Management and surgical procedures of Hirschsprung disease

Hirschsprung disease

1. Management and Surgical procedures of HD Dr S asidhar Moderator: Dr Babu Rao

2. Anatomy of anal canal

3. The three loops of the external anal sphincter. Continence depends on the preservation of at least one of the three. Some subcutaneous muscle fibers encircle the anus; some attach to the perianal skin anteriorly at C.

4. Diagram of the extrinsic muscles of the surgical anal canal. (1) Coccyx; (2) pubis; (3) levator ani muscle; (4) puborectalis muscle; (5) deep external sphincter; (6) superfi cial external sphincter; (7) subcutaneous external sphincter; (8) anococcygeal ligament; (9) anal verge; (10) rectum.

5. The interior of the anal canal showing the rectal columns, anal valves, and anal sinuses (crypts). They form the pectinate line The pectinate line is the most important landmark in the anal canal. It marks the transition between the visceral area above and the somatic area below. The arterial supply, the venous and lymphatic drainage, the nerve supply, and the character of the lining all change at or very near the pectinate line

6. Management and surgical procedures of HD

7. Surgical interventions – Historical Perspective Drs. Swenson, Neuhauser (a radiologist) and Pickett Boston 1948 Recognized an area of spasm in the rectum or Rectosigmoid that defined the site of obstruction in patients with congenital megacolon using a barium enema and fluoroscopy Swenson & Bill 1948 First successful operative procedure. The operation was undertaken based on careful clinical observations and thoughtful deduction ignoring the controversy at the time regarding the influence of bowel innervation and the presence or absence of ganglion cells in this disorder State (Minneapolis, Minnesota) 1952 Described low anterior resection Sandegard 1953 Reported the first successful operation in a patient with total colonic aganglionosis (TCA) by performing a total colectomy and an ileoanal anastomosis Bernard Duhamel (St Denis, France.) 1956 Described the retrorectal transanal pull-though procedure.

8. Surgical interventions – Historical Perspective Rehbein (Bremen, Germany) 1958 Low anterior resection taking the anastomosis down to 3–4 cm above the pectinate line Grob (Zurich, Switzerland ) 1959 Used a different location for the posterior incision. He made the incision 2.0–2.5 cm above the pectinate line. Pagès in Paris & Duhamel 1960 Rectal incision 1.5 cm above the pectinate line to avoid incontinence and constipation Soave of Genoa, Italy 1963 Described the endorectal pull-through procedure Boley (New York). 1964 Modified the procedure by performing a primary anastomosis at the time of the pull-through procedure Martin 1968 Described a modification of the Duhamel procedure utilizing a side-to-side anastomosis between normally innervated small bowel and aganglionic colon upto the level of splenic flexure for TCA

9. Surgical interventions – Historical Perspective Kimura 1981 used an aganglionic right colon patch. Boley 1984 Left colon as patch So et al 1981 First to report a one-stage pull-through procedure in neonates with HD without a preliminary colostomy Ziegler 1987 described the concept of myotomy / myectomy of aganglionic bowel for patients with near total aganglionosis (NTAG) with less than 40 cm of normally innervated small bowel Georgeson et al 1995 Described a laparoscopically assisted Soave endorectal pull-through procedure avoiding an open laparotomy De la Torre- Mondregon & Ortega-Salgado of Mexico 1998 First to perform a one-stage totally transanal pull-through procedure

10. Surgeries available for Hirschsprungs Disease Rectal Biopsy – submucosal , full-thickness Colostomy , Ileostomy Definitive surgeries -Swenson’s procedure -Duhamel’s procedure -Soave’s procedure - Rehbein , State – outdated

11. Minimal Access Approaches - LAPAROSCOPIC PULL-THROUGH -TRANSANAL (PERINEAL) PULL-THROUGH -Ultra short - Sphincterotomy -Short - Extended myomectomy - Total colonic aganglionosis -Martin Duhamel -Kimura Stringel -Extended myotomy / Myomectomy

12. Rectal suction biopsy Less than 4 years of age May be performed in ward or clinic without anaesthesia Painless if taken 2.5 cm above anal verge in neonate and 3.5 cm in older child Preoperative care Gentle bowel washout with 10ml/kg of warm saline Vitamin k in neonates Original suction biopsy instrument was devised by Helen Noblett in 1969 Variants: Solo-RBT, rbi2 Blunt ended tube with a 3mm side hole 2 cm from the tip Lithotomy position for infants Left lateral knees bent position for older children

