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Megacystis-Microcolon-intestinal hypoperistalsis syndrome
Journal of Pediatric Surgery Case Reports 41 (2019) 4–7
Contents lists available at ScienceDirect
Journal of Pediatric Surgery Case Reports journal homepage: www.elsevier.com/locate/epsc
Megacystis-Microcolon-intestinal hypoperistalsis syndrome Ahmed Elrouby , Mostafa Ahmed Kotb, Mostafa Abdelatty, Saber Waheeb
T
∗
Pediatric Surgery Department, Faculty of Medicine, Alexandria University
ARTICLE INFO
ABSTRACT
Keywords: Megacystitis Microcolon Hypoperistalysis
Megacystis Microcolon Intestinal Hypoperistalsis Syndrome (MMIHS) is a rare congenital anomaly that presents with severe form of functional obstruction of the gastrointestinal tract which is usually fatal. Here, we report a full term female infant with MMIHS presented with feeding intolerance, huge abdominal distension, bile-stained vomiting, absent bowel sounds and a large abdominal cystic mass. Exploration revealed hugely distended urinary bladder, micro colon, short bowel and malrotation of the small intestine. Ladd's procedure, ileostomy and vesicostomy were performed.
Geospatial data: Pediatric Surgery Department, Elshatby University Hospital for Children, Faculty of Medicine, Alexandria University.
Corresponding author. E-mail addresses: [email protected], [email protected] (A. Elrouby), [email protected] (M.A. Kotb), [email protected] (M. Abdelatty), [email protected] (S. Waheeb). ∗
https://doi.org/10.1016/j.epsc.2018.11.002 Received 30 October 2018; Received in revised form 2 November 2018; Accepted 8 November 2018 Available online 20 November 2018 2213-5766/ © 2018 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/BY-NC-ND/4.0/).
Journal of Pediatric Surgery Case Reports 41 (2019) 4–7
A. Elrouby et al.
1. Introduction
2. Case report
Megacystis-Microcolon -Intestinal Hypoperistalsis -Syndrome (MMIHS) is a rare autosomal recessive disorder that was first described in 1976 by Berdon in five newborn girls [1].It is considered the most severe form of functional intestinal obstruction in neonates [2].It is characterized by abdominal distension resulting from distended nonobstructive urinary bladder, microcolon, intestinal hypo- or aperistalsis and may be associated with other anomalies like malrotation of the small intestine [3].It has been found that the incidence is approximately 4 times more common in females than males [4].
A 1 day-old female full term infant, weighting 3250 g delivered by an uncomplicated cesarean section, presented to Elshatby University Hospital with severe abdominal distension at birth, feeding intolerance, bilious vomiting and inability to void spontaneously with no history of passage of meconium. There was no family history of congenital anomalies and no consanguinity between the parents. Physical examination of the baby revealed a large, ill defined, mobile pelvi-abdominal cystic mass extending from the pelvis up to the middle part of abdomen with absent bowel sounds. No tenderness could be elicited all over the abdomen. The infant was normally responsive and no other abnormal physical findings could be identified. A prenatal ultrasound was done showing a large pelvi-abdominal mass, with no definite origin could be identified (Fig. 1). Naso-gastric suctioning together with urinary catheterization were performed with the evacuation of approximately 30 ml of bilious fluid and 145 ml of urine respectively. Laboratory investigations showed anemia, hypoprotenemia, elevated blood urea nitrogen (BUN) and increased level of C-reactive protein (CRP). Otherwise, other laboratory results were unremarkable. The performed radiological investigations included an erect plain Xray abdomen in and an upper (GIT) series with contrast. It showed several mildly dilated intestinal loops within the right upper quadrant, dilated stomach with non-passage of contrast material in the distal intestine together with a large soft tissue density in the left hemi-abdomen exerting a mass like effect on the dilated stomach (Fig. 2a and b). Post-natal abdominal ultrasonography confirmed the presence of the cystic mass extending from the pelvis up to the epigastrium with bilateral hydrouretronephrosis more evident on the left side. However, the origin of the mass could not be identified. Normal liver, gall
Fig. 1. Prenatal ultrasound image showing a large pelvi-abdominal mass.
