Complications, risks, and consequences
Estimated frequency
Most significant/serious complications
Bleeding or hematoma formationa
1–5 %
Hernia recurrencea (10 year)
For open surgery
0.1–1 %
For laparoscopic surgery
1–5 %
Testicular ischemia, testicular atrophy
Incarcerated, but forcibly reducible
1–5 %
Incarcerated, but forcibly not reducible
5–20 %
Ovarian torsion/loss (ovarian loss in the newborn has been reported to be up to 14 %)
5–20 %
Small bowel obstruction
In the newborn
1–5 %
In > newborns
0.1–1 %
Laparotomya (bowel injury or adhesion-related strangulation or ischemia as for the newborn)
1–5 %
Rare significant/serious problems
Infectiona
0.1–1 %
Neural injurya
Ilioinguinal nerve
0.1–1 %
Iliohypogastric nerve
0.1–1 %
Vascular injury – artery or vein
0.1–1 %
Spermatic cord injurya (parts of vas have been found in 0.5 % of operative specimens of the sac)
0.1–1 %
Less serious complications
Pain/discomfort/tenderness(<2 months; usually only days)a
5–20 %
Pain/discomfort/tenderness(>2 months)
0.1–1 %
Scrotal/labial swelling
5–20 %
Urinary retention
0.1–1 %
Dehiscencea (rare, may occur after testicular infarction)
<0.1 %
Dimpling/deformity of the skina
0.1–1 %
Wound scarring (all)
0.1–1 %
Drain tube(s)a
0.1–1 %
Perspective
See Table 3.1. Herniotomy is a much simpler procedure than open inguinal hernia repair in adults. Complications from herniotomy are infrequent and usually minor in nature. However, precise recording of these is limited by under-reporting. Spermatic cord injury occurs in at least 1 in 200 children (as the histopathology of the resected sac does not take into account internal blockage of the vas from handling). Therefore, the practice of exploring both sides in males under 2–4 years of age is disappearing. Bowel injury is rare, but when it does occur in the compromised ventilated neonate, it can trigger necrotizing enterocolitis and major ongoing problems. Infection and bleeding are other complications, but are usually minor. True recurrence rates are not known, as the follow-up must extend into later adult life to accurately analyze these, and most patients are lost to late follow-up. But, within childhood, the recurrence rate for healthy children could be as low as 0.1 % for open surgery and 3–5 % for laparoscopic closure of the deep ring, thereby implying that laparoscopic closure has a greatly increased recurrence rate in children. In the male, an asynchronous hernia occurs in approximately 8 % on the right if the index (initial) hernia repair was on the left and 4 % on the left if the previous index repair was on the right. Gonadal infarction and injury is usually a consequence of the problem, rather than a surgical complication per se, but is important to discuss preoperatively where practicable.
Major Complications
The loss of the gonad from venous infarction or torsion is a serious consequence. Bowel injury is rare in children, but can be significant especially after bowel incarceration, obstruction, or infarction with perforation. In the neonate, an appendix in the hernia can cause problems with deep-seated and systemic infection (and the rare T-antigen exposure) if the appendix has infarcted in the hernia, which cannot be predicted preoperatively. Infection also increases the risk of dehiscence, especially if nonabsorbable sutures are used.
Consent and Risk Reduction
Main Points to Explain
GA risk
Pain/discomfort
Bleeding/hematoma*
Infection (local/systemic)*
Urinary obstruction*
Risk of other abdominal organ injury*
Gonad loss
Hernia recurrence*
Risks without surgery*
*Dependent on pathology, comorbidities, and surgery performed
Surgery for Exomphalos (Omphalocele)
Description
Exomphalos (also termed omphalocele) is an abdominal wall defect that is nearly always picked up by antenatal ultrasound, if one has been done. Exomphalos occurs in approximately 1: 5,000–6,000 live births. There is a field defect in the abdominal wall, which may be small – “exomphalos minor” – or may be large and remain within the abdominal area – “exomphalos major” (which is large enough to contain liver). One subgroup, the “giant exomphalos,” has a lesion bigger than the head, the lungs are hypoplastic, and the mortality is high. An even more massive defect is where the lesion extends up into the chest – the “thoracoabdominal cleft.” As the exomphalos with its field defect extends further and further, so more and more structures are involved, and the mortality rises. With thoracoabdominal cleft, for example, when picked up antenatally, most fetuses will die before birth, and those that are live-born will have a high mortality.
In a large exomphalos, there is a broad-based midline defect that can extend extensively up and down the abdomen, thorax, or pelvis, with defects in the organs within that field. The lesion is covered by a membrane that is probably the remnant of the ectoderm and the endoderm, where the muscle somites did not grow between these two layers. Severe versions in the lower abdomen are often associated with exstrophy of the urinary bladder; severe lesions in the upper abdomen and thorax are associated with exstrophy of the heart, heart defects, midline chest wall defects, and underdeveloped lungs. There may be associated lethal chromosome defects, but these are surprisingly more often associated with the smaller exomphalos minor.
