Laparoscopy-assisted treatment of Hirschsprung's disease: Duhamel method

The description of the laparoscopy-assisted treatment of Hirschsprung's disease: Duhamel method covers all aspects of the surgical procedure used for the management of rectosigmoid Hirschsprung's disease. Operating room set up, position of patient and equipment, instruments used are thoroughly described. The technical key steps of the surgical procedure are presented in a step by step way: laparoscopic exploration, laparoscopic rectal dissection, laparoscopic sigmoid colon dissection, transection of rectum, retrorectal pull-through, anastomosis, rectal closure. Consequently, this operating technique is well standardized for the management of this condition.

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Laparoscopy-assisted   treatment   of   Hirschsprung's   disease:   Duhamel   method

Authors
N Bax, DC Van der Zee
Abstract
The description of the laparoscopy-assisted treatment of Hirschsprung's disease: Duhamel method covers all aspects of the surgical procedure used for the management of rectosigmoid Hirschsprung's disease.
Operating room set up, position of patient and equipment, instruments used are thoroughly described. The technical key steps of the surgical procedure are presented in a step by step way: laparoscopic exploration, laparoscopic rectal dissection, laparoscopic sigmoid colon dissection, transection of rectum, retrorectal pull-through, anastomosis, rectal closure.
Consequently, this operating technique is well standardized for the management of this condition.
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2003-03
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E-publication
WeBSurg.com, Mar 2003;3(03).
URL: http://www.websurg.com/doi-ot02en226.htm

Laparoscopy-assisted   treatment   of   Hirschsprung's   disease:   Duhamel   method

1. Introduction
Bernard Duhamel's technique for the treatment of Hirschsprung's disease consists of the resection of the aganglionic colon above the peritoneal reflection, retrorectal pull-through of the ganglionated colon and partial distal side-to-side anastomosis between the pulled-through ganglionated colon and the remaining distal aganglionic rectum (1956, 1960).
In 1968, Steichen et al. described the use of a stapler to create a complete side-to-side anastomosis. A laparoscopy-assisted version of the technique was published in 1995 by Bax and van der Zee, and subsequently by de Lagausie et al. in 1999.This is a minimal access technique that allows for easy determination of the extent of the disease, and for intra-abdominal mobilization of the colon to be resected and pulled through. In this operative technique, we focus mainly on the treatment of rectosigmoid Hirschsprung's disease.
2. Anatomy
• Physiopathology
Hirschsprung’s disease is a congenital disturbance of the innervation of the colon. Histologically it is characterized by an absence of ganglion cells and by hyperplasia of the parasympathetic nerve fibers. In 80% of cases, the disease is limited to the rectosigmoid colon. In the rest, the disease extends more proximally to involve varying portions of the colon. This may even involve the whole colon and, rarely, the small bowel (Teitelbaum et al., 1998).
• Rectosigmoid colon
• Transverse aspect
1. Sigmoid colon
2. Bladder
3. Right deep inguinal ring
4. Vas deferens
5. Spermatic vessels
6. Cecum
7. Ileum
8. Rectum
9. Peritoneal reflection
Classic Hirschsprung’s disease involves the rectum and the sigmoid colon, collectively known as the rectosigmoid colon. The sigmoid colon is divisible into a fixed iliac segment and a mobile pelvic segment, at the junction of which the sigmoid mesentery crosses the left ureter and iliac vessels. Both ureters run along the lateral walls of the pelvis in the direction of the bladder. The rectum lies posterior to the uterus in the female, and posterior to the bladder in the male. In the male, the right and left vas deferens approach each other posterior to the bladder just at the peritoneal reflection, between the bladder and the rectum.
• Sagittal aspect
1. Toldt’s fascia
2. Presacral fascia
3. Mesorectum
4. Fascia propria
5. Denonvilliers’ fascia
6. Peritoneum
7. Parietal fascia
Below the peritoneal reflection, in the male, the rectum is bordered anteriorly by the seminal vesicles and the prostate, but is separated from these structures by Denonvilliers’ fascia (rectogenital septum).
• Innervation
1. Superior hypogastric plexus
2. Inferior hypogastric plexus
3. Right sympathetic trunk
4. Left sympathetic trunk
5. Inferior mesenteric plexus
6. Left superior hypogastric nerve
7. Right superior hypogastric nerve
8. Rectal branches
9. Urogenital branches
10. Sacral nerves
11. Inferior hypogastric plexus
12. Deeper branches of pelvic plexuses
13. Erectile nerves
Close to the lateral walls of the rectum, the pelvic nerve plexus branches out to the rectum, bladder, prostate and erectile organs. Extensive dissection of the rectum beyond the peritoneal reflection puts the vas deferens, seminal vesicles and pelvic nerves at risk. A major advantage of the Duhamel technique is that the dissection of the distal rectum is limited to the retrorectal space (Duhamel, 1956; Duhamel, 1960; Teitelbaum et al., 1998).
Nevertheless, dissection should proceed as close to the colon and rectal wall as possible.
• Vasculature
1. Abdominal aorta
2. Inferior mesenteric artery
3. Sigmoid vessels
4. Internal iliac artery
5. Rectal vessels
6. Left colic artery
7. Inferior mesenteric vessels
8. Sigmoid trunk
9. Superior rectal artery (SRA)
10. Middle rectal artery
11. Inferior rectal artery
12. Left branch of the SRA
13. Right branch of the SRA
14.Pelvic floor
15. Anal canal
The rectosigmoid colon is mainly supplied by the inferior mesenteric artery and its branches, and mostly drained by the inferior mesenteric vein and its branches. The marginal vessels run along the mesenteric border of the colon. The rectosigmoid colon and even part of the distal left colon can be removed, but the marginal blood supply must be preserved. This is achieved by dividing the sigmoid vessels and if necessary the left colic vessels close to their origin.

