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Combined living-related segmental liver and bowel transplantation for megacystis-microcolon-intestinal hypoperistalsis syndrome
Journal of Pediatric Surgery (2008) 43, E9–E11
www.elsevier.com/locate/jpedsurg
Combined living-related segmental liver and bowel transplantation for megacystis-microcolon-intestinal hypoperistalsis syndrome Vandad Raofi a,⁎, Elizabeth Beatty a , Giuliana Testa a , Herand Abcarian b , Jose Oberholzer a , Howard Sankary a , Mark Grevious c , Enrico Benedetti a a
Division of Transplant Surgery, Department of Surgery (M/C 958), University of Illinois College of Medicine, Chicago, IL 60612, USA b Division of Colorectal Surgery, Department of Surgery (M/C 958), University of Illinois College of Medicine, Chicago, IL 60612, USA c Division of Plastic Surgery, Department of Surgery (M/C 958), University of Illinois College of Medicine, Chicago, IL 60612, USA Received 18 July 2007; accepted 16 September 2007
Index words: Pediatric; Intestinal pseudoobstruction; Living donor; Combined liver and intestinal transplant
Abstract Background: Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) is the most severe form of functional intestinal obstruction in the newborn. To date, multivisceral transplantation has been the only accepted treatment modality for these patients, and the results have met with marginal success. We report the first case of a patient affected by MMIHS and cholestatic liver failure treated by a combined living-related liver and intestinal transplant (CLRLITx). Case Report: The patient was a 1-year-old Hispanic girl born with MMIHS and maintained on total parenteral nutrition since birth. Once liver failure developed, she was referred for evaluation for possible CLRLITx. The patient's mother volunteered as the donor. The left lateral segment was used for the liver transplant. The intestinal graft consisted of the terminal 180 cm of the ileum with a single vascular pedicle. Initially, the patient continued to have severe gastroparesis; however, by 8 months posttransplant, stomach function had returned to normal. Currently, at 2 years posttransplant, she is tolerating an oral diet with gastric tube supplementation. Results of absorption studies are within normal, and she has shown catch-up growth. Conclusion: A CLRLITx can be a viable alternative for infants diagnosed with MMIHS. This procedure can help avoid the 25% wait-list mortality for children who are in need of a combined transplant. © 2008 Published by Elsevier Inc.
Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS), first described by Berdon and coworkers [1], is a severe and often lethal form of neonatal ⁎ Corresponding author. Tel.: +1 312 996 6771; fax: +1 312 413 3483. E-mail address: [email protected] (V. Raofi). 0022-3468/$ – see front matter © 2008 Published by Elsevier Inc. doi:10.1016/j.jpedsurg.2007.09.073
pseudoobstruction. It is characterized by hypoperistalsis or aperistalsis of the gastrointestinal (GI) tract, malrotation, microcolon, and genitourinary abnormalities—namely, a nonobstructed dilated bladder [2]. The prognosis is extremely poor, with most patients dying within the first 6 months without treatment [3]. Use of prokinetic agents, hormonal
E10 treatment, and surgical manipulation are usually futile [4], and to date, multivisceral transplantation has been generally accepted as the lone long-term option for those who cannot tolerate total parenteral nutrition (TPN) [5]. However, Gillis and Grantmyre [6] reported a case of a male infant whose GI function gradually improved and who was weaned off intravenous nutrition by 8 months of age. Furthermore, in a University of Nebraska study of pediatric bowel transplant recipients for intestinal pseudoobstruction, where the use of stomach and colon were excluded, only 1 of 5 long-term survivors had ongoing gastroparesis [7]. To this effect, we report the first case of a combined living-related liver and intestinal transplantation (CLRLITx) in a newborn with a diagnosis of MMIHS.
