Handbook of Surgical Consent
Annotate
Cite
Details to consider when consenting for neonatal surgery 490
Primary repair of oesophageal atresia and tracheo-oesophageal fistula 491
Closure of abdominal wall defects: exomphalos and gastroschisis 493
Congenital diaphragmatic hernia repair 499
Ladd's procedure for malrotation with or without volvulus 501
Necrotizing enterocolitis 504
Duodenal and small intestinal atresias 506
Surgery for Hirschsprung's disease 510
Details to consider when consenting for neonatal surgery
Neonatal surgical procedures are often performed in the first few hours or days of life and this is usually a time of great parental anxiety and stress.
Many neonatal conditions are now antenatally diagnosed, and there may have been an opportunity for antenatal counselling to help prepare the parents for surgery.
Where possible, consent should be obtained face-to-face. However, sick neonates often require transportation to tertiary referral centres for a surgical opinion and parents may be unable to accompany their baby at the time. In these circumstances it may be necessary to obtain consent over the telephone. In such cases, the following things should happen:
The parents must identify themselves and confirm their relationship to the patient and that they have parental responsibility
They must confirm that they understand the nature of the call (they understand it is for consent for surgery)
The patient's diagnosis, the treatment plan, the complications, risks, and alternatives should be disclosed
There should be a specific request for consent, which is agreed to
The call should be witnessed and agreement of consent should be repeated to the witness
It is then acceptable for the person obtaining consent and the witness to sign the consent form on behalf of the parents
Primary repair of oesophageal atresia and tracheo-oesophageal fistula
Description
Oesophageal atresia represents a congenital failure of the oesophagus to develop in continuity with the stomach, ending as a blind pouch. Although oesophageal atresia can be an isolated anomaly it most commonly occurs with a tracheo-oesophageal fistula (TOF), an abnormal communication between the trachea and oesophagus (usually distal).
The diagnosis is suspected on antenatal ultrasound scans when there is an absent stomach bubble and polyhydramnios
Postnatally the infant may present with frothing and inability to swallow their own secretions or feed
A chest X-ray will often show the nasogastric tube curled up in the upper pouch at the level of T3–T5
Diagnosis should be confirmed following passage of a Replogle tube. Once the Replogle tube is in place continuous suction should be initiated to reduce the risk of aspiration
Parents should be counselled that there might be other abnormalities present, for example VACTERL association (Vertebral, Anorectal, Cardiac, Tracheo-Esophageal, Renal and Limb anomalies).
Surgery
Some surgeons perform preliminary bronchoscopy to identify the fistula
1. Right posterolateral muscle-sparing thoracotomy
2. Extrapleural approach
3. Ligation of the azygous vein
4. Identification, division, and over-sewing of the TOF
5. Mobilization of the upper oesophageal pouch
6. Oesophageal anastomosis if gap allows
7. Trans-anastomotic feeding tube (use will vary according to unit or surgeon preference)
Additional procedures that may become necessary
In an unstable infant requiring respiratory support, it may be necessary to perform emergency TOF ligation alone to allow stabilization. If the baby's condition allows, subsequent anastomosis may be attempted, otherwise delayed repair will be necessary
Babies with a ‘long gap’ (greater than three vertebral bodies) may require a delayed primary closure at several weeks of age. An initial gastrostomy will be required to allow the baby to feed enterally
Formal oesophageal anastomosis after gap assessment demonstrates this to be possible
Oesophageal replacement will be required when anastomosis is not possible. This requires an initial cervical oesophagostomy to allow drainage of saliva followed by a delayed gastric transposition or intestinal interposition
Benefits
Restoring oesophageal continuity
Alternative procedures/conservative measures
Untreated this condition is incompatible with life
Significant/frequently occurring risks
Early complications
Bleeding
Anastomotic leak occurs in around 10%1,2
Radiologic leaks are usually not clinically significant
Minor leaks with persistent drainage of saliva into a chest drain make up the majority of significant leaks. They heal with conservative management
Complete disruption of the anastomosis. These are major leaks and commonly present within the first few days of surgery, often with a tension pneumothorax. They will require re-exploration when the baby is stable
Wound infection
Death (coexisting cardiac anomalies and low birthweight (<1500g) increase risk of death)
Late complications
Type of anaesthesia/sedation
General anaesthesia
Follow-up/need for further procedure
Long-term outcome depends on presence and severity of associated anomalies. Multidisciplinary follow-up is required in order to diagnose and manage some of the long-term complications of both the condition and the surgery
References
Closure of abdominal wall defects: exomphalos and gastroschisis
Description
During the 6th week of gestation rapid growth of the midgut causes a physiological herniation of the intestine through the umbilical ring. The midgut rotates as it re-enters the abdominal cavity and the intestine then migrates so that the small intestine and colon come to lie in their correct anatomical positions by the end of the 10th week of development.
