two subgroups, those experiencing EO (group A, 10 patients) and those without EO (Group B, 33 patients). All cases of EO occurred after reconstruction, and the patients’ age at first episode ranged between 4 and 11 years after the completion of the surgery (colostomy closure). There was no side predilection. Five patients experienced more than two episodes of EO (up to five in two patients). In four cases, emergency admission was required, with one patient requiring surgical exploration to rule out testicular torsion. The other three cases had EO confirmed by testicular US. In the remaining patients, EO was successfully managed at home with antibiotics and/or common analgesics, in accordance with the pediatrician’s advice. Symptomatic VUR occurred in 9/10 cases in Group A and in 13/33 cases in group B (Chi-square 7.8658, p = 0.005038), and no anomalies, other than reflux, were found when reviewing the X rays. SD was present in 4/10 cases in group A and in 13/33 cases in Group B (Chi-square 0.0434, p = 0.83491). In three cases, SD occurred in association with symptomatic reflux. BM was required in 8/10 cases in Group A and in 12/33 cases in Group B (Chi-square 5.87, p = 0.0015).
Discussion With increasing data available on the follow-up of ARM patients, EO is being reported with increasing frequency, and it has an estimated incidence of 1.2–6.1 %, which is much higher than what was previously reported in the pediatric population [3]. In particular, when considering only the subset of patients with RU fistula (the only ones of our series in which EO occurred), the percentage rises to approximately 23 %. In other words, one in four patients with this condition developed EO. To date, case reports of EO are either anecdotal or collected from different series. Raveenthiram et al. [4] published 6 personal cases and reviewed an additional 35 in the literature. They identified the following significant risk factors for EO onset: congenital malformations, in particular, ureterovasal anastomosis [5], and the so called ‘‘persistent mesonephric duct syndrome,’’ which includes ectopic ureter, dysplastic kidney, renal malformations, the absence of hemitrigone, ureterovasal reflux, and the absence of the seminal vesicle on the ipsilateral side [6]. In their own series, only two cases of EO occurred after completing all steps of the reconstruction, whereas the other three EO occurred when the colostomy was still in place. In one case, the chronology was not known.
Pediatr Surg Int (2015) 31:305–309
Oguzcurt et al. [7] reported two cases of EO, one occurring before surgical repair and the other shortly thereafter, but before colostomy closure. Kyian et al. [8] described a series of 58 male patients who were operated upon for imperforated anus and reported four cases of EO. Of interest, in three cases, EO occurred prior to surgical repair, and in the remaining case, there was no communication with the urinary tract. At follow-up, no EO recurrences were seen after PSARP. Our data are significantly different from these findings for three main reasons. First, we were not able to identify any underlying anomaly when performing instrumental examinations. Second, no cases of EO occurred before completing reconstruction. Third, all the EO cases were reported long after surgery (up to 11 years after colostomy closure). With respect to congenital anomalies, some of the cases in the literature were detected by VCUG and a few others by distal colostogram demonstrating urethro ejaculatory reflux. It must be noted that our workup policy prior to reconstruction was that of performing a distal colostogram before PSARP, and VCUG is usually reserved for symptomatic (febrile UTIs) patients. Therefore, we might have missed the vas/trigone anomalies that have been repeatedly described in the literature. There is the opinion that a VCUG be incorporated in the workup of all imperforate anus patients prior to undertaking reconstruction to identify such anomalies, as Mickelson et al. advocate [9]; however, we do not agree with this view, and our attitude is corroborated by the findings of Wiersma, who, in his review of 360 voiding cystourethrograms performed in male patients over a 6 year period, found 54 cases of urethro— ejaculatory reflux (UER), nine of which had anorectal malformations [10], while six had EO and UER without any underlying pathology. Based on these findings, we believe that preliminary VCUG should probably be reserved for cases with difficult catheterization at the time of colostomy, multiple anomalies and EO occurring prior to PSARP procedure. In other words, the chances of preventing EO by performing VCUG in all male subjects with imperforate anus are small. The follow-up of asymptomatic patients with urethroejaculatory duct anomalies to determine to what extent these subjects will be prone to future EO remains to be clarified. On the other hand, VCUG may retain its validity in cases of recurrent EO, where chances of detecting ureterovasal anomalies are far greater. Even though we did not recall our EO patients for such an examination after the telephone interview, it will certainly be our policy in future cases. Other options for EO onset have been suggested over the years. In 2002, Hong et al. [11] reported that urethral injuries occurred in 20 % of patients and posterior urethra
