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  • at 2 1/2 cm from anal verge: no ganglion cells identified, large nerve trunks present in submucosal tissue with focal ingrowth of nerve fibers into lamina propria
  • At submucosal level (Meissner’s Plexuus) Myenteric level (Auerbach’s plexus) 5:1 male to female ratio. First described by Dr. Harald Hirschsprung in 1886 Explosive release of feces and flatus may follow exam
  • AXR: massive colon distention with gas and feces, air-fluid levels may be present Contrast enema: spastic transitional segment with irregular saw-toothed outline, barium may be retained in proximal bowel more than 24 hours Rectal biopsy: absence of Meissner and Auerbach’s plexus, marked hypertrophy of nerve trunks Rectal suction biopsy: more difficult than pathologically diagnosing Hirschsprung disease from samples obtained by a fulll- thickness biopsy Acetylcholinesterase staining: intensely stains the hypertrophied nerve fibers thoughout the lamina propria and muscularis propria Rectal biopsy: definitive method Rectal Manometry : loss of normal reflex relaxation, involves internal anal sphincter on rectal distention, fallen out of use Complications following use of the rectal suction biopsy technique in the diagnosis of Hirschsprung's disease, are rare. In a series of 1,340 consecutive biopsies, complications included three clinical perforations of the bowel, one resulting in death, and three rectal hemorrhages requiring transfusion. A plea is made for the use of greater care in this technique. (0.45% total risk for complications) of that, 0.2% for perforation, 0.2% for significant bleeding
  • Early colostomy with resection of aganglionic segment Swenson procedure The Swenson procedure was the original pull-through procedure used to treat Hirschsprung disease. The aganglionic segment is resected down to the sigmoid colon and the remaining rectum, and an oblique anastomosis is performed between the normal colon to the low rectum. Duhamel procedure The Duhamel procedure was first described in 1956 as a modification to the Swenson procedure. Key points are that a retrorectal approach is used and a significant portion of aganglionic rectum is retained. The aganglionic bowel is resected down to the rectum, and the rectum is oversewn. The proximal bowel is then brought through the retrorectal space (between the rectum and sacrum), and an end-to-side anastomosis is performed on the remaining rectum. Soave (endorectal) procedure The Soave procedure was introduced in the 1960s and consists of removing the mucosa and submucosa of the rectum and pulling the ganglionic bowel through the aganglionic muscular cuff of the rectum. The original operation did not include a formal anastomosis, but the procedure has been modified by Boley to include a primary anastomosis at the anus.
  • Retrospective chart review

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  • 1. Pediatric Case Management The Children’s Hospital at Sinai Joseph Wiley, MD Cynthia Roldan, MD November 29, 2005
  • 2. November Cases-ER
    • 4 month old infant with HIE with severe neurologic sequelae presented in respiratory arrest
  • 3. November Cases-NICU
    • FT infant with known congenital heart defect, with double outlet right ventricle (transfer)
    • FT infant with Hydrops transferred for ECMO (mortality)
    • 24 week ex premature infant with NEC (mortality)
  • 4. Pediatric Case Management Conference November 29, 2005 Julia Trintis, D.O.
  • 5. CC: H.W. 9 day old with abdominal distension and bilious emesis
    • Returning 6 days after discharge from NICU
    • Passed meconium after first 24 hours
    • Evaluation for abdominal distention and bilious emesis in FTN
      • Transfer to NICU
      • Rectal exam-large meconium plug evacuated
  • 6. NICU Course
    • Work up:
      • AXR -gaseous distention of small and large bowel loops; opacification of rectum and sigmoid colon
      • Barium Enema - no evidence of microcolon; possible rectal web
      • Sigmoidoscopy -normal-no web or stricture
      • CF Studies -ordered (ultimately negative)
  • 7. Neonatal Assessment
    • FT infant with probable meconium plug syndrome.