1. Case report The patient has been affected by GI dysmotility and inability to tolerate oral feedings since birth and had to be maintained on TPN since that time. She underwent several abdominal explorations and bowel resections, all with no success. The patient was eventually diagnosed with MMIHS. Near the end of her first year, she began developing TPNassociated liver disease, and by her first birthday, she had biopsy-proven grade IV fibrosis of the liver. At that time, she was referred for evaluation for CLRLITx at our institution. Her mother volunteered as the donor. A standard left lateral hepatectomy and resection of a 180-cm segment of terminal ileum with a single vascular pedicle consisting of the terminal branch of the superior mesenteric artery and vein were performed. The detailed surgical technique for this procedure has been previously described [8,9]. The patient initially underwent a total enterectomy up to the third portion of the duodenum. Subsequently, the hepatectomy and liver transplant were performed without anastomosing the bile duct at this time. After the liver was revascularized, the segmental intestinal transplantation was performed. The donor artery was anastomosed to the infrarenal aorta, followed by venous anastomosis of the vein to the inferior vena cava. After reperfusion of the intestinal graft, an 8-cm proximal segment of the bowel was left to create the bile duct anastomosis. Distal to this segment, bowel continuity was established by a doublelayer hand-sewn duodenoileostomy. Because preoperative studies had shown stomach dysmotility, a posterior gastrojejunostomy was also created using the same 2-layered technique. A feeding jejunostomy was passed through the anastomosis and into the transplanted small bowel, and a gastrostomy tube was left in place to decompress the stomach. An end ileostomy was then performed. The native distal microcolon was left undisturbed. Finally, a hepaticojejunostomy was performed between the donor bile duct and the proximal portion of the transplanted bowel. The abdomen was then closed with Vicryl mesh. The patient eventually underwent skin grafting over this mesh once sufficient granulation tissue had developed.
V. Raofi et al. Immunosuppression included induction with Thymoglobulin 3 mg/kg the night before surgery and then 2 mg/kg on postoperative day 1. The patient also underwent a rapid prednisone taper. This consisted of methylprednisone 1 mg/kg on the first postoperative day, followed by 0.5 mg/kg on days 2 and 3, and finally, 0.25 mg/kg on days 4 and 5. All steroids were discontinued by postoperative day 6. Maintenance immunosuppression consisted of mycophenolate mofetil and tacrolimus. Mycophenolate mofetil was started at 25 mg/m2 given 4 times daily and tapered off by 2 months because the patient had no episodes of acute rejection. Tacrolimus dose was adjusted to keep the trough levels at 15 to 20 ng/mL for the first month and then gradually weaned down to a level of 8 to 12 ng/mL thereafter. The patient is currently on Prograf monotherapy only. Gastrointestinal motility studies, including gastric emptying scans performed before transplant, showed markedly abnormal and delayed gastric emptying. This gastroparesis persisted postoperatively for several months. However, gastric motility gradually improved, and by the seventh posttransplant month, stomach function had returned to normal. The patient was gradually weaned off of jejunostomy feedings, and the jejunostomy tube was removed. Today, the patient is fully sustained with oral feedings and gastric tube supplementation, and the results of her absorption studies are within normal limits. At the time of transplant, the patient was in less than the fifth percentile in both height and weight. By 2 years posttransplant, she has shown not only linear growth but actually catch-up growth. She is currently in the 25th to 50th percentile for weight and 50th to 75th percentile for height.
2. Discussion Gastrointestinal dysmotility syndrome encompasses a group of disorders characterized by failure of the GI tract to produce normal peristalsis. Overall, patients with motility disorders comprise about 10% of bowel transplants performed as of May 2003 [10], and this is also reflected in a retrospective review of all pediatric intestinal transplants at the University of Miami [5]. Of these disorders, MMIHS is the most severe form and includes urinary abnormalities in addition to the GI dysmotility. In all cases, mechanical obstruction, Hirschsprung disease, and secondary aperistalsis syndromes such as muscular dystrophy and Chagas disease have been excluded. On histologic examination, most patients have normal ganglion cells in the myenteric and submucosal plexuses; however, there have case reports of decreased ganglia in some and hyperganglionosis and giant ganglia in others [4]. Other hypothesis for MMIHS have included disturbances in myocellular contractile fiber synthesis [11], imbalance between gut peptides [12], intestinal myopathy [13], neuroaxonal dystrophy [14], and defective autonomic inhibitory neuroeffector activity [15].