13. Lubricated instrument inserted into the anus and side hole positioned at 3 cm from anal verge This is the minimum distance and avoids the normal hypoganglionic zone and diagnostic confusion Side hole should face posterior wall or lateral wall of rectum Suction applied by withdrawing the syringe attached to the suction biopsy instrument to 3-5ml (~150cmH2O) After 2-3 seconds the knife is triggered

14. Syringe suction is released to neutral pressure before removing from the patient. Instrument is withdrawn and specimen removed 3X1mm Submucosa- whitish layer Procedure repeated at 3.5cm and 4cm above the anal verge 2-4 specimens are collected Specimens marked with the level of collection

15. Postoperative care A rectal examination should be carried out after completing the biopsy to exclude active bleeding . Observations should be continued for at least 2 hours, to ensure complete hemostasis . Complications Inadequate specimen retrieval. 10 and 20 % Perforation. Bleeding . Pelvic sepsis .

16. Solo RBT * Solo-RBT : A New Instrument for Rectal Suction Biopsies in the Diagnosis of Hirschsprung’s Disease, A . Pini Prato, G. Martucciello, and V. Jasonni Genova, Italy. Journal of Pediatric Surgery, Vol 36, No 9 (September), 2001: pp 1364-1366

17. Open rectal biopsy under general anesthesia is required when the specimen obtained with the RSB instrument is inadequate or the child is older . Preoperative preparation: Rectum irrigated with saline or very dilute povidone-iodine solution. Position: The infant is held in the lithotomy position while an older child will need to be placed in stirrups in the lithotomy position . Prophylactic antibiotics are given . Open rectal biopsy Procedure The anal orifice is digitally dilated. It is held open either with a Parks’ retractor (or similar self-retaining retractor) or by an assistant holding two small Langenbeck’s retractors .

18. The superior aspect of the dentate line is identified and marked with a polyglactin suture (3/0), which is used for traction. Two additional polyglactin sutures are placed on the posterior wall of the rectum at 1 and 2 cm proximal to the dentate line. Retain the needle on the most cephalad of these sutures, as it can be used to begin the closure of the defect after the biopsy is obtained.

19. hold the middle suture. Using sharp curved scissors, a full-thickness incision is made along the lower half of the rectal wall, between the dentate line and the middle suture. The rectal defect is closed in a single, full thickness running or interrupted layer with an absorbable suture (e.g. 4/0 polyglactin ( Vicryl )

20. Hemostasis may be achieved with bipolar diathermy or, more usually, by suturing the defect with a running locking suture from above. Complications Hemorrhage Infection

21. New born with delayed passage of meconium Enterocolitis Older infants/children with constipation (+or-)Rectal biopsy (suction/full thickness) Urgent decompression by rectal irrigation +/_ colostomy Fluid resuscitation NG aspiration Antibiotics Plain X ray Contrast enema Primary pull through Leveling colostomy R. Transverse colostomy Three stage procedure Two stage procedure Frozen section facility Anorectal manometry for RAIR (screening)

22. Three stage procedure Advantages Colostomy can be made even if transition is not evident & frozen section facility is not available Anal anastomosis is well protected by colostomy The risk of the error of opening the colostomy in an aganglionic area is much reduced Disadvantages Multiple admissions & operations Molibilisation of colostomy is needed in cases with long segment HD Distal segment may undergo disuse atrophy if left long or child lost for follow-up May not function properly after colostomy closure

23. Two stage procedure Initial colostomy is performed at the junction of the ganglionic & aganglionic colon (Leveling colostomy) Second stage – pull-through done after taking down the stoma & using the proximal end for pull-through 6 – 12 months age

24. Levelling Colostomy Indications Severe enterocolitis Markedly dilated proximal colon due to delayed diagnosis Advantages Allows determination of aganglionic level- facilitating subsequent pull- through Allows the proximal bowel to grow Colostomy closed during pull-through thus avoiding a 3 rd operation Max. amount of colon for absorption Assures colostomy is in functioning bowel Avoids risk of second pull through.

25. Leveling colostomy – Disadvantages No benefits of protective colostomy Length of colon mobilized and removed may be longer O peration technique Colostomy performed at the level of normally innervated ganglion cells as ascertained on frozen section i.e., just proximal to the transition zone.

26. Leveling colostomy – operation technique Preoperative preparation Rectal washouts Broad-spectrum, intravenous antibiotics just prior to incision No formal bowel preperation is required or effective Incision Oblique incision in left lower quadrant If level of aganglionosis is not readily apparent, incision can be extended transversely across the midline .

