- Email: [email protected]
Percutaneous endoscopic cecostomy
Percutaneous Endoscopic Cecostomy S t e v e n L. W e r l i n ,
M.D.
Chronic constipation and encopresis are common problems in children with spina bifida and anorectal anomalies. Commonly used therapies include complicated bowel regimens and antegrade continence enemas delivered via surgically placed appendicostomies and radiologically placed cecostomies. We have described a technique for percutaneous placement of cecostomies for the delivery of continence enemas or venting. Percutaneous cecostomies were placed in 22 patients. Improvement in bowel management occurred in all patients. Copyright 2002, Elsevier Science (USA). All rights reserved.
ecal incontinence and chronic constipation are common problems in children with spina bifida, anorectal malformations, and neurological handicaps. Traditional management with diet modifications, laxatives, enemas, and biofeedback is frequently unsuccessful. >2 The antegrade continence enema (ACE) delivered through a surgically placed appendicostomy or cecostomy was first described by Malone et aP in children with spina bifida and anorectal anomalies. Ths procedure formerly entailed a major surgical procedure but now can be done laparoscopically. Shandling et al described the successful insertion of percutaneous cecostomies under fluoroscopic guidance for the delivery of the ACE in a group of children primarily with spina bifida. 4,5 With the use of ACE through either an appendicostomy or cecostomy most children develop continence. A recent report describes the use of radiologically placed cecostomies for the delivery of ACE in neurologically normal children with constipation unresponsive to medical therapy. 6 There are case reports in the adult literature, which describe the colonoscopic placement of percutaneous cecostomies for colonic decompression in Ogilvie's syndrome, left-sided coIonic obstruction due to malignancy and pseudo-obstruction, r We adapted this technique to children to simplify the insertion of percutaneous endoscopic cecostomy (PEC) tubes and to make ACE more readily available. 8
F
Patient Population Twenty five patients with chronic constipation with or without encopresis, or with encopresis alone who had failed medical
management with laxatives, enemas and dietary modification or who required colonic venting for Hirschsprung's disease or Olgivie's syndrome had PEC's placed. The underlying medical problems are summarized in Table 1. PECs were placed for venting in one patient with Hirschsprung's disease and recurrent enterocolitis and in one patient with cystic fibrosis and recurrent distal intestinal obstruction syndrome.
Methods Since most of our patients could not or would not ingest a bowel prep, children were admitted to the hospital for cleanout. Colonoscopy to the cecum was performed under general endotracheal anesthesia or conscious sedation. Because of the neurological and behavioral problems found in many of our patients, general anestesia was usually used. The standard pull technique for percutaneous gastrostomy was used. The anterior abdominal wall was transilluminated, prepped and draped. A small incision was made and a trocar was passed into the cecum under direct vision. Unlike percutaneous gastrostomy placement, the passage of the trocar into the cecum was not always easy. Several passes were frequently required. A guide wire, passed through the trocar, was grasped with a snare and pulled out through the anus. The guide wire was tied to a 20 French pull type PEG tube, and pulled retrograde through the colon and out the anterior abdominal wall (Figs 1 and 2). All patients received prophylactic antibiotics (eg, metronidazole and gentamicin) for 24 hours. Most patients were discharged within 24 hours. The cecostomy tube was flushed with 20 ml of tap water twice a day for the first 7-14 days. 5,s Prior to ACE the tube was usually converted to a low profile device (Genie; Bard, Billerica, MA). As reported by Shandling et al 4 ACEs were then given using Fleet's Phospho-Soda (0.3-0.6 mL/kg) followed by tap water, saline or oral colonic lavage solution (0.5-1.5 L) daily or every other day. The regimen was individualized so that the patient would pass a stool within 1 hour and remain continent for the rest of the day. For convenience, most patients received the ACE in the evening
Results From the Division of Gastroenterology, Department of Pediatrics, The Medical College of Wisconsin; and Department of Pediatrics, The Children's Hospital of Wisconsin, Milwaukee, Wl. Address reprint requests to Steven L. Werlin, MD, Department of Pediatrics, The Medical College of Wisconsin, 8701 Watertown Plank Rd, Milwaukee, Wl 53226; e-mail: swerlin @mcw.edu. Copyright 2002, Elsevier Science (USA). All rights reserved. 1096-2883/02/0404-0009535.00/0 doi:l 0.1053/tgie.2002.37441
PEC was attempted in 25 patients (12 males ) with an age range of 17 months to 22 years. The procedure was successful in all but 3. The 3 failures were due to inability to find an acceptable window because of failure to transilluminate (2) and inability by 3 endoscopists to reach the cecum due to tortuosity of the colon (1). These patients were treated with surgical cecostomy (2) and radiologically placed cecostomy (1).
