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Management of rectal stenosis with endoscopic balloon dilatation
Journal of Pediatric Surgery (2013) 48, E13–E16
www.elsevier.com/locate/jpedsurg
Management of rectal stenosis with endoscopic balloon dilatation Guy Hidas a,1 , David Gibbs b , Alamsahebpour Alireza a , Antoine E. Khoury a,⁎ a
Urology Department, University of California, Irvine, Children's Hospital of Orange County, Orange California, CA 92868, USA b Surgery Department, University of California, Irvine, Children's Hospital of Orange County, Orange, CA 92868, USA Received 23 November 2012; revised 24 December 2012; accepted 17 January 2013
Key words: Anal web; Anal stenosis; Balloon dilatation; Endoscopy
Abstract We present a rare case of female newborn that presented with atypical genitalia, vaginal atresia and a short segment rectal stenosis which was successfully managed in a minimally invasive fashion using urological endoscopic and balloon dilatation technology. We believe that this technique is safe and feasible for short rectal stenotic segments and might prevent the need for major reconstruction surgery. © 2013 Elsevier Inc. All rights reserved.
1. Clinical presentation A full term female, was born via normal vaginal delivery, to a 31-year-old healthy mother. Prenatal ultrasound (US) had demonstrated a single umbilical artery and minimal hydronephrosis (HN) that resolved on follow-up prenatal US. Physical examination revealed stable vital signs, newborn weight of 3500 g, and examination of the head, neck, chest and limbs was unremarkable. The abdomen was distended but soft and non tender. The genitals appeared atypical with an enlarged clitoris and a urethral meatus opening at the tip of the clitoris, and no vaginal opening was found (Fig. 1). The anus was noted to be in a normal anatomical position. At post delivery day two, the baby still ⁎ Corresponding author. Tel.: + 1 714 509 3914; fax: + 1 714 509 3916. E-mail addresses: [email protected] (G. Hidas), [email protected] (A.E. Khoury). 1 CHOC Children's Urology, University of California, Irvine, Orange, CA 92868. Tel.: +1 714 512 3919; fax: +1 714 512 3916, +949 726 2627(CellPhone). 0022-3468/$ – see front matter © 2013 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpedsurg.2013.01.035
had not passed meconium and had two bilious episodes of vomiting. Rectal examination was performed with a Q-tip without difficulty. Laboratory studies showed a karyotype of 46 XX and normal electrolytes. A hormonal panel evaluation revealed normal levels of 17-hydroxyprogesterone, testosterone FSH, LH and AMH profile. US at the age of 3 days demonstrated grade one HN in the right kidney and grade two in the left with dilatation of the proximal ureter. On pelvic US a 5 cm fluid filled dilated vagina and diffuse dilated loops of bowel within the abdomen were seen. A barium enema showed a dilated rectum and colon containing meconium, on sharp transition in the rectum with persistent hold up of contrast after 2.5 h (Fig. 2). Extrinsic compression of the rectum was suspected and our aim was to decompress the distended vagina in order to relieve the obstructed urinary and gastrointestinal system. Interventional Radiology was consulted with a request to place a percutaneous tube vaginostomy. A computed tomography was performed which demonstrated no presacral mass, Sacral or vertebral anomaly. Since the bowel loops
E14
G. Hidas et al.
Fig. 1 Atypical appearance of the genitals. Enlarged clitoris and a urethral meatus opening at the tip of the clitoris. No vaginal opening was found.
were anterior to the dilated vagina a percutaneous approach was deemed unsafe. At the age of 6 days the baby was taken to the operating room in order to decompress the urogenital sinus by placing a percutaneous tube vaginostomy with laparoscopic guidance and to better understand the anatomical relations of the pelvic organs in planning for future reconstruction. Cystoscopy was performed through the urethral meatus at the tip of
Fig. 2 A barium enema showed a dilated rectum and colon containing meconium, on sharp transition in the rectum with persistent hold up of contrast after 2.5 h.
