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“Poop fail” A Case Discussion

Ryan Em C. Dalman MD MBA - 070070. “Poop fail” A Case Discussion. Objectives. Present a case of Imperforate Anus Discuss the pathophysiology and management of Imperforate Anus. Case Presentation. Patient History. General Data.

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“Poop fail” A Case Discussion

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  1. Ryan Em C. Dalman MD MBA - 070070 “Poop fail” A Case Discussion

  2. Objectives • Present a case of Imperforate Anus • Discuss the pathophysiology and management of Imperforate Anus

  3. Case Presentation Patient History

  4. General Data • Live preterm baby boy, born via primary CS for non-reassuring-fetal-heart-rate pattern and IUGR, at 35 weeks AOG, born on January 25, 2011

  5. Maternal and Birth History • 30 year-old G3P1 (0121) • Pre-ecclampsia • Maternal PMH: unremarkable

  6. History of Present Pregnancy • 1st trimester • Premature contractions—admitted for 1 week, given duphaston • 2nd trimester • UTI by urinalysis 3x, given 7 days unrecalled antibiotics for each week • ~30weeks AOG, BP 150/100, started on methyldopa 250mg BID • BP at home was uncontrolled • 3 days PTA, (+) headache, methyldopa increased to 500mg q6h, but was persistent and was admitted

  7. Family History • unremarkable

  8. Case Presentation Physical Exam

  9. General Infant Size • BW 1040g • BL 35 cm • HC 28 cm • AC 21 cm • AS 9,9 • MT 35 weeks, AGA

  10. General PE • Active, good cry • Fontanels - flat • Pupils - brisk • Muscle tone - normal • Strength of extremities- normal • Extremities – no deformities • Chest expansion- normal • Breath sounds clear and equal • Abdomen - soft • Anus - imperforate • Spine – intact • Color - pink • Cord - dry • Skin - clear • Pulses - strong • CRT <3 sec • Edema - none

  11. Case Presentation Case Discussion

  12. Admitting Impression • Imperforate anus

  13. Differential Daignosis • None • There is no differential diagnosis for an imperforate anus

  14. Imperforate Anus • Abnormal termination of the anorectum • Ranges from anal stenosisto persistence of cloaca • Most common defect • Imperforate anus with a fistula between the distal colon and urethra in boys or the vestibule of the vagina in girls

  15. Imperforate Anus • Associated anomalies: VACTER-L • Vertebral • Anal • Cardiac • Tracheal • Esophageal • Renal • Limb

  16. Imperforate Anus • Male defects: • Perineal Fistula – rectum opens in the perineum • Rectourethral bulbar fistula – rectum communicates with the lower posterior portion of the urethra called bulbar

  17. Imperforate Anus • Female defects: • Perineal Fistula – rectum opens in the perineum • Vestibular fistula – rectum opens through an abnormally narrow orifice located in the vestibule of the genitalia immediately outside the hymen

  18. Imperforate Anus • Female defects: • Rectovaginal Fistula – fistula between rectum and vagina

  19. Imperforate Anus • Pathophysiology • Embryogenesis of malformations still unclear • Rectum and anus develop from hindgut or cloacal cavity when lateral ingrowth of the mesenchyme forms the urorectal septum in the midline. • Bladder&urethra septum  rectum&anal canal • Cloacal duct – small communications bet. these 2 • Should close by 7th week of gestation

  20. Imperforate Anus • Pathophysiology • Ventral urogenital external opening forms first; dorsal anal membrane opens later • Anal development • Fusion of the anal tubercles and an external invagination (proctoderm) which deepens toward the rectum but separated from it by an anal membrane • Anal membrane should desintegrate by 8th week

  21. Imperforate Anus • There are known risk factors that predispose a person to have a child with imperforate anus • A genetic linkage is sometimes present

  22. Diagnostics/ workup • CBC, blood typing and screening • Presence of meconium in the urine (males) • Filtering with a gauze pad • Urinalysis • If a patient has perineal fistulas, vestibular fistula, or a single patent orifice, UA is unot indicated

  23. Diagnostics/ workup • Invertogram • Cross table lateral on prone position

  24. Diagnostics/ workup • Prone cross table buttocks with elevation

  25. Diagnostics/ workup • Abdominal Ultrasound • Visualized liver, gallbladder, kidneys • Obscured pancreas probably due to overlying bowel gas • Undefined gallbladder • Bilateral hyrocoele, both testicles within scrotal sac • Minimal ascites • No frank congenital problems on solid organs

  26. Management • Medical • NPO, IV hydration • Treat other life-threatening co-morbidities first • If urinary fistula is suspected, give broad-spectrum antibiotics

  27. Management • Invertogram • < 1cm: Immediate Anoplasty • > 1cm: colostomy, then definitive surgery after a few months • Males with meconium in urine: colostomy, then definitive surgery after a few months

  28. Epidemiology • 1 newborn per 5000 live births (US)

  29. Prognosis • All patients with anorectal malformations with no significant life-threatening co-morbidities should survive • Prognosis best determined by the probability of primary fecal incontinence

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