      • Differential Diagnosis: Hirschsprung’s Disease, Cystic Fibrosis, Isolated Meconium Plug
    • Monitored for additional 24 hours; had normal stools and resolution of symptoms
    • Plan:
      • Discharge home
      • Follow up with PMD, GI
      • Further evaluation including rectal suction biopsy for recurrence of symptoms
      • F/U CF Genetic studies
  • 8. HPI
    • Presentation to Pediatric Floor
      • Initially doing well
      • BM’s occurred every feed until 1 day PTA
      • Developed Poor feeding
      • Decreased activity
      • Watery, mucousy stool
      • One episode of bilious emesis
    • SHx/FHx/Allergies/Meds -non contributory
  • 9. Physical Exam
    • T:36.5 HR140 RR40 BP N.O. Ox Sat 98%
    • General: Alert, Awake, in NAD
    • Abdomen: hyperactive bowel sounds, distended but soft, no palpable masses or HSM
    • Perianal exam-normally placed anus
    • Rectal: normal; hemoccult negative; no narrowing or explosive stool
    • Neuro: intact
  • 10. Abdominal X-Ray B A Abnormal Colonic Caliber
  • 11. Hospital Course
    • Normal saline enemas BID
    • IV fluids
    • Rectal suction biopsy
    • Acetylcholinesterase stain
    • Discharged home
      • Readmitted for full thickness biopsy of sigmoid colon
      • Scheduled for definitive surgery today
  • 12. Points for Discussion
    • Differential Diagnosis of Meconium Plug
    • Diagnostic Methods for Hirschsprung’s Disease
      • Choice of biopsy: Rectal Suction vs. Full thickness
      • Anal rectal Manometry
  • 13. Meconium Plug Syndrome
    • Transient disorder of the newborn colon characterized by delayed passage of meconium and intestinal dilatation
    • Epidemiology: 1/500
    • Incidence increased in premature infants of diabetic mothers and in infants whose mothers received magnesium sulfate
    • Immaturity of myenteric plexus nerve cells or their hormonal receptors
  • 14. Clinical Features-MPS
    • Abdominal distention
    • Failure to pass significant meconium in the first 24 hours of life
    • Bilious vomiting
    • Associated with
    • Cystic Fibrosis
    • Hirschsprung Disease is eventually diagnosed in 10-30%
  • 15. Hirschsprung Disease
    • Aganglionic megacolon: lack of intramural ganglionic cells
    • Occurs in 1:5000 births
    • Associated with Down syndrome
    • Signs: distended abdomen, palpable loops of bowel, rectal exam without stool in ampulla
  • 16. Diagnostic Workup/ Dilemmas
    • Abdominal X-ray
    • Contrast enema- demonstrates the retained meconium as a filling defect or plug.
      • Must done in an “unprepped patient”
      • MPS diagnosis of exclusion: enema findings in neonatal Hirschsprung disease can be indistinguishable from meconium plug syndrome
    • Rectal suction biopsy-risk of perforation, bleeding
    • Full thickness biopsy
  • 17. Meconium Plug Obstruction: Retrospective Case Review 21 patients with Large Bowel Obstruction Relieved by Passage of Meconium Plugs Conclusion: Essential for all babies with MP obstruction to have HD excluded. Burge, D. Meconium Plug obstruction. Pediatric Surg Int(2004) 20:108-110 2 8 6 Bilious emesis 4 8 7 Abdominal Distention 3403 3363 3369 Mean BW (gms) 37 39 37 Mean gestation (wks) 4 (19%) 8 (38%) 9 (43%) Number SLCS HD MPS Diagnosis
  • 18. Diagnosis of Hirschsprung’s Disease: a prospective, comparative accuracy study of common tests 111 Infants suspected of HD Anal Manometry 83% sensitive 93% specific Rectal Suction Biopsy 93% sensitive 100% specific Contrast Enema 76% sensitive 97% specific Conclusion: Rectal Suction Biopsy is the most accurate test for diagnosing HD, with lowest rate of inconclusive results.
    • De Lorijn, et al. “Diagnosis of Hirschsprung’s disease: a prospective, comparative accuracy study of common tests” J. Pediatrics. 2005, 146 (6): 787-92.