the clitoris. The urethra was normal; no communication was identified between the vagina and the urethra. The ureteric orifices and bladder were normal. At this point a diagnostic laparoscopy was performed. We observed distended loops of bowel, particularly the colon and rectum. The proximal vagina was distended. A laparoscopic guided percutaneous tube vaginostomy was placed using the Seldinger technique [1]. With an empty bladder, a 16 gauge Angiocath was passed through the abdominal wall into the anterior vaginal wall under direct laparoscopic vision. A 0.35 guide wire was passed through the Angiocath into the vagina and the Angiocath was removed. A 10 French dilator peel away sheath (Cook Medical Inc. IN, USA) was passed over the wire with laparoscopic control. The dilator and the wire were removed and an eight French feeding tube was placed through the peel away sheath. 50 ml of milky colored fluid was drained from the upper vagina. After draining the vagina, the rectal dilation persisted. We therefore questioned whether the vaginal dilatation was significant enough to cause extrinsic compression and obstruction of the rectum. In order to better understand the cause of the rectal obstruction we tried to pass Hegar dilators into the rectum and found that they would not pass higher than 3 cm. We used a 9.5 French pediatric cystoscope with water irrigation, to scope the rectum. We found a stenotic rectal segment 3 cm from the anus (Fig. 3). We passed a 0.35 guide wire through the stenotic area and then passed the scope over the guide wire. The stenotic segment was carefully examined and found to be less than 5 mm in length. The rectum proximal to the stenotic area was dilated. Since the stenotic segment was very short we decided to attempt to manage it using an endoscopic balloon dilatation approach. We placed a 30 French NephroMax® Balloon dilator (Boston Scientific, MA, and USA) over the wire and
Managing rectal stenosis with endoscopic balloon dilatation
E15 observed. The patient underwent serial rectal dilatation up to number 13 Hegar dilator twice daily for four months at home by the parents. At six month follow-up assessment the child was passing normal bowel movements without the need for diversion. She is awaiting repair of the distal vaginal atresia.
2. Discussion
Fig. 3 Rectoscopic view demonstrated stenotic rectal segment 3 cm from the anus.
inflated it to a pressure of 15 atm for two minutes to dilate the rectum from the stenotic segment down to the anus (Fig. 4). Once the balloon was deflated the baby immediately passed a significant amount of stool. In the following days she continued to stool normally and the abdomen was decompressed. Cefotaxime and metronidazole were used perioperatively for 48 h. No post operative complications were
Fig. 4 Perineal view. NephroMax® Balloon dilator (Boston Scientific, MA, USA) is protruding from the anus during dilatation of the rectum from the stenotic segment down to the anus.
Rectal stenosis is a rare anomaly in the spectrum of anorectal malformations with less than 2% prevalence in the entire anorectal malformation spectrum. Rectal stenosis can present as an isolated malformation or part of a urogenital sinus or cloacal complex [2]. Presentation is variable and may range from a newborn presenting with complete bowel obstruction and meconium ileus to an older child presenting with chronic constipation [3,4]. Anal stenosis is part of low anorectal malformation. In this anomaly, the stenotic anal opening is located in the normal anatomic position and along the voluntary muscle sphincter complex. The stenotic part is a median bar or a membrane that is situated at the level of the dentate line. The median bar should be excised as it interferes with fecal outflow. Complete anal membrane (rectal atresia) requires surgical incision, while partial membranes usually may be managed by dilatations only. Traditionally these cases are managed initially by diverting colostomy followed by distal colostogram and formal reconstruction. Balloon dilatation is a common endourological procedure. It is used mainly to dilate post traumatic or postoperative urethral or ureteral strictures as well as to establish a percutaneous tract for management of renal stones. The advantage of using a balloon versus rigid sequential dilators is that the deflated balloon can be passed over the wire through the stenotic segment. The balloon allows in situ radial dilation without repeated shear forces associated with the risk of false passage and hemorrhage [5]. A guide wire is initially positioned under direct endoscopic vision followed by fluoroscopic guided positioning of the balloon with the aid of the proximal and distal radiopaque markers. The balloon is then inflated with contrast fluid via a pressure inflation device. As the balloon is inflated a “waist” appears at the site of the greatest resistance (the stricture site). Once the balloon is fully inflated and the “waist” disappears, the balloon is kept fully inflated for two to five minutes before deflating and removing it. To our knowledge there are no reported cases using this technology to treat anorectal obstruction. Many different surgical procedures have been described to manage rectal stenosis; most of them involve multiple stages and colostomy diversion [5]. We present our experience with a female neonate born with a long distal segment of vaginal atresia and very short annular rectal stenosis managed endoscopically
E16 with balloon dilatation. She will require ongoing careful follow-up for possible development of restenosis In this case the decision to use an endoscopic approach instead of the traditional open approach was based on the endoscopic observation that the stenotic area was short and appeared like an intra-luminal web of mucosal tissue. We believe that this technique is primarily indicated for similar lesions with short stenotic segments. The successful correction of this stenotic lesion using a minimally invasive technique well established in endourology, offers a reasonable alternative and a useful addition to the armamentarium of surgeons involved in the care of this patient population.