The closure of a major exomphalos (one containing most of the liver) or giant exomphalos (where the lesion is bigger than the baby’s head) can be very difficult. Where the exomphalos is large, a staged procedure is often used to gradually force the viscera back into the abnormally small abdomen over a period of 7–10 days. The covering membrane is usually removed at birth to be replaced with a tough Silastic sheet or polyvinyl bag (the silo), which in turn is used to force the viscera back into the abdomen as fast as can be tolerated. Usually a muscle or fascial sheath (using flaps of rectus sheath turned medially) can be achieved, but for giant exomphalos, the procedure may have to be staged over years after initial primary skin closure alone. The mortality can be high, especially with large, repeated, or complicated procedures.
Anatomical Points
For omphalocele (exomphalos) additional defects are present in up to 70 % of patients (higher when detected in utero, as the most complex cases die in utero). The size of the defect determines the anatomical disturbance necessitating surgical correction. The extent of herniation and amount of abdominal wall available for reconstruction can vary considerably with the degree of the field defect involved. Accordingly, the surgery has to be individualized. For thoracoabdominal clefts, the heart defects, the pericardial defect, the diaphragmatic defects, and pulmonary hypoplasia increase risks and may make surgery almost impossible.
Table 3.2
Surgery for exomphalos (omphalocele) estimated frequency of complications, risks, and consequences
Complications, risks, and consequences | Estimated frequency |
|---|---|
Most significant/serious complications | |
Infection (higher with more severe defects)a | 20–50 % |
Bleeding or hematoma formationa | 5–20 % |
Numbness/altered sensation | 1–5 % |
Hernia recurrencea (10 year) | 1–5 % |
Cardiorespiratory failure (especially high with congenital heart disease)a | 5–20 % |
Multisystem organ failure (especially high with congenital heart disease)a | 5–20 % |
Prolonged ventilationa | >80 % |
TPN (total parenteral nutrition)a | 50–80 % |
Suture abscess +/− suture sinusa | 1–5 % |
Small bowel obstruction (later) | 20–50 % |
Dehiscencea | 20–50 % |
Deatha | 20–50 % |
Less serious complications | |
Pain/discomfort/tenderness(<2 months) | 20–50 % |
Pain/discomfort/tenderness(>2 months) | 0.1–1 % |
Seroma formation | 5–20 % |
Scarring/dimpling/deformity of the skina | >80 % |
Drain tube(s)a | 50–80 % |
Perspective
See Table 3.2. Exomphalos, in its various forms, has complications proportional to the amount of anatomical disturbance and associated organ defects. The early complications of abdominal wall repair are mainly related to respiratory insufficiency, either due to the force used to reduce the viscera or due to primary pulmonary hypoplasia or both. Infection can be problematic when the defect cannot be closed quickly, especially where the closure is not ideal. Silastic may be used as a device to force the contents in, as a temporary device to get the abdomen closed placed beneath the skin, but it commonly becomes infected. Staged repairs are used and collagen matrix “Surgi-sis®” is getting some usage to leave a stronger scar where the muscles cannot be brought together for exomphalos. Absorbable materials are preferred for closure where possible, and nonabsorbable patch materials are usually avoided. For patients in multi-organ failure, skin closure with later definitive surgery is often used. Nonabsorbable patches nearly always become infected and nonabsorbable sutures may cause suture erosion where there has been difficult closure. Even “PDS®” (polydioxanone suture) may last too long and cause problems. There are often small incisional herniae especially where there has been a flapped fascial repair. Where the muscles cannot be brought together at the first session, then a large ventral hernia has to be dealt with as a later staged repair. Even if the muscles can be brought together, they often drift apart again leaving a low-profile dome of scar tissue with the centrally placed liver lying directly underneath this. Inability to close the abdominal wall with tearing of the Silastic sac from the abdominal wall muscles can occur, causing subsequent increased difficulty in later surgery, with sepsis and multi-organ failure. Most babies have to be ventilated for a prolonged period postoperatively and especially where the viscera are being forced back in. Total parenteral nutrition (TPN) is often required, with its associated metabolic and septic complications. Death occurs in about 30 % of live-born babies with a giant exomphalos, and in utero mortality occurs in about 50 % of complex patients detected on antenatal ultrasound, that is, death occurs before or at birth.
Major Complications
Death, early and late bowel obstruction, failure to close the defect, respiratory insufficiency, and ureteric obstruction can arise from the pressure being used. Infection may be a problem, with skin organisms predominating, unless bowel injury has occurred. The presence of a major foreign body (the silo) may cause infection where it is sutured to the muscle and can contaminate the bowel in the silo. Nonabsorbable sutures can increase bacterial colonization and the risk of infection. Bleeding is seldom severe unless an omental or organ injury occurs. Portosystemic anastomoses around the umbilicus can produce annoying bleeding, but is rarely severe. Hernia recurrence rates for these herniae are rather high and further increased in the presence of extensive abdominal wall deficiencies and infection. When an exomphalos major is being forced back, then abdominal compartment syndromes can arise from high pressures. If the liver is being forced more than the rest of the viscera, then liver infarction can occur, especially in those with anatomical anomalies of the blood supply. Where the pressure is more severe below the liver, the ureteric obstruction can arise causing postrenal failure. Obviously the pressure being utilized has to be tailored to achieve reduction, while avoiding these major issues. Death is a serious risk without surgery, but postoperative multisystem organ failure and sepsis remain major determinants of mortality.