27. Leveling Colostomy In general, a 1 × 0.5-cm biopsy specimen is taken and interrupted silk or polyglactin sutures are placed to close the biopsy site

28. Leveling colostomy - Postoperative care The stoma usually begins to function within 24 hours, and feeding can begin shortly thereafter. It is occasionally helpful to perform intermittent dilatations of the proximal ostomy to prevent narrowing of the opening and allow the dilated proximal colon to return to normal size.

29. Surgical procedures Rectosigmoidectomy (Swenson and Bill -1948) Retrorectal – transanal approach (Duhamel -1956) Endorectal procedure (Soave -1964). Laparoscopic assisted transanal endorectal pull-through (LATEP) – Georgeson et al(1990’s) Transanal endorectal pull-through (TERPT) - De la Torre and Ortega-Salgado and Langer et al in the late 1990s. The basic principle of all the procedures is to bring the ganglionic bowel down to the anus

30. Schematic illustrations of several operative pull-through techniques for the treatment of Hirschsprung disease.

31. Preoperative assessment during definitive surgery in staged surgery History & physical examination Records of operative findings, procedures done, HPE reports X-ray abdomen – to identify any fecaloma Distal cologram - to outline the adequacy of the distal bowel for pull-through without the need to mobilize the colostomy. (if initially right Transverse colostomy )

32. Preoperative preparation for pull-through First generation cephalosporin or ampicillin + gentamycin or amikacin + metronidazole started 24hrs prior to surgery Mechanical bowel preparation Twice daily rectal washes with NS starting at least a week or two prior to surgery/ Distal colostomy washouts Low residue diet for 3days prior to surgery Gut irrrigation using polyethylene glycol solution (70ml/kg) on the day prior to surgery On the morning of the surgery a rectal flatus tube is inserted to ensure complete evacuation of colon.

33. Swenson’s pull hrough Surgical principle is to remove the diseased portion of the bowel that is a aganglionic distal rectum & anastomosing normal colon to lower rectum to maintain the continuity to allow normal defecation Orvar Swenson, MD, 1909-2012 The first ever pull through procedure described for treating HD & published by Swenson & Bill in 1948

34. Swenson’s Pull-through Initially anastomosis used to be completed at the anorectum (2cm from dentate line)  High incidence of enterocolitis (early 16%, late 27%). Modified by Resecting virtually whole of the posterior rectal wall (very top aspect of IAS) Making an oblique anastomosis leaving 1.5 to 2cm anteriorly, 1 to 1.5cm on both sides & 0.5 to 1cm posteriorly from dentate line

35. Swenson’s Pull-through Position Should provide simultaneous exposure of the perineum & abdomen Lithotomy position Pelvis is allowed to drop back over the lower end of the table & legs are strapped over the sandbags/ stirrups. Foley catheter is inserted into the bladder

36. Swenson’s Pull-through Anal dilatation – slow & gentle anal dilatation Incision Oblique /Modified hockey stick incision in LLQ incorporating the colostomy. Colostomy mobilised Denis Browne retractor applied Urinary bladder is lifted forward out of abdomen by stay sutures Sigmoid colon is mobilised by dividing sigmoid vessels & retaining the marginal vessels It may be necessary to mobilise the splenic flexure to obtain adequate length.

37. Swenson’s Pull-through Bowel is divided at the rectosigmoid junction & removed Peritoneum is divided around its lateral & anterior reflection from the rectum exposing the muscle coat of rectum Dissection extends around the rectum, keeping very close to the bowel wall. to prevent damage to the pelvic splanchnic innervation .

38. Swenson’s Pull-through Dissection should commence from side to side & in an anterior to a posterior direction with the rectum being gradually dissected out in a circumferential manner. Dissection is done extensively posteriorly & on both sides but less anteriorly

39. Swenson’s Pull-through The mobilized rectum is intussuscepted through the anus by passing a curved clamp or a Babcock forceps

40. Swenson’s Pull-through An incision is made anteriorly through the rectal wall about 2 cm from the dentate line, extending halfway through the rectal circumference (9 to 3o’clock) A clamp is inserted through this incision to grasp multiple sutures placed through the cut end of the proximal colon

41. Swenson’s Pull-through The normal colon is pulled through the pelvis and out through the incision in the everted rectum. An outer layer of interrupted 4-0 absorbable sutures is placed through the cut muscular edge of the rectum and the muscular wall of the pull-through colon.