307
diverticula in 18 % of patients following repair of imperforate anus with the posterior sagittal approach. In a review of 20 infertile men operated upon for imperforate anus, Holt [12] documented three caudal epididymal blocks in four men with a history of recurrent epididymitis. Even though no details were given about the surgical technique, the author raised concerns about the possible obstruction of the ejaculatory ducts by the pull through procedure or by the repair of the recto-urethral fistula. Alternatively, surgery may perturb the oblique course of the ejaculatory ducts as they enter the prostatic urethra, preventing reflux of urine, and the susceptibility of the prepubertal vas to any form of trauma, including simple manipulation, has been repeatedly described in the literature [13]. Data on this topic are, however, contradictory because anomalies of the angle of entry of the ejaculatory ducts joining the urethra have also been reported as being congenital [14]. Our findings seem to support the hypothesis of the involvement of the ejaculatory ducts at the time of the repair of the recto-urethral fistula. All EO episodes occurred after reconstruction, and we did not find any cases of EO in cases of recto-bladderneck communication or in cases without communication between the rectum and urinary tract. Based on these considerations, a precise anatomical assessment of the fistula site, with differentiation between the recto-prostatic and recto-bulbar fistula, could help identify those cases more at risk for EO. Unfortunately, neither in the literature nor in our surgical case notes was this difference taken into consideration. In addition, the precise identification of the fistula site, both at distal colostogram and at surgery, has proven very difficult, given the progressively younger age at which these babies are repaired. Regarding the timing of reconstruction, we performed PSARP at approximately 3 months of age, with colostomy closure shortly thereafter. This could partly explain why we did not observe cases of preoperative EO. In the previously mentioned series described by Raveenthiram et al. [4], PSARP was effective in curing EO in 36 % of cases, but the surgery was carried out at one year of age. These findings seem to support the hypothesis of a non-completely diverting colostomy contaminating the genitourinary tract [15]. In summary, early repair appears to be effective in preventing preoperative EO. However, on the other hand, the identification of the fistula site may be more difficult, and the risk of the involvement of the ejaculatory ducts becomes more significant. As previously mentioned, four of our patients with EO experienced emergency admission because of recurrent EO, and in one case, surgical exploration was required. Therefore, a correlation was sought with other conditions,
123
308
such as symptomatic VUR and neurogenic bladder, which are frequently associated with imperforate anus. In their collective review of 41 cases, Raveenthiram et al. [4] found an incidence of associated risk factors, specifically, UTIs and spinal dysraphism, of 54 and 34 %, respectively. Unfortunately, as data were collected from different series, the details regarding surgical approach were not available, and no statistical analysis was performed. In our series, all cases were operated upon by the same surgical team with the same three-step approach at approximately the same age, thus making our patient population more homogenous. Our findings confirm that there is an association between VUR and EO, and this reached statistical significance. It must be noted; however, that we only performed VCUG in cases of febrile UTIs, and therefore, the incidence could be even greater. With regard to SD, we did not find a statistically significant difference between patients with and without EO. However, it must be considered that we included all patients with spinal cord malformations, regardless if they were symptomatic or not. Thus, the variations in the data reported in the literature may reflect differences in definitions. Three cases also presented with an association between SD and symptomatic VUR. Because this association is frequently reported in the literature, we speculate that the disturbances in bladder cycling, which are typical of neurogenic bladder, could be responsible for EO onset. We suggest that a better characterization with videourodynamic studies should replace traditional imaging in these cases. Because our follow-up period spans approximately 11 years, we were also interested in lifestyle changes which may be associated with a higher