G. Hidas et al.
References [1] Seldinger SI. Catheter replacement of the needle in percutaneous arteriography; a new technique. Acta Radiol 1953;39:368. [2] Murphy F, Puri P, Hutson JM, et al. Incidence and frequency of different types, and classification of anorectal malformations. In: Holschneider AM, Hutson JM, editors. Anorectal malformations in children. Berlin: Springer-Verlag; 2006. p. 163-84. [3] Zia-w-Miraj Ahmad M, Brereton RJ, et al. Rectal atresia and stenosis. J Pediatr Surg 1995;30:1546-50. [4] Rashid KA, Wakhlu A, Tandon RK, et al. Anorectal junction stenosis: diagnosis and management. Eur J Pediatr Surg 2008;18:303-6. [5] Wolf Jr S. Percutaneous approaches to the upper urinary tract collecting system. Campbell-Walsh urology. 10th ed. Saunders; 2011. p. 1324-56.
www.elsevier.com/locate/jpedsurg
Management of rectal stenosis with endoscopic balloon dilatation Guy Hidas a,1 , David Gibbs b , Alamsahebpour Alireza a , Antoine E. Khoury a,⁎ a
Urology Department, University of California, Irvine, Children's Hospital of Orange County, Orange California, CA 92868, USA b Surgery Department, University of California, Irvine, Children's Hospital of Orange County, Orange, CA 92868, USA Received 23 November 2012; revised 24 December 2012; accepted 17 January 2013
Key words: Anal web; Anal stenosis; Balloon dilatation; Endoscopy
Abstract We present a rare case of female newborn that presented with atypical genitalia, vaginal atresia and a short segment rectal stenosis which was successfully managed in a minimally invasive fashion using urological endoscopic and balloon dilatation technology. We believe that this technique is safe and feasible for short rectal stenotic segments and might prevent the need for major reconstruction surgery. © 2013 Elsevier Inc. All rights reserved.