Fig. 2. (a, b): an upper (GIT) series on the left and a plain X-Ray abdomen in erect position on the right. The right directed arrows refer to the hugely dilated stomach, while the left directed arrows refer to a large white shadow denoting a huge pelvi-abdominal mass. Notice also the non-passage of contrast material on the left side while the gasless abdomen on the right side denotes collapsed bowel. 5
Journal of Pediatric Surgery Case Reports 41 (2019) 4–7
A. Elrouby et al.
Fig. 5. A double barrel ileostomy and supra-pubic vesicostomy.
post operatively, feeding was not tolerated with the appearance of signs of septicemia despite the strong antibiotic coverage and antiseptic techniques used while delivering care to the patient. The baby started total parenteral nutrition (TPN) on the 10th day post operatively. After that, the baby ran a stationary course with daily follow up of her laboratory investigations and correction of any identified abnormalities. The baby girl completed her first month, till date she survived. However, long term survival cannot yet be ensured, because most of the cases died during their first year, as reported in literature.
Fig. 3. CAT scan image showing the hugely distended bladder reaching middle abdomen.
bladder, biliary system and spleen were noted. There was no ascites. An abdominal computed axial tomography (CAT) scan with intra-venous contrast was performed, revealing, a hugely distended bladder occupying the whole left hemi abdomen corresponding to the mass seen on radiograph and abdominal ultrasonograophy. Together with bilateral mild hydronephrosis which was more evident on the left side. The ureters could be traced and seen dilated on both sides. However, no definitive obstructing lesion could be identified and bowel loops seen mildly dilated. The radiologist recommended urodynamic study for the patient (Fig. 3). The decision was taken to perform a surgical abdominal exploration via midline incision. It revealed a hugely distended bladder, small intestinal malrotation, short bowel and micro colon, features consistent with (MMIHS), together with dilated ureters on both sides (Fig. 4). Ladd's procedure, a double barrel ileostomy and supra-pubic vesicostomy were performed (Fig. 5). Post-operative follow up in the first week showed feeding tolerance up to 30 ml via NGT/3 h and passage of stool and urine via the performed ileostomy and vesicostomy respectively. Intra-venous fluids, meropenem, vancomycin and prokinetic drugs were given to promote bowel motility and protect against infections. However, by the 8th day
3. Discussion Till date, 230 cases of MMIHS have been reported in the literature [3]. The is frequently a history of consanguinity among parents suggesting an autosomal recessive mode of inheritance, although most cases are sporadic [5]. Usually, neonates present with bilious emesis and abdominal distension resulting from either functional intestinal obstruction &/or bladder distension. This distention usually fills the whole abdomen and even it can reach the xiphisternum. Plain X-ray abdomen would reveal dilated bowel loops, huge distended stomach or a gasless abdomen. Contrast enema reveals a microcolon with or without malrotation [6]. Although the exact etiology is unknown, but several theories arose to explain the pathogenesis. Visceral myopathy is the most accepted theory, supported by the evidence of myopathy involving both the circular and longitudinal layers of the small bowel muscularis propria on histologic studies [7].This can be in the form of excessive smooth muscle glycogen storage suggesting a defect of glycogen-energy utilization [8], absence of the contractile and cytoskeletal proteins in the smooth muscle layers [9]or vacuolar degenerative changes in the smooth muscle cells with excessive connective tissue between muscle cells in the bowel and bladder [10].A possible cause of intestinal hypoperistalsis is the imbalance in intestinal peptides [11].Moreover, absence of Interstitial Cell of Cajal in the bowel and urinary bladder has been reported as a possible causative factor [9]. Some manifestations of (MMIHPS) can be identified prenatally by routine prenatal ultrasonography as distended stomach and urinary bladder with or without hydronephrosis. Amniotic fluid volume is increased or normal in most patients, although oligo-hydramnions has occasionally been reported [6]. The mainstay of treatment is nutritional support via total parenteral nutrition (TPN). Palliative surgery in the form of jejunostomy&/or vesicostomy is commonly indicated [12]. Our patient had directly a diverting ileostomy due to hugely distended ileum which resulted into severely distended abdomen causing neonatal respiratory distress. Also, it's been proved in literature that long term survivors of this syndrome