incidence of EO. Interestingly, an association was found between patients on a BM regimen and EO. As previously mentioned, we defined BM patients as those requiring enemas on a daily basis to be clean, and this was a common finding in our patient population. References in the literature regarding colonic washouts causing EO in imperforate anus patients are scant and limited to 2 % of cases in the series by Raveenthiram et al. Of interest, washouts causing EO were performed by the colostomy, or in other words, before the completion of all the steps of reconstruction. To our knowledge, no other evidence exists in the literature regarding the relationships between EO and bowel management. Effects of repeated colonic washouts have been extensively studied in subjects undergoing bowel preparation for colorectal surgery [16], and it is well known that one of the complications of an enema is inflammation of the mucosal lining of the colon. However, because this procedure is usually limited to the immediate preoperative period, little is known about the effects of its long-term use. In this
123
Pediatr Surg Int (2015) 31:305–309
respect, it must be noted that imperforate anus patients actually start mechanical bowel cleansing very early on and usually continue with this regimen indefinitely because this will enable them to keep themselves clean for up to 36–48 h. This, in turn, may lead to mechanical irritation induced by the repeated insertion of enema catheters. Relationships between colonic inflammation and changes in urinary smooth muscle function have been repeatedly described in animal models [17], as have abnormalities in bladder detrusor smooth muscle contractility. We speculate that such inflammation may extend to the surrounding structures, such as the ejaculatory ducts, vas, and seminal vesicles. Enemas that are carried out on a regular basis may also act as key factors in introducing environmental bacteria to the human gut. As such, the mechanical action of the repeated insertion of enema catheters should also take into consideration. This mechanism could partly explain why some of our patients experienced repeated episodes of EO long after surgery. In fact, bowel management is usually started after 2 years and is to be conducted indefinitely. As such, the trigger effect never stops. Experimental studies have also demonstrated that bladder changes are not permanent and that they are reversed after recovery from the colitis. With respect to treatment of EO, even though most of the patients and/or their families have now learned how to promptly recognize and treat recurrent episodes at home, hospitalization, and even surgery for suspected testicular torsion (four and one case in our series, respectively) are still of concern. Nevertheless, the prevention of recurrences is much more controversial: in cases of documented anomalies, such as ureterovasal reflux, the few reported attempts of endoscopic correction have yielded unsatisfactory results [18]. For intractable cases, vasectomy is now being considered the treatment of choice. However, we believe that if this option is now straightforward for unilateral cases, cases without side predilection, which were all the cases in our series, pose some ethical issues that must be presented to families and caregivers. Any aggressive attitude must be weighed against the long-term effects of recurrent EO on spermatogenesis, which are currently unknown. This paper has several limitations. First, telephone contact may have introduced some bias, such as the missing of mild cases that were difficult to recall. Second, EO is a rare condition, and therefore, the statistical analysis may be limited. Third, clinical evidence of the relationship between SC and EO cannot be confirmed clinically by withdrawing bowel management because these patients are highly dependent on this method of bowel cleansing. Most importantly, because none of our patients has currently reached pubertal age, seminal parameters are unavailable,
Pediatr Surg Int (2015) 31:305–309
and therefore, data on fertility, which would be the most critical issue in this patient population, are still missing. Nevertheless, based on these considerations, some indication may be given to families and/or caregivers about the possibility of EO onset, even long after surgery, and prompt recognition may help to avoid unnecessary anxiety, hospitalization, and even surgery. The extent to which prevention can be effective in cases with increased risk remains unknown.
Conclusions EO occurs approximately in 20 % of all male cases with ARM and communication between the rectum and urethra. In patients with anorectal malformation and testicular pain, EO should be considered the primary diagnosis, even long after reconstruction. Therefore, information about this condition should be provided to caregivers and families throughout the follow-up period to prevent unnecessary anxiety and hospitalization. Conflict of interest of interest.