Techniques in Gastrointestinal Endoscopy, Vol 4, No 4 (October), 2002: pp 221-224
221
TABLE 1. Indication for Cecostomy Imperforate anus Spina bifida Cerebral palsy Asperger's Syndrome Nemaline myopathy Colonic psuedo-obstruction Neurofibromatosis with segmental colonic dysmotility Hirschsprung's disease and recurrent enterocolitis Cystic fibrosis
8 6 5 1 1 1 1 1 1
The duration of the procedure was typically 20 to 30 minutes (range, 15 to 90 minutes). The longer procedures were early in our experience. The total hospital stay averaged 2 days (range, 1-7 days). Early postoperative complications included urinary tract, infection (1), seizures (1), and fever with negative blood culture, abdominal distention and pain, with spontaneous resolution on the fifth day (1). The patient with repaired Hirschsprung's disease and recurrent enterocolitis developed enterocolitis 9 days after PEC placement, prior to use of his cecostomy tube. Severe abdominal distension led to pressure necrosis from the external bumper. He was successfully treated with irrigation, venting of the PEC tube and an oral antibiotic. One child died 9 months after PEC placement of his underlying disease. Development of granulation tissue around the stomas
was common and was treated with silver nitrate or triamcinolone cream. Six of our patients had ventriculoperitoneal shunts. None of these patients developed CSF infections after placement of the PEC. The only long-term complication occurred in a teenage girl with imperforate anus. During a 2-year period she gained 20 pounds but did not come in for follow-up visits nor show her stoma to her parents. Her stoma developed severe pressure necrosis and infection and required surgical treatment. All surviving patients are improved on their ACE regimen with a follow-up period of 5 months to 5 years. All patients and families were pleased with their cecostomy tubes and ACEs. For example, one 7-year-old boy with repaired imperforate anus, who had never achieved continence, was referred for evaluation for colectomy. After PEC placement and ACE, he has been continent for 4 years and no longer requires diapers.
Discussion Intractable constipation can be painful and difficult to manage.1 Encopresis is humiliating and devastating both to the individual and family. Traditional management consists of one or a combination of the following: diet modification, laxatives, enemas, suppositories and biofeedback. These therapies require patience and long term compliance, which can be very frustrating both to patients and families. In children with spina bifida
A
D
E
Fig 1. A) Colonoscopy is performed to the cecum and the anterior abdominal wall is transilluminated. 13) After prepping and draping the anterior abdominal wall, a 0.5 cm incision is made and a trocar is passed into the cecum. C) A guide wire is passed through the trocar. The guide wire is grasped with a snare passed through the biopsy channel of the colonoscope. The colonoscope is removed. D) The guide wire is tied to a 20 French pull type PEG tube and pulled retrograde through the colon and out the anterior abdominal wall. E) Properly placed percutaneous cecostomy. (Reprinted with permission, s)
222
STEVEN L. WERLIN
Fig 2. Internal portion of cecostomy tube within cecum after percutaeous cecostomy.
or anorectal anomalies lack of control of the anal sphincter often leads to incontinence. The ACE procedure, first described by Malone et aP others, 9 has been a very successful method for dealing with this problem. The Malone procedure, is performed by resecting the appendix with a cuff of cecum. The remaining cecum is then reversed and sutured to the skin. A modified Malone procedure is preferred by others. In this procedure, the appendix is left in situ and sutured to the skin. A circular flush stoma is created either at the appendiceal tip or the reversed cecum followed by an antireflux procedure. A 10 Fr catheter is left in this conduit for two weeks. Unfortunately, the Malone procedure is typically done at laparotomy. This procedure can be performed laparoscopically if the appendix is intact. Complications of surgically placed cecostomy or appendicostomy, which include stomal stenosis, stoma leakage, difficulty in intubating the stoma, and appendiceal necrosis may occur in up to 20% of patients. Shandling et al 4 simplified the delivery of ACE by inserting a percutaneous cecostomy under fluoroscopic guidance after a bowel clean out. A cecostomy is placed by the push technique also used for percutaneous gastrostomy placement. The catheter is left to drain, and flushed with 10 mL of saline twice daily. Most patients are discharged on the second hospital day. The use of the ACE is begun one week after placement. 4,5 The complications encountered are similar to those we reported.
