Anorectal malformation (ARM): Spectrum of congenital anomalies related to abnormal development of anorectal canal
Diagnosis of ARM is made by clinical exam
Imaging is used to identify associated anomalies, plan operations, & evaluate postoperative complications
Evolution of ARM classification: Traditionally as high, intermediate, or low (relative to pubococcygeal line)
Inconsistent, inaccurate, & of little prognostic importance
Current (Peña or Krickenbeck): By specific malformation with therapeutic & prognostic implications
Cloaca: Subtype of ARM in girls; rectum, vagina, & bladder converge into common channel → single perineal orifice
Definitive repair as neonate vs. divided colostomy with delayed distal colostogram & definitive repair
Posterior sagittal anorectoplasty (PSARP): Definitive surgical reconstruction for most ARMs
IMAGING
General Features
Radiographic Findings
Fluoroscopic Findings
MR Findings
Ultrasonographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
CLINICAL ISSUES
Presentation
Demographics
Treatment
DIAGNOSTIC CHECKLIST
Consider
Selected References
Canning DA: Re: Cloaca reconstruction: a new algorithm which considers the role of urethral length in determining surgical planning. J Urol. 204(6):1368, 2020
Jarboe M et al: Imaged-guided and muscle sparing laparoscopic anorectoplasty using real-time magnetic resonance imaging. Pediatr Surg Int. 36(10):1255-60, 2020
Hosokawa T et al: Ultrasound imaging of the anorectal malformation during the neonatal period: a comprehensive review. Jpn J Radiol. 36(10):581-91, 2018
Kraus SJ et al: Augmented-pressure distal colostogram: the most important diagnostic tool for planning definitive surgical repair of anorectal malformations in boys. Pediatr Radiol. 48(2):258-69, 2018
Bischoff A: The surgical treatment of cloaca. Semin Pediatr Surg. 25(2):102-7, 2016
Buyukbese Sarsu S et al: A very rare cause of anal atresia: Currarino syndrome. J Clin Med Res. 8(5):420-3, 2016
Giuliani S et al: Long term follow-up and transition of care in anorectal malformations: an international survey. J Pediatr Surg. 51(9):1450-7, 2016
Govender S et al: Delayed diagnosis of anorectal malformations (ARM): causes and consequences in a resource-constrained environment. Pediatr Surg Int. 32(4):369-75, 2016
King SK et al: Colonic perforation in a neonate with an anorectal malformation. ANZ J Surg. 86(5):418-9, 2016
Lane VA et al: Rectal atresia and anal stenosis: the difference in the operative technique for these two distinct congenital anorectal malformations. Tech Coloproctol. 20(4):249-54, 2016
Lee MY et al: Sonographic determination of type in a fetal imperforate anus. J Ultrasound Med. 35(6):1285-91, 2016
Liechty ST et al: The morbidity of a divided stoma compared to a loop colostomy in patients with anorectal malformation. J Pediatr Surg. 51(1):107-10, 2016
Peiro JL et al: Prenatal diagnosis of cloacal malformation. Semin Pediatr Surg. 25(2):71-5, 2016
Yang G et al: Imperforate anus with rectopenile fistula: a case report and systematic review of the literature. BMC Pediatr. 16(1):65, 2016
Bischoff A et al: End stage renal disease and kidney transplant in patients with anorectal malformation: is there an alternative route? Pediatr Surg Int. 31(8):725-8, 2015
Bischoff A et al: Laparoscopic approach in the management of anorectal malformations. Pediatr Surg Int. 31(5):431-7, 2015
De la Torre-Mondragón L et al: Unexpected megarectum: a potential hidden source of complications in patients with anorectal malformation. J Pediatr Surg. 50(9):1560-2, 2015
Gine C et al: Two-port laparoscopic descending colostomy with separated stomas for anorectal malformations in newborns. Eur J Pediatr Surg. 26(5):462-4, 2015
Thomeer MG et al: High resolution MRI for preoperative work-up of neonates with an anorectal malformation: a direct comparison with distal pressure colostography/fistulography. Eur Radiol. 25(12):3472-9, 2015
Wang C et al: Anorectal malformation: the etiological factors. Pediatr Surg Int. 31(9):795-804, 2015
Fernandez E et al: Esophageal atresia in patients with anorectal malformations. Pediatr Surg Int. 30(8):767-71, 2014
Sanchez S et al: Vesicoureteral reflux and febrile urinary tract infections in anorectal malformations: a retrospective review. J Pediatr Surg. 49(1):91-4; discussion 94, 2014