The authors declare that they have no conflict
References 1. Rubio-Cordero JL, Nunez Nunez R, Blesa Sanchez E (1944) Ano-rectal malformation and recurring orchioepdidymitis in infancy. Eur J Ped Surg 4:46–48 2. Parrott TS (1985) Urological implications of anorectal malformation Urol. Clin N Am 12:13–21
309 3. Gislason T, Noronha RF, Gregory JG (1980) Acute epididymitis in boys: a 5-year retrospective study. J Urol 124:533–534 4. Raveenthiram V (2011) Sam CJ:Epydidimo-orchitis complicating anorectal malformations :collective review of 41 cases. J Urol 186:1467–1472 5. Hicks CM, Skoog Sj, Done S (1989) Ectopic vas deferens, imperforate anus and hypospadias: a new triad. J Urol 141:586–588 6. Vordermark J (1983) The persistent mesonephric duct syndrome: a description of a new syndrome. J Urol 130:958–960 7. Oguzcurt P, Tanyel FC, Buyukpamkcu N (1998) Acute scrotum due to epidydimo-orchitis associated with vasal anomalies in children with ano-rectal malformations. J Pediatr Surg 33:1834–1836 8. Kyian G, Dagli TE, Serdar H, Tugtepe H (2003) Epididymitis in infants with anorectal malformation. Eur Urol 43:576–579 9. Mickelson JJ, MacNeily AE, Blair GK (2007) The posterior urethra in anorectal malformations. J Pediatr Surg 42:585–587 10. Wiersma R (2009) Urethro ejaculatory duct reflux in children: an updated review. Eur J Pediatr Surg 24:374–376 11. Hong AR, Acuna MF, Pena A (2002) Urologic injuries associated with repair of anorectal malformations in male patients. J Pediatr Surg 37:339–344 12. Holt B, Pryor JP, Hendry WF (1995) Male infertility after surgery for imperforate anus. J Pediatr Surg 30:1677–1679 13. Pryor J, Mills SE, Howard SS (1991) Injury to the prepubertal vas deferens. I. Histological analysis of prepubertal vas. J Urol 146:473–476 14. Williams DI, Grant J (1969) Urological complications of imperforate anus. Br J Urol 41:660–665 15. Pena A (2011) Editorial comment. J Urol 186:1472 16. Duncan JE (2009) Quietmeyer CM: Bowel preparation: current status. Clin Colon Rectal Surg 22:14–20 17. Noronha R, Akbarali H, Malykhina A, Foreman RD, GreenwoodVan Meerveld B (2007) Changes in urinary bladder smooth muscle function in response to colonic inflammation. Am J Renal Physiol 293:1461–1467 18. Kajbafzadeh AM, Payadvash S (2006) Endoscopic treatment of vesicovasal and vesico ureteric reflux in infants with persistent mesonephric duct. J Urol 176:2657
123
ORIGINAL ARTICLE
Epidydimo-orchitis and anorectal malformations: when and in whom? A. Zaccara • S. Ragozzino • B. D. Iacobelli • F. Rivosecchi • M. L. Capitanucci • G. Mosiello M. Silveri • M. De Gennaro • P. Bagolan
•
Accepted: 27 January 2015 / Published online: 5 February 2015 Ó Springer-Verlag Berlin Heidelberg 2015
Abstract Purpose Epididymo-orchitis (EO) is infrequently reported in anorectal malformation (ARM) cases. Therefore, it is difficult to assess its risk factors. Methods A total of 110 male patients who were operated on for ARM at the same Institution over a period of 13 years were contacted. Association was assessed between EO and the following: spinal dysraphism (SD), symptomatic VUR (VUR), and bowel management (BM) requiring enemas. The data were analyzed with the Chi-square test. Results A total of 89 patients were contacted. Ten cases of EO were found, and all occurred in patients with rectourethral (RU) fistula after reconstruction. The patients’ age at first episode ranged between 4 and 11 years. RU fistula patients experiencing EO (Group A, 10 patients) were compared with those without EO (Group B, 33 patients). VUR occurred in 9/10 cases in Group A and in 13/33 cases A. Zaccara (&) M. L. Capitanucci G. Mosiello M. De Gennaro Department of Urology and Nephrology, Bambino Gesu’ Children’s Hospital, Rome, Italy e-mail: [email protected] S. Ragozzino Department of Surgery, Meyer Children’s Hospital, Florence, Italy B. D. Iacobelli P. Bagolan Newborn Surgery Unit, Bambino Gesu’ Children’s Hospital, Rome, Italy
in group B (Chi-square 7.8658, p = 0.005038). SD was present in 4/10 cases in group A and in 13/33 cases in Group B (Chi-square 0.0434, p = 0.83491). A total of 8/10 cases in Group A and 12/33 cases in Group B were on BM (Chi-square 5.87, p = 0.0015). Conclusions EO occurs in approximately in 20 % of male cases with ARM, and recto-urinary communication and should be considered the primary diagnosis in the presence of testicular pain. This could avoid unnecessary surgical exploration, and the family should be counseled about this subject Keywords Anorectal malformations Epididymoorchitis Bowel management
Introduction Among the different sequelae of surgery for treating imperforate anus, epididymo-orchitis (EO) is infrequently reported in the literature. To date, most of the cases are either anecdotal or collected from different series, and it has an estimated incidence of 1.2–6.1 % [1, 2]. Because of its rarity, clinical findings of EO are often unexpected and worrisome for patients and families. However, even though imperforate anus patients usually suffer from many associated conditions, which often affect them for their entire lifetime (e.g., spinal dysraphism, constipation, and recurrent UTIs), attempts to identify risk factors have only been made on the basis of the sparse data available in the literature.