Combined living-related segmental liver and bowel transplantation In their case presentation, in which they described a child with MMIHS who was gradually weaned off of TPN, Gillis and Grantmyre [6] suggested that the primary defect may be a reflection of neuromuscular immaturity, which in certain instances may correct over time. Still, despite the various hypotheses, the exact etiology remains unknown. What is known, though, is that most of these patients undergo surgical exploration and various trials of prokinetic drug therapy with no success. By February 2005, a total of 182 cases of MMIHS had been reported in the literature. Of these, only 23 were still alive, and more than 90% (21/23) had to be maintained by total or partial parenteral nutrition [4]. Over time, it has become clear that with few exceptions, the long-term survival of these patients depends on TPN, and those who develop complications from prolonged intravenous nutrition require intestinal or multivisceral (MVI) transplantation. Depending on the transplant center's preference, these procedures have either included the stomach and colon or have excluded them from the transplant. Although results are improving with increased experience and better immunosuppression regimens, MVI transplant outcomes are still far from satisfactory, with only 50% patient survival being reported at 3 years posttransplant [5,7,16]. This is further compounded by the unacceptably high mortality of children awaiting combined liver and bowel or MVI transplants. This pretransplant mortality has approached 25% and is even higher for smaller children, specifically those younger than 1 year [17-19]. There is concern that children with MMIHS or dysmotility syndromes will require an MVI transplant including the stomach because of poor function of the native stomach and upper GI tract [2,20]. However, in the study by Iyer et al [7], a total of 8 patients with the diagnosis of intestinal pseudoobstruction underwent isolated small bowel or combined small bowel –liver transplantation without the inclusion of the stomach. Of the 5 long-term survivors, only 1 needs daily venting of the stomach. A second patient has poor gastric emptying but does vomit or need gastric decompression. She maintains her diet mainly on oral intake, with occasional jejunal tube feeding supplements. The final 3 patients are fully maintained on an oral diet. This report, along with that of Gillis and Grantmyre [6], may indicate that an MVI transplant may not be required for patients with MMIHS who develop complications from TPN. To that effect, herein, we have reported the first case of a CLRLITx in a 1-year-old infant with TPN-induced liver failure and an underlying diagnosis of MMIHS. We believe that there are several potential advantages of a living-related donor transplant. First and foremost, the high wait-list mortality associated with a combined liver and intestinal transplants in children can be avoided. Secondly, based on the intestinal transplant registry, patients with isolated small bowel transplants have better overall outcomes compared with combined or MVI grafts. Finally, these children will
E11
need to be on lifelong immunosuppression, and better HLA matching may reduce their overall risk of rejection episodes and need for heavy immunosuppression.
References [1] Berdon WE, Baker DH, Blanc WA, et al. Megacystis microcolon intestinal hypoperistalsis syndrome: a new cause of intestinal obstruction in the newborn. Report of radiologic findings in five newborn girls. AJR Am J Roentgenol 1976;126:957-64. [2] Masetti M, Rodriguez M, Thompson J, et al. Multivisceral transplantation for megacystis microcolon intestinal hypoperistalsis syndrome. Transplantation 1999;68(2):228-32. [3] Anneren G, Meurling S, Olsen L. Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS), an autosomal recessive disorder: clinical reports and review of the literature. Am J Med Genet 1991;41: 251-4. [4] Puri P, Shinkai M. Megacystis microcolon intestinal hypoperistalsis syndrome. Semin Pediatr Surg 2005;14:58-63. [5] Loinaz C, Rodriguez M, Kato T, et al. Intestinal and multivisceral transplantation in children with severe gastrointestinal dysmotility. J Pediatr Surg 2005;40:1598-604. [6] Gillis DA, Grantmyre EB. Megacystis microcolon intestinal hypoperistalsis syndrome: survival of a male infant. J Pediatr Surg 1985;20: 279-81. [7] Iyer K, Kaufman S, Sudan D, et al. Long-term results of intestinal transplantation for pseudo-obstruction in