VanderBrink BA et al: Epididymitis in patients with anorectal malformations: a cause for urologic concern. Int Braz J Urol. 40(5):676-82, 2014
Wetherill C et al: Hirschsprung disease and anorectal malformation. Early Hum Dev. 90(12):927-32, 2014
Alves JC et al: Comparison of MR and fluoroscopic mucous fistulography in the pre-operative evaluation of infants with anorectal malformation: a pilot study. Pediatr Radiol. 43(8):958-63, 2013
Bischoff A et al: Update on the management of anorectal malformations. Pediatr Surg Int. 29(9):899-904, 2013
Podberesky DJ et al: Magnetic resonance imaging of anorectal malformations. Magn Reson Imaging Clin N Am. 21(4):791-812, 2013
Podberesky DJ et al: MRI of acquired posterior urethral diverticulum following surgery for anorectal malformations. Pediatr Radiol. 41(9):1139-45, 2011
Bischoff A et al: Prenatal diagnosis of cloacal malformations. Pediatr Surg Int. 26(11):1071-5, 2010
Levitt MA et al: The morbidity of constipation in patients with anorectal malformations. J Pediatr Surg. 45(6):1228-33, 2010
Raschbaum GR et al: Magnetic resonance imaging-guided laparoscopic-assisted anorectoplasty for imperforate anus. J Pediatr Surg. 45(1):220-3, 2010
Eltomey MA et al: Postoperative pelvic MRI of anorectal malformations. AJR Am J Roentgenol. 191(5):1469-76, 2008
Levitt MA et al: Anorectal malformations. Orphanet J Rare Dis. 2:33, 2007
Peña A et al: Reoperations in anorectal malformations. J Pediatr Surg. 42(2):318-25, 2007
Levitt MA et al: Outcomes from the correction of anorectal malformations. Curr Opin Pediatr. 17(3):394-401, 2005
Sydorak RM et al: Laparoscopic repair of high imperforate anus. Semin Pediatr Surg. 11(4):217-25, 2002
Pena A et al: Advances in the management of anorectal malformations. Am J Surg. 180(5):370-6, 2000
Kluth D et al: Current concepts in the embryology of anorectal malformations. Semin Pediatr Surg. 6(4):180-6, 1997
Lerone M et al: The genetics of anorectal malformations: a complex matter. Semin Pediatr Surg. 6(4):170-9, 1997
Pena A: Management of anorectal malformations during the newborn period. World J Surg. 17(3):385-92, 1993
Gross GW et al: Augmented-pressure colostogram in imperforate anus with fistula. Pediatr Radiol. 21(8):560-2, 1991
Related Anatomy
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References
Tables
Tables
KEY FACTS
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
TERMINOLOGY
Synonyms
Imperforate anus
Definitions
Anorectal malformation (ARM): Spectrum of congenital anomalies related to abnormal development of anorectal canal
Diagnosis of ARM is made by clinical exam
Imaging is used to identify associated anomalies, plan operations, & evaluate postoperative complications
Evolution of ARM classification: Traditionally as high, intermediate, or low (relative to pubococcygeal line)
Inconsistent, inaccurate, & of little prognostic importance
Current (Peña or Krickenbeck): By specific malformation with therapeutic & prognostic implications
Cloaca: Subtype of ARM in girls; rectum, vagina, & bladder converge into common channel → single perineal orifice
Definitive repair as neonate vs. divided colostomy with delayed distal colostogram & definitive repair
Posterior sagittal anorectoplasty (PSARP): Definitive surgical reconstruction for most ARMs
IMAGING
General Features
Radiographic Findings
Fluoroscopic Findings
MR Findings
Ultrasonographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
CLINICAL ISSUES
Presentation
Demographics
Treatment
DIAGNOSTIC CHECKLIST
Consider
Selected References
Canning DA: Re: Cloaca reconstruction: a new algorithm which considers the role of urethral length in determining surgical planning. J Urol. 204(6):1368, 2020
Jarboe M et al: Imaged-guided and muscle sparing laparoscopic anorectoplasty using real-time magnetic resonance imaging. Pediatr Surg Int. 36(10):1255-60, 2020
Hosokawa T et al: Ultrasound imaging of the anorectal malformation during the neonatal period: a comprehensive review. Jpn J Radiol. 36(10):581-91, 2018
Kraus SJ et al: Augmented-pressure distal colostogram: the most important diagnostic tool for planning definitive surgical repair of anorectal malformations in boys. Pediatr Radiol. 48(2):258-69, 2018
Bischoff A: The surgical treatment of cloaca. Semin Pediatr Surg. 25(2):102-7, 2016
Buyukbese Sarsu S et al: A very rare cause of anal atresia: Currarino syndrome. J Clin Med Res. 8(5):420-3, 2016