Fig. 4. An Intra-operative picture showing the hugely distended bladder. 6
Journal of Pediatric Surgery Case Reports 41 (2019) 4–7
A. Elrouby et al.
References
are those who have ileostomies. Diverting ileostomy allows the patient to start oral feeding earlier and avoids long term TPN, with all of its complications, which is recommended in case of starting bowel management program instead of ileostomy [13]. Again we committed vesicostomy directly before attempt to do repeated urinary catheterization as the patient had elevated BUN&CRP so we decided to relieve the functional urinary obstruction surgically to save the kidneys and stop the progress of sepsis which may be aggravated by repeated urinary catheterization. Currently, the only accepted treatment modality is multivisceral transplantation. During the last few years, the improved neonatal total parenteral nutrition and success of bowel transplantation has significantly increased the rate of survival. MMIHS has a poor prognosis where most patients died in the first year of life from severe overwhelming sepsis resulting from malnutrition, TPN induced liver failure, sepsis or renal failure secondary to hydronephrosis with majority of patients [4].
[1] Berdon W, Baker D, Blanc W, Gay B, Santulli T, Donovan C. Megacystis-microcolonintestinal hypoperistalsis syndrome: a new cause of intestinal obstruction in the newborn. Report of radiologic findings in five newborn girls. Am J Roentgenol [Internet] 1976;126(5):957–64. Available from: http://www.ajronline.org/doi/10. 2214/ajr.126.5.957. [2] Gosemann J-H, Puri P. Megacystis microcolon intestinal hypoperistalsis syndrome: systematic review of outcome. Pediatr Surg Int [Internet] 2011;27(10):1041–6. Available from: http://link.springer.com/10.1007/s00383-011-2954-9. [3] Mc Laughlin D, Puri P. Familial megacystis microcolon intestinal hypoperistalsis syndrome: a systematic review. Pediatr Surg Int 2013;29:947–51. [4] Al-Salem AH. Megacystis microcolon intestinal hypoperistalsis syndrome: a report of a variant. Ann Pediatr Surg 2014;10(2):57–60. [5] Thorson W, Diaz-Horta O, Foster J, Spiliopoulos M, Quintero R, Farooq A, et al. De novo ACTG2 mutations cause congenital distended bladder, microcolon, and intestinal hypoperistalsis. Hum Genet 2014;133(6):737–42. [6] García Delgado R, García Rodríguez R, Armas Roca M, Romero Requejo A, García Escribano P, Santana Suárez A, et al. Megacystis-Micro colon-Intestinal Hypoperistalsis syndrome: a case report. J Perinat Med [Internet]. 2015;43. Available from: http://www.embase.com/search/results?subaction=viewrecord& from=export&id=L72185658%5Cnhttps://doi.org/10.1515/jpm-2015-2003% 5Cnhttp://sfx.library.uu.nl/utrecht?sid=EMBASE&issn=03005577&id=doi:10. 1515%2Fjpm-2015-2003&atitle=Megacystis-Micro+colon-Intesti. [7] Piotrowska AP, Rolle U, Solari V, Puri P. Interstitial cells of Cajal in the human normal urinary bladder and in the bladder of patients with megacystis-microcolon intestinal hypoperistalsis syndrome. BJU Int 2004;94(1):143–6. [8] Ciftci AO, Cook RCM, 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. [9] Piaseczna Piotrowska A, Rolle U, Chertin B, De Caluwé D, Bianchi A, Puri P. Alterations in smooth muscle contractile and cytoskeleton proteins and interstitial cells of Cajal in megacystis microcolon intestinal hypoperistalsis syndrome. J Pediatr Surg 2003:749–55. [10] Puri P, Lake BD, Gorman F, O'Donnell B, Nixon HH. Megacystis-microcolon-intestinal hypoperistalsis syndrome: a visceral myopathy. J Pediatr Surg 1983;18(1):64–9. [11] Taguchi T, Ikeda K, Shono T, Goto S, Kubota M, Kawana T, et al. Autonomic innervation of the intestine from a baby with megacystis microcolon intestinal hypoperistalsis syndrome: I. Immunohistochemical study. J Pediatr Surg 1989;24(12):1264–6. [12] Puri P, Gosemann JH. Megacystis microcolon intestinal hypoperistalsis syndrome. Newborn surgery. fourth ed.2017. p. 737–43. [13] Michael F, Wangler MD, Arthur L, Beaudet M. GeneReviews [Internet]. Available from:. http://www.ncbi.nlm.nih.gov/books/NBK299311/.
Patient consent Consent to publish the case report was not obtained. This report does not contain any personal information that could lead to the identification of the patient. Funding No funding or grant support. Authorship All authors attest that they meet the current ICMJE criteria for Authorship. Conflict of interest The following authors have no financial disclosures: (Authors initials).