42. First the anterior muscular anastomosis is completed. Incision on the rectum is extended 1-1.5cm from dentate line on both sides & 0.5 – 1cm posteriorly Through the diagonal excision of the rectum, the internal sphincter is preserved to maintain fecal continence & partial sphincterectomy is done in posterior direction to reduce the risk of post-op HAEC With traction on the three o'clock and six o'clock sutures, the left posterior quadrant of the muscular anastomosis is done. In a similar manner, the right posterior quadrant of the anastomosis is completed

43. Swenson’s Pull-through When the outer layer (muscular) anastomosis is completed, the end of the pulled-through colon is incised a few millimeters from the muscular suture line. The mucosa of the cuff of the everted rectum is anastomosed to the mucosa of the pulled-through colon ( interrupted 4-0 absorbable sutures). When anastomosis is completed, the sutures are cut, allowing the anastomosis to retract within the anus.

44. Post op care keep the infant on total parenteral nutrition for 7 days postoperatively and then gradually start oral feeds. The urethral catheter is removed after 3 days. Antibiotics are discontinued after 5 days. Rectal examination is performed 2 weeks later during an outpatient visit.

45. Experience with Swenson’s procedure Mortality The mortality after Swenson’s operation is reported to be 0–5.8% Postoperative mortality is considerably increased in patients in whom anastomotic leaks occur Significantly higher in babies with Down’s synd Bowel control Bowel control increases with age A followup of 5 years is required for complete evaluation Best predictors of abnormal bowel habits by sherman et al are – temporary soiling after discharge and rectal stricture.

46. Experience with Swenson’s procedure constipation is the most common late complication Soiling Temporary soiling after discharge was the only factor which influenced the occurrence of soiling at follow-up Influence of Trisomy 21 on Bowel Control Studies show 10-15% of association More than 3/4 th of patients with Down’s synd had completely unsatisfactory outcome. Attainment of normal post op defecation is dependent on – intensity of bowel training, social back ground, intelligence of child and motivation to be socially clean which are lacking in these children.

47. Persistent Bowel Symptoms due to Associated Intestinal Neuronal Dysplasia HD is associated with intestinal neuronal dysplasia (IND) in about 25% of patients Patients have constipation, enterocolitis or soiling. Rectal stricture 9.5% after swenson’s procedure, 4.3% required operation, higher incidence is seen in patients operated before 4 months of age. Fistulae were rare Other rare complications are – intestinal obstruction, urinary incontinence, sexual dysfnction Enterocolitis remains the most serious complication .

48. Duhamel’s procedure Principle of Duhamel’s technique is to exclude the rectum instead of removing it

49. Duhamel operation Bernard Duhamel first described his operation for Hirschsprung‘s disease in 1956. The procedure consisted of a retrorectal dissection, whereby a significant portion of the aganglionic rectum was preserved and anastomosed to ganglionated proximal colon. The advantages of this procedure included Ease of performance, Reduction of anastomotic leaks and strictures, Retention of anal sensory receptors, and Preservation of the nervi erigentes .

50. Duhamel operation Operative principles Minimal pelvic dissection Retrorectal approach for the pulled-through colon to anal opening Partial disruption of internal anal sphincter posteriorly Preservation of the anterior wall of the rectum & its nerve supply Elimination of colo -rectal septum with wide side-to-side anastomosis (stapled/crushed) between the anterior aganglionic & pulled down ganglionic colon

51. Modifications In 1956 1 st description - Anal transverse incision was made at the ano-cutaneous junction- high anal incontinence & prolapse In 1959 - Grob modification- post. anal wall incision 2-2.5cm above anocutaneous junction- to preserve IAS – high incidence constipation & fecalomas In 1960,Duhamel-Pages modification - Anal incision 1-1.5cm above pectinate - preserves 1/3 rd of IAS- less soiling & less constipation.

52. Modifications Centered around the elimination of the common wall of the rectal pouch spur 1964 Zachary & Lister - O shaped clamp 1966 Talbert - Suturing & stapling device 1987 Steichen - EEA Martin & Caudill – End-to-side anastomosis

53. Duhamel procedure. Operation generally done when the child is 6-12 months age & wt 10kg Prior to surgery rectal examination should be done to ensure rectum is empty Child placed in lithotomy position with proper padding Child cleaned, draped & catheterized Incision – muscle cutting hockey stick incision is given