Giuliani S et al: Long term follow-up and transition of care in anorectal malformations: an international survey. J Pediatr Surg. 51(9):1450-7, 2016
Govender S et al: Delayed diagnosis of anorectal malformations (ARM): causes and consequences in a resource-constrained environment. Pediatr Surg Int. 32(4):369-75, 2016
King SK et al: Colonic perforation in a neonate with an anorectal malformation. ANZ J Surg. 86(5):418-9, 2016
Lane VA et al: Rectal atresia and anal stenosis: the difference in the operative technique for these two distinct congenital anorectal malformations. Tech Coloproctol. 20(4):249-54, 2016
Lee MY et al: Sonographic determination of type in a fetal imperforate anus. J Ultrasound Med. 35(6):1285-91, 2016
Liechty ST et al: The morbidity of a divided stoma compared to a loop colostomy in patients with anorectal malformation. J Pediatr Surg. 51(1):107-10, 2016
Peiro JL et al: Prenatal diagnosis of cloacal malformation. Semin Pediatr Surg. 25(2):71-5, 2016
Yang G et al: Imperforate anus with rectopenile fistula: a case report and systematic review of the literature. BMC Pediatr. 16(1):65, 2016
Bischoff A et al: End stage renal disease and kidney transplant in patients with anorectal malformation: is there an alternative route? Pediatr Surg Int. 31(8):725-8, 2015
Bischoff A et al: Laparoscopic approach in the management of anorectal malformations. Pediatr Surg Int. 31(5):431-7, 2015
De la Torre-Mondragón L et al: Unexpected megarectum: a potential hidden source of complications in patients with anorectal malformation. J Pediatr Surg. 50(9):1560-2, 2015
Gine C et al: Two-port laparoscopic descending colostomy with separated stomas for anorectal malformations in newborns. Eur J Pediatr Surg. 26(5):462-4, 2015
Thomeer MG et al: High resolution MRI for preoperative work-up of neonates with an anorectal malformation: a direct comparison with distal pressure colostography/fistulography. Eur Radiol. 25(12):3472-9, 2015
Wang C et al: Anorectal malformation: the etiological factors. Pediatr Surg Int. 31(9):795-804, 2015
Fernandez E et al: Esophageal atresia in patients with anorectal malformations. Pediatr Surg Int. 30(8):767-71, 2014
Sanchez S et al: Vesicoureteral reflux and febrile urinary tract infections in anorectal malformations: a retrospective review. J Pediatr Surg. 49(1):91-4; discussion 94, 2014
VanderBrink BA et al: Epididymitis in patients with anorectal malformations: a cause for urologic concern. Int Braz J Urol. 40(5):676-82, 2014
Wetherill C et al: Hirschsprung disease and anorectal malformation. Early Hum Dev. 90(12):927-32, 2014
Alves JC et al: Comparison of MR and fluoroscopic mucous fistulography in the pre-operative evaluation of infants with anorectal malformation: a pilot study. Pediatr Radiol. 43(8):958-63, 2013
Bischoff A et al: Update on the management of anorectal malformations. Pediatr Surg Int. 29(9):899-904, 2013
Podberesky DJ et al: Magnetic resonance imaging of anorectal malformations. Magn Reson Imaging Clin N Am. 21(4):791-812, 2013
Podberesky DJ et al: MRI of acquired posterior urethral diverticulum following surgery for anorectal malformations. Pediatr Radiol. 41(9):1139-45, 2011
Bischoff A et al: Prenatal diagnosis of cloacal malformations. Pediatr Surg Int. 26(11):1071-5, 2010
Levitt MA et al: The morbidity of constipation in patients with anorectal malformations. J Pediatr Surg. 45(6):1228-33, 2010
Raschbaum GR et al: Magnetic resonance imaging-guided laparoscopic-assisted anorectoplasty for imperforate anus. J Pediatr Surg. 45(1):220-3, 2010
Eltomey MA et al: Postoperative pelvic MRI of anorectal malformations. AJR Am J Roentgenol. 191(5):1469-76, 2008
Levitt MA et al: Anorectal malformations. Orphanet J Rare Dis. 2:33, 2007
Peña A et al: Reoperations in anorectal malformations. J Pediatr Surg. 42(2):318-25, 2007
Levitt MA et al: Outcomes from the correction of anorectal malformations. Curr Opin Pediatr. 17(3):394-401, 2005
Sydorak RM et al: Laparoscopic repair of high imperforate anus. Semin Pediatr Surg. 11(4):217-25, 2002
Pena A et al: Advances in the management of anorectal malformations. Am J Surg. 180(5):370-6, 2000
Kluth D et al: Current concepts in the embryology of anorectal malformations. Semin Pediatr Surg. 6(4):180-6, 1997
Lerone M et al: The genetics of anorectal malformations: a complex matter. Semin Pediatr Surg. 6(4):170-9, 1997