7
Contents lists available at ScienceDirect
Journal of Pediatric Surgery Case Reports journal homepage: www.elsevier.com/locate/epsc
Megacystis-Microcolon-intestinal hypoperistalsis syndrome Ahmed Elrouby , Mostafa Ahmed Kotb, Mostafa Abdelatty, Saber Waheeb
T
∗
Pediatric Surgery Department, Faculty of Medicine, Alexandria University
ARTICLE INFO
ABSTRACT
Keywords: Megacystitis Microcolon Hypoperistalysis
Megacystis Microcolon Intestinal Hypoperistalsis Syndrome (MMIHS) is a rare congenital anomaly that presents with severe form of functional obstruction of the gastrointestinal tract which is usually fatal. Here, we report a full term female infant with MMIHS presented with feeding intolerance, huge abdominal distension, bile-stained vomiting, absent bowel sounds and a large abdominal cystic mass. Exploration revealed hugely distended urinary bladder, micro colon, short bowel and malrotation of the small intestine. Ladd's procedure, ileostomy and vesicostomy were performed.
Geospatial data: Pediatric Surgery Department, Elshatby University Hospital for Children, Faculty of Medicine, Alexandria University.
Corresponding author. E-mail addresses: [email protected], [email protected] (A. Elrouby), [email protected] (M.A. Kotb), [email protected] (M. Abdelatty), [email protected] (S. Waheeb). ∗
https://doi.org/10.1016/j.epsc.2018.11.002 Received 30 October 2018; Received in revised form 2 November 2018; Accepted 8 November 2018 Available online 20 November 2018 2213-5766/ © 2018 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/BY-NC-ND/4.0/).
Journal of Pediatric Surgery Case Reports 41 (2019) 4–7
A. Elrouby et al.
1. Introduction
2. Case report
Megacystis-Microcolon -Intestinal Hypoperistalsis -Syndrome (MMIHS) is a rare autosomal recessive disorder that was first described in 1976 by Berdon in five newborn girls [1].It is considered the most severe form of functional intestinal obstruction in neonates [2].It is characterized by abdominal distension resulting from distended nonobstructive urinary bladder, microcolon, intestinal hypo- or aperistalsis and may be associated with other anomalies like malrotation of the small intestine [3].It has been found that the incidence is approximately 4 times more common in females than males [4].
A 1 day-old female full term infant, weighting 3250 g delivered by an uncomplicated cesarean section, presented to Elshatby University Hospital with severe abdominal distension at birth, feeding intolerance, bilious vomiting and inability to void spontaneously with no history of passage of meconium. There was no family history of congenital anomalies and no consanguinity between the parents. Physical examination of the baby revealed a large, ill defined, mobile pelvi-abdominal cystic mass extending from the pelvis up to the middle part of abdomen with absent bowel sounds. No tenderness could be elicited all over the abdomen. The infant was normally responsive and no other abnormal physical findings could be identified. A prenatal ultrasound was done showing a large pelvi-abdominal mass, with no definite origin could be identified (Fig. 1). Naso-gastric suctioning together with urinary catheterization were performed with the evacuation of approximately 30 ml of bilious fluid and 145 ml of urine respectively. Laboratory investigations showed anemia, hypoprotenemia, elevated blood urea nitrogen (BUN) and increased level of C-reactive protein (CRP). Otherwise, other laboratory results were unremarkable. The performed radiological investigations included an erect plain Xray abdomen in and an upper (GIT) series with contrast. It showed several mildly dilated intestinal loops within the right upper quadrant, dilated stomach with non-passage of contrast material in the distal intestine together with a large soft tissue density in the left hemi-abdomen exerting a mass like effect on the dilated stomach (Fig. 2a and b). Post-natal abdominal ultrasonography confirmed the presence of the cystic mass extending from the pelvis up to the epigastrium with bilateral hydrouretronephrosis more evident on the left side. However, the origin of the mass could not be identified. Normal liver, gall
Fig. 1. Prenatal ultrasound image showing a large pelvi-abdominal mass.