  • 19. Management
    • Primary pull-through procedure
      • Soave (endorectal) procedure
      • Swenson procedure
      • Duhamel procedure
    • Early colostomy with resection of aganglionic segment & Re-establishment of continuity
  • 20. References
    • Diament, M. Emedicine. “Meconium Plug Syndrome.” 3/05.
    • Hekmatnia, Ali. Emedicine. “Meconium Ileus.” 7/05.
    • De Lorijn, et al. “Diagnosis of Hirschsprung’s disease: a prospective, comparative accuracy study of common tests” J. Pediatrics. 6/05, 146 (6): 787-92.
    • Gomella, et al. Neonatology: Management, Procedures, On-Call Problems, Diseases, and Drugs . 2004.
    • Behrman, et. Al. Nelson Textbook of Pediatrics: 17 th edition. 2004
    • Lee, Stephen. Emedicine. “Hirschsprung disease.” 8/05.
    • Burge, D. Meconium Plug obstruction. Pediatric Surg Int(2004) 20:108-110
  • 21. Case Management Conference Jaime Lanzillotta, DO November 29, 2005
  • 22. D.C. 6 month old female with Bilious Emesis
    • HPI: Ex-23 week premature female
    • 1 day h.o. initial nonbilious emesis (4-5 episodes) (yellow in color & occurred after each feed)
    • Decreased wet diapers, decreased activity
    • Normal intake-4 ounces q2-3 hours
    • Normal stools
    • Temperature 99 °
  • 23. HPI, cont’d.
    • Admitted 1 week prior with similar symptoms:
    • Diagnosed with partial small bowel obstruction.
    • Decompressed with NGT and feeds were re-started.
    • Discharged home on full feeds.
  • 24. Past Medical/Surgical Hx
    • 23 weeks premature
    • Chronic lung disease-home O2
    • Necrotizing enterocolitis-s/p bowel resection, ileostomy & bowel re-anastomosis
    • Retinopathy of prematurity-s/p laser
    • Patent ductus arteriosis - s/p ligation
  • 25. History, Continued
    • Allergies-NKDA
    • Family History-non-contributory
    • Immunizations: UTD
    • Meds: Poly-vi-sol, Calcium, Phosphorus
  • 26. Physical Exam
    • T 38.3 (ER) 37.3 (peds) P 175 RR 68 Sat 99% on 0.1L NC
    • General: Awake, active, no distress
    • Lungs: Increased upper airway transmitted sounds; mild subcostal retractions
    • Abd: Distended, non-tender, + bowel sounds, reducible ventral hernia, Ø masses
    • Ext: warm, well perfused, cap refill <3 seconds
  • 27. Imaging and Laboratory Studies
    • Lumbar Puncture
        • glucose 93
        • protein 39
        • 0 WBC /325 RBC
        • Lactate 1.7
        • Gram stain negative
    • Urine -, Rota-, RSV-
    Nonspecific film: No air fluid levels Dilated loops of bowel present 4.3 23 .3 141 108 15 150 N 76 L 16 M 7.5 9.5 11.6 35 421
  • 28. Assessment/ Plan
    • 6 month old ex 23 week premature female with bilious emesis, rule out bowel obstruction
    • Plan:
      • IV Ceftriaxone
      • NG Tube decompression
      • Surgical consult
      • Serial abdominal exam
      • NPO
      • Guiac stools
      • NG tube output replacement
  • 29. Hospital Course
    • HD#1
      • increasingly irritable
      • Mom reported change in activity and behavior to staff
    • HD #2
      • Increased abdominal distension becoming more tense
      • Poor perfusion
      • Repeat x-ray was ordered –showed signs of obstruction, with air fluid levels
      • Transferred to the PICU for presumed obstruction and signs of shock
      • Intubated and taken to the OR emergently
  • 30. Hospital Course, cont’d.