54. Duhamel procedure. Colostomy mobilised & dismanteled , transected Alternatively the colostomy site is removed with an automatic stapling device. Proximal colon closed with a running silk suture 4 stages Mobilisation of upper colon & closure of the rectum Preparation of retrorectal space Endoanal incision Retrorectal pull-through procedure

55. Duhamel procedure - Mobilisation of colon Proximal mobilisation is done- it can be brought easily to below the pubic symphysis May sometimes need division of left colic artery/IMA at aortic root Marginal artery of Drumond should be preserved so that colon viability is based on the left branch of middle colic artery

56. Duhamel procedure – Closure of rectum Peritoneum over the mesosigmoid is incised both laterally & medially, joined anteriorly in the colo-vesical pouch Both the ureters are identified Colon mobilised distally upto the level of pelvic peritoneum The rectum is transected just above the pelvic peritoneum (and closed).

57. Duhamel procedure- preparation of retrorectal space Opening of the mesorectum provides access to the retrorectal space The blunt dissection is carried out with index finger/ long curverd forceps with a small sponge/ long kelly’s clamp The dissection is carried down to the pelvic floor so that the assistant’s finger can be felt when inserted no further than 1 – 1.5cm into the anus

58. Duhamel procedure- Endoanal incision A semicircular incision (posterior 180 ) is made on the rectal wall between silk stay sutures placed at 3, 6 & 9 O’ clock positions The incision should be 0.5 – 1.0 cm proximal to the dentate line .

59. Retrorectal pull-through The kelly’s clamp/long curved forceps with sponge is passed from abdomen through the retrorectal space The sponge is grasped with another long curved forceps through the incision Using sponge as a guide, forceps is drawn in a retrograde direction into the abdomen

60. Duhamel procedure. The proximal colon is grasped with the forceps and drawn downward into the retrorectal space to the level of endoanal incision Care should be taken that bowel is not twisted, not in tension, mesentry to be directed posteriorly in sagittal plane.

61. Duhamel procedure. Anastomosis technique Two long kocher clamps are inserted with one blade in the rectum & other in the drawn colon The bases of the clamps are held apart so that Their end points meet in an inverted V at the apex of the rectal pouch The position of the clamps is checked by direct abdominal palpation

62. Duhamel procedure. The walls of the colon & rectum deprived from circulation necrotize & the clamps with necrotic tissue slides out of the anus (after 4 – 10 days) By this way a large enterotomy is carried out with a longitudinal anastomosis between the drawn colon & native rectum Disadvantages – The protrusion of the handles of the clamps through the anus-inconvenient to the child. Incomplete division of the colo -rectal septum Occurrence of stenosis

63. Alternative method The proximal end of the rectal stump is kept open A longitudinal colotomy is made on the pulled down colon opposite the open rectal stump GIA linear cutter is inserted through the anus with one blade in the rectum & other in pulled down colon.

64. Alternative method Care to be taken that the entire length is included and the tip of the stapler is protruding well beyond the rectal stump & colostomy in abdomen The stapler is then fired to complete the longitudinal anastomosis & division of the common wall. In case the entire common wall is not divided, the same needs to be completed by another firing with the stapler now applied from the abdominal end in prograde fashion.

65. Duhamel procedure. The open proximal end of the rectal stump is anastomosed to the colostomy on the pulled down colon in end-to-side fashion … Martin’s modification The anastomosis is extraperitonealized after placing a small pelvic drain of CRD Peritoneum on both the medial & lateral aspects of pulled down colon is repaired Abdominal wound closed in layers Rectal pack of paraffin gauze is applied, removed after 24hrs

66. Complications and results Anastomotic leak – 2.2% Necrosis – 0.09% Stricture – 0.7% Mortality rate – 1.6% Majority of post op deaths are related to enterocolitis (incidence – 5-26%) Incontinence – 0-20% Constipation and fecal impaction due to larger reservoir – 5-8% Lower UTI, ? Partial detrussor denervation

67. Primary vs staged Duhamel’s Limited studies are available. Laparoscopic Duhamel’s procedure Laparoscopic techniques have mainly been described for Swenson’s procedure and less frequently for Duhamel’s. Bowel biopsies are taken laparoscopically to determine the extent of aganglionosis before ablation. Further steps – laparoscopic mobilization, dissection and closure of rectal stump and laparoscopically controlled pull through.