Fig. 2. (a, b): an upper (GIT) series on the left and a plain X-Ray abdomen in erect position on the right. The right directed arrows refer to the hugely dilated stomach, while the left directed arrows refer to a large white shadow denoting a huge pelvi-abdominal mass. Notice also the non-passage of contrast material on the left side while the gasless abdomen on the right side denotes collapsed bowel. 5
Journal of Pediatric Surgery Case Reports 41 (2019) 4–7
A. Elrouby et al.
Fig. 5. A double barrel ileostomy and supra-pubic vesicostomy.
post operatively, feeding was not tolerated with the appearance of signs of septicemia despite the strong antibiotic coverage and antiseptic techniques used while delivering care to the patient. The baby started total parenteral nutrition (TPN) on the 10th day post operatively. After that, the baby ran a stationary course with daily follow up of her laboratory investigations and correction of any identified abnormalities. The baby girl completed her first month, till date she survived. However, long term survival cannot yet be ensured, because most of the cases died during their first year, as reported in literature.
Fig. 3. CAT scan image showing the hugely distended bladder reaching middle abdomen.
bladder, biliary system and spleen were noted. There was no ascites. An abdominal computed axial tomography (CAT) scan with intra-venous contrast was performed, revealing, a hugely distended bladder occupying the whole left hemi abdomen corresponding to the mass seen on radiograph and abdominal ultrasonograophy. Together with bilateral mild hydronephrosis which was more evident on the left side. The ureters could be traced and seen dilated on both sides. However, no definitive obstructing lesion could be identified and bowel loops seen mildly dilated. The radiologist recommended urodynamic study for the patient (Fig. 3). The decision was taken to perform a surgical abdominal exploration via midline incision. It revealed a hugely distended bladder, small intestinal malrotation, short bowel and micro colon, features consistent with (MMIHS), together with dilated ureters on both sides (Fig. 4). Ladd's procedure, a double barrel ileostomy and supra-pubic vesicostomy were performed (Fig. 5). Post-operative follow up in the first week showed feeding tolerance up to 30 ml via NGT/3 h and passage of stool and urine via the performed ileostomy and vesicostomy respectively. Intra-venous fluids, meropenem, vancomycin and prokinetic drugs were given to promote bowel motility and protect against infections. However, by the 8th day
3. Discussion Till date, 230 cases of MMIHS have been reported in the literature [3]. The is frequently a history of consanguinity among parents suggesting an autosomal recessive mode of inheritance, although most cases are sporadic [5]. Usually, neonates present with bilious emesis and abdominal distension resulting from either functional intestinal obstruction &/or bladder distension. This distention usually fills the whole abdomen and even it can reach the xiphisternum. Plain X-ray abdomen would reveal dilated bowel loops, huge distended stomach or a gasless abdomen. Contrast enema reveals a microcolon with or without malrotation [6]. Although the exact etiology is unknown, but several theories arose to explain the pathogenesis. Visceral myopathy is the most accepted theory, supported by the evidence of myopathy involving both the circular and longitudinal layers of the small bowel muscularis propria on histologic studies [7].This can be in the form of excessive smooth muscle glycogen storage suggesting a defect of glycogen-energy utilization [8], absence of the contractile and cytoskeletal proteins in the smooth muscle layers [9]or vacuolar degenerative changes in the smooth muscle cells with excessive connective tissue between muscle cells in the bowel and bladder [10].A possible cause of intestinal hypoperistalsis is the imbalance in intestinal peptides [11].Moreover, absence of Interstitial Cell of Cajal in the bowel and urinary bladder has been reported as a possible causative factor [9]. Some manifestations of (MMIHPS) can be identified prenatally by routine prenatal ultrasonography as distended stomach and urinary bladder with or without hydronephrosis. Amniotic fluid volume is increased or normal in most patients, although oligo-hydramnions has occasionally been reported [6]. The mainstay of treatment is nutritional support via total parenteral nutrition (TPN). Palliative surgery in the form of jejunostomy&/or vesicostomy is commonly indicated [12]. Our patient had directly a diverting ileostomy due to hugely distended ileum which resulted into severely distended abdomen causing neonatal respiratory distress. Also, it's been proved in literature that long term survivors of this syndrome
Fig. 4. An Intra-operative picture showing the hugely distended bladder. 6
Journal of Pediatric Surgery Case Reports 41 (2019) 4–7
A. Elrouby et al.
References
are those who have ileostomies. Diverting ileostomy allows the patient to start oral feeding earlier and avoids long term TPN, with all of its complications, which is recommended in case of starting bowel management program instead of ileostomy [13]. Again we committed vesicostomy directly before attempt to do repeated urinary catheterization as the patient had elevated BUN&CRP so we decided to relieve the functional urinary obstruction surgically to save the kidneys and stop the progress of sepsis which may be aggravated by repeated urinary catheterization. Currently, the only accepted treatment modality is multivisceral transplantation. During the last few years, the improved neonatal total parenteral nutrition and success of bowel transplantation has significantly increased the rate of survival. MMIHS has a poor prognosis where most patients died in the first year of life from severe overwhelming sepsis resulting from malnutrition, TPN induced liver failure, sepsis or renal failure secondary to hydronephrosis with majority of patients [4].