F. Rivosecchi Department of Pediatrics, Bambino Gesu’ Children’s Hospital, Rome, Italy
Materials and methods
M. Silveri Department of Surgery, Bambino Gesu’ Children’s Hospital, Rome, Italy
In the period ranging from 2000 to 2013, 110 male patients with imperforate anus were operated upon at our
123
306
Institution. In order to investigate the occurrence of signs and/or symptoms of EO, an appropriately designed questionnaire was administered over the telephone to patients and/or families. EO was defined as at least two recurrent episodes of acute scrotum (scrotal pain and swelling), with or without concomitant dysuria. All the patients were treated by the same surgical team with the same surgical techniques, specifically, posterior sagittal anorectoplasty (PSARP) for intermediate and high conditions and limited PSARP for low forms. The former were treated with a three-step approach (colostomy-PSARP-colostomy closure), whereas the latter were operated upon with a single-stage procedure. In the three-step approach, PSARP was carried out at 3 months of age, and colostomy was closed shortly thereafter. In all cases requiring colostomy prior to reconstruction, a totally diverting (divided) colostomy was carried out. The level of communication between the gastrointestinal and urinary tract was assessed by a distal colostogram prior to surgical reconstruction. The association was assessed between EO and the following: Symptomatic Vesico Ureteral Reflux (VUR), Spinal Dysraphism (SD), and the necessity of bowel management (BM). Symptomatic VUR was defined as VUR leading to at least to an episode of acute Pyelonephritis (APN). VCUG was not performed in asymptomatic cases. The films of VUR patients were further reviewed for associated anomalies (ureteral ectopia, reflux of urine into the vas, and/or seminal vesicles). SD was defined as spinal cord malformations and was assessed by screening MRI (with or without neurological signs and/or symptoms). According to our follow-up policy, all patients with ARM were screened with MRI. BM was defined as the necessity for regular colonic washouts, along with dietary adjustments, for complete cleanliness. Enemas were administered daily or every other day and mostly consisted of 40 ml/kg warm saline with the addition of petroleum jelly products. The data were analyzed with the Chi-square test. Informed consent was obtained from all the individual participants included in the study.
Results Eighty-nine patients could be traced and were included in the study. Of these, 42 had no communication between the gastrointestinal and urinary tract, while in 47, a communication was present (recto-bladderneck fistula in 4 cases and Recto-Urethral (RU) fistula in 43 cases). Ten cases of
123
Pediatr Surg Int (2015) 31:305–309
EO were found, all of which occurred in patients with RU fistula. The patients with RU fistula were thus divided into two subgroups, those experiencing EO (group A, 10 patients) and those without EO (Group B, 33 patients). All cases of EO occurred after reconstruction, and the patients’ age at first episode ranged between 4 and 11 years after the completion of the surgery (colostomy closure). There was no side predilection. Five patients experienced more than two episodes of EO (up to five in two patients). In four cases, emergency admission was required, with one patient requiring surgical exploration to rule out testicular torsion. The other three cases had EO confirmed by testicular US. In the remaining patients, EO was successfully managed at home with antibiotics and/or common analgesics, in accordance with the pediatrician’s advice. Symptomatic VUR occurred in 9/10 cases in Group A and in 13/33 cases in group B (Chi-square 7.8658, p = 0.005038), and no anomalies, other than reflux, were found when reviewing the X rays. SD was present in 4/10 cases in group A and in 13/33 cases in Group B (Chi-square 0.0434, p = 0.83491). In three cases, SD occurred in association with symptomatic reflux. BM was required in 8/10 cases in Group A and in 12/33 cases in Group B (Chi-square 5.87, p = 0.0015).
Discussion With increasing data available on the follow-up of ARM patients, EO is being reported with increasing frequency, and it has an estimated incidence of 1.2–6.1 %, which is much higher than what was previously reported in the pediatric population [3]. In particular, when considering only the subset of patients with RU fistula (the only ones of our series in which EO occurred), the percentage rises to approximately 23 %. In other words, one in four patients with this condition developed EO. To date, case reports of EO are either anecdotal or collected from different series. Raveenthiram et al. [4] published 6 personal cases and reviewed an additional 35 in the literature. They identified the following significant risk factors for EO onset: congenital malformations, in particular, ureterovasal anastomosis [5], and the so called ‘‘persistent mesonephric duct syndrome,’’ which includes ectopic ureter, dysplastic kidney, renal malformations, the absence of hemitrigone, ureterovasal reflux, and the absence of the seminal vesicle on the ipsilateral side [6]. In their own series, only two cases of EO occurred after completing all steps of the reconstruction, whereas the other three EO occurred when the colostomy was still in place. In one case, the chronology was not known.