68. Postulated advantages Visualization Atraumatic dissection Less post op pain Faster recovery Duhamel’s procedure for re-do pull through is Indicated in patients with Recurrent fecoloma formation Recurrent episodes of enterocolitis Retained aganglionosis Segmental bowel dysfunction with bowel dilatation

69. Soave’s ( Boley Scot) Endorectal Pull-through The operation based on removing the mucosa and submucosa of the rectum and pulling ganglionic intestine through the aganglionic muscular cuff By remaining within the muscular cuff of the aganglionic segment, important sensory fibers and the integrity of the internal sphincter are preserved. Avoids injury to the pelvic nerves

70. Indications Hirschsprung’s disease Multiple Juvenile polyposis Ulcerative colitis Familial poliposis . History 1955 Roumaldi proposed it at Roman society of surgery 1957 Soave used it for ARM with fistula & in 1961 he performed this tech for HD. 1964 Boley modification- Primary anastomosis

71. Coran & Weintraub modification – eversion of submucosal -mucosal tube onto the perineum to facilitate anastomosis Rintala & Lindahl - Transanal approach combined with an open laparotomy for mobilization of aganglionic segment Georgeson - Lap assisted mobilization of aganglionic segment Torre-Mondragon & Ortega-Salgado– entire dissection & mobilisation via transanal route

72. In Original Soave procedure the pulled through colon is left hanging beyond the anal verge. After a period (~ 2 wks) to allow adherence of the bowel to the anal tissues,the protruding segment was resected No anastomosis. Boley (1964)- performed a primary anastomosis at the anus. Endorectal Pull-through

73. Endorectal Pull-through Preparation Upto 1980’s - >5 months & weight > 8 kg If soave’s procedure is performed with in 3 months of life, chronic inflammatory process of rectum (because of chronic proctitis leading to tenaceous adhesions on submucosal layer) are generally avoided. Procedure does’t require any protective colostomy Aim- achieve radical treatment with out contaminating the operative field Colostomy is indicated if – acute enterocolitis / intestinal obstruction Colostomy is done in the most distal part of the ganglionic colon  leveling colostomy. In order to achieve this it is necessary to perform multiple seromuscular biopsies intra operatively.

74. Preparation starts from one week before the procedure to reduce complications Rectal probing should be repeated and alternated with evacuating enemas. Perioperative IV antibiotic prophylaxis is started 1 hour before surgery.

75. Soave’s – operative tech General anesthesia Catheter placement Performing wide dilatation of the anal canal using two fore fingers – essential for sucessful pull through procedure. Supine position with buttocks lying at the extreme edges of operating table and legs hanging freely wrapped in drapes and fixed to prevent slipping of patient pelvis.

76. Laparotomy Paramedian left incision/ pfannensteil incision In patients with level ileostomy for total colonic aganglionosis , a xiphopubic median incision is required in order to perform an endorectal ileal pull-through procedure. All mesocolic adhesions to the left parietal peritoneum are dissected up to the splenic flexure (classic form of HSCR). Before starting endorectal dissection, it is essential to perform multiple seromuscular biopsies of the rectum and colon in order to evaluate the length of the aganglionic and associated hypoganglionic segments

77. Seperation of seromuscular from mucosal layer of rectum Most technically difficult and peculiar step mepivacaina 2% with epinephrine 1:100,000 (10 μg ) in 10 ml of normal saline solution is injected between the layers in order to facilitate initial seperation

78. Endorectal Pull-through The endorectal dissection is started approximately 2 cm below the peritoneal reflection The seromuscular layer incised with either sharp dissection or cautery . Once the submucosal layer is reached the seromuscular layer is divided circumferentially using blunt dissection with hemostat/ Kitner dissector.

79. Endorectal Pull-through After the plane is established, dissection is continued distally. Once muscular cuff begins to develop, traction sutures placed in muscle in each quadrant. Communicating vessels coagulated. Dissection is carried down within 0.5cm (in neonates) – 1cm( older chidren ) from dentate line

80. Endorectal Pull-through – Perineal dissection Narrow retractors placed at anal- mucocutaneous junction Kelly clamp is inserted into the rectum The mucosal- submucosal tube is then everted onto the perineum.