[1] Berdon W, Baker D, Blanc W, Gay B, Santulli T, Donovan C. Megacystis-microcolonintestinal hypoperistalsis syndrome: a new cause of intestinal obstruction in the newborn. Report of radiologic findings in five newborn girls. Am J Roentgenol [Internet] 1976;126(5):957–64. Available from: http://www.ajronline.org/doi/10. 2214/ajr.126.5.957. [2] Gosemann J-H, Puri P. Megacystis microcolon intestinal hypoperistalsis syndrome: systematic review of outcome. Pediatr Surg Int [Internet] 2011;27(10):1041–6. Available from: http://link.springer.com/10.1007/s00383-011-2954-9. [3] Mc Laughlin D, Puri P. Familial megacystis microcolon intestinal hypoperistalsis syndrome: a systematic review. Pediatr Surg Int 2013;29:947–51. [4] Al-Salem AH. Megacystis microcolon intestinal hypoperistalsis syndrome: a report of a variant. Ann Pediatr Surg 2014;10(2):57–60. [5] Thorson W, Diaz-Horta O, Foster J, Spiliopoulos M, Quintero R, Farooq A, et al. De novo ACTG2 mutations cause congenital distended bladder, microcolon, and intestinal hypoperistalsis. Hum Genet 2014;133(6):737–42. [6] García Delgado R, García Rodríguez R, Armas Roca M, Romero Requejo A, García Escribano P, Santana Suárez A, et al. Megacystis-Micro colon-Intestinal Hypoperistalsis syndrome: a case report. J Perinat Med [Internet]. 2015;43. Available from: http://www.embase.com/search/results?subaction=viewrecord& from=export&id=L72185658%5Cnhttps://doi.org/10.1515/jpm-2015-2003% 5Cnhttp://sfx.library.uu.nl/utrecht?sid=EMBASE&issn=03005577&id=doi:10. 1515%2Fjpm-2015-2003&atitle=Megacystis-Micro+colon-Intesti. [7] Piotrowska AP, Rolle U, Solari V, Puri P. Interstitial cells of Cajal in the human normal urinary bladder and in the bladder of patients with megacystis-microcolon intestinal hypoperistalsis syndrome. BJU Int 2004;94(1):143–6. [8] Ciftci AO, Cook RCM, 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. [9] Piaseczna Piotrowska A, Rolle U, Chertin B, De Caluwé D, Bianchi A, Puri P. Alterations in smooth muscle contractile and cytoskeleton proteins and interstitial cells of Cajal in megacystis microcolon intestinal hypoperistalsis syndrome. J Pediatr Surg 2003:749–55. [10] Puri P, Lake BD, Gorman F, O'Donnell B, Nixon HH. Megacystis-microcolon-intestinal hypoperistalsis syndrome: a visceral myopathy. J Pediatr Surg 1983;18(1):64–9. [11] Taguchi T, Ikeda K, Shono T, Goto S, Kubota M, Kawana T, et al. Autonomic innervation of the intestine from a baby with megacystis microcolon intestinal hypoperistalsis syndrome: I. Immunohistochemical study. J Pediatr Surg 1989;24(12):1264–6. [12] Puri P, Gosemann JH. Megacystis microcolon intestinal hypoperistalsis syndrome. Newborn surgery. fourth ed.2017. p. 737–43. [13] Michael F, Wangler MD, Arthur L, Beaudet M. GeneReviews [Internet]. Available from:. http://www.ncbi.nlm.nih.gov/books/NBK299311/.
Patient consent Consent to publish the case report was not obtained. This report does not contain any personal information that could lead to the identification of the patient. Funding No funding or grant support. Authorship All authors attest that they meet the current ICMJE criteria for Authorship. Conflict of interest The following authors have no financial disclosures: (Authors initials).
7