Consent and Risk Reduction
Main Points to Explain
GA risk
Pain/discomfort
Bleeding/hematoma*
Infection (local/systemic)*
Urinary obstruction*
Risk of organ ischemia/infarction
Risk of other abdominal organ injury*
Possible further surgery*
Hernia recurrence
Risks without surgery*
Death*
*Dependent on pathology, comorbidities, and surgery performed
Surgery for Gastroschisis
Description
Gastroschisis is a condition that is a mechanical accident where the midgut loop ruptures out through the side of an apparently normal umbilical cord with a normal abdominal wall. This leads to protrusion of small bowel and occasionally other viscera into the amniotic cavity, from the base of the umbilical cord. The defect is becoming much more common and appears to be a vascular accident, where the frequency has been shown to increase in young mothers taking vasoactive drugs, such as cocaine. The incidence of gastroschisis in the general female reproductive age range is about 1 per 5,000 live births, but 1 per 200 live births in pregnant women under 18 years of age.
Because the intestine is protruding, irritated by amniotic fluid, it is thick walled, edematous, and shortened. As a result, there is a prolonged period of bowel dysmotility after birth that can last for life. Sections of the gut can lie over the sharp edge of the small defect and can infarct in utero. Short bowel syndrome may then occur so that 5–10 % may die or require a small bowel transplant. Up to 70 % of gastroschisis patients can have the herniated viscera returned to the abdomen primarily without the use of polyvinyl bag “silos” as the abdominal wall has its normal potential.
Anatomical Points
The size of the defect can vary, as can the site, degree and length of bowel affected. Anatomical variants causing vascular or bowel obstruction may complicate the surgical anatomy and dictate resection of small bowel or more complex repairs, with attendant associated complications.
Table 3.3
Surgery for gastroschisis estimated frequency of complications, risks, and consequences
Complications, risks, and consequences | Estimated frequency |
|---|---|
Most significant/serious complications | |
Infection (higher with more severe defects) | 20–50 % |
Bleeding or hematoma formationa | 5–20 % |
Numbness/altered sensation | 1–5 % |
Hernia recurrencea (10 year) | 1–5 % |
Cardiorespiratory failure (especially high with congenital heart disease)a | 5–20 % |
Multisystem organ failure (especially high with congenital heart disease)a | 5–20 % |
Prolonged ventilationa | 50–80 % |
TPN (total parenteral nutrition)a | 50–80 % |
Bowel dysmotilitya | >80 % |
Malabsorption and failure to thrivea | 5–20 % |
Suture abscess +/− suture sinusa | 1–5 % |
Small bowel obstruction (later) or pseudo-obstruction for lifea | 20–50 % |
Multiple hospital admissions (for all complications)a | 20–50 % |
Liver failure (often from short bowel syndrome and TPN)a | 5–20 % |
Small bowel transplanta | 5–20 % |
Dehiscencea | 20–50 % |
Deatha | 5–20 % |
Less serious complications | |
Pain/discomfort/tenderness(<2 months) | 20–50 % |
Pain/discomfort/tenderness(>2 months) | 0.1–1 % |
Seroma formation | 5–20 % |
Scarring/dimpling/deformity of the skina | 1–5 % |
Drain tube(s)a | 1–5 % |
Perspective
See Table 3.3. Gastroschisis is not usually associated with pulmonary hypoplasia, so lung problems are less frequent overall. The abdominal cavity is fully developed, but tends to be small because the gut has not been present inside to expand it, as the body grows. Even staged closure is relatively easy in comparison to exomphalos. Gastroschisis is a problem especially for young mothers. They require a great deal of care to get the child growing properly, and nearly all have problems with constipation because of the motility problems. Those with short bowel are often hospitalized frequently and for months, with or without transplantation. The abdominal wall repair can be associated with respiratory insufficiency, primarily due to the tightness when the viscera are reduced. Infection can occur from cutaneous or bowel organisms, especially when bowel injury occurs in utero or at surgery. Abdominal prosthetic patches are rarely required, and absorbable sutures are typically preferred.
Major Complications
Infection may be a problem, with skin organisms predominating unless bowel injury has occurred. Since foreign material is seldom used, infection rates are generally lower than for exomphalos repairs. Nonabsorbable sutures can increase bacterial colonization and the risk of infection. Bleeding is seldom severe unless an omental, bowel, or organ injury occurs. Hernia recurrence rates for these herniae are moderate and further increased in the presence of infection, poor healing, multisystem organ failure, or nutritional deficiency. Lifelong immunosuppressants following SB transplantation or home chronic TPN may be necessary if short bowel syndrome occurs. Liver failure may ensue. Overall, multisystem organ failure and death are not common. Nutritional deficiency, short bowel syndrome, and bowel dysmotility can be significant chronic problems. Repeated or prolonged hospitalization can be a significant consequence.