PERCUTANEOUS ENDOSCOPIC CECOSTOMY
One patient had his cecostomy tube placed in the terminal ileum but it functioned well. We modified the technique of percutaneous placement of cecostomy based on the adult literature and inserted cecostomies at colonoscopy. 8 With regards to hospital stay and recovery time, our method is comparable to that employed by Shandling. Placement at cecostomy offers the advantage of direct visualization of the cecostomy site, which avoids inadvertent placement of the cecostomy in the terminal ileum or other undesirable sites. Having the cecostomy tube in place at all times prevents the complication of stomal stenosis and difficulty of intubating the stoma encountered in a surgically placed cecostomy. For aesthetic reasons, the PEC tube can be changed to a low profile gastrostomy device. Our patients and families have been very satisfied both with the procedure and the ease and efficacy of ACE. Teenage patients have become more independent. Prior to PEC, most of our patients were on complicated home bowel regimens, which at times required several hours. After PEC placement, the home bowel regimen is reduced to 45-60 minutes several times a week. ACE through PEC has dramatically changed the quality of life of these patients and their caregivers. Antegrade continence enemas have been shown to reduce fecal incontinence and constipation in children with spina bifida. Even more importantly improvement in self esteem and psychosocial function has been reported. 1~ Children with intractable constipation or fecal incontinence because of spina bifida, anorectal anomalies or severe neurodevelopmental problems should no longer be allowed to suffer needlessly. Antegrade continence enemas are recommended for selected patients with spina bifida, spinal cord injury, anorectal anomalies, and severe neurological handicaps. In children with Hirschsprung's disease and recurrent enterocolitis or colonic pseudo-obstruction, cecostomies can be used for irrigation and venting. In neurologically normal children with intractable constipation ACE can be an effective treatmetn. The ease of insertion and the widespead availability of pediatric gastroenterologists, the low complication rate and short recovery time make colonoscopic cecostomy the method of choice for the delivery of ACE in children. ACE through the use of PEC is not recommended for normal individuals with constipation who fail medical management because of non-compliance, since compliance is also required for a successful ACE program.
References 1. Liptak GS, Revell GM: Management of bowel dysfunction in children with spinal cord disease or injury by means of the enema continence catheter. J Pediatr 120:190-194, 1992 2. Shankar KF, Losty PD, Kenny SE, et al: Functional results following the antegrade continence enema procedure. Br J Surg 85:980-982, 1988 3. Malone PS, Ransley PG, Kiely EM: Preliminary report: The antegrade continence enema. Lancet 336:1217-1218, 1990 4. Shandling B, Chait PG, Richards HF: Percutaneous cecostomy: A new technique in the management of fecal incontinence. J Pediatr Surg 31:534-537, 1996 5. Chait PG, Shandling B, Richards M, et al: A fecal incontinence in children: Treatment with percutaneous cecostomy tube placement--a prospective study. Radiology 203:621-624, 1997 6. Youssef N, Barksdale Jr E, Griffiths J, et al: Management of intrac-
223
table constipation with antegrade enemas in neurologically intact children. J Pediatr Gastroenterol Nutr 34:402-405, 2002 7. Ponsky JL, Aszodi A, Perse D: Percutaneous endoscopic cecostomy: A new approach to nonobstructive colonic dilatation. Gastrointest Endosc 32:108-110, 1986 8. P,ivera M, Kugathasan S, Berger W, et al: Percutaneous colono-
224
scopic cecostomy for management of chronic constipation in children. Gastrointest Endosc 53:225-228, 2001 9. Webb H, Barraza M, Stevens P, et al: Bowel dysfunction in spina bifida--An american experience with the ace procedure. Eur J Pediatr Surg 8:37-38, 1998 (suppl) 10. Aksnes G, Diseth T, Helseth A, et at: Pediatrics 109:484-489, 2002
STEVEN L. WERLIN
M.D.