children. J Pediatr Surg 2001;36(1):174-7. [8] Testa G, Panaro F, Schena S, et al. Living related small bowel transplantation—donor surgical technique. Ann Surg 2004;240(5): 779-84. [9] Testa G, Holterman M, John E, et al. Combined living donor liver/ small bowel transplantation. Transplantation 2005;79(10):1401-4. [10] www.intestinaltransplant.org. [11] Ciftci A, Cook R, van Velzen D. Megacystis microcolon intestinal hypoperistalsis syndrome: evidence of a primary myocellular defect of contractile fiber synthesis. J Pediatr Surg 1996;31(12):1706-11. [12] Taguchi T, Ikeda K, Shono T, et al. Autonomic innervation of the intestine from a baby with megacystis microcolon intestinal hypoperistalsis syndrome: immunohistochemical study. J Pediatr Surg 1989;24(12):1264-6. [13] Rolle U, O'Briain S, Pearl R, et al. Megacystis-microcolon-intestinal hypoperistalsis syndrome: evidence of intestinal myopathy. Pediatr Surg Int 2002;18:2-5. [14] Al Rayess M, Ambler M. Axonal dystrophy presenting as the megacystis microcolon-intestinal hypoperistalsis syndrome. Pediatr Pathol 1992;12:743-50. [15] Kubota M, Ikeda K, Ito Y. Autonomic innervation of the intestine from a baby with megacystis microcolon intestinal hypoperistalsis syndrome: electrophysiological study. J Pediatr Surg 1989;24(12):1267-70. [16] Nathan J, Rudolph J, Kocoshis S, et al. Isolated liver and multivisceral transplantation for total parenteral nutrition–related end-stage liver disease. J Pediatr Surg 2007;42:143-7. [17] Horslen S. Organ allocation for liver-intestine candidates. Liver Transpl 2004;10(10 Suppl 2):S86-9. [18] Fryer J, Pellar S, Ormond D, et al. Mortality in candidates waiting for combined liver-transplants exceeds that for other candidates waiting for liver transplants. Liver Transpl 2003;9:748-53. [19] Sokal E, Cleghorn G, Goulet O, et al. Liver and intestinal transplantation in children: Working Group Report of the First World Congress of Pediatric Gastroenterology, Hepatology, and Nutrition. J Pediatr Gastroenterol Nutr 2002;35(Supple):S159-72. [20] Sigurdsson L, Reyes J, Kocoshis S, et al. Intestinal transplantation in children with chronic intestinal pseudo-obstruction. Gut 1999;45: 570-4.
www.elsevier.com/locate/jpedsurg
Combined living-related segmental liver and bowel transplantation for megacystis-microcolon-intestinal hypoperistalsis syndrome Vandad Raofi a,⁎, Elizabeth Beatty a , Giuliana Testa a , Herand Abcarian b , Jose Oberholzer a , Howard Sankary a , Mark Grevious c , Enrico Benedetti a a
Division of Transplant Surgery, Department of Surgery (M/C 958), University of Illinois College of Medicine, Chicago, IL 60612, USA b Division of Colorectal Surgery, Department of Surgery (M/C 958), University of Illinois College of Medicine, Chicago, IL 60612, USA c Division of Plastic Surgery, Department of Surgery (M/C 958), University of Illinois College of Medicine, Chicago, IL 60612, USA Received 18 July 2007; accepted 16 September 2007
Index words: Pediatric; Intestinal pseudoobstruction; Living donor; Combined liver and intestinal transplant
Abstract Background: Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) is the most severe form of functional intestinal obstruction in the newborn. To date, multivisceral transplantation has been the only accepted treatment modality for these patients, and the results have met with marginal success. We report the first case of a patient affected by MMIHS and cholestatic liver failure treated by a combined living-related liver and intestinal transplant (CLRLITx). Case Report: The patient was a 1-year-old Hispanic girl born with MMIHS and maintained on total parenteral nutrition since birth. Once liver failure developed, she was referred for evaluation for possible CLRLITx. The patient's mother volunteered as the donor. The left lateral segment was used for the liver transplant. The intestinal graft consisted of the terminal 180 cm of the ileum with a single vascular pedicle. Initially, the patient continued to have severe gastroparesis; however, by 8 months posttransplant, stomach function had returned to normal. Currently, at 2 years posttransplant, she is tolerating an oral diet with gastric tube supplementation. Results of absorption studies are within normal, and she has shown catch-up growth. Conclusion: A CLRLITx can be a viable alternative for infants diagnosed with MMIHS. This procedure can help avoid the 25% wait-list mortality for children who are in need of a combined transplant. © 2008 Published by Elsevier Inc.
Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS), first described by Berdon and coworkers [1], is a severe and often lethal form of neonatal ⁎ Corresponding author. Tel.: +1 312 996 6771; fax: +1 312 413 3483. E-mail address: [email protected] (V. Raofi). 0022-3468/$ – see front matter © 2008 Published by Elsevier Inc. doi:10.1016/j.jpedsurg.2007.09.073
pseudoobstruction. It is characterized by hypoperistalsis or aperistalsis of the gastrointestinal (GI) tract, malrotation, microcolon, and genitourinary abnormalities—namely, a nonobstructed dilated bladder [2]. The prognosis is extremely poor, with most patients dying within the first 6 months without treatment [3]. Use of prokinetic agents, hormonal
E10 treatment, and surgical manipulation are usually futile [4], and to date, multivisceral transplantation has been generally accepted as the lone long-term option for those who cannot tolerate total parenteral nutrition (TPN) [5]. However, Gillis and Grantmyre [6] reported a case of a male infant whose GI function gradually improved and who was weaned off intravenous nutrition by 8 months of age. Furthermore, in a University of Nebraska study of pediatric bowel transplant recipients for intestinal pseudoobstruction, where the use of stomach and colon were excluded, only 1 of 5 long-term survivors had ongoing gastroparesis [7]. To this effect, we report the first case of a combined living-related liver and intestinal transplantation (CLRLITx) in a newborn with a diagnosis of MMIHS.
1. Case report The patient has been affected by GI dysmotility and inability to tolerate oral feedings since birth and had to be maintained on TPN since that time. She underwent several abdominal explorations and bowel resections, all with no success. The patient was eventually diagnosed with MMIHS. Near the end of her first year, she began developing TPNassociated liver disease, and by her first birthday, she had biopsy-proven grade IV fibrosis of the liver. At that time, she was referred for evaluation for CLRLITx at our institution. Her mother volunteered as the donor. A standard left lateral hepatectomy and resection of a 180-cm segment of terminal ileum with a single vascular pedicle consisting of the terminal branch of the superior mesenteric artery and vein were performed. The detailed surgical technique for this procedure has been previously described [8,9]. The patient initially underwent a total enterectomy up to the third portion of the duodenum. Subsequently, the hepatectomy and liver transplant were performed without anastomosing the bile duct at this time. After the liver was revascularized, the segmental intestinal transplantation was performed. The donor artery was anastomosed to the infrarenal aorta, followed by venous anastomosis of the vein to the inferior vena cava. After reperfusion of the intestinal graft, an 8-cm proximal segment of the bowel was left to create the bile duct anastomosis. Distal to this segment, bowel continuity was established by a doublelayer hand-sewn duodenoileostomy. Because preoperative studies had shown stomach dysmotility, a posterior gastrojejunostomy was also created using the same 2-layered technique. A feeding jejunostomy was passed through the anastomosis and into the transplanted small bowel, and a gastrostomy tube was left in place to decompress the stomach. An end ileostomy was then performed. The native distal microcolon was left undisturbed. Finally, a hepaticojejunostomy was performed between the donor bile duct and the proximal portion of the transplanted bowel. The abdomen was then closed with Vicryl mesh. The patient eventually underwent skin grafting over this mesh once sufficient granulation tissue had developed.