Exomphalos and gastroschisis are congenital abdominal wall defects. Exomphalos arises as a result of failure of closure of the abdominal wall following return of viscera
In exomphalos the viscera are covered with a clear sac made of amnion, Wharton's jelly, and peritoneum, to which the umbilical chord is attached. In exomphalos minor the defect is less than 5cm in diameter and can usually be closed at one operation. Exomphalos major is greater than 5cm, may contain stomach, liver, and bowel and may require staged closure
Exomphalos is associated with chromosome abnormalities in about 50% of cases (commonly trisomy 13, 18, or 21) and other congenital anomalies in up to 70% of cases.1,2 Beckwith–Wiedemann syndrome (BWS) is usually associated with exomphalos minor, and includes:
Exomphalos
Macroglossia
Macrosomia
Visceromegaly
Hemihypertrophy
Hypoglycaemia
Various types of solid tumour
Pentalogy of Cantrell includes:
Exomphalos
Anterior diaphragmatic hernia (Bochdalek)
Sternal cleft
Ectopia cordis
Intracardiac anomaly
Giant exomphalos, with the whole liver out, is often associated with significant pulmonary hypoplasia
Gastroschisis is thought to be due to a vascular accident involving the abdominal musculature following full closure. There is no sac covering bowel protruding through a defect, which is usually to the right of a normally sited umbilical cord. The intestine has been exposed to amniotic fluid and as a result may be foreshortened, thickened and matted together
Unlike exomphalos, infants with gastroschisis do not have an increased risk of chromosomal abnormalities but do have an increased incidence of atresias, felt to be due to vascular compromise of the abnormal bowel or a closing defect
Exomphalos minor
The goal of surgical management is primary closure with fascial apposition and complete skin closure, without causing excessive intra-abdominal pressure.
Additional procedures that may become necessary
Ladd's procedure after assessment of intestinal rotation
Benefits
Therapeutic: Closure of abdominal defect
Alternative procedures/conservative measures
A very small defect (hernia into the chord) may be amenable to simple ligation of the sac at the base of the cord after reduction of bowel
Significant/frequently occurring risks2
Early complications:
Bleeding
Wound infection
Postoperative sepsis (respiratory, central venous catheter related)
Late complications:
Ventral hernia
Adhesive bowel obstruction
Type of anaesthesia/sedation
General anaesthesia
Follow-up/need for further procedure
Medical follow-up depends on severity of associated congenital anomalies
Patients with BWS have lifelong risk of solid tumours, e.g. Wilms’, hepatoblastoma, and as a result must have lifelong surveillance
Cosmetic scar revision
Major exomphalos
Again the goal of surgery is to achieve fascial and skin closure. However, with exomphalos major this may not be possible without causing excessively raised intra-abdominal pressure and the risk of abdominal compartment syndrome. If skin closure is possible, fascial repair can be achieved with a prosthetic patch. Otherwise, a staged surgical closure using a surgical silo will be necessary.