Consent and Risk Reduction
Main Points to Explain
GA risk
Pain/discomfort
Bleeding/hematoma*
Infection (local/systemic)*
Nutritional deficiency
Risk of other abdominal organ injury*
Hernia recurrence
Possible further surgery*
Risks without surgery*
Death*
*Dependent on pathology, comorbidities, and surgery performed
General Abdominal Surgery
Open Pyloromyotomy for Pyloric Stenosis
Description
General anesthesia is used. Pyloric stenosis is hypertrophy of the pyloric muscle and occurs in approximately 1:200 male and about 1:1,000 female live births (i.e., 85 % are male). It typically develops in the first 4–6 weeks of life in the first-born male. The aim is to divide the constricting hypertrophic muscle that encircles the pyloric region, using a longitudinal (Ramstedt) pyloromyotomy, exposing the underlying intact mucosa. The pyloromyotomy allows drainage by incising longitudinally, approximately 1–2 cm, through the muscle wall of the pyloric canal from immediately proximal to the duodenal bulb and then back to the antrum. This longitudinal incision deliberately leaves the muscle wall gaping, down to the intact mucosa, which then balloons into the muscle defect allowing passive drainage of gastric contents.
Anatomical Points
The anatomy of the pylorus is relatively constant; however, the extent of the pyloric hypertrophy may vary, proximally up the antral wall. The hypertrophy always ends abruptly at the junction of the pylorus and duodenum, with no duodenal extension, and there is a normal duodenal bulb. The degree of preoperative obstruction, projectile vomiting, and consequent acid-base and electrolyte imbalance will vary considerably and has to be corrected prior to surgery.
Table 3.4
Open pyloromyotomy for pyloric stenosis estimated frequency of complications, risks, and consequences
Complications, risks, and consequences | Estimated frequency |
|---|---|
Most significant/serious complications | |
Infectiona | |
Subcutaneous/wound | 5–20 % |
Intra-abdominal | 0.1–1 % |
Systemic | 0.1–1 % |
Bleeding or hematoma formationa | 1–5 % |
Wound breakdown and evisceration | 5–20 % |
Perforation of the mucosa or duodenum | 5–20 % |
Reflux esophagitis | 5–20 % |
Delayed gastric emptyinga | 20–50 % |
Recurrence/persistence of pyloric obstructiona | 1–5 % |
Mortalitya | 0.1–1 % |
Mortality without surgery | 20–50 % |
Rare significant/serious problems | |
Liver injurya | 0.1–1 % |
Small bowel obstruction (early or late; lifetime)a [Adhesion formation] | 0.1–1 % |
Subphrenic abscess | <0.1 % |
Multisystem failure (renal, pulmonary, cardiac failure) | <0.1 % |
Less serious complications | |
Paralytic ileus | 1–5 % |
Slow recovery requiring prolonged feeding with small frequent feeds | 5–20 % |
Wound scarring (poor cosmesis/wound deformity) | 1–5 % |
Incisional hernia | 5–20 % |
Prolonged use of nasogastric tubea | 1–5 % |
Perspective
See Table 3.4. Most of the complications are of a minor nature, and children usually recover rapidly from surgery. Major complications are related to perforation of the mucosa, wound infection, recurrence of pyloric obstruction, and rarely later complications of small bowel obstruction. Correction of preoperative electrolyte disturbances and ensuring adequate nutrition are important considerations.
Major Complications
Inadvertent perforation and leakage may be serious and occasionally not recognized at operation, leading to intra-abdominal infection and abscess formation. If the perforation is recognized and closed during operation, the risk of infection is approximately doubled, but if not recognized, then intra-abdominal sepsis is almost inevitable. Infection and multisystem failure may be catastrophic, although death is very rare. Bleeding is rare and is usually controlled at surgery. Wound infection is common and occurs especially in malnourished infants with severe prolonged vomiting prior to presentation and diagnosis. Wound dehiscence and consequent hernia formation may result. Persistent pyloric stenosis can result from inadequate division of the hypertrophic muscle, but true recurrent pyloric stenosis is very rare. Small bowel obstruction is rare but may occur from postoperative adhesions, even many years later.
Consent and Risk Reduction
Main Points to Explain
GA risk
Pain/discomfort
Bleeding/hematoma*
Infection (local/systemic)*
Risk of other abdominal organ injury*
Perforation*
Recurrence of stenosis
Possible further surgery*
Risks without surgery*
*Dependent on pathology, comorbidities, and surgery performed
Laparoscopic Pyloromyotomy for Pyloric Stenosis
Description
General anesthesia is used. Pyloric stenosis is hypertrophy of the pyloric muscle and occurs in approximately 1:200 male and about 1:1,000 female live births (i.e., 85 % are male). It typically develops in the first 4–6 weeks of life in the first-born male. The aim is to divide the constricting hypertrophic muscle that encircles the pyloric region, using a longitudinal (Ramstedt) pyloromyotomy, exposing the underlying imperforated mucosa. The pyloromyotomy allows drainage by incising longitudinally, approximately 1–2 cm, through the muscle wall of the pyloric canal from immediately proximal to the duodenal bulb and then back to the antrum. This longitudinal incision deliberately leaves the muscle wall gaping, down to the intact mucosa, which then balloons into the muscle defect allowing passive drainage of gastric contents. Approximately half of all pyloromyotomies are now carried out laparoscopically even in premature babies. This frequency will continue to increase, as it is a good training procedure for younger surgeons starting laparoscopy in pediatric patients. In all babies (down to 1.0 Kg), there is no need for access ports other than for the 3 mm telescope. The other 3 mm instruments are placed directly through small stab wounds so that the cosmetic results are superior.