Pediatr Surg Int (2015) 31:305–309
Oguzcurt et al. [7] reported two cases of EO, one occurring before surgical repair and the other shortly thereafter, but before colostomy closure. Kyian et al. [8] described a series of 58 male patients who were operated upon for imperforated anus and reported four cases of EO. Of interest, in three cases, EO occurred prior to surgical repair, and in the remaining case, there was no communication with the urinary tract. At follow-up, no EO recurrences were seen after PSARP. Our data are significantly different from these findings for three main reasons. First, we were not able to identify any underlying anomaly when performing instrumental examinations. Second, no cases of EO occurred before completing reconstruction. Third, all the EO cases were reported long after surgery (up to 11 years after colostomy closure). With respect to congenital anomalies, some of the cases in the literature were detected by VCUG and a few others by distal colostogram demonstrating urethro ejaculatory reflux. It must be noted that our workup policy prior to reconstruction was that of performing a distal colostogram before PSARP, and VCUG is usually reserved for symptomatic (febrile UTIs) patients. Therefore, we might have missed the vas/trigone anomalies that have been repeatedly described in the literature. There is the opinion that a VCUG be incorporated in the workup of all imperforate anus patients prior to undertaking reconstruction to identify such anomalies, as Mickelson et al. advocate [9]; however, we do not agree with this view, and our attitude is corroborated by the findings of Wiersma, who, in his review of 360 voiding cystourethrograms performed in male patients over a 6 year period, found 54 cases of urethro— ejaculatory reflux (UER), nine of which had anorectal malformations [10], while six had EO and UER without any underlying pathology. Based on these findings, we believe that preliminary VCUG should probably be reserved for cases with difficult catheterization at the time of colostomy, multiple anomalies and EO occurring prior to PSARP procedure. In other words, the chances of preventing EO by performing VCUG in all male subjects with imperforate anus are small. The follow-up of asymptomatic patients with urethroejaculatory duct anomalies to determine to what extent these subjects will be prone to future EO remains to be clarified. On the other hand, VCUG may retain its validity in cases of recurrent EO, where chances of detecting ureterovasal anomalies are far greater. Even though we did not recall our EO patients for such an examination after the telephone interview, it will certainly be our policy in future cases. Other options for EO onset have been suggested over the years. In 2002, Hong et al. [11] reported that urethral injuries occurred in 20 % of patients and posterior urethra
307
diverticula in 18 % of patients following repair of imperforate anus with the posterior sagittal approach. In a review of 20 infertile men operated upon for imperforate anus, Holt [12] documented three caudal epididymal blocks in four men with a history of recurrent epididymitis. Even though no details were given about the surgical technique, the author raised concerns about the possible obstruction of the ejaculatory ducts by the pull through procedure or by the repair of the recto-urethral fistula. Alternatively, surgery may perturb the oblique course of the ejaculatory ducts as they enter the prostatic urethra, preventing reflux of urine, and the susceptibility of the prepubertal vas to any form of trauma, including simple manipulation, has been repeatedly described in the literature [13]. Data on this topic are, however, contradictory because anomalies of the angle of entry of the ejaculatory ducts joining the urethra have also been reported as being congenital [14]. Our findings seem to support the hypothesis of the involvement of the ejaculatory ducts at the time of the repair of the recto-urethral fistula. All EO episodes occurred after reconstruction, and we did not find any cases of EO in cases of recto-bladderneck communication or in cases without communication between the rectum and urinary tract. Based on these considerations, a precise anatomical assessment of the fistula site, with differentiation between the recto-prostatic and recto-bulbar fistula, could help identify those cases more at risk for EO. Unfortunately, neither in the literature nor in our surgical case notes was this difference taken into consideration. In addition, the precise identification of the fistula site, both at distal colostogram and at surgery, has proven very difficult, given the progressively younger age at which these babies are repaired. Regarding the timing of reconstruction, we performed PSARP at approximately 3 months of age, with colostomy closure shortly thereafter. This could partly explain why we did not observe cases of preoperative EO. In the previously mentioned series described by Raveenthiram et al. [4], PSARP was effective in curing EO in 36 % of cases, but the surgery was carried out at one year of age. These findings seem to support the hypothesis of a non-completely diverting colostomy contaminating the genitourinary tract [15]. In summary, early repair appears to be effective in preventing preoperative EO. However, on the other hand, the identification of the fistula site may be more difficult, and the risk of the involvement of the ejaculatory ducts becomes more significant. As previously mentioned, four of our patients with EO experienced emergency admission because of recurrent EO, and in one case, surgical exploration was required. Therefore, a correlation was sought with other conditions,
123
308
such as symptomatic VUR and neurogenic bladder, which are frequently associated with imperforate anus. In their collective review of 41 cases, Raveenthiram et al. [4] found an incidence of associated risk factors, specifically, UTIs and spinal dysraphism, of 54 and 34 %, respectively. Unfortunately, as data were collected from different series, the details regarding surgical approach were not available, and no statistical analysis was performed. In our series, all cases were operated upon by the same surgical team with the same three-step approach at approximately the same age, thus making our patient population more homogenous. Our findings confirm that there is an association between VUR and EO, and this reached statistical significance. It must be noted; however, that we only performed VCUG in cases of febrile UTIs, and therefore, the incidence could be even greater. With regard to SD, we did not find a statistically significant difference between patients with and without EO. However, it must be considered that we included all patients with spinal cord malformations, regardless if they were symptomatic or not. Thus, the variations in the data reported in the literature may reflect differences in definitions. Three cases also presented with an association between SD and symptomatic VUR. Because this association is frequently reported in the literature, we speculate that the disturbances in bladder cycling, which are typical of neurogenic bladder, could be responsible for EO onset. We suggest that a better characterization with videourodynamic studies should replace traditional imaging in these cases. Because our follow-up period spans approximately 11 years, we were also interested in lifestyle changes which may be associated with a higher incidence of EO. Interestingly, an association was found between patients on a BM regimen and EO. As previously mentioned, we defined BM patients as those requiring enemas on a daily basis to be clean, and this was a common finding in our patient population. References in the literature regarding colonic washouts causing EO in imperforate anus patients are scant and limited to 2 % of cases in the series by Raveenthiram et al. Of interest, washouts causing EO were performed by the colostomy, or in other words, before the completion of all the steps of reconstruction. To our knowledge, no other evidence exists in the literature regarding the relationships between EO and bowel management. Effects of repeated colonic washouts have been extensively studied in subjects undergoing bowel preparation for colorectal surgery [16], and it is well known that one of the complications of an enema is inflammation of the mucosal lining of the colon. However, because this procedure is usually limited to the immediate preoperative period, little is known about the effects of its long-term use. In this
123
Pediatr Surg Int (2015) 31:305–309
respect, it must be noted that imperforate anus patients actually start mechanical bowel cleansing very early on and usually continue with this regimen indefinitely because this will enable them to keep themselves clean for up to 36–48 h. This, in turn, may lead to mechanical irritation induced by the repeated insertion of enema catheters. Relationships between colonic inflammation and changes in urinary smooth muscle function have been repeatedly described in animal models [17], as have abnormalities in bladder detrusor smooth muscle contractility. We speculate that such inflammation may extend to the surrounding structures, such as the ejaculatory ducts, vas, and seminal vesicles. Enemas that are carried out on a regular basis may also act as key factors in introducing environmental bacteria to the human gut. As such, the mechanical action of the repeated insertion of enema catheters should also take into consideration. This mechanism could partly explain why some of our patients experienced repeated episodes of EO long after surgery. In fact, bowel management is usually started after 2 years and is to be conducted indefinitely. As such, the trigger effect never stops. Experimental studies have also demonstrated that bladder changes are not permanent and that they are reversed after recovery from the colitis. With respect to treatment of EO, even though most of the patients and/or their families have now learned how to promptly recognize and treat recurrent episodes at home, hospitalization, and even surgery for suspected testicular torsion (four and one case in our series, respectively) are still of concern. Nevertheless, the prevention of recurrences is much more controversial: in cases of documented anomalies, such as ureterovasal reflux, the few reported attempts of endoscopic correction have yielded unsatisfactory results [18]. For intractable cases, vasectomy is now being considered the treatment of choice. However, we believe that if this option is now straightforward for unilateral cases, cases without side predilection, which were all the cases in our series, pose some ethical issues that must be presented to families and caregivers. Any aggressive attitude must be weighed against the long-term effects of recurrent EO on spermatogenesis, which are currently unknown. This paper has several limitations. First, telephone contact may have introduced some bias, such as the missing of mild cases that were difficult to recall. Second, EO is a rare condition, and therefore, the statistical analysis may be limited. Third, clinical evidence of the relationship between SC and EO cannot be confirmed clinically by withdrawing bowel management because these patients are highly dependent on this method of bowel cleansing. Most importantly, because none of our patients has currently reached pubertal age, seminal parameters are unavailable,
Pediatr Surg Int (2015) 31:305–309
and therefore, data on fertility, which would be the most critical issue in this patient population, are still missing. Nevertheless, based on these considerations, some indication may be given to families and/or caregivers about the possibility of EO onset, even long after surgery, and prompt recognition may help to avoid unnecessary anxiety, hospitalization, and even surgery. The extent to which prevention can be effective in cases with increased risk remains unknown.