If the disease extends more proximally, more length can be gained by taking down the splenic flexure of the colon. If this is insufficient, then the middle colic and even right colic vessels are divided close to their origin and the ileocecal region is mobilized. The right colon can then be turned counterclockwise and brought down into the pelvis. However, division of the sigmoid or inferior mesenteric vessels close to their origins and mobilization of the ileocecal region may put the ureters at risk.
3. Indications
Hirschsprung’s disease is an indication for surgery. While several techniques are in use for rectosigmoid disease, many pediatric surgeons use a Duhamel-type technique for extended aganglionosis (Martin, 1972; Teitelbaum et al. 1998).
However, in extended aganglionosis it may be wise to take multiple biopsies during a first operative session and to defer further surgery until the definitive results of the pathological examination of the biopsies are known.
4. Preop period
Timing of the operation
This depends on the timing of the diagnosis and on the effectiveness of bowel decompression achieved by rectal washouts. For infants diagnosed within the neonatal period, the operation should be performed between the age of 1 and 4 months. If bowel washouts are troublesome or ineffective, then the operation is not postponed, irrespective of patient age or body weight.

Preoperative preparation
The day before surgery, an antegrade whole bowel lavage is carried out.
5. Operating room set-up
• Patient
Older children are placed in a supine position at the end of the operating table. Small children can be placed transversely at the end of the operating table (not shown).
A Trendelenburg position is used for rectosigmoid disease, but the position is adjusted in extended aganglionosis.

Anesthetic considerations:
a. General anesthesia is used in combination with locoregional techniques;
b. Mask ventilation is avoided during induction;
c. Endotracheal intubation is used;
d. N2O is not used in order to avoid N2O bowel distention;
e. Optimal muscle relaxation is mandatory in order to create an optimal working space.
Broad spectrum intravenous antibiotics are given prophylactically.

The rectum is emptied with a large double-lumen suction device. If there is any remaining feces, irrigation is carried out until the effluent is clear.

The skin is prepped from the nipples to the ankles. The feet are wrapped in sterile sheets.

A urine catheter is inserted after draping. The bladder is emptied regularly during the operation by the surgeon.
• Team
The operating table is in a Trendelenburg position.
1. The surgeon stands on the patient’s right and faces the lower end of the table.
2. The assistant stands on the patient’s left and faces the lower end of the table.
3. The scrub nurse stands on the surgeon’s right.
4. The anesthesiologist stands at the upper end of the table.
If the infant has been placed transversally, the surgeon stands at the head of the patient with the assistant to the left and the scrub nurse to the right.
• Equipment
1. Monitor
2. Operating table
3. Anesthetic equipment
4. Electrocautery
5. Laparoscopic unit
6. Instrument table
The laparoscopic unit is located behind the surgeon. A second monitor is at the lower end of the table. In extended aganglionosis, the laparoscopic unit is moved along the patient’s right side, while the other monitor is moved along the patient’s left side.
The anesthetic equipment is at the upper end of the operating table.
The electrocautery unit and the suction and irrigation unit are located on the right of the patient’s head.
All cables come from the same direction and pass along the right side of the patient, at least during rectosigmoid dissection. There are 8 cables in total; camera, light, CO2, external monopolar high frequency electrocogulation (HFE), internal monopolar HFE, internal bipolar HFE, suction, irrigation. An additional cable may be required for systems such as ultrasonic energy.
6. Trocar placement
• Pneumoperitoneum
A small incision is made just below the umbilicus for an open introduction of the first trocar. A sleeve is put around the trocar to prevent it from being pushed in. A suture around the stopcock prevents it from being pulled out. The pneumoperitoneum is then started. With good muscle relaxation a pressure of 8 mm Hg suffices. Flow is set at 2L/min in infants up to a year old, and at 5L/min in older children.
• Trocar placement
Trocar placement is modified according to the age of the patient.

Younger children
A: 6 mm, inferior umbilical fold
B: 3.8 mm, immediately lateral to the left rectus sheath at the umbilical level
C: 3.8 mm, immediately lateral to the left rectus sheath at the umbilical level
D: 3.8 mm, mid-epigastrium

Older children
A: 6 mm, inferior umbilical fold
B: 6 mm, immediately lateral to the left rectus sheath in the left iliac fossa
C: 6 mm, immediately lateral to the left rectus sheath in the right iliac fossa
D: 6 mm, right lower epigastrium
• Rectal cannula
All trocars have been inserted in this infant. A suction cannula is placed in the rectum. This can also be used for manipulation of the rectum during dissection.
7. Instrumentation
• Optical
Trocar A
1. 5 mm 30° laparoscope
• Retracting
Trocar B
1. Holding (fenestrated) forceps with ratchet for the assistant.
• Operating
Internal use
Trocar C
1. Metzenbaum scissors
2. Monopolar hook
3. Bipolar forceps
4. Needle holder
5. Suction-irrigation

Trocar D
6. Kelly tissue holder

Transanal use
7. Endoscopic linear stapler
8. Long curved artery forceps
8. Major principles
The objectives of the laparoscopy-assisted Duhamel method are:
1. Laparoscopic exploration and determination of the extent of the disease by biopsy
2. Laparoscopic rectal dissection
3. Laparoscopic dissection of the sigmoid colon
4. Transection of the rectum at the peritoneal reflection:
- either internally after ligature of the proximal rectum,
- or after transanal eversion.
5. Transanal opening of the distal posterior rectum, development of the retrorectal space from below, and retrorectal pull-through of colon till ganglionated colon becomes visible.
6. Anastomosis
9. Laparoscopic exploration
• Visual exploration
Usually it is immediately clear whether the child has rectosigmoid disease or extended aganglionosis. In classic Hirschsprung’s disease the rectum is narrow while the rectosigmoid colon becomes progressively dilated. The colonic wall in the transition zone between aganglionic and ganglionic bowel is thickened.

The quality of the preoperative antegrade whole bowel lavage is evaluated. There may be air accumulation in the bowel just above the aganglionic colon, which can be emptied by transanal insertion of a double-lumen suction device. It is advantageous to leave the suction cannula in the distal colon for guidance.
• Biopsy
Frozen section pathological studies of 1 or serial seromuscular biopsies are needed to determine the proximal extension of the disease. Usually 2 seromuscular biopsies suffice; one is taken at the beginning of the dilatation and thickening of the colon to confirm the diagnosis of aganglionosis, and the other is taken higher up where the bowel wall looks normal and possesses normal ganglion cells. If the proximal biopsy does not show ganglion cells, another should be taken further up. If a full thickness biopsy is taken inadvertently, the hole should be closed with a 5.0 polyglactin 910 suture to prevent leakage. It is a good principle to oversew all biopsy sites that are not exteriorized later on to prevent unrecognized leakage.
10. Lap. rectal dissection
The assistant retracts the sigmoid colon cephalad. The rectal dissection is started on the right. A window is created in the mesentery at the rectosigmoid junction close to the bowel wall, the dissection is continued anteriorly, and the peritoneal reflection is divided. The rectum is mobilized circumferentially, and stops just inferior to the peritoneal reflection. Next, the dissection is continued caudad, close to the posterior wall of the rectum, and ends just superior to the anus.
11. Lap sigmoid dissection
1. Sigmoid colon
2. Sigmoid vessels
3. Inferior mesenteric artery
The dissection now proceeds cephalad. If the upper sigmoid colon has to be mobilized, Toldt's line is incised first, to mobilize the mesentery. The dissection of the sigmoid colon mesentery is carried out close to the colon wall until the normal ganglionic colon, as confirmed by biopsy, is reached (the 'good biopsy site'). If more mobilization of the proximal colon is warranted the inferior mesenteric artery should be divided close to the aorta leaving the marginal vessels intact. Smaller vessels can be divided with monopolar HFE. Larger vessels can be divided between ligatures or clips, or with ultrasonic energy. They can also be sealed with bipolar HFE and cut with scissors.
12. Transection of rectum
This can be done internally after ligature or stapling of the more proximal rectum.
Alternatively, the mobilized rectum can be everted transanally and transected outside the body. In this case the everted rectum is amputated close to the anus, after which the aganglionic bowel can be further exteriorized and removed. The distal end of the bowel is then closed and pushed back into the abdomen as is the rectum. The amputated rectum remains open in the abdomen.
13. Retrorectal pull-through
A transverse incision is made 0.5 to 1 cm (depending on the patient's age) above the dentate line in the posterior rectal wall. The retrorectal space is opened and a curved artery forceps is inserted to grasp the closed end of the mobilized colon under laparoscopic view. The mobilized colon is pulled down through the retrorectal space and opening in the posterior rectal wall till the 'good biopsy site' is reached.
14. Anastomosis
• Anastomosis 1
Transanal end-to-side:
When the 'good biopsy site' is reached the pulled-through colon is amputated and anastomosed circumferentially to the opening in the posterior rectal wall.
• Anastomosis 2
Transanal side-to-side:
A linear stapler is then inserted with one part of the beak in the pulled through retrorectal bowel and the other part in the remaining rectum. The side-to-side anastomosis is complete when the top of the beak in the rectum is seen in the abdomen. After firing, the stapled side-to-side anastomosis can be seen through the open rectal stump.
Finally, the rectal stump is closed laparoscopically.
15. Rectal closure
As the rectum has been amputated and pushed back into the abdomen, the open upper portion has to be closed. This is done laparoscopically with a running 3X0 polyglactin 910 suture. The needle and suture are inserted directly through the abdominal wall.
16. Postop period
Postoperative management
Epidural analgesia or intravenous morphine is given for 48 hours.
The urine catheter is left in place until the next morning, unless the patient has an epidural catheter for pain relief. In this case, the urine catheter is left in situ until the epidural catheter is removed. There is no need for a nasogastric tube. Oral feeding is commenced as soon as there is evidence of gastrointestinal passage.
The patient is discharged when full oral feeding is commenced.
The operation leaves tiny wounds, the scars of which are hardly visible after a few months.

Complications
Possible early complications are:
- damage to the ureter, vas deferens or pelvic nerves;
- leaking biopsy sites;
- torsion of the pulled-through colon;
- pelvic sepsis/anastomotic leak.

Possible late complications are:
- insufficient resection;
- enterocolitis;
- soiling;
- incontinence.
17. Conclusion
The Duhamel technique is based on sound principles and is considered the best available technique for extended Hirschsprung’s disease. However, the laparoscopy-assisted technique is attractive for both rectosigmoid and extended forms of the pathology. The disadvantage is that it requires considerable laparoscopic surgical skills.
18. Reference