81. Endorectal Pull-through – Perineal dissection The mucosal- submucosal tube is incised on the anterior half 0.5cm – 1cm from the dentate line

82. Endorectal Pull-through – Perineal dissection A Kelly clamp is inserted into this opening for grasping the two previously placed traction sutures. Great care is taken not to twist

83. Endorectal Pull-through – Perineal dissection Anterior ½ of the ganglionic colon is incised & anastomosed to the anterior half of the mucosal- submucosal tube with interrupted absorbable sutures One ¼ of the remaining Final ¼ invert the neorectum into correct position

84. Endorectal Pull-through The pulled through colon is attached with seromuscular bites to the muscular cuff to prevent prolapse . No drain is placed

85. Primary Pull through in neonates All the surgical procedures described have been performed as primary/single stage with good & comparable outcome

86. Primary Versus Staged Pull-through Principle Colonic dilatation can be quickly controlled by rectal washouts - Calibre of the pull-through bowel is near normal-allows accurate anastomosis -minimizes leakage & cuff infection Advantages of primary pull-through in neonates Specific stoma complications are avoided No. of admissions & hospital stay reduced Anal function and the anorectal reflex are reestablished as early as possible. If attempted in older children hugely dilated colon segment (normal ganglionic segment)needs to be resected

87. Selection criteria for Primary Pull-through Parameter Primary Staged Presentation Early Delayed presentation (very dilated bowel) Disease invovlement Rectosigmoid Long segment, TCA Enterocolitis Absent/resolved Active/severe Rectal washes Effective decompression No decompression General condition Stable Deteriorating Local expertise Frozen section histology Not available

88. Laparoscopic pull-through Avoidance of a painful abdominal incision, Rapid return of bowel function, Shorter postoperative recovery, Improved cosmetic appearance.

89. In the early 1990s, Georgeson et al described a minimal access approach, consisting of a Laparoscopic biopsy to identify the transition zone, Laparoscopic mobilization of the rectum below the peritoneal reflection, and Short endorectal mucosal dissection from below. The anastomosis was done from below after prolapsing and excising the rectum.

90. Multiple reports documented a short time in the hospital, and early results were equivalent to those reported for the open procedures. Subsequently, laparoscopic approaches have been described for the Duhamel and Swenson operations, with excellent short-term results reported

91. Laparoscopic Endorectal Technique With the patient positioned transversely at the end of the operating table, CO2 insufflation of the peritoneal cavity was performed using a Varess needle inserted through the abdominal wall. Three 5mm (3mm) trocars were inserted as shown Additional midline suprapubic trochar site is useful to provide pelvic retraction & hold the colon in traction during dissection of pelvis

92. Laparoscopic Endorectal Technique Dissection and leveling of the aganglionic segment are performed as in the open technique. Blood vessels may be ligated with surgical clips or cautery for smaller vessels . Once mobilization is complete, the surgeon moves to the perineum, where a trans anal dissection and anastomosis are performed

93. TRANSANAL ENDORECTAL PULL THROUGH

94. Transanal Endorectal Pull-through Selection criterion Neonates, infants (proximal bowel not much dilated) A barium enema study must suggest the diagnosis of HD & also the level of aganglionsis Confirmed diagnosis of HD HD limited to rectosigmoid or short segment

95. Transanal Endorectal Pull-through Advantages of one-stage anal procedures Avoidance of multiple laparotomies with its associated complications Avoidance of colostomy with its associated complications Reduced operating time Less blood loss No pelvic structure damage Decreased need of analgesics No external scars & Improved cosmetic appearance Single hospital admission Short hospital stay Lower hospital costs High degree of parental acceptance

96. Transanal endorectal pull through - procedure Preparation diagnosis is confirmed by rectal biopsy Prior to surgery, the colon must be decompressed and enterocolitis , if present, controlled Nutritional status must also be evaluated and optimized. In an older child with severe enterocolitis or massive colonic distension, a defunctioning stoma should be considered routine preoperative mechanical bowel preparation is unnecessary Mechanical irrigation of the bowel can be accomplished with equal effectiveness from below once the child has undergone anesthesia. Intravenous prophylactic broad spectrum antibiotics are used

97. Transanal endorectal pull through - procedure General anesthesia Lithotomy position/ prone jackknife position Urinary catheterization is optional

98. Submucosal Dissection anal retractor or retraction sutures are placed submucosal injection of a dilute epinephrine solution or air rectal mucosa is circumferentially incised using cautery approximately 3–5 mm from the dentate line fine sutures are placed in the proximal cut edge of the mucosal cuff, and traction is applied while the endorectal submucosal dissection is carried proximally

99. Mobilization of the Rectum When the submucosal dissection has been completed, the rectal muscle is divided circumferentially Dissection then continues proximally, dividing all vessels When the peritoneal reflection is reached, the sigmoid is then mobilized in the same fashion and the rectum and sigmoid are delivered through the anus

100. Anastomosis colonic dissection is completed when the transition zone is reached colon is divided at least 2 cm above the most distal normal biopsy to prevent the possibility of a transition zone pull-through rectal muscular cuff is then split longitudinally standard Soave- Boley anastomosis is then performed.