Chronic constipation and encopresis are common problems in children with spina bifida and anorectal anomalies. Commonly used therapies include complicated bowel regimens and antegrade continence enemas delivered via surgically placed appendicostomies and radiologically placed cecostomies. We have described a technique for percutaneous placement of cecostomies for the delivery of continence enemas or venting. Percutaneous cecostomies were placed in 22 patients. Improvement in bowel management occurred in all patients. Copyright 2002, Elsevier Science (USA). All rights reserved.
ecal incontinence and chronic constipation are common problems in children with spina bifida, anorectal malformations, and neurological handicaps. Traditional management with diet modifications, laxatives, enemas, and biofeedback is frequently unsuccessful. >2 The antegrade continence enema (ACE) delivered through a surgically placed appendicostomy or cecostomy was first described by Malone et aP in children with spina bifida and anorectal anomalies. Ths procedure formerly entailed a major surgical procedure but now can be done laparoscopically. Shandling et al described the successful insertion of percutaneous cecostomies under fluoroscopic guidance for the delivery of the ACE in a group of children primarily with spina bifida. 4,5 With the use of ACE through either an appendicostomy or cecostomy most children develop continence. A recent report describes the use of radiologically placed cecostomies for the delivery of ACE in neurologically normal children with constipation unresponsive to medical therapy. 6 There are case reports in the adult literature, which describe the colonoscopic placement of percutaneous cecostomies for colonic decompression in Ogilvie's syndrome, left-sided coIonic obstruction due to malignancy and pseudo-obstruction, r We adapted this technique to children to simplify the insertion of percutaneous endoscopic cecostomy (PEC) tubes and to make ACE more readily available. 8
F
Patient Population Twenty five patients with chronic constipation with or without encopresis, or with encopresis alone who had failed medical
management with laxatives, enemas and dietary modification or who required colonic venting for Hirschsprung's disease or Olgivie's syndrome had PEC's placed. The underlying medical problems are summarized in Table 1. PECs were placed for venting in one patient with Hirschsprung's disease and recurrent enterocolitis and in one patient with cystic fibrosis and recurrent distal intestinal obstruction syndrome.
Methods Since most of our patients could not or would not ingest a bowel prep, children were admitted to the hospital for cleanout. Colonoscopy to the cecum was performed under general endotracheal anesthesia or conscious sedation. Because of the neurological and behavioral problems found in many of our patients, general anestesia was usually used. The standard pull technique for percutaneous gastrostomy was used. The anterior abdominal wall was transilluminated, prepped and draped. A small incision was made and a trocar was passed into the cecum under direct vision. Unlike percutaneous gastrostomy placement, the passage of the trocar into the cecum was not always easy. Several passes were frequently required. A guide wire, passed through the trocar, was grasped with a snare and pulled out through the anus. The guide wire was tied to a 20 French pull type PEG tube, and pulled retrograde through the colon and out the anterior abdominal wall (Figs 1 and 2). All patients received prophylactic antibiotics (eg, metronidazole and gentamicin) for 24 hours. Most patients were discharged within 24 hours. The cecostomy tube was flushed with 20 ml of tap water twice a day for the first 7-14 days. 5,s Prior to ACE the tube was usually converted to a low profile device (Genie; Bard, Billerica, MA). As reported by Shandling et al 4 ACEs were then given using Fleet's Phospho-Soda (0.3-0.6 mL/kg) followed by tap water, saline or oral colonic lavage solution (0.5-1.5 L) daily or every other day. The regimen was individualized so that the patient would pass a stool within 1 hour and remain continent for the rest of the day. For convenience, most patients received the ACE in the evening
Results From the Division of Gastroenterology, Department of Pediatrics, The Medical College of Wisconsin; and Department of Pediatrics, The Children's Hospital of Wisconsin, Milwaukee, Wl. Address reprint requests to Steven L. Werlin, MD, Department of Pediatrics, The Medical College of Wisconsin, 8701 Watertown Plank Rd, Milwaukee, Wl 53226; e-mail: swerlin @mcw.edu. Copyright 2002, Elsevier Science (USA). All rights reserved. 1096-2883/02/0404-0009535.00/0 doi:l 0.1053/tgie.2002.37441
PEC was attempted in 25 patients (12 males ) with an age range of 17 months to 22 years. The procedure was successful in all but 3. The 3 failures were due to inability to find an acceptable window because of failure to transilluminate (2) and inability by 3 endoscopists to reach the cecum due to tortuosity of the colon (1). These patients were treated with surgical cecostomy (2) and radiologically placed cecostomy (1).