Additional procedures that may become necessary
Ladd's procedure after assessment of intestinal rotation
Prosthetic patch to facilitate fascial closure
Surgical silo formation
Benefits
Therapeutic: Closure of abdominal defect
Alternative procedures/conservative measures
Initial non-operative management: A small unstable preterm infant or a neonate with a very large sac can be managed by application of topical agents to promote epithelialization from the skin edges This conservative management creates a large ventral hernia, which can then be closed when the child is older. Surgery is often staged and represents a technical challenge3
Significant/frequently occurring risks2
Early complications:
Significant risk of bleeding when dissecting sac from the liver
Skin flap haematoma
Wound infection and breakdown
Patch infection
Postoperative sepsis (respiratory, CVC-related)
Drug toxicity due to systemic absorption of active agents such as silver, iodine, or mercury has been reported
Late complications:
Ventral hernia
Adhesive bowel obstruction
Gastro-oesophageal reflux
Type of anaesthesia/sedation
General anaesthesia
Follow-up/need for further procedure
Delayed closure where a surgical silo was required
Long-term follow-up to observe for late complications
Revision surgery of abdominal scar
Gastroschisis
Protection of the eviscerated bowel after birth is paramount in these babies, quickly followed by stabilization with fluid resuscitation and warming.
A variety of closure techniques are available.4 Currently, two strategies are employed in the main: staged reduction and closure, and primary closure. If there is volvulus, necrosis, or perforation, or a closing defect noted at birth, emergency surgery is required.
Staged reduction and closure involves placing the bowel inside a preformed Silastic silo (PFS) at the cot-side using only analgesia or sedation. This allows gradual reduction of the intestine over a period of 3–5 days. When the intestine is fully reduced the defect can be closed either as an operative procedure in theatre or using a cot-side closure technique.
PFS application
A silo large enough to contain the volume of herniated bowel is selected
Bowel is passed into the silo, maintaining alignment and rotation
The ring is deformed into an elongated shape to allow insertion through the defect after ensuring there are no congenital bands between the bowel and edge of defect
The silo is suspended vertically inside cot
Additional procedures that may become necessary
An umbilical defect may need to be incised to make it large enough to accept a PFS at the cot-side
Benefits
Therapeutic: reducing abdominal viscera and closing defect
Allows enteral feeding
Alternative procedures/conservative measures
Primary closure
Risks of PFS:
Missed intestinal atresia
Bowel ischaemia within the silo, due to inappropriate choice of size (volume of silo must be adequate)
Silo detachment
Entrapment of viscus between silo ring and abdominal wall
Sepsis
Type of anaesthesia/sedation
Analgesia or sedation
Follow-up/need for further procedure
All patients treated with a silo will need either surgical closure or cot-side closure
Cot-side closure
Carefully remove the silo with one person ensuring bowel stays reduced
Clean and dry abdomen and apply tincture of benzoin compound to the skin surrounding the defect
Lift and pull the umbilical cord to the opposite side of the defect to oppose skin edges
Place large 12mm Steri-strips horizontally to keep skin edges together
Vertical Steri-strips can be used to reinforce this
Clear dressings over Steri-strips, allowing the cord to protrude, are used to cover the Steri-strips
Leave dressing for 5–7 days
Additional procedures that may become necessary
Surgical/primary closure may be required
Benefits
Therapeutic: reducing abdominal viscera and closing defect
No need for anaesthesia
Alternative procedures/conservative measures
Primary closure
Significant/frequently occurring risks
Risks of cot-side closure:
Skin closed only, not the fascia so increased risk of umbilical hernia
Dressings lifting from the skin due to serum build up beneath
Raised intra-abdominal pressure if dressings too tight
Failure of closure
Type of anaesthesia/sedation
Analgesia or sedation
Follow-up/need for further procedure
Umbilical hernia repair
Cosmetic revision
Primary closure
Primary closure involves urgent surgery soon after birth. The intestines are carefully examined for atresias and first the muscle and then the fascia are closed. The umbilical skin is then closed and an attempt is made to re-fashion the umbilicus. The same technique is used to close the abdomen after a PFS has been used to reduce the bowel.