V. Raofi et al. Immunosuppression included induction with Thymoglobulin 3 mg/kg the night before surgery and then 2 mg/kg on postoperative day 1. The patient also underwent a rapid prednisone taper. This consisted of methylprednisone 1 mg/kg on the first postoperative day, followed by 0.5 mg/kg on days 2 and 3, and finally, 0.25 mg/kg on days 4 and 5. All steroids were discontinued by postoperative day 6. Maintenance immunosuppression consisted of mycophenolate mofetil and tacrolimus. Mycophenolate mofetil was started at 25 mg/m2 given 4 times daily and tapered off by 2 months because the patient had no episodes of acute rejection. Tacrolimus dose was adjusted to keep the trough levels at 15 to 20 ng/mL for the first month and then gradually weaned down to a level of 8 to 12 ng/mL thereafter. The patient is currently on Prograf monotherapy only. Gastrointestinal motility studies, including gastric emptying scans performed before transplant, showed markedly abnormal and delayed gastric emptying. This gastroparesis persisted postoperatively for several months. However, gastric motility gradually improved, and by the seventh posttransplant month, stomach function had returned to normal. The patient was gradually weaned off of jejunostomy feedings, and the jejunostomy tube was removed. Today, the patient is fully sustained with oral feedings and gastric tube supplementation, and the results of her absorption studies are within normal limits. At the time of transplant, the patient was in less than the fifth percentile in both height and weight. By 2 years posttransplant, she has shown not only linear growth but actually catch-up growth. She is currently in the 25th to 50th percentile for weight and 50th to 75th percentile for height.
2. Discussion Gastrointestinal dysmotility syndrome encompasses a group of disorders characterized by failure of the GI tract to produce normal peristalsis. Overall, patients with motility disorders comprise about 10% of bowel transplants performed as of May 2003 [10], and this is also reflected in a retrospective review of all pediatric intestinal transplants at the University of Miami [5]. Of these disorders, MMIHS is the most severe form and includes urinary abnormalities in addition to the GI dysmotility. In all cases, mechanical obstruction, Hirschsprung disease, and secondary aperistalsis syndromes such as muscular dystrophy and Chagas disease have been excluded. On histologic examination, most patients have normal ganglion cells in the myenteric and submucosal plexuses; however, there have case reports of decreased ganglia in some and hyperganglionosis and giant ganglia in others [4]. Other hypothesis for MMIHS have included disturbances in myocellular contractile fiber synthesis [11], imbalance between gut peptides [12], intestinal myopathy [13], neuroaxonal dystrophy [14], and defective autonomic inhibitory neuroeffector activity [15].
Combined living-related segmental liver and bowel transplantation In their case presentation, in which they described a child with MMIHS who was gradually weaned off of TPN, Gillis and Grantmyre [6] suggested that the primary defect may be a reflection of neuromuscular immaturity, which in certain instances may correct over time. Still, despite the various hypotheses, the exact etiology remains unknown. What is known, though, is that most of these patients undergo surgical exploration and various trials of prokinetic drug therapy with no success. By February 2005, a total of 182 cases of MMIHS had been reported in the literature. Of these, only 23 were still alive, and more than 90% (21/23) had to be maintained by total or partial parenteral nutrition [4]. Over time, it has become clear that with few exceptions, the long-term survival of these patients depends on TPN, and those who develop complications from prolonged intravenous nutrition require intestinal or multivisceral (MVI) transplantation. Depending on the transplant center's preference, these procedures have either included the stomach and colon or have excluded them from the transplant. Although results are improving with increased experience and better immunosuppression regimens, MVI transplant outcomes are still far from satisfactory, with only 50% patient survival being reported at 3 years posttransplant [5,7,16]. This is further compounded by the unacceptably high mortality of children awaiting combined liver and bowel or MVI transplants. This pretransplant mortality has approached 25% and is even higher for smaller children, specifically those younger than 1 year [17-19]. There is concern that children with MMIHS or dysmotility syndromes will require an MVI transplant including the stomach because of poor function of the native stomach and upper GI tract [2,20]. However, in the study by Iyer et al [7], a total of 8 patients with the diagnosis of intestinal pseudoobstruction underwent isolated small bowel or combined small bowel –liver transplantation without the inclusion of the stomach. Of the 5 long-term survivors, only 1 needs daily venting of the stomach. A second patient has poor gastric emptying but does vomit or need gastric decompression. She maintains her diet mainly on oral intake, with occasional jejunal tube feeding supplements. The final 3 patients are fully maintained on an oral diet. This report, along with that of Gillis and Grantmyre [6], may indicate that an MVI transplant may not be required for patients with MMIHS who develop complications from TPN. To that effect, herein, we have reported the first case of a CLRLITx in a 1-year-old infant with TPN-induced liver failure and an underlying diagnosis of MMIHS. We believe that there are several potential advantages of a living-related donor transplant. First and foremost, the high wait-list mortality associated with a combined liver and intestinal transplants in children can be avoided. Secondly, based on the intestinal transplant registry, patients with isolated small bowel transplants have better overall outcomes compared with combined or MVI grafts. Finally, these children will
E11
need to be on lifelong immunosuppression, and better HLA matching may reduce their overall risk of rejection episodes and need for heavy immunosuppression.
References [1] Berdon WE, Baker DH, Blanc WA, et al. Megacystis microcolon intestinal hypoperistalsis syndrome: a new cause of intestinal obstruction in the newborn. Report of radiologic findings in five newborn girls. AJR Am J Roentgenol 1976;126:957-64. [2] Masetti M, Rodriguez M, Thompson J, et al. Multivisceral transplantation for megacystis microcolon intestinal hypoperistalsis syndrome. Transplantation 1999;68(2):228-32. [3] Anneren G, Meurling S, Olsen L. Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS), an autosomal recessive disorder: clinical reports and review of the literature. Am J Med Genet 1991;41: 251-4. [4] Puri P, Shinkai M. Megacystis microcolon intestinal hypoperistalsis syndrome. Semin Pediatr Surg 2005;14:58-63. [5] Loinaz C, Rodriguez M, Kato T, et al. Intestinal and multivisceral transplantation in children with severe gastrointestinal dysmotility. J Pediatr Surg 2005;40:1598-604. [6] Gillis DA, Grantmyre EB. Megacystis microcolon intestinal hypoperistalsis syndrome: survival of a male infant. J Pediatr Surg 1985;20: 279-81. [7] Iyer K, Kaufman S, Sudan D, et al. Long-term results of intestinal transplantation for pseudo-obstruction in children. J Pediatr Surg 2001;36(1):174-7. [8] Testa G, Panaro F, Schena S, et al. Living related small bowel transplantation—donor surgical technique. Ann Surg 2004;240(5): 779-84. [9] Testa G, Holterman M, John E, et al. Combined living donor liver/ small bowel transplantation. Transplantation 2005;79(10):1401-4. [10] www.intestinaltransplant.org. [11] Ciftci A, Cook R, van Velzen D. Megacystis microcolon intestinal hypoperistalsis syndrome: evidence of a primary myocellular defect of contractile fiber synthesis. J Pediatr Surg 1996;31(12):1706-11. [12] Taguchi T, Ikeda K, Shono T, et al. Autonomic innervation of the intestine from a baby with megacystis microcolon intestinal hypoperistalsis syndrome: immunohistochemical study. J Pediatr Surg 1989;24(12):1264-6. [13] Rolle U, O'Briain S, Pearl R, et al. Megacystis-microcolon-intestinal hypoperistalsis syndrome: evidence of intestinal myopathy. Pediatr Surg Int 2002;18:2-5. [14] Al Rayess M, Ambler M. Axonal dystrophy presenting as the megacystis microcolon-intestinal hypoperistalsis syndrome. Pediatr Pathol 1992;12:743-50. [15] Kubota M, Ikeda K, Ito Y. Autonomic innervation of the intestine from a baby with megacystis microcolon intestinal hypoperistalsis syndrome: electrophysiological study. J Pediatr Surg 1989;24(12):1267-70. [16] Nathan J, Rudolph J, Kocoshis S, et al. Isolated liver and multivisceral transplantation for total parenteral nutrition–related end-stage liver disease. J Pediatr Surg 2007;42:143-7. [17] Horslen S. Organ allocation for liver-intestine candidates. Liver Transpl 2004;10(10 Suppl 2):S86-9. [18] Fryer J, Pellar S, Ormond D, et al. Mortality in candidates waiting for combined liver-transplants exceeds that for other candidates waiting for liver transplants. Liver Transpl 2003;9:748-53. [19] Sokal E, Cleghorn G, Goulet O, et al. Liver and intestinal transplantation in children: Working Group Report of the First World Congress of Pediatric Gastroenterology, Hepatology, and Nutrition. J Pediatr Gastroenterol Nutr 2002;35(Supple):S159-72. [20] Sigurdsson L, Reyes J, Kocoshis S, et al. Intestinal transplantation in children with chronic intestinal pseudo-obstruction. Gut 1999;45: 570-4.