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Post–serial transverse enteroplasty bowel redilatation treated by longitudinal intestinal lengthening and tailoring procedure
Journal of Pediatric Surgery (2012) 47, E19–E22
www.elsevier.com/locate/jpedsurg
Post–serial transverse enteroplasty bowel redilatation treated by longitudinal intestinal lengthening and tailoring procedure Fabio Fusaro a,⁎, Dominique Hermans b , Catherine Wanty b , Francis Veyckemans c , Jacques Pirenne d , Raymond Reding a a
Pediatric Intestinal Rehabilitation Unit, Department of Abdominal Surgery and Transplantation, St Luc University Hospital, Catholic University of Louvain (UCLouvain), 1200 Brussels, Belgium b Pediatric Intestinal Rehabilitation Unit, Department of Pediatrics, St Luc University Hospital, Catholic University of Louvain (UCLouvain), 1200 Brussels, Belgium c Pediatric Intestinal Rehabilitation Unit, Department of Anesthesiology, St Luc University Hospital, Catholic University of Louvain (UCLouvain), 1200 Brussels, Belgium d Department of Abdominal Transplant Surgery, University Hospitals Leuven, Catholic University Leuven (KULeuven), Campus Gasthuisberg Herestraat 4, 3000 Leuven, Belgium Received 2 March 2012; revised 3 April 2012; accepted 7 May 2012
Key words: Short bowel syndrome; Bowel lengthening procedures; Intestinal failure; Parenteral nutrition
Abstract The serial transverse enteroplasty (STEP) is a safe and successful procedure to lengthen the small bowel. Several patients develop postoperative bowel redilatation with loss of bowel adaptation. We describe a 2-month-old male infant with short bowel syndrome who developed dilatation of the lengthened segment after STEP, which was successfully treated by a longitudinal intestinal lengthening and tailoring procedure. To the best of our knowledge, this is the first described case of longitudinal intestinal lengthening and tailoring procedure after STEP. © 2012 Elsevier Inc. All rights reserved.
Serial transverse enteroplasty (STEP) is one of the most frequently used procedures to lengthen the small bowel in short bowel syndrome (SBS) [1]. At long-term follow-up, several patients present with postoperative bowel redilatation which induces stasis, bacterial overgrowth, malabsorption, dependence of parenteral nutrition (PN) with high risk of liver failure. We report a case of lengthened bowel redilatation
⁎ Corresponding author. Tel.: +32 27641401; fax: +32 27648954. E-mail addresses: [email protected], [email protected] (F. Fusaro). 0022-3468/$ – see front matter © 2012 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpedsurg.2012.05.023
after a first STEP successfully treated by a longitudinal intestinal lengthening and tailoring (LILT) procedure.
1. Case report A 2-month-old boy with SBS following surgical treatment of type IIIa proximal jejunal atresia with a V-shaped gap in the mesentery, absence of the ileum and ileocecal valve, and a residual complete microcolon was referred to our institution for persistent feeding intolerance with total
E20
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parenteral nutrition (TPN) dependence. Enteral nutrition was only possible up to 10% of the patient's caloric needs. He was born at 32 weeks of gestation with a birth weight of 1738 g. During his current admission, his weight and length were 2.622 g and 47.5 cm, respectively. The clinical examination showed, jaundice, and abdominal distension. An abdominal ultrasound (US) and an upper gastrointestinal contrast study showed a dilated, thickened hypoperistaltic small bowel and a patent jejunocolonic anastomosis and microcolon. A “hepatosparing” low-fat TPN regimen containing 1 g/kg per week of fat, 15 g/kg per day of glucose, and 1.4 g/kg per day of protein was started. At laparotomy, we confirmed the presence of a dilated and thickened residual jejunum, no obstructive mechanical causes or stenosis of the anastomotic site, and a microcolon. A STEP procedure was performed on the dilated jejunum in order to increase the length from 30 to 48 cm and to reduce the diameter from 4 to 1.5 cm. The stasis improved with consequent feeding tolerance; enteral nutrition had been advanced to 80 kcal/kg per day, with diminishing the PN. Six months after STEP procedure, the infant presented with progressive feeding intolerance, periodic bilious vomiting, weight loss, increased parenteral nutrition requirements, and a history of 3 episodes of sepsis related to bacterial translocations. An abdominal US and a contrast study showed marked redilatation of the lengthened bowel without evidence of an anastomotic stenosis. A surgical exploration confirmed that the lengthened intestine from the first STEP was markedly dilated, 6 cm of diameter, and thickened with loss of the “zigzag” configuration without signs of mechanical obstruction or anastomotic stenosis (Fig. 1). At this time, the residual nondilated small bowel and the dilated segment measured 60 and 26 cm, respectively. Considering the length and dilatation of the STEP segment, the absence of mechanical obstruction, and a normal-appearing mesentery, a LILT procedure was performed. After separation of the mesentery, the dilated segment was opened longitudinally (Fig. 2) and sutured in 2
Fig. 1 line.
Fig. 2
Longitudinal opening of the dilated STEP segment.
separate well vascularized loops that were anastomosed in an S-configuration with the proximal nondilated bowel and the residual colon (Fig. 3). This resulted in an increase of intestinal length from 86 to 112 cm. The postoperative course was uneventful. Enteral feeding was established on the fifth postoperative day, and TPN was then gradually reduced according to increased enteral tolerance. Two months after the operation, full enteral feeding was possible with normal liver function tests and approximately 6 to 8 stools per day. At 7 months of follow-up after LILT, the patient is in excellent clinical condition, and his z score for body weight and body mass index moved from −3.5 to −2.0 SDS and from −2.6 to −0.4 SDS, respectively (Figs. 4 and 5). An abdominal US showed nondilated bowel with peristalsis.
2. Discussion Patients with SBS develop bowel dilatation secondary to the intestinal adaptation process. Dysmotility associated with dilated bowel leads to stasis and bacterial overgrowth
Dilated STEP segment, arrow indicates the scars of staple Fig. 3
Anastomosis of the 2 hemiloops together and with the colon.
Post-STEP bowel redilatation treated by LILT procedure
Fig. 4
E21
Weight evolution; arrows indicate the time of the first STEP procedure and the second LILT procedure.
resulting in malabsorption, failure to thrive, bacterial translocation, and sepsis. Intestinal lengthening procedures (ILPs) aim to treat the bowel dilatation and promote adaptation, optimizing the volume-to-surface-area ratio of the intestine. They improve intestinal motility, enhance tolerance and absorption of nutrients, remove stasis and related bacterial overgrowth, reduce/eliminate parenteral nutrition dependence with improvement in general health, liver status, and growth. The 2 main types are the LILT and the STEP procedures described by Bianchi and Kim, respectively [1,2]. Despite good tolerance of both procedures and improved outcome, a number of related complications are observed [3-5]. A few reports focused on the problem related to redilatation of the operated segment [6,7]. Because one of the fundamental principles of these techniques is to reduce the calibre of the intestine to promote bowel motility and reduce stasis and consequent bacterial overgrowth, redilatation of the operated segment is associated with motility problems indicating a return to a dysfunctional status. Six months after the STEP procedure, our patient developed progressive feeding intolerance, episodes of bacterial overgrowth and translocations with the necessity to increase PN. Redilatation of the lengthened segment became evident. At the second laparotomy we confirmed the large redilatation of lengthened segment with loss of “zigzag” configuration, thick scars in the staple line sites and a persistent small calibre colon. We decided to perform a
second lengthening procedure to eliminate the dilatation and create a uniform lumen with normal intestinal diameter. Regarding the choice of the type of re-do operative technique, several reports describe a second STEP procedure as treatment for the redilated lengthened segment [8-10]. A recent report of Miyasaka et al analyzed the problem of the redilatation after ILPs. They concluded that the patients with redilated bowel after ILPs treated by a second STEP procedure have a worse clinical outcome: only 1 of the 8 patients analyzed were successfully weaned off PN [7]. Because of linear conformation of the redilated initially lengthened bowel, absence of mechanical causes of obstruction, and a small calibre colon, we speculate that a LILT procedure, in this case, could be more suitable because it is likely the motility in the narrowed segment is restored in a more linear direction to ameliorate the transit into the small calibre colon, reducing the risk of redilatation in the lengthened segment. Also if it is well demonstrated by Modi et al that after the STEP procedure the bowel motility is not impaired, we believed that it is possible in this patient, that the functional obstacles created by persistent small calibre colon had a negative impact on the "zigzag" postSTEP bowel configuration, creating redilatation [11]. The preservation of the mesentery during first STEP and the width of the small bowel allowed the creation of two fully vascularized isopropulsive hemiloops during LILT without technical problems. After the operation, intestinal tolerance
E22
F. Fusaro et al.
Fig. 5
Body mass index evolution; arrows indicate the time of the first STEP procedure and the second LILT procedure.
was quickly restored and the patient was weaned off parenteral nutrition. This case emphasizes once again as the bowel adaptation in short bowel syndrome is a dynamic process that requires a combination of medical management and surgical procedures all addressed to promote adaptation over the time. The use of a single procedure to treat this condition may be outdated. This case emphasizes that lengthened bowel redilatation should be considered as a potentially serious complication after ILPs. The attitude of “wait and see” may expose the patient to the risk of serious complications such as translocation, sepsis, and TPN-related complications, reducing the chances of intestinal adaptation. A reoperation should be done as soon as possible to quickly restore the bowel adaptation process and avoid life-threatening complications. This case suggests the LILT and STEP procedures should be considered complementary procedures and LILT can be performed safely after a STEP procedure to restore bowel function. To the best of our knowledge, this is the first described case of LILT procedure after STEP. ILPs are important surgical adjuncts as part of a multidisciplinary intestinal rehabilitation program for patients affected by SBS, and their sequential use increase the chances of intestinal adaptation.
References [1] Kim HB, Lee PW, Garza J, et al. Serial transverse enteroplasty for short bowel syndrome: a case report. J Pediatr Surg 2003;38:881-5. [2] Bianchi A. Intestinal loop lengthening—a technique for increasing small intestinal length. J Pediatr Surg 1980;15:145-51. [3] Sudan D, Thompson JS, Botha J, et al. Comparison of intestinal lengthening procedures for patients with short bowel syndrome. Ann Surg 2007;246:593-604. [4] Walker SR, Nucci A, Yaworski JA, et al. The Bianchi procedure: a 20 year single institution experience. J Pediatr Surg 2006;41:113-9. [5] Ching YA, Fitzgibbons S, Valim C, et al. Long-term nutritional and clinical outcomes after serial transverse enteroplasty at a single institution. J Pediatr Surg 2009;44:939-43. [6] Waag KL, Hosie S, Wessel L. What do children look like after longitudinal intestinal lengthening? Eur J Pediatr Surg 1999;9:260-2. [7] Miyasaka EA, Brown PI, Teitelbaum DH. Redilatation of bowel after intestinal lengthening procedures—an indicator for poor outcome. J Pediatr Surg 2011;46:145-9. [8] Ehrlich PF, Mychaliska GB, Tetelbaum DH. The 2 STEP: an approach to repeating a serial transverse enteroplasty. J Pediatr Surg 2007;42:819-22. [9] Piper H, Modi BP, Kim HB, et al. The second STEP: the feasibility of repeat serial transverse enteroplasty. J Pediatr Surg 2006;41:1951-6. [10] Andres AM, Thompson J, Grant W, et al. Repeat surgical bowel lengthening with the STEP procedure. Transplantation 2008;85:1294-9. [11] Modi BP, Ching YA, Langer M, et al. Preservation of intestinal motility after the serial transverse enteroplasty procedure in a large animal model of short bowel syndrome. J Pediatr Surg 2009;44:229-35.
www.elsevier.com/locate/jpedsurg
Post–serial transverse enteroplasty bowel redilatation treated by longitudinal intestinal lengthening and tailoring procedure Fabio Fusaro a,⁎, Dominique Hermans b , Catherine Wanty b , Francis Veyckemans c , Jacques Pirenne d , Raymond Reding a a
Pediatric Intestinal Rehabilitation Unit, Department of Abdominal Surgery and Transplantation, St Luc University Hospital, Catholic University of Louvain (UCLouvain), 1200 Brussels, Belgium b Pediatric Intestinal Rehabilitation Unit, Department of Pediatrics, St Luc University Hospital, Catholic University of Louvain (UCLouvain), 1200 Brussels, Belgium c Pediatric Intestinal Rehabilitation Unit, Department of Anesthesiology, St Luc University Hospital, Catholic University of Louvain (UCLouvain), 1200 Brussels, Belgium d Department of Abdominal Transplant Surgery, University Hospitals Leuven, Catholic University Leuven (KULeuven), Campus Gasthuisberg Herestraat 4, 3000 Leuven, Belgium Received 2 March 2012; revised 3 April 2012; accepted 7 May 2012
Key words: Short bowel syndrome; Bowel lengthening procedures; Intestinal failure; Parenteral nutrition
Abstract The serial transverse enteroplasty (STEP) is a safe and successful procedure to lengthen the small bowel. Several patients develop postoperative bowel redilatation with loss of bowel adaptation. We describe a 2-month-old male infant with short bowel syndrome who developed dilatation of the lengthened segment after STEP, which was successfully treated by a longitudinal intestinal lengthening and tailoring procedure. To the best of our knowledge, this is the first described case of longitudinal intestinal lengthening and tailoring procedure after STEP. © 2012 Elsevier Inc. All rights reserved.
Serial transverse enteroplasty (STEP) is one of the most frequently used procedures to lengthen the small bowel in short bowel syndrome (SBS) [1]. At long-term follow-up, several patients present with postoperative bowel redilatation which induces stasis, bacterial overgrowth, malabsorption, dependence of parenteral nutrition (PN) with high risk of liver failure. We report a case of lengthened bowel redilatation
⁎ Corresponding author. Tel.: +32 27641401; fax: +32 27648954. E-mail addresses: [email protected], [email protected] (F. Fusaro). 0022-3468/$ – see front matter © 2012 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpedsurg.2012.05.023
after a first STEP successfully treated by a longitudinal intestinal lengthening and tailoring (LILT) procedure.
1. Case report A 2-month-old boy with SBS following surgical treatment of type IIIa proximal jejunal atresia with a V-shaped gap in the mesentery, absence of the ileum and ileocecal valve, and a residual complete microcolon was referred to our institution for persistent feeding intolerance with total
E20
F. Fusaro et al.
parenteral nutrition (TPN) dependence. Enteral nutrition was only possible up to 10% of the patient's caloric needs. He was born at 32 weeks of gestation with a birth weight of 1738 g. During his current admission, his weight and length were 2.622 g and 47.5 cm, respectively. The clinical examination showed, jaundice, and abdominal distension. An abdominal ultrasound (US) and an upper gastrointestinal contrast study showed a dilated, thickened hypoperistaltic small bowel and a patent jejunocolonic anastomosis and microcolon. A “hepatosparing” low-fat TPN regimen containing 1 g/kg per week of fat, 15 g/kg per day of glucose, and 1.4 g/kg per day of protein was started. At laparotomy, we confirmed the presence of a dilated and thickened residual jejunum, no obstructive mechanical causes or stenosis of the anastomotic site, and a microcolon. A STEP procedure was performed on the dilated jejunum in order to increase the length from 30 to 48 cm and to reduce the diameter from 4 to 1.5 cm. The stasis improved with consequent feeding tolerance; enteral nutrition had been advanced to 80 kcal/kg per day, with diminishing the PN. Six months after STEP procedure, the infant presented with progressive feeding intolerance, periodic bilious vomiting, weight loss, increased parenteral nutrition requirements, and a history of 3 episodes of sepsis related to bacterial translocations. An abdominal US and a contrast study showed marked redilatation of the lengthened bowel without evidence of an anastomotic stenosis. A surgical exploration confirmed that the lengthened intestine from the first STEP was markedly dilated, 6 cm of diameter, and thickened with loss of the “zigzag” configuration without signs of mechanical obstruction or anastomotic stenosis (Fig. 1). At this time, the residual nondilated small bowel and the dilated segment measured 60 and 26 cm, respectively. Considering the length and dilatation of the STEP segment, the absence of mechanical obstruction, and a normal-appearing mesentery, a LILT procedure was performed. After separation of the mesentery, the dilated segment was opened longitudinally (Fig. 2) and sutured in 2
Fig. 1 line.
Fig. 2
Longitudinal opening of the dilated STEP segment.
separate well vascularized loops that were anastomosed in an S-configuration with the proximal nondilated bowel and the residual colon (Fig. 3). This resulted in an increase of intestinal length from 86 to 112 cm. The postoperative course was uneventful. Enteral feeding was established on the fifth postoperative day, and TPN was then gradually reduced according to increased enteral tolerance. Two months after the operation, full enteral feeding was possible with normal liver function tests and approximately 6 to 8 stools per day. At 7 months of follow-up after LILT, the patient is in excellent clinical condition, and his z score for body weight and body mass index moved from −3.5 to −2.0 SDS and from −2.6 to −0.4 SDS, respectively (Figs. 4 and 5). An abdominal US showed nondilated bowel with peristalsis.
2. Discussion Patients with SBS develop bowel dilatation secondary to the intestinal adaptation process. Dysmotility associated with dilated bowel leads to stasis and bacterial overgrowth
Dilated STEP segment, arrow indicates the scars of staple Fig. 3
Anastomosis of the 2 hemiloops together and with the colon.
Post-STEP bowel redilatation treated by LILT procedure
Fig. 4
E21
Weight evolution; arrows indicate the time of the first STEP procedure and the second LILT procedure.
resulting in malabsorption, failure to thrive, bacterial translocation, and sepsis. Intestinal lengthening procedures (ILPs) aim to treat the bowel dilatation and promote adaptation, optimizing the volume-to-surface-area ratio of the intestine. They improve intestinal motility, enhance tolerance and absorption of nutrients, remove stasis and related bacterial overgrowth, reduce/eliminate parenteral nutrition dependence with improvement in general health, liver status, and growth. The 2 main types are the LILT and the STEP procedures described by Bianchi and Kim, respectively [1,2]. Despite good tolerance of both procedures and improved outcome, a number of related complications are observed [3-5]. A few reports focused on the problem related to redilatation of the operated segment [6,7]. Because one of the fundamental principles of these techniques is to reduce the calibre of the intestine to promote bowel motility and reduce stasis and consequent bacterial overgrowth, redilatation of the operated segment is associated with motility problems indicating a return to a dysfunctional status. Six months after the STEP procedure, our patient developed progressive feeding intolerance, episodes of bacterial overgrowth and translocations with the necessity to increase PN. Redilatation of the lengthened segment became evident. At the second laparotomy we confirmed the large redilatation of lengthened segment with loss of “zigzag” configuration, thick scars in the staple line sites and a persistent small calibre colon. We decided to perform a
second lengthening procedure to eliminate the dilatation and create a uniform lumen with normal intestinal diameter. Regarding the choice of the type of re-do operative technique, several reports describe a second STEP procedure as treatment for the redilated lengthened segment [8-10]. A recent report of Miyasaka et al analyzed the problem of the redilatation after ILPs. They concluded that the patients with redilated bowel after ILPs treated by a second STEP procedure have a worse clinical outcome: only 1 of the 8 patients analyzed were successfully weaned off PN [7]. Because of linear conformation of the redilated initially lengthened bowel, absence of mechanical causes of obstruction, and a small calibre colon, we speculate that a LILT procedure, in this case, could be more suitable because it is likely the motility in the narrowed segment is restored in a more linear direction to ameliorate the transit into the small calibre colon, reducing the risk of redilatation in the lengthened segment. Also if it is well demonstrated by Modi et al that after the STEP procedure the bowel motility is not impaired, we believed that it is possible in this patient, that the functional obstacles created by persistent small calibre colon had a negative impact on the "zigzag" postSTEP bowel configuration, creating redilatation [11]. The preservation of the mesentery during first STEP and the width of the small bowel allowed the creation of two fully vascularized isopropulsive hemiloops during LILT without technical problems. After the operation, intestinal tolerance
E22
F. Fusaro et al.
Fig. 5
Body mass index evolution; arrows indicate the time of the first STEP procedure and the second LILT procedure.
was quickly restored and the patient was weaned off parenteral nutrition. This case emphasizes once again as the bowel adaptation in short bowel syndrome is a dynamic process that requires a combination of medical management and surgical procedures all addressed to promote adaptation over the time. The use of a single procedure to treat this condition may be outdated. This case emphasizes that lengthened bowel redilatation should be considered as a potentially serious complication after ILPs. The attitude of “wait and see” may expose the patient to the risk of serious complications such as translocation, sepsis, and TPN-related complications, reducing the chances of intestinal adaptation. A reoperation should be done as soon as possible to quickly restore the bowel adaptation process and avoid life-threatening complications. This case suggests the LILT and STEP procedures should be considered complementary procedures and LILT can be performed safely after a STEP procedure to restore bowel function. To the best of our knowledge, this is the first described case of LILT procedure after STEP. ILPs are important surgical adjuncts as part of a multidisciplinary intestinal rehabilitation program for patients affected by SBS, and their sequential use increase the chances of intestinal adaptation.
References [1] Kim HB, Lee PW, Garza J, et al. Serial transverse enteroplasty for short bowel syndrome: a case report. J Pediatr Surg 2003;38:881-5. [2] Bianchi A. Intestinal loop lengthening—a technique for increasing small intestinal length. J Pediatr Surg 1980;15:145-51. [3] Sudan D, Thompson JS, Botha J, et al. Comparison of intestinal lengthening procedures for patients with short bowel syndrome. Ann Surg 2007;246:593-604. [4] Walker SR, Nucci A, Yaworski JA, et al. The Bianchi procedure: a 20 year single institution experience. J Pediatr Surg 2006;41:113-9. [5] Ching YA, Fitzgibbons S, Valim C, et al. Long-term nutritional and clinical outcomes after serial transverse enteroplasty at a single institution. J Pediatr Surg 2009;44:939-43. [6] Waag KL, Hosie S, Wessel L. What do children look like after longitudinal intestinal lengthening? Eur J Pediatr Surg 1999;9:260-2. [7] Miyasaka EA, Brown PI, Teitelbaum DH. Redilatation of bowel after intestinal lengthening procedures—an indicator for poor outcome. J Pediatr Surg 2011;46:145-9. [8] Ehrlich PF, Mychaliska GB, Tetelbaum DH. The 2 STEP: an approach to repeating a serial transverse enteroplasty. J Pediatr Surg 2007;42:819-22. [9] Piper H, Modi BP, Kim HB, et al. The second STEP: the feasibility of repeat serial transverse enteroplasty. J Pediatr Surg 2006;41:1951-6. [10] Andres AM, Thompson J, Grant W, et al. Repeat surgical bowel lengthening with the STEP procedure. Transplantation 2008;85:1294-9. [11] Modi BP, Ching YA, Langer M, et al. Preservation of intestinal motility after the serial transverse enteroplasty procedure in a large animal model of short bowel syndrome. J Pediatr Surg 2009;44:229-35.