Additional procedures that may become necessary
Prosthetic patch
Bowel resection and anastomosis
Enterostomy formation
Silo placement if abdominal compartment is not large enough to house all the eviscerated organs
Benefits
Therapeutic: reducing abdominal viscera and closing defect
Allows enteral feeding
Alternative procedures/conservative measures
Staged reduction with preformed silo
Significant/frequently occurring risks
Risks of surgical closure
Early complications:
Bleeding
Wound infection or dehiscence
Anastomotic leak
Abdominal compartment syndrome
Late complications:
Sepsis
Anastomotic stricture
Adhesional bowel obstruction
NEC
Gastro-oesophageal reflux
Abdominal wall hernias
Short-bowel syndrome as a result of multiple atresias or a ‘closing’ defect
Parenteral nutrition-associated liver disease (PNALD) as a result of prolonged parenteral nutrition
Type of anaesthesia/sedation
General anaesthesia for primary closure
Analgesia or sedation for preformed silo application
Follow-up/need for further procedure
All children need long-term follow-up to assess feeding, growth, and bowel habit
Cosmetic revision may be required
References
Congenital diaphragmatic hernia repair
Description
Congenital diaphragmatic hernia.
There remains a high mortality and morbidity for a ‘severe’ subset of patients despite recent advances in the antenatal and postnatal management of these babies including:
Where diagnosed antenatally, parents will have had extensive input from fetal medicine specialists and counselling.
Some of these babies are not diagnosed until after birth, often presenting with varying degrees of respiratory difficulty.
Surgery is only offered if the neonate achieves a degree of stability in terms of oxygenation and ventilation, usually after 48h. Surgery is not indicated in those patients who do not achieve this.
Surgery
1. Open, thoracoscopic, or laparoscopic
2. Return bowel to abdominal cavity
3. Excise hernial sac if present
4. Dissect a diaphragmatic rim if possible and aim for primary repair using non-absorbable sutures
5. A prosthetic or bioprosthetic patch can be used if the defect is too large for primary closure
Additional procedures that may become necessary
Ladd's procedure after assessment of intestinal rotation
Insertion of prosthetic patch
Benefits
Close diaphragmatic defect and return abdominal viscera to the abdomen
Alternative procedures/conservative measures
Untreated, this condition is not compatible with life
Early complications:
Bleeding
Wound infection
Chylous effusion
Death
Late complications:
Recurrence of hernia
Need for patch revision
Adhesive bowel obstruction
Gastro-oesophageal reflux
Chronic lung disease
Hearing difficulties
Neurocognitive deficits
Chest asymmetry/pectus deformity
Nutritional morbidity/growth failure
Tracheomegaly in infants treated by FETO
Death
Type of anaesthesia/sedation
General anaesthesia
Follow-up/need for further procedure
Patients will usually need to be reviewed every 3–6 months for the first 1–2 years and annually thereafter
With increased survival a range of complications may be seen at follow up, and long-term multidisciplinary follow-up is therefore required
References
Ladd's procedure for malrotation with or without volvulus
Description
Following normal intestinal development, the small bowel mesentery runs from the ligament of Treitz at the duodenojejunal flexure on the left of the second lumbar vertebra to the ileocaecal region. The caecum is then fixed in the right iliac fossa. This gives a long base to the small bowel mesentery, which provides a stable arrangement for the superior mesenteric vessels, which lie within it
If normal rotation does not occur the caecum can lie near the duodeno-jejunal flexure with a narrow base to the small bowel mesentery, making this more susceptible to volvulus. Acute torsion can lead to obstruction of the superior mesenteric vessels and catastrophic midgut ischaemia if untreated
Elective surgery may be performed in order to reduce the risk of volvulus once malrotation is diagnosed. In a collapsed infant emergency laparotomy is indicated
The goal of surgery is to produce a more stable mesenteric pedicle, and in the acute situation to restore gut perfusion and preserve as much bowel length as possible
Malrotation volvulus.
