1 / 73

MEGACOLON

MEGACOLON. VIKAS.K.M 2002 MBBS. MEGACOLON. DEFINITION Distention of the colon to greater than 6 or 7 cm in diameter. HIRSCHSPRUNG’S DISEASE. CONGENITAL. HIRSCHSPRUNG’S DISEASE

marinat
Download Presentation

MEGACOLON

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  1. MEGACOLON VIKAS.K.M 2002 MBBS

  2. MEGACOLON • DEFINITION Distention of the colon to greater than 6 or 7 cm in diameter

  3. HIRSCHSPRUNG’S DISEASE

  4. CONGENITAL HIRSCHSPRUNG’S DISEASE • Neurogenic form of intestinal obstruction in which there is an absence of ganglion cells in the myenteric & submucosal plexus • 1 in 4500 • Sex ratio 4:1 Harald Hirschsprung (1830-1916)

  5. HIRSCHSPRUNG’S DISEASE GENETICS • Hetrogeneous • Mutations RET gene & RET ligands Endothelin receptor system • 3-5% have down’s syndrome

  6. HIRSCHSPRUNG’S DISEASE • Hydrocephalus VSD Meckel’s diverticulum • Definite family history

  7. PATHOLOGY • FAILURE OF MIGRATION of neuroblasts into the gut from vagal nerve trunks • ABSENCE of ganglion cells in neural plexus • HYPERTROPHY of nerve trunks

  8. MACROSCOPICALLY • The affected segment is NOTDISTENDED • Properly innervated upstream segment DILATES • Wall may be thinned or thickened • Stercoral ulcers

  9. HIRSCHSPRUNG’S DISEASE

  10. HIRSCHSPRUNG’S DISEASE Dilation of bowel proximal to the affected region

  11. HIRSCHSPRUNG’S DISEASE

  12. TOTAL COLONIC HIRSCHSPRUNG’S DISEASE The transition zone (arrow) is in the small intestine

  13. STERCORAL ULCERS

  14. MICROSCOPICALLY ABSENCE OF GANGLION CELLS

  15. CLINICAL FEATURES • Delayed passage of meconium(95%) • Abdominal distension • Bilious vomiting • Severe diarrhoea altrenating with constipation(10-15%) Enterocolitis of hirchsprung’s disease

  16. DIAGNOSIS

  17. DIAGNOSIS ABDOMINAL RADIOGRAPH • Dilated bowel loops with fluid levels • Intramural gas – enterocolitis • Free peritonial gas - perforation

  18. ABDOMINAL RADIOGRAPH Dilated bowel loops Fluid levels

  19. DIAGNOSIS BARIUM ENEMA • Indicate length & site • No definitive cutoff point indicating transition zone • Evacuation of contrast may take 24 – 48 hours • Transition zone clear on delayed x-ray

  20. BARIUM ENEMA • Coning down of transition zone • Irregularity in the mucosa • Abnormal contractions TZ

  21. BARIUM ENEMA Contracted diseased segment (black arrow), dilatation of normal bowel segment (red arrow) and the transitional zone (TZ)

  22. BARIUM ENEMA TZ

  23. DIAGNOSIS RECTAL BIOPSY • Submucosal suction biopsy is adequate in 90% • Full thickness operative biopsy in more emergent circumstances • Absence of ganglion cells in at least 10 sections – diagnosis confirmed • Increased Ach staining of neurofibrils

  24. RECTAL BIOPSY

  25. Ach STAINING NORMAL INSCREASED Ach STAINING

  26. DIAGNOSIS ANORECTAL MANOMETRY • Measures anorectal intraluminal pressure • Absent rectoanal inhibitory reflex indicating a lack of relaxation of the internal sphincter characteristic of aganglionosis

  27. ANORECTAL MANOMETRY

  28. DIFFRENTIAL DIAGNOSIS • HYPOTHYROIDISM • MECONIUM PLUG SYNDROME • COLONIC NEURONAL DYSPLASIA • ADYNAMIC ILEUS WITH SEPSIS • INTESTINAL PSEUDO-OBSTRUCTION

  29. TREATMENT

  30. TREATMENT • Depends on Age Length of involved segment Severity of symptoms Presence of enterocolitis

  31. TREATMENT NEONATAL PERIOD • TEMPORARY DECOMPRESSING COLOSTOMY • At least 10 cm proximal to transition zone

  32. COLOSTOMY

  33. TREATMENT 6 MONTH – ONE YEAR A definitive pull-through procedure using • SOAVE(endorectal) • DUHAMEL(retrorectal) • SWENSON(rectosigmoidectomy)

  34. PULL-THROUGH PROCEDURE • Each is done a little differently, but all involve removing the part of the intestine that isn't working and connecting the healthy part that's left to the anus. After pull-through surgery, the child has a working intestine

  35. SOAVE PROCEDURE

  36. DUHAMELPROCEDURE

  37. SWENSON PROCEDURE

  38. OPERATIVE FINDING OF TRANSITION ZONE

  39. PROGNOSIS • Overall survival in > 90% cases • Rare deaths due to – Delayed diagnosis Complications • > 96% continent • Long term follow up is important

  40. ACQUIRED MEGACOLON

  41. ACQUIRED MEGACOLON CAUSES • Chagas disease • Organic obstruction of bowel • Toxic megacolon • Fuctional psychosomatic disorder

  42. ACQUIRED MEGACOLON • Can occour at any age • Except for chagas disease,where inflammatory involvment of ganglia is evident,the remaining forms are not associated with deficiency of mural ganglia

  43. CHAGAS DISEASE • Protozoosis • Flagellate protozoa Trypanosoma cruzi • Destruction of the autonomic nervous system innervation of the colon leads to a loss of the normal smooth muscle tone of the wall and subsequent gradual dilation REDUVID BUG

  44. MEGACOLON IN CHAGAS DISEASE

  45. TOXIC MEGACOLON • DEFINITION Toxic megacolon is the clinical term for an acute toxic colitis with dilatation of the colon • Total or segmental • Hallmarks - nonobstructive colonic dilatation larger than 6 cm and signs of systemic toxicity

  46. TOXIC MEGACOLON TOXIC MEGACOLON

  47. TOXIC MEGACOLON Colon is dilated and shows hemorrhagic necrosis

  48. TOXIC MEGACOLON CLASSIC ETIOLOGIES • Ulcerative colitis • Crohn colitis • Pseudomembranous colitis Ulcerative colitis Crohns disease Pseudomembranous colitis

  49. INFECTIOUS CAUSES Salmonella species Shigella species Campylobacter species Yersinia species Clostridium difficile Entamoeba histolytica Cytomegalovirus OTHER CAUSES Radiation colitis Ischemic colitis Nonspecific colitis secondary to chemotherapy TOXIC MEGACOLON

More Related