Anatomical Points
The anatomy of the pylorus is relatively constant; however, the extent of the pyloric hypertrophy may vary, proximally up the antral wall. The hypertrophy always ends abruptly at the junction of the pylorus and duodenum, with no duodenal extension, and there is a normal duodenal bulb. The degree of preoperative obstruction, projectile vomiting, and consequent acid-base and electrolyte imbalance will vary considerably and has to be corrected prior to surgery. Adhesions from previous surgery or an enlarged liver may make laparoscopic access more difficult.
Table 3.5
Laparoscopic pyloromyotomy for pyloric stenosis estimated frequency of complications, risks, and consequences
Complications, risks, and consequences | Estimated frequency |
|---|---|
Most significant/serious complications | |
Infectiona | |
Subcutaneous/wound | 5–20 % |
Intra-abdominal | 0.1–1 % |
Systemic | 0.1–1 % |
Bleeding or hematoma formationa | 1–5 % |
Wound breakdown and evisceration | 5–20 % |
Perforation of the mucosa or duodenum | 5–20 % |
Reflux esophagitis | 5–20 % |
Delayed gastric emptying | 20–50 % |
Conversion to open operation | 1–5 % |
Recurrence/persistence of pyloric obstruction | 1–5 % |
Mortality without surgery | 20–50 % |
Rare significant/serious problems | |
Pneumothorax | 0.1–1 % |
Gas embolus | 0.1–1 % |
Liver injury | 0.1–1 % |
Small bowel obstruction (early or late; lifetime)a [Adhesion formation] | 0.1–1 % |
Subphrenic abscess | <0.1 % |
Multisystem failure (renal, pulmonary, cardiac failure) | <0.1 % |
Mortality | 0.1–1 % |
Less serious complications | |
Paralytic ileus | 1–5 % |
Slow recovery requiring prolonged feeding with small frequent feeds | 5–20 % |
Surgical emphysema | 1–5 % |
Pain/tenderness | |
Acute (<4 weeks) | 5–20 % |
Chronic (>4 weeks) | <0.1 % |
Port-site herniae | 5–20 % |
Wound scarring (poor cosmesis/wound deformity) | 1–5 % |
Incisional hernia | 5–20 % |
Prolonged use of nasogastric tubea | 1–5 % |
Perspective
See Table 3.5. Most of the complications are of a minor nature, and children usually recover rapidly from surgery. With laparoscopic approaches, the risk of wound infection, wound dehiscence, and incisional hernia is reduced. Major complications are related to perforation of the mucosa, wound infection, recurrence of pyloric obstruction, and rarely later complications of small bowel obstruction. Correction of preoperative electrolyte disturbances and ensuring adequate nutrition are important considerations. With laparoscopy, there is an increased risk of mucosal perforation in the pyloric canal and of tears to the duodenal cap as the grasper holds the duodenum during division and spreading of the very mobile, hypertrophied pylorus muscle. Laparoscopic entry is associated with the complication of herniation of the omentum, or rarely bowel, through any of the port-site wounds, of about 5–10 %.
Major Complications
Inadvertent perforation and leakage may be serious and occasionally not recognized at operation, leading to intra-abdominal infection and abscess formation. If the perforation is recognized and closed during operation, the risk of infection is approximately doubled, but if not recognized, then intra-abdominal sepsis is almost inevitable. Infection and multisystem failure may be catastrophic, although death is very rare. Bleeding is rare and is usually controlled at surgery. Wound infection is common and occurs especially in malnourished infants with severe prolonged vomiting prior to presentation and diagnosis. Wound dehiscence and consequent hernia formation may result. Persistent pyloric stenosis can result from inadequate division of the hypertrophic muscle, but true recurrent pyloric stenosis is very rare. Small bowel obstruction is rare but may occur from postoperative adhesions, even many years later. Gas embolus and major vascular injury are additional serious, although very rare, complications of the laparoscopic approach. Conversion to open operation is a small but significant risk rather than a complication per se.
Consent and Risk Reduction
Main Points to Explain
GA risk
Pain/discomfort
Bleeding/hematoma*
Infection (local/systemic)*
Risk of other abdominal organ injury*
Perforation*
Recurrence of stenosis
Possible further surgery*
Risks without surgery*
*Dependent on pathology, comorbidities, and surgery performed
Congenital Diaphragmatic Hernia Repair
Description
General anesthesia is used. Congenital diaphragmatic hernia is a defect in the diaphragm that occurs before birth, leaving a free communication between the chest and abdominal cavities. The most common form is a posterolateral defect through the foramen of Bochdalek and occurs on the left side in over ¾ of cases. Abdominal contents in the chest often inhibit lung development and growth. After birth, this lack of lung volume and maturation makes gas exchange difficult or impossible. Even if the baby survives the immediate period after birth, higher than normal pulmonary vascular resistance promotes persistence of the right to left ductal and cardiac shunting of blood through a ductus arteriosus and patent foramen ovale (persistence of fetal circulation). The size of the defect and degree of herniation of contents are directly associated with the risk of complications and mortality. Larger-sized defects are less likely to close. The larger the defect, the more medially it extends until the liver herniates upward through a large hole with medial extension. If the liver has rotated up into the chest, then the baby is more likely to die than if the liver is in the abdomen. CDH is often associated with many other abnormalities: cardiac and chromosomal which often prove to be lethal. The mortality is probably 30–50 % for those detected antenatally (as long as there is no other lethal defect – the isolated CDH), but for those seen to have liver in the chest on antenatal ultrasound, the mortality is greater than 90 %.