Surgery
1. Open for emergency. Laparoscopic approach is an option for malrotation without volvulus
2. Assess gut for viability
3. Untwist volvulus
4. Assess position of the duodenojejunal flexure
5. After blood supply has been restored, resect any gangrenous bowel
6. Primary anastomosis or enterostomy formation
7. Divide Ladd's bands (peritoneal bands fixing the duodenum)
8. Straighten duodenal loop and mobilize colon
9. Broaden the base of the mesentery
10. Place the large bowel in the left side of the abdomen and the small bowel on the right (Ladd's position2)
11. Appendicectomy, inversion, or resection
Additional procedures that may become necessary
Bowel resection (may be massive)
Enterostomy formation
Primary anastomosis
If there is doubt over the viability of the bowel, a second-look laparotomy can be done in 24–48h after initial untwisting
Further bowel resection
Central venous access for parenteral nutrition
Benefits
Restore blood flow to the gut
Place bowel in a safe position so this cannot happen again
Alternative procedures/conservative measures
‘Open and shut’ laparotomy in overwhelming gut necrosis
Significant/frequently occurring risks1
Early complications:
Bleeding
Wound infection
Anastomotic leak
Death
Late complications:
Anastomotic stricture
Adhesional bowel obstruction
Short bowel syndrome
PNALD
Type of anaesthesia/sedation
General anaesthesia
Follow-up/need for further procedure
Early outpatient review to check the patient is thriving
Patients with stomas may have major fluid and electrolyte losses and will require close monitoring and nutritional support
Patients with short bowel syndrome may need long-term total parenteral nutrition and may benefit from bowel-lengthening surgery
References
Necrotizing enterocolitis
Description
NEC is one of the most common surgical emergencies in neonates. It can affect small sections of the intestine, be multifocal or affect the intestine in its entirety. Seen more commonly in premature neonates,1 symptoms include feed intolerance, increasing nasogastric aspirates, abdominal distension, and bloody diarrhoea. Treatment is mainly conservative with a long period of bowel rest, intravenous total parenteral nutrition, and antibiotics.
Surgery is reserved for those with evidence of:
Perforation
Obstruction
Palpable mass
Failure to progress with medical management
Aim of surgery is removal of necrotic bowel and anastomosis where possible, or defunctioning enterostomies and preservation of as much intestinal length as possible.
Additional procedures that may become necessary
Bowel resection (may be massive)
Single resection and anastomosis
Multiple resections and creation of enterostomies
Limited resection or ‘clip and drop’ in multifocal NEC aims to salvage bowel length by retaining areas of questionable viability to be reassessed at a second look laparotomy in 24–48h2
Benefits
To remove diseased bowel while attempting to preserve as much length as possible
Alternative procedures/conservative measures
Initial treatment is conservative
Peritoneal drain insertion for stabilization followed by a timely ‘rescue’ laparotomy3
‘Open and shut’ laparotomy for NEC totalis
Significant/frequently occurring risks
Early complications
Bleeding
Massive bowel resection
Wound infection
Anastomotic leak
Sepsis
Enterostomy complications (
see‘Enterostomy complications’, p.505)
Death
Late complications
Anastomotic stricture
Post-NEC stricture
Abscess or fistula formation
Adhesional bowel obstruction
Gastro-oesophageal reflux
Recurrence of NEC (5%)
Short-bowel syndrome
PNALD
Death
Enterostomy complications
Prolapse
Stricture
Retraction
Parastomal hernia
Fluid and electrolyte disturbance
Inadequate weight gain
Peristomal skin excoriation and bleeding
Type of anaesthesia/sedation
General anaesthesia
Follow-up/need for further procedure
Patients with a peritoneal drain will require a laparotomy
Stoma closure
Strictureplasty or stricture resection for post-NEC stricture
Short-bowel syndrome babies will have a prolonged hospital stay requiring total parenteral nutrition, central venous access
Surgery that may be required to ameliorate short-bowel syndrome includes bowel tapering, bowel lengthening, and intestinal transplantation
Multidisciplinary follow-up is required to monitor nutrition and growth
Patients with stomas may have major fluid and electrolyte losses and will require close monitoring and nutritional support
These patients may also have developmental and intellectual delay
References
Duodenal and small intestinal atresias
Description
Duodenal atresia occurs in around 1 in 5000 live births and is associated with other congenital anomalies.
Half of these are detected on antenatal ultrasound scans
The remainder will present in the first day of life with:
Vomiting (bilious or non-bilious depending on the relation of the atresia to the ampulla of Vater)
Abdominal distension
‘Double bubble’ on the abdominal X-ray
Improved operative techniques along with enhanced provision of neonatal care have improved survival rates to around 90%1
Half of all patients with duodenal atresia will have chromosomal abnormalities; trisomy 21 occurs in about a third
Jejuno-ileal atresias occur due to vascular accidents usually late in the second or third trimester.