Conclusions EO occurs approximately in 20 % of all male cases with ARM and communication between the rectum and urethra. In patients with anorectal malformation and testicular pain, EO should be considered the primary diagnosis, even long after reconstruction. Therefore, information about this condition should be provided to caregivers and families throughout the follow-up period to prevent unnecessary anxiety and hospitalization. Conflict of interest of interest.
The authors declare that they have no conflict
References 1. Rubio-Cordero JL, Nunez Nunez R, Blesa Sanchez E (1944) Ano-rectal malformation and recurring orchioepdidymitis in infancy. Eur J Ped Surg 4:46–48 2. Parrott TS (1985) Urological implications of anorectal malformation Urol. Clin N Am 12:13–21
309 3. Gislason T, Noronha RF, Gregory JG (1980) Acute epididymitis in boys: a 5-year retrospective study. J Urol 124:533–534 4. Raveenthiram V (2011) Sam CJ:Epydidimo-orchitis complicating anorectal malformations :collective review of 41 cases. J Urol 186:1467–1472 5. Hicks CM, Skoog Sj, Done S (1989) Ectopic vas deferens, imperforate anus and hypospadias: a new triad. J Urol 141:586–588 6. Vordermark J (1983) The persistent mesonephric duct syndrome: a description of a new syndrome. J Urol 130:958–960 7. Oguzcurt P, Tanyel FC, Buyukpamkcu N (1998) Acute scrotum due to epidydimo-orchitis associated with vasal anomalies in children with ano-rectal malformations. J Pediatr Surg 33:1834–1836 8. Kyian G, Dagli TE, Serdar H, Tugtepe H (2003) Epididymitis in infants with anorectal malformation. Eur Urol 43:576–579 9. Mickelson JJ, MacNeily AE, Blair GK (2007) The posterior urethra in anorectal malformations. J Pediatr Surg 42:585–587 10. Wiersma R (2009) Urethro ejaculatory duct reflux in children: an updated review. Eur J Pediatr Surg 24:374–376 11. Hong AR, Acuna MF, Pena A (2002) Urologic injuries associated with repair of anorectal malformations in male patients. J Pediatr Surg 37:339–344 12. Holt B, Pryor JP, Hendry WF (1995) Male infertility after surgery for imperforate anus. J Pediatr Surg 30:1677–1679 13. Pryor J, Mills SE, Howard SS (1991) Injury to the prepubertal vas deferens. I. Histological analysis of prepubertal vas. J Urol 146:473–476 14. Williams DI, Grant J (1969) Urological complications of imperforate anus. Br J Urol 41:660–665 15. Pena A (2011) Editorial comment. J Urol 186:1472 16. Duncan JE (2009) Quietmeyer CM: Bowel preparation: current status. Clin Colon Rectal Surg 22:14–20 17. Noronha R, Akbarali H, Malykhina A, Foreman RD, GreenwoodVan Meerveld B (2007) Changes in urinary bladder smooth muscle function in response to colonic inflammation. Am J Renal Physiol 293:1461–1467 18. Kajbafzadeh AM, Payadvash S (2006) Endoscopic treatment of vesicovasal and vesico ureteric reflux in infants with persistent mesonephric duct. J Urol 176:2657
123