Techniques in Gastrointestinal Endoscopy, Vol 4, No 4 (October), 2002: pp 221-224
221
TABLE 1. Indication for Cecostomy Imperforate anus Spina bifida Cerebral palsy Asperger's Syndrome Nemaline myopathy Colonic psuedo-obstruction Neurofibromatosis with segmental colonic dysmotility Hirschsprung's disease and recurrent enterocolitis Cystic fibrosis
8 6 5 1 1 1 1 1 1
The duration of the procedure was typically 20 to 30 minutes (range, 15 to 90 minutes). The longer procedures were early in our experience. The total hospital stay averaged 2 days (range, 1-7 days). Early postoperative complications included urinary tract, infection (1), seizures (1), and fever with negative blood culture, abdominal distention and pain, with spontaneous resolution on the fifth day (1). The patient with repaired Hirschsprung's disease and recurrent enterocolitis developed enterocolitis 9 days after PEC placement, prior to use of his cecostomy tube. Severe abdominal distension led to pressure necrosis from the external bumper. He was successfully treated with irrigation, venting of the PEC tube and an oral antibiotic. One child died 9 months after PEC placement of his underlying disease. Development of granulation tissue around the stomas
was common and was treated with silver nitrate or triamcinolone cream. Six of our patients had ventriculoperitoneal shunts. None of these patients developed CSF infections after placement of the PEC. The only long-term complication occurred in a teenage girl with imperforate anus. During a 2-year period she gained 20 pounds but did not come in for follow-up visits nor show her stoma to her parents. Her stoma developed severe pressure necrosis and infection and required surgical treatment. All surviving patients are improved on their ACE regimen with a follow-up period of 5 months to 5 years. All patients and families were pleased with their cecostomy tubes and ACEs. For example, one 7-year-old boy with repaired imperforate anus, who had never achieved continence, was referred for evaluation for colectomy. After PEC placement and ACE, he has been continent for 4 years and no longer requires diapers.
Discussion Intractable constipation can be painful and difficult to manage.1 Encopresis is humiliating and devastating both to the individual and family. Traditional management consists of one or a combination of the following: diet modification, laxatives, enemas, suppositories and biofeedback. These therapies require patience and long term compliance, which can be very frustrating both to patients and families. In children with spina bifida
A
D
E
Fig 1. A) Colonoscopy is performed to the cecum and the anterior abdominal wall is transilluminated. 13) After prepping and draping the anterior abdominal wall, a 0.5 cm incision is made and a trocar is passed into the cecum. C) A guide wire is passed through the trocar. The guide wire is grasped with a snare passed through the biopsy channel of the colonoscope. The colonoscope is removed. D) The guide wire is tied to a 20 French pull type PEG tube and pulled retrograde through the colon and out the anterior abdominal wall. E) Properly placed percutaneous cecostomy. (Reprinted with permission, s)
222
STEVEN L. WERLIN
Fig 2. Internal portion of cecostomy tube within cecum after percutaeous cecostomy.
or anorectal anomalies lack of control of the anal sphincter often leads to incontinence. The ACE procedure, first described by Malone et aP others, 9 has been a very successful method for dealing with this problem. The Malone procedure, is performed by resecting the appendix with a cuff of cecum. The remaining cecum is then reversed and sutured to the skin. A modified Malone procedure is preferred by others. In this procedure, the appendix is left in situ and sutured to the skin. A circular flush stoma is created either at the appendiceal tip or the reversed cecum followed by an antireflux procedure. A 10 Fr catheter is left in this conduit for two weeks. Unfortunately, the Malone procedure is typically done at laparotomy. This procedure can be performed laparoscopically if the appendix is intact. Complications of surgically placed cecostomy or appendicostomy, which include stomal stenosis, stoma leakage, difficulty in intubating the stoma, and appendiceal necrosis may occur in up to 20% of patients. Shandling et al 4 simplified the delivery of ACE by inserting a percutaneous cecostomy under fluoroscopic guidance after a bowel clean out. A cecostomy is placed by the push technique also used for percutaneous gastrostomy placement. The catheter is left to drain, and flushed with 10 mL of saline twice daily. Most patients are discharged on the second hospital day. The use of the ACE is begun one week after placement. 4,5 The complications encountered are similar to those we reported.
PERCUTANEOUS ENDOSCOPIC CECOSTOMY
One patient had his cecostomy tube placed in the terminal ileum but it functioned well. We modified the technique of percutaneous placement of cecostomy based on the adult literature and inserted cecostomies at colonoscopy. 8 With regards to hospital stay and recovery time, our method is comparable to that employed by Shandling. Placement at cecostomy offers the advantage of direct visualization of the cecostomy site, which avoids inadvertent placement of the cecostomy in the terminal ileum or other undesirable sites. Having the cecostomy tube in place at all times prevents the complication of stomal stenosis and difficulty of intubating the stoma encountered in a surgically placed cecostomy. For aesthetic reasons, the PEC tube can be changed to a low profile gastrostomy device. Our patients and families have been very satisfied both with the procedure and the ease and efficacy of ACE. Teenage patients have become more independent. Prior to PEC, most of our patients were on complicated home bowel regimens, which at times required several hours. After PEC placement, the home bowel regimen is reduced to 45-60 minutes several times a week. ACE through PEC has dramatically changed the quality of life of these patients and their caregivers. Antegrade continence enemas have been shown to reduce fecal incontinence and constipation in children with spina bifida. Even more importantly improvement in self esteem and psychosocial function has been reported. 1~ Children with intractable constipation or fecal incontinence because of spina bifida, anorectal anomalies or severe neurodevelopmental problems should no longer be allowed to suffer needlessly. Antegrade continence enemas are recommended for selected patients with spina bifida, spinal cord injury, anorectal anomalies, and severe neurological handicaps. In children with Hirschsprung's disease and recurrent enterocolitis or colonic pseudo-obstruction, cecostomies can be used for irrigation and venting. In neurologically normal children with intractable constipation ACE can be an effective treatmetn. The ease of insertion and the widespead availability of pediatric gastroenterologists, the low complication rate and short recovery time make colonoscopic cecostomy the method of choice for the delivery of ACE in children. ACE through the use of PEC is not recommended for normal individuals with constipation who fail medical management because of non-compliance, since compliance is also required for a successful ACE program.
References 1. Liptak GS, Revell GM: Management of bowel dysfunction in children with spinal cord disease or injury by means of the enema continence catheter. J Pediatr 120:190-194, 1992 2. Shankar KF, Losty PD, Kenny SE, et al: Functional results following the antegrade continence enema procedure. Br J Surg 85:980-982, 1988 3. Malone PS, Ransley PG, Kiely EM: Preliminary report: The antegrade continence enema. Lancet 336:1217-1218, 1990 4. Shandling B, Chait PG, Richards HF: Percutaneous cecostomy: A new technique in the management of fecal incontinence. J Pediatr Surg 31:534-537, 1996 5. Chait PG, Shandling B, Richards M, et al: A fecal incontinence in children: Treatment with percutaneous cecostomy tube placement--a prospective study. Radiology 203:621-624, 1997 6. Youssef N, Barksdale Jr E, Griffiths J, et al: Management of intrac-
223
table constipation with antegrade enemas in neurologically intact children. J Pediatr Gastroenterol Nutr 34:402-405, 2002 7. Ponsky JL, Aszodi A, Perse D: Percutaneous endoscopic cecostomy: A new approach to nonobstructive colonic dilatation. Gastrointest Endosc 32:108-110, 1986 8. P,ivera M, Kugathasan S, Berger W, et al: Percutaneous colono-
224
scopic cecostomy for management of chronic constipation in children. Gastrointest Endosc 53:225-228, 2001 9. Webb H, Barraza M, Stevens P, et al: Bowel dysfunction in spina bifida--An american experience with the ace procedure. Eur J Pediatr Surg 8:37-38, 1998 (suppl) 10. Aksnes G, Diseth T, Helseth A, et at: Pediatrics 109:484-489, 2002
STEVEN L. WERLIN