1. Clinical presentation A full term female, was born via normal vaginal delivery, to a 31-year-old healthy mother. Prenatal ultrasound (US) had demonstrated a single umbilical artery and minimal hydronephrosis (HN) that resolved on follow-up prenatal US. Physical examination revealed stable vital signs, newborn weight of 3500 g, and examination of the head, neck, chest and limbs was unremarkable. The abdomen was distended but soft and non tender. The genitals appeared atypical with an enlarged clitoris and a urethral meatus opening at the tip of the clitoris, and no vaginal opening was found (Fig. 1). The anus was noted to be in a normal anatomical position. At post delivery day two, the baby still ⁎ Corresponding author. Tel.: + 1 714 509 3914; fax: + 1 714 509 3916. E-mail addresses: [email protected] (G. Hidas), [email protected] (A.E. Khoury). 1 CHOC Children's Urology, University of California, Irvine, Orange, CA 92868. Tel.: +1 714 512 3919; fax: +1 714 512 3916, +949 726 2627(CellPhone). 0022-3468/$ – see front matter © 2013 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpedsurg.2013.01.035
had not passed meconium and had two bilious episodes of vomiting. Rectal examination was performed with a Q-tip without difficulty. Laboratory studies showed a karyotype of 46 XX and normal electrolytes. A hormonal panel evaluation revealed normal levels of 17-hydroxyprogesterone, testosterone FSH, LH and AMH profile. US at the age of 3 days demonstrated grade one HN in the right kidney and grade two in the left with dilatation of the proximal ureter. On pelvic US a 5 cm fluid filled dilated vagina and diffuse dilated loops of bowel within the abdomen were seen. A barium enema showed a dilated rectum and colon containing meconium, on sharp transition in the rectum with persistent hold up of contrast after 2.5 h (Fig. 2). Extrinsic compression of the rectum was suspected and our aim was to decompress the distended vagina in order to relieve the obstructed urinary and gastrointestinal system. Interventional Radiology was consulted with a request to place a percutaneous tube vaginostomy. A computed tomography was performed which demonstrated no presacral mass, Sacral or vertebral anomaly. Since the bowel loops
E14
G. Hidas et al.
Fig. 1 Atypical appearance of the genitals. Enlarged clitoris and a urethral meatus opening at the tip of the clitoris. No vaginal opening was found.
were anterior to the dilated vagina a percutaneous approach was deemed unsafe. At the age of 6 days the baby was taken to the operating room in order to decompress the urogenital sinus by placing a percutaneous tube vaginostomy with laparoscopic guidance and to better understand the anatomical relations of the pelvic organs in planning for future reconstruction. Cystoscopy was performed through the urethral meatus at the tip of
Fig. 2 A barium enema showed a dilated rectum and colon containing meconium, on sharp transition in the rectum with persistent hold up of contrast after 2.5 h.
the clitoris. The urethra was normal; no communication was identified between the vagina and the urethra. The ureteric orifices and bladder were normal. At this point a diagnostic laparoscopy was performed. We observed distended loops of bowel, particularly the colon and rectum. The proximal vagina was distended. A laparoscopic guided percutaneous tube vaginostomy was placed using the Seldinger technique [1]. With an empty bladder, a 16 gauge Angiocath was passed through the abdominal wall into the anterior vaginal wall under direct laparoscopic vision. A 0.35 guide wire was passed through the Angiocath into the vagina and the Angiocath was removed. A 10 French dilator peel away sheath (Cook Medical Inc. IN, USA) was passed over the wire with laparoscopic control. The dilator and the wire were removed and an eight French feeding tube was placed through the peel away sheath. 50 ml of milky colored fluid was drained from the upper vagina. After draining the vagina, the rectal dilation persisted. We therefore questioned whether the vaginal dilatation was significant enough to cause extrinsic compression and obstruction of the rectum. In order to better understand the cause of the rectal obstruction we tried to pass Hegar dilators into the rectum and found that they would not pass higher than 3 cm. We used a 9.5 French pediatric cystoscope with water irrigation, to scope the rectum. We found a stenotic rectal segment 3 cm from the anus (Fig. 3). We passed a 0.35 guide wire through the stenotic area and then passed the scope over the guide wire. The stenotic segment was carefully examined and found to be less than 5 mm in length. The rectum proximal to the stenotic area was dilated. Since the stenotic segment was very short we decided to attempt to manage it using an endoscopic balloon dilatation approach. We placed a 30 French NephroMax® Balloon dilator (Boston Scientific, MA, and USA) over the wire and
Managing rectal stenosis with endoscopic balloon dilatation
E15 observed. The patient underwent serial rectal dilatation up to number 13 Hegar dilator twice daily for four months at home by the parents. At six month follow-up assessment the child was passing normal bowel movements without the need for diversion. She is awaiting repair of the distal vaginal atresia.