    • OR course:
    • closed loop bowel obstruction
    • large areas of ischemic bowel- no resection
    • abdominal compartment syndrome
    • multiple adhesions-lysed
    • Transferred back to the PICU
  • 31. PICU course: post op
    • Resp:
      • intubated until POD #7.
      • Weaned to nasal cannula.
      • lasix prn for fluid retention.
      • albuterol & flovent.
    • CV:
      • Stable; Negative echo
    • ID:
      • Broad spectrum antibiotics
      • E.coli bacteremia
    • Heme:
      • Anemia, thrombocytopenia
      • PRBC, platelet transfusions
    • FEN:
      • TPN x 2 weeks.
      • NG feeds 1 wk post-op
      • Advanced to full nipple feeds
      • Metoclopramide
    • Neuro:
      • sedated for intubation
  • 32. Post-operative film Multiple air fluid levels Dilated loops of bowel
  • 33. Hospital course, continued
    • HD # 26
    • Transferred back to pediatric floor
    • Hospital course on B3:
    • Tolerated full feeds
    • Intermittent abdominal distension with stable x-rays
    • Discharged home after 5 days.
    • Readmitted 1 week after discharge, with fever, r/o SBI
  • 34. Key Points
    • Irritability in an infant with changing clinical exam warrants further investigation.
    • What are the signs/symptoms of a closed loop bowel obstruction vs. partial bowel obstruction?
    • Could a different diagnostic test have been performed to detect closed loop obstruction?
    • Repeat examinations by surgical team is essential.
    • Follow clinical judgement especially with changing exam/history.
  • 35. Differential Diagnosis of Small Bowel Obstruction in Infants
    • Intussusception
    • Incarcarated hernias
    • Malrotation with midgut volvulus
    • Postoperative adhesions
    • Annular pancreas
    • Mesocolic hernia
    • Necrotizing enterocolitis
    • Cecal volvulus
    • Duplication cysts
  • 36. Types of Obstruction
    • Simple -blocked in 1 place
    • Closed-loop-blocked in 2 places
    • Strangulated -Decreased blood flow
    • Incarcerated -When obstruction is not relieved and bowel becomes necrotic
  • 37. Closed Loop Bowel Obstruction 2 sites of bowel obstruction
  • 38. Pathophysiology of Small Bowel Obstruction Obstruction Accumulation of chyle, salivary,gastric, biliary,pancreatic & intestinal secretions Peristaltic contractions There is also: Impaired perfusion Ischemia/necrosis Perforation
  • 39. Pathophysiology of Small Bowel Obstruction
  • 40. Clinical Features of Bowel Obstruction
    • Colicky abdominal pain
    • Irritable, fussy or inconsolable
    • Decreased activity
    • Vomiting (bilious in proximal obstruction, feculent in distal obstruction)
    • Anorexia
    • Diarrhea
    • Constipation (complete obstruction)
    • Fever (with bowel strangulation/necrosis)
  • 41. Diagnostic Work Up
    • Plain abdominal film-flat and upright
    • Upper GI series
    • Ultrasound
    • CT
    • Labs: CBC, electrolytes, stool guiac
  • 42. Closed Loop Obstruction
    • Diffuse abdominal tenderness
    • Increased irritablility in an infant
    • Absence of bowel sounds
    • Fever
    • Tachycardia
    • Leukocytosis
    • Acidosis
    • Blood in stool
    These clinicial features are non-specific and may NOT be present even when ischemia and necrosis is occurring
  • 43. Diagnosis of Closed Loop Bowel Obstruction with CT
    • 19 cases of closed loop obstruction imaged with CT & x-ray
    • ABDOMINAL X-RAY CT
    • Non-specific findings of SBO Signs of closed loop in 8
    • in 10 pts pts
    • Finding specific to closed Signs of closed loop &
    • bowel loop obstruction in 1 pt strangulation in 7 pts
    Closed Loop and Strangulating Intestinal Obstruction: CT Signs.Radiology 1992,185:769-775 Conclusion: CT is a promising modality for diagnosis of closed-loop and strangulating small bowel obstruction