101. Transanal Endorectal Pull-through Contraindications Redo pull-through Grossly dilated bowel in older chidren Without confirmed diagnosis of HD Long segment HD/TCA Any H/O bleeding disorders

102. Postoperative care Oral feeding can be started as soon as child starts to pass stools if there is no abdominal distension Examination of perineal region To look for erythema / cellulitis Early sign of an anastomotic leak (leak uncommon) In cases of ERPT Per rectal examination with cotton tip applicator to ensure patency of rectal anastomosis befor discharge Instructing the parents to apply thick coat of barrier creams Educating the parents regarding enterocolitis . Preventive measures Follow up to late complications

103. Stooling pattern change in the first 2 yrs after pull-through Frequency 5-10 bowel movements/day – immidiately after pull-through 1 - 4 bowel movements/day – within 6-12 months Normal pattern Constipation Slowly improves

104. Complications. All post-operative complications which are recognised during the 1 st 4weeks following the surgery Factors which influence Extent of aganglionsis General condition of the child Technical expertise & experience of the surgeon Age at the time of operation. Whether or not colostomy done The administration of prophylactic antibiotics Family care The late follow-up offers the best oppurtunity to critically evaluate the efficiancy & results of any particular surgical procedure.

105. Early complications Wound infection (4%) Wound dehiscence(1%) Hemorrhage (<2%) Intestinal obstruction (7.5% - 10%) Anastomotic leak or disruption (1-10%) Cuff abscess (5%) Retraction of bowel(2 -10%) Rectal stenosis (10 -20%) Enterocolitis (5-42%) Minor complications Perianal excoriation UTI/bladder dysfunction Mortality

106. Late complications Constipation Incontinence (1-39%) Enterocolitis Urological complications Incontinence Retention Impotence Anastomotic complications/fistula Intestinal obstruction Moratlity (07%)

107. Enterocolitis (HAEC) Can be severe or life threatening Clinical features Fever, Abdominal distention, Diarrhea, Elevated white blood cell count, and Evidence of intestinal edema on abdominal radiograph Reported incidence varies in literature (2% - 40%) (depending on the definition) HAEC score may be useful

108. Enterocolitis (HACE) Majority of the episodes occur within 2yrs ofter pull-through There is no correlation between enterocolitis before & after surgery The incidence of enterocolitis directly correlates with mortality In a survey by AAP (1196 cases) , 14% developed enterocolitis with a mortality of 30% Swenson’s series of 880 cases death after discharge from enterocolitis – 1% Early recognition with prompt management influence the outcome

109. Enterocolitis Swenson & Fisher (1956) advocated rectal tube decompression for initial management Other measures Aggressive fluid resusciation Bowel rest Broad spectrum antibiotics Resection with Diversion if peritonitis or clinical worsening occurs Children with repeated bouts of enterocolitis needs to be evaluated for mechanical obstruction Contrast enema, manometry , rectal biopsy

110. Enterocolitis Most patients will improve with time Patients with persistent enterocolitis despite investigation Can be managed temporarily with botulism toxin Permanently by internal sphincterotomy ( Polley et al & Marty et al)/posterior myectomy Preventive measures to minimise the risk of HACE Chronic administration of metronidazole ( 1-2months) or probiotic agents Prophylactic rectal irrigations(Marty et al) Should be advised particularly in those who are thought to be at higher risk for this complication on the basis of clinical or histologic grounds. It is extremely important that the surgeon educate the family about the risk of this complication

111. Voiding & sexual dysfunction Many of the operations for Hirschsprung's disease have been designed specifically to avoid pelvic nerve injury. Overall, the incidence of impotence and urinary dysfunction is quite low. Postoperative enuresis ~9.5%. Swenson 10.4% Duhamel 14.3% Soave 15.3% More recent series report no incidence of impotence or urinary problems.

112. Late mortality Most common cause is enterocolitis Other causes of death Intestinal obstruction Pneumonia Nonrelated medical disorders. In most studies the differentiation of early & late deaths is lacking No specific pull-through procedure is associated with higher rate of late deaths

113. Overall quality of life Is difficult to assess Overall quality of life was described as quite good with ~94% of children becoming well adjusted members of the society Developmental milestones are satisfactory in ~95% cases School performanace is satisfactory in ~ 82% Clearly children with poor functional outcome have psychosocial problems

114. Comparison of different procedures

115. Thank you