Emergency surgery is no longer the treatment of choice. The patient must be stabilized prior to surgery, and this may take days or even weeks. When there is sufficient respiratory reserve for the patient to be able to tolerate the surgery, then a transverse upper abdominal incision is used. In those that have a good prognosis, closure of the defect is usually straightforward as the defect will not be big, and there will usually be good muscle shelves that can be unfolded. If the defect is large or the diaphragm is completely absent, then partial closure can be obtained as above, and the rest (or the whole diaphragm) can be closed using an abdominal wall flap or Gerota’s fascia (the perirenal fascia is dissected up from the lower pole of the kidney to the top, leaving the attachment to the upper pole of the kidney as a hinge from the kidney which has become more popular recently). Using a muscle flap leaves a weakness in the abdominal wall that produces a permanent bulge and possibly scoliosis later in life as the child grows. Use of Gerota’s fascia has only been introduced recently, but appears to be an effective closure without such side effects. Closing the defect with synthetic material has been used in the past, but this material does not grow with the child so that there is a very high rate of recurrence as the attachments give way. Suturing the anterior ribs to the posterior ribs at the lateral end of the defect can be used, but may lead to severe chest wall deformity and a small volume stomach later, as there is no space between the ribs for the stomach to act as a reservoir.
Anatomical Points
The diaphragm is formed from the pleuroperitoneal membranes, the septum transversum, the dorsal mesentery of the esophagus, and the body wall. The closure of the pleuroperitoneal canals joining the thorax and abdomen starts to occur by about the 6th week of gestation and should be complete by approximately 10 weeks of gestation. Failure to close fully leads to persistence of the posterolateral pleuroperitoneal canal – the foramen of Bochdalek – which occurs in approximately 1:2,200 conceptions and 1:4,000 live births, as many die before birth of multiple anomalies. Some 80 % occur on the left side. Herniation through the retrosternal area – foramen of Morgagni – is a rare defect.
Table 3.6
Surgery for congenital diaphragmatic hernia estimated frequency of complications, risks, and consequences
Complications, risks, and consequences | Estimated frequency |
|---|---|
Most significant/serious complications | |
Infection | |
Subcutaneous/wound | 1–5 % |
Intrathoracic (pneumonia; pleural) | 1–5 % |
Mediastinitis | 0.1–1 % |
Systemic | 0.1–1 % |
Prolonged assisted ventilationa,b | 80 % |
Gastroesophageal reflux | 50–80 % |
Chronic lung disease; pulmonary failurea | 50–80 % |
Diaphragmatic injury/dysfunction/paresis | 5–20 % |
Small bowel obstruction | 5–20 % |
Scoliosis and chest wall deformities (postoperative)a (especially where prosthetic patches or muscle flaps have been used) | 5–20 % |
Volvulus | 1–5 % |
Multisystem organ failure (renal, pulmonary, cardiac failure)a (ultimate cause of death in approximately 50 % of neonates) | 20–50 % |
Mortality | |
Term infantsa | 20–50 % |
Preterm infantsa (includes those detected antenatally – usual today) | 50–80 % |
Mortality without surgery (almost 100 % for severe early defects)a | >80 % |
Rare significant/serious problems | |
Bleeding/hematoma formation | |
Wound | 0.1–1 % |
Hemothorax | 0.1–1 % |
Pulmonary contusion | 0.1–1 % |
Surgical emphysema | 0.1–1 % |
Persistent air leaka | 0.1–1 % |
Deep venous thrombosis | 0.1–1 % |
Osteomyelitis of ribsa | <0.1 % |
Less serious complications | |
Pain/tendernessa,b | |
Acute (<4weeks) | 5–20 % |
Chronic (>4 weeks) | <0.1 % |
Wound scarring | 5–20 % |
Deformity of rib/chest or skin (poor cosmesis) | 1–5 % |
Perspective
See Table 3.6. Death occurs in approximately 50 % of cases when CDH is discovered antenatally and higher in preterm infants. The time at which the defect stops closing determines the outcome. If the defect stops closing early in gestation, the liver rotates into the chest, and being more solid than intestine causes more damage. The abdominal viscera (liver and intestine) push the left lung up and the heart over to the right and compress the right lung as well. So both lungs are compressed. As a result both lungs are small but also undergo a maturation arrest. The larger the defect, the more immature and small the lungs and the more complex the surgery to close it effectively. The presence of additional anomalies also contributes to the mortality and complications. Delayed lung maturation leads to a lung histology that is similar to the lung development of a very preterm baby. In addition, the lungs are smaller, so the clinician has to support a full-term baby with lungs that are far too small, and have arrested development (in the pseudoglandular phase) so that ventilator-induced chronic lung disease (of the newborn) frequently occurs, but usually resolves by a year of age – in survivors. This may be responsible for late deaths weeks and months after birth. Viral infections during this period may well tip the baby into respiratory failure and death. Coexistence of cardiac anomalies increases the mortality. The neonate is typically completely unaware of this as he/she will be paralyzed, ventilated, and sedated. When CDH is detected later (up to 20 years after birth), then pain and awareness are often more of an issue.
Major Complications
Death occurs in up to 50 % of those born alive, and death has already occurred in another 20 % before birth or immediately after birth due to the basic inability to ventilate these babies. If the liver was seen to be in the chest before birth, and if the lung to head ratio is less than 0.8 on antenatal ultrasound, death before, during, or after birth is likely in more than 90 % of fetuses. In those that survive the immediate period after birth, the next major hurdle is the high pulmonary vascular resistance, which maintains a “persistent fetal circulation”. There is often a supra-systemic pulmonary blood pressure, so that blood shunts away from the lungs at the level of the ductus arteriosus and the foramen ovale. Because of the high pressure, the right side of the heart also fails. Support therefore becomes complex, often involving pulmonary vasodilators such as inhaled nitric oxide, longer-term sildenafil therapy, and right ventricular support. Long before this occurs, the baby is often placed on oscillating ventilation to try to reduce lung damage, but long-term data has not shown any improvement in survival for those that are oscillated. This situation of hypoxia, shunting, and cardiac failure (leading to multi-organ failure) has a high mortality and is exceptionally difficult to treat. Secondary lung infection is not uncommon and may affect either lung. Viral pneumonitis is common months after the surgery, and in those that have only just survived the initial problems, the infection may well tip the baby into terminal respiratory failure. If the fluid in the almost empty left hemithorax becomes infected, this rapidly leads to generalized sepsis and multi-organ failure. Overventilation leading to pneumothorax or persistent air leak creates a situation that cannot be resolved on conventional ventilation. Oscillation or ECMO may have to be used to retrieve this situation. ECMO as a primary treatment for CDH to get the neonate through the first few days or weeks of life has not achieved universal acceptance and is rarely used in Australia. ECMO as a retrieval from an air leak can be effective.
There is an emerging group of neonates that now only just survive, as we get gradually better at retrieving the hypoxic shunting baby. These babies are now managed with permissive hypercapnea and apparently less than ideal hypoventilation (which drastically reduces lung damage and mimics the levels of tissue oxygenation that was present before birth). These babies have previously rarely seen problems.
Moderate to severe tracheobronchomalacia, where major airway collapse can occur from the soft walls of the airways, from the larynx to the major bronchi can occur. To take these patients off a ventilator, a tracheostomy may be required for months until the trachea and bronchi become more rigid and are able to support themselves. This will take months or up to a year. Another problem is persisting pulmonary hypertension that may go on for months. In the past this was an acute problem only, as the patient either recovered or died. Now, the patients are surviving through the acute phase (weeks) only for the problem to persist for months, if not a year or so.
Multisystem organ failure is extremely serious and is the usual mode of death in those that survive long enough to be ventilated. This is usually the result of the inability to oxygenate the patient or generalized sepsis. Cardiac anomalies will contribute to multi-organ failure, so that the ultimate cause of death in these patients is often a combination of problems leading to this scenario. Once established, there is a high mortality. Mortality is greater for infants with large herniae, those with a large volume of liver in the chest, cardiac disease, bilateral herniae, and in very preterm/low-birth-weight infants (<1,500 g). Those with chromosomal anomalies, e.g., trisomy 13 or 18, rarely survive birth and often die in utero. Even if they survive birth, they soon die, and treatment would not be a viable option.
For repeat surgery: In the neonate, bowel perforation is rare, but may occur in repeat surgery especially where a prosthetic patch has been used, has torn off its attachments as the baby grows, and then has to be dissected off the colon. Then the subsequent leakage and infection may be devastating. Pleural space infection (empyema) may follow leakage from the bowel, but is virtually unheard of in the first operation.
Consent and Risk Reduction
Main Points to Explain
GA risk
Pain/discomfort
Bleeding/hematoma*
Infection (local/systemic)*
Nutritional deficiency
Risk of other abdominal organ injury*
Hernia recurrence
Persistent pneumothorax
Possible further surgery*
Risks without surgery*
Multisystem organ failure
Death*
*Dependent on pathology, comorbidities, and surgery performed
Biliary Atresia and Choledochal Cyst Surgery: Biliary Bypass Drainage: Roux-en-Y Hepaticojejunostomy (The Kasai Procedure)
Description
The aim is to restore bile flow from the liver to the intestine by resecting, bypassing, or relieving the obstruction.
Biliary atresia occurs in about 1 in 10,000 live births (and may be higher in Asian populations), resulting in fibrous obliteration of the biliary tree (may occur early in life probably as a form of neonatal sclerosing cholangitis).
Choledochal cysts are at least fivefold (1:50,000) less common (but also relatively higher in Asians and females) and comprise a group of congenital dilatations of the bile ducts. The most common in the neonate is a “fusiform” dilation of the common bile duct and common hepatic duct up to the confluence of the left and right hepatic ducts. In the neonate, choledochal cysts may show some of the features of biliary atresia, and the two can coexist. For example, it is not uncommon to see a choledochal cyst in the CBD, accompanied with classical histological biliary atresia in the more distal CBD.
Long-term cholangiocarcinomas may develop in unresected choledochal cysts, a few before age 25. But, only 57 % of the cholangiocarcinomas occur in the area of the cyst. Therefore, nearly 50 % will occur in the residual biliary system after the cyst is removed and bypassed. This implies that the whole of the biliary tree is at risk. Surgery depends on the type of abnormality and extent of obstruction. For the more common choledochal cysts in childhood, the choice is limited to (i) a Roux-en-Y jejunostomy anastomosed to the common hepatic duct or (difficult in a neonate) to the right and left hepatic ducts after “fish-mouthing” the inferior borders of those ducts or (ii) a cholecocho-duodenostomy, where the common hepatic duct is joined to the upper border of the duodenum. The latter can be done quite effectively laparoscopically in the child. Close follow-up is usually required to detect late stenosis, which may produce multiple stones within the biliary tree above the stenosis; or in the case of choledochal cysts, cholangiocarcinoma of the remaining bile duct(s) can occur. Occasionally, resection of the short, affected portion of extrahepatic bile duct can be performed with primary end-end anastomosis.
In biliary atresia, the Kasai portoenterostomy joins the porta hepatis to a Roux-en-Y jejunal loop, after removing the scar tissue at the hepatic plate, leaving only the inner layers of the plate, so as to avoid entering the liver parenchyma. The dissection is carried out as far laterally as possible, and the lymphatics are preserved (not coagulated) as they may be the source of future bile drainage. In biliary atresia, one third of patients will never drain bile and go into liver failure within months of birth. Of the two thirds that drain bile, only half of them (one third or those operated on) will continue to drain bile beyond 5 years of age and may reach 20–30 years of age before they require transplant.
Anatomical Points
Knowledge of the common and uncommon biliary and vascular anatomical variants is essential for reducing mishap during biliary surgery. For choledochal cyst, the cyst is dissected out by peeling it away from its surrounding tissues, bearing in mind the anatomical variants that can occur. For biliary atresia, scar tissue is present, so the fibrous band that represents the GB is dissected down to the “fibrous pyramid” that represents the CHD and the right and left hepatic ducts, and then the vessels are identified one by one around this pyramid, taking care to identify the small arteries in the neonate (and up to 3 months of age). Once that is done, the fibrous pyramid is removed down to the last inner layer of the hepatic plate. Correct identification of the common bile duct, rather than the common hepatic duct, should be confirmed to ensure that a choledochotomy is not performed in a bile duct that is too small (no luxury of choice may exist as the cyst may well go up into the CHD and even the confluence of the hepatic ducts). Even CT cholangiograms or MRCP will not usually clarify the anatomy particularly well in the very young. Preoperatively in the neonate, the choledochal cyst can occupy the whole of the abdomen, and the whole system is distorted. For the common form of choledochal cysts, the CBD, the GB and part of the CHD are involved. For intrahepatic cysts, the cyst would probably be left until the child is older, or if causing cholangitis, a liver resection (if practical with multiple cysts) may be preferable.
Table 3.7
Surgery for biliary atresia and choledochal cyst estimated frequency of complications, risks, and consequences
Complications, risks, and consequences | Estimated frequency |
|---|---|
Most significant/serious complications | |
Death in biliary atresia as above | |
Late stenosis, cholelithiasis, and liver failure in choledochal cysts | |
Infectiona overall | 1–5 % |
Subcutaneous/wound | 1–5 % |
Intra-abdominal/liver bed/pelvic | 0.1–1 % |
Liver(hepatitis; abscess) | 0.1–1 % |
Cholangitis | 20–50 % |
Systemic | 0.1–1 % |
Bleeding/hematoma formation | |
Wound | 1–5 % |
Anastomotic; raw liver surface | 1–5 % |
Gastrointestinal (incl. variceal) hemorrhage | 20–50 % |
Liver failure long term (cirrhosis)a | 20–50 % |
Portal hypertension | 20–50 % |
Bile leak/collection | 20–50 % |
Biliary fistulaa | 5–20 % |
Biliary ischemia/stenosis/obstruction/cholelithiasisa | 20–50 % |
Small bowel fistulaea | 1–5 % |
Later cholangiocarcinomaa | 1–5 % |
Mortality (10 year)a | 20–50 % |
Mortality without surgerya | >80 % |
Rare significant/serious problems | |
Injury to the bowel or blood vessels | 0.1–1 % |
Gastric/duodenal/small bowel/colonic | |
Bile/hepatic duct injury | 0.1–1 % |
Liver injury | 0.1–1 % |
Biliary ascites | 0.1–1 % |
Failure to detect/remove calculi | 0.1–1 % |
Jejunal fistula | 0.1–1 % |
Small bowel ischemia | 0.1–1 % |
Multisystem organ failure (renal, pulmonary, cardiac failure)a | 0.1–1 % |
Small bowel obstruction (early or late)a [Anastomotic stenosis/ischemic stenosis/adhesion formation] | 0.1–1 % |
Operative cholangiogram | |
Dye reaction/cholangitis/pancreatitis/radiation exposure | <0.1 % |
Possibