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Shawn Werner, MD ATC 11.29.2012
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Aristotle first described Anorectal Malfromations (ARM) Soranus treated in 2 nd century CE Amussat: proctoplasty, 1835 Mid-1900s to 1980s: puborectalis ring DeVries and Pena Posterior sagittal anorectoplasty, 1982 Georgeson Laparoscopic approach, 2000
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Abnormality of Hindgut Descending colon, rectum, upper anal canal, lining of bladder, urethra 5-10% incidence in genetic dz Trisomy 21 and 22q11.2 http://www.duke.edu/web/anatomy/embryology/GI/GI.html
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1:4000-5000 live births High association with other congenital anomalies Males > Females High vs Low lesion http://www.ajronline.org
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Urogenital: 81% Spinal dysraphism VACTERL Vertebral: 33-50% ▪ Tethered cord: 20-30% ▪ Sacral agenesis ▪ Spina bifida ▪ hemivertebrae duodenal Atresia: Cardiac: 10-30% ▪ ASD & VSD Tracheoesophageal: 5-10% Rectal Limb malformation http://radiopaedia.org/articles/hemivertebra
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Prevention Halt progression Reverse symptoms
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Spinal cord below L2 Filum terminale diffusely thickened Cord fixed by spinal lipoma Can be asymptomatic
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Asymptomatic Deterioration in gait Spasticity Weakness Back pain Incontinence Limb deformities
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Debate on imaging: XR, US, MRI Cost-effectiveness Availability of resources Risk to the patient Indications of preventative surgery
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Abn sacral anatomy abn cord BUT Normal sacral anatomy Plain radiograph 80% sens., 18% spec. Better imaging modalities Technology is beautiful Abnormal cord Normal cord
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Relatively cheap Best test for neonates US 86.5% sensitive, 92.9% specific
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Level and type of ARM Fistula location Sphincter complex Spinal anomalies MRI 95.6% sensitive, 90.9% specific Increased cost Requires sedation
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3 risk groups Low: simple skin dimple, DM mother Intermed: complex skin lesion, low ARM High: high ARM, cloacal malform./exstophy Compared 1. MRI 2. Plain radiograph 3. Ultrasound 4. No imaging with close follow-up
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US as neonate Spinal operation at 2-3 mo better if before 3 yo MRI days prior to surgery
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89 pts with ARM Eval at birth with sacral XR and spinal US MRI performed btwn 6-12 months 53 sacral abnormalities 54 spinal cord abnormalities Neg XR, + MRI in 4 pts Neg US, + MRI in 3 pts
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MRI best quality and more accurate > 3mo MRI for all children 6-12 mo US 1-3 mo Prior to complete posterior ossification Recommendations for urodynamics
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Retrospective 63 pts in 13 years with ARM 22 tethered cord MRI prior to 3 months of age + tethered cord surgical release Similar incidence between high and low lesions Pediatric Surgery 2001
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Pain Most likely to improve 75% in symptomatic pts Neurological Stabilize or improve 80-90% Early intervention= greater recovery Bowel & Bladder 16-67% Spasticity 63%
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High Association between tethered cord and ARM Multiple different imaging strategies US in neonate MRI around 2-3 months Most would recommend surgery
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1. Golonka NR, Haga LJ, Keating RP, et al. Routine MRI evaluation of low imperforate anus reveals unexpected high incidence of tethered spinal cord. J Pediatr Surg. 2002;37(7):966-9; discussion 966-9. 2. Herman RS, Teitelbaum DH. Anorectal malformations. Clin Perinatol. 2012;39(2):403-422. doi: 10.1016/j.clp.2012.04.001. 3. Lew SM, Kothbauer KF. Tethered cord syndrome: An updated review. Pediatr Neurosurg. 2007;43(3):236-248. doi: 10.1159/000098836. 4. Medina LS. Spinal dysraphism: Categorizing risk to optimize imaging. Pediatr Radiol. 2009;39 Suppl 2:S242-6. doi: 10.1007/s00247-008-1115-3. 5. Medina LS, Crone K, Kuntz KM. Newborns with suspected occult spinal dysraphism: A cost- effectiveness analysis of diagnostic strategies. Pediatrics. 2001;108(6):E101. 6. Miyasaka M, Nosaka S, Kitano Y, et al. Utility of spinal MRI in children with anorectal malformation. Pediatr Radiol. 2009;39(8):810-816. doi: 10.1007/s00247-009-1287-5. 7. Mosiello G, Capitanucci ML, Gatti C, et al. How to investigate neurovesical dysfunction in children with anorectal malformations. J Urol. 2003;170(4 Pt 2):1610-1613. doi: 10.1097/01.ju.0000083883.16836.91. 8. Nievelstein RA, Vos A, Valk J, Vermeij-Keers C. Magnetic resonance imaging in children with anorectal malformations: Embryologic implications. J Pediatr Surg. 2002;37(8):1138-1145. 9. Pacheco-Jacome E, Ballesteros MC, Jayakar P, Morrison G, Ragheb J, Medina LS. Occult spinal dysraphism: Evidence-based diagnosis and treatment. Neuroimaging Clin N Am. 2003;13(2):327-34, xii.
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Shawn Werner, MD ATC Orthopaedic Surgery Intern
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