2. Discussion
Fig. 3 Rectoscopic view demonstrated stenotic rectal segment 3 cm from the anus.
inflated it to a pressure of 15 atm for two minutes to dilate the rectum from the stenotic segment down to the anus (Fig. 4). Once the balloon was deflated the baby immediately passed a significant amount of stool. In the following days she continued to stool normally and the abdomen was decompressed. Cefotaxime and metronidazole were used perioperatively for 48 h. No post operative complications were
Fig. 4 Perineal view. NephroMax® Balloon dilator (Boston Scientific, MA, USA) is protruding from the anus during dilatation of the rectum from the stenotic segment down to the anus.
Rectal stenosis is a rare anomaly in the spectrum of anorectal malformations with less than 2% prevalence in the entire anorectal malformation spectrum. Rectal stenosis can present as an isolated malformation or part of a urogenital sinus or cloacal complex [2]. Presentation is variable and may range from a newborn presenting with complete bowel obstruction and meconium ileus to an older child presenting with chronic constipation [3,4]. Anal stenosis is part of low anorectal malformation. In this anomaly, the stenotic anal opening is located in the normal anatomic position and along the voluntary muscle sphincter complex. The stenotic part is a median bar or a membrane that is situated at the level of the dentate line. The median bar should be excised as it interferes with fecal outflow. Complete anal membrane (rectal atresia) requires surgical incision, while partial membranes usually may be managed by dilatations only. Traditionally these cases are managed initially by diverting colostomy followed by distal colostogram and formal reconstruction. Balloon dilatation is a common endourological procedure. It is used mainly to dilate post traumatic or postoperative urethral or ureteral strictures as well as to establish a percutaneous tract for management of renal stones. The advantage of using a balloon versus rigid sequential dilators is that the deflated balloon can be passed over the wire through the stenotic segment. The balloon allows in situ radial dilation without repeated shear forces associated with the risk of false passage and hemorrhage [5]. A guide wire is initially positioned under direct endoscopic vision followed by fluoroscopic guided positioning of the balloon with the aid of the proximal and distal radiopaque markers. The balloon is then inflated with contrast fluid via a pressure inflation device. As the balloon is inflated a “waist” appears at the site of the greatest resistance (the stricture site). Once the balloon is fully inflated and the “waist” disappears, the balloon is kept fully inflated for two to five minutes before deflating and removing it. To our knowledge there are no reported cases using this technology to treat anorectal obstruction. Many different surgical procedures have been described to manage rectal stenosis; most of them involve multiple stages and colostomy diversion [5]. We present our experience with a female neonate born with a long distal segment of vaginal atresia and very short annular rectal stenosis managed endoscopically
E16 with balloon dilatation. She will require ongoing careful follow-up for possible development of restenosis In this case the decision to use an endoscopic approach instead of the traditional open approach was based on the endoscopic observation that the stenotic area was short and appeared like an intra-luminal web of mucosal tissue. We believe that this technique is primarily indicated for similar lesions with short stenotic segments. The successful correction of this stenotic lesion using a minimally invasive technique well established in endourology, offers a reasonable alternative and a useful addition to the armamentarium of surgeons involved in the care of this patient population.
G. Hidas et al.
References [1] Seldinger SI. Catheter replacement of the needle in percutaneous arteriography; a new technique. Acta Radiol 1953;39:368. [2] Murphy F, Puri P, Hutson JM, et al. Incidence and frequency of different types, and classification of anorectal malformations. In: Holschneider AM, Hutson JM, editors. Anorectal malformations in children. Berlin: Springer-Verlag; 2006. p. 163-84. [3] Zia-w-Miraj Ahmad M, Brereton RJ, et al. Rectal atresia and stenosis. J Pediatr Surg 1995;30:1546-50. [4] Rashid KA, Wakhlu A, Tandon RK, et al. Anorectal junction stenosis: diagnosis and management. Eur J Pediatr Surg 2008;18:303-6. [5] Wolf Jr S. Percutaneous approaches to the upper urinary tract collecting system. Campbell-Walsh urology. 10th ed. Saunders; 2011. p. 1324-56.