Multiple atresias may occur if different segments are involved with viable portions of intestine surviving in between
Most patients will be diagnosed antenatally but those who are not, typically present in the first days of life with symptoms of intestinal obstruction. They may still pass meconium
An abdominal X-ray taken at least 24h after birth will demonstrate multiple dilated loops
An upper gastro-intestinal contrast study can be used if there is diagnostic doubt
A preoperative contrast enema may also be useful
Unlike duodenal atresia, associated anomalies are uncommon
Duodenal atresia
The preferred operation for duodenal atresia is a duodeno-duodenostomy, which can be performed open or laparoscopically.2
Surgery
1. Mobilize duodenum
2. Duodenoduodenostomy
3. Check distal intestine for further atresias
Additional procedures that may become necessary
Further distal anastomoses
Benefits
Restore intestinal continuity
Enable enteral feeding
Alternative procedures/conservative measures
Can be performed laparoscopically or open
Without surgical correction duodenal atresia is incompatible with life
Significant/frequently occurring risks1
Early complications
Bleeding
Wound infection
Anastomotic leak
Prolonged ileus
Damage to the bile ducts has been reported
Late complications
Anastomotic stricture
Adhesive bowel obstruction
Gastro-oesophageal reflux
Duodenogastric reflux
Gastric and duodenal ulcers
Megaduodenum
Type of anaesthesia/sedation
General anaesthesia
Follow-up/need for further procedure
Late duodenal dysmotility resulting in megaduodenum may require tapering duodenoplasty
Multidisciplinary follow-up may be required for ongoing care of associated abnormalities
Jejunal or ileal atresias
Jejunal and ileal atresias are classified into five types (Table 16.1).
Type I | A complete occluding web (Fig. 16.3a) |
Type II | Proximal and distal segments (Fig. 16.3b) |
Type IIIa | Complete separation with a mesenteric defect (Fig. 16.3c) |
Type IIIb | Proximal jejunal atresia with complete absence of the mesentery to the distal bowel—‘Christmas tree’ or ‘apple peel’ atresia |
Type IV | Multiple atresias |
Type I | A complete occluding web (Fig. 16.3a) |
Type II | Proximal and distal segments (Fig. 16.3b) |
Type IIIa | Complete separation with a mesenteric defect (Fig. 16.3c) |
Type IIIb | Proximal jejunal atresia with complete absence of the mesentery to the distal bowel—‘Christmas tree’ or ‘apple peel’ atresia |
Type IV | Multiple atresias |
Associated anomalies are uncommon with jejuno-ileal atresias. However, some atresias are as a result of complicated meconium ileus in utero. Genetic testing for cystic fibrosis should be performed.
Surgical repair involves anastomosing the proximal and distal atretic segments to restore continuity and aims to preserve intestinal length.
Surgery
1. Usually a transverse supra-umbilical incision
2. Assess length
3. Trace dilated bowel distally to the atresia
4. Resect proximal dilated bowel in case of major size discrepancy if sufficient length allows
5. Perform tapering or imbrication enteroplasty of proximal bowel if necessary to preserve bowel length
6. Perform enterostomy if baby is unstable
7. Examine for further atresias by flushing distal loop
Additional procedures that may become necessary
Bowel resection
Anastomosis
Formation of enterostomy
Benefits
Restore intestinal continuity
Enable enteral feeding
Alternative procedures/conservative measures
Without surgical correction intestinal atresias are incompatible with life
Significant/frequently occurring risks3
Early complications
Bleeding
Wound infection
Anastomotic leak
Late complications
Anastomotic stricture
Adhesive bowel obstruction
Gastro-oesophageal reflux
Short bowel syndrome
PNALD
Sepsis
Death
Type of anaesthesia/sedation
General anaesthesia
Follow-up/need for further procedure
Bowel length influences the long-term outcome in these patients: 5–10% of children may develop short-bowel syndrome and need long-term parenteral nutrition or even a liver or small bowel transplant
Motility problems may occur many years after surgical repair. Dilatation of the proximal bowel with anastomotic narrowing may require anastomotic revision, tapering, or plication
Bland–Sutton classification of intestinal atresia: (a) complete occluding web, mural continuity; (b) cord joining proximal and distal segments; and (c) complete separation with mesenteric defect.
References
Surgery for Hirschsprung's disease
Description
Hirschsprung's disease is a congenital gut motility disorder characterized by absence of ganglion cells in a variable length of distal large bowel.
Incidence is around 1 in 5000 live births1
Classical triad:
Failure to pass meconium in the first 48h of life
Bilious vomiting
Abdominal distension
A very small number have chronic constipation and are eventually diagnosed later in infancy or childhood
Rectal biopsy
Although diagnosis may be suggested by a contrast enema showing the ‘transitional zone’, the gold standard is by histological examination of a rectal biopsy to ascertain aganglionosis. A suitable diagnostic specimen of rectal mucosa and submucosa is required. A rectal suction biopsy can usually be performed at the cot-side in infants.
Additional procedures that may become necessary
Inadequate biopsy will require further biopsy to be taken
Benefits
To enable diagnosis
Alternative procedures/conservative measures
Rectal suction biopsy
Open rectal biopsy
Significant/frequently occurring risks
Bleeding
Inadequate biopsy
Infection
Type of anaesthesia/sedation
Anaesthesia is not required for rectal suction biopsy in infants as biopsy is taken from above the dentate line
General anaesthesia—for larger children having open biopsy
Follow-up/need for further procedure
If Hirschsprung's disease is confirmed, a daily rectal washout regimen will need to be initiated. Parents can be taught to perform rectal washout and definitive surgery discussed. This is commonly performed as a single procedure primary pull-through
If adequate bowel decompression cannot be achieved by washouts, defunctioning enterostomy will be required
The aim of surgery is to resect aganglionic bowel and to re-anastomose the remaining ganglionic bowel to the anal canal. Both of these operations aim to preserve the innervation of the pelvic organs by minimizing the pelvic dissection.
In the Duhamel procedure (Fig. 16.4c) the distal rectum is left in situ and the normal bowel is brought down in a retro-rectal tunnel. An end-to-side anastomosis is performed before stapling the common septum to create a rectal pouch
In the Soave operation (Fig. 16.4a) the seromuscular layer is separated from the mucosal layer of the rectum, which is then removed leaving a sheath of muscle. The normal bowel is then pulled through this muscle and anastomosed to the anus
(a) Soave, (b) Swenson, and (c) Duhamel procedures for Hirschsprung's disease.
Both techniques are suitable for laparoscopic assisted repair.
Additional procedures that may become necessary
A colostomy may be fashioned to protect the pelvic anastomosis
Closure of stoma
Benefits
Remove aganglionic colon
Enable patients to pass stool
Alternative procedures/conservative measures
Can be performed laparoscopically or open
Can be done as a one-stage or a two-stage procedure with a covering stoma in the newborn
A levelling colostomy can be performed followed by definitive pull through at a later stage for those patients who present late and have significant colonic distension
Can be performed via a abdominal and transanal approach or totally via the transanal approach
Significant/frequently occurring risks
Early complications
Bleeding
Wound infection
Perianal excoriation
Rectal prolapse
Anastomotic leak
Pelvic sepsis
Late complications
Anastomotic stricture
Rectocolonic spur formation (Duhamel)
Adhesional bowel obstruction
Constipation (∼30%)
Enterocolitis (15–30%)
Incontinence
Death
Type of anaesthesia/sedation
General anaesthesia
Urinary incontinence and sexual dysfunction may affect a small number of adult and adolescent patients
Patients who have had a Duhamel may develop constipation due to a recurrent rectocolonic spur which may need re-division
Constipation can be a problem following both procedures
Frequent bowel movements can also be a problem but should usually subside within 6–12 months; 80% of patients should have fewer than three to four bowel motions per day 2–3 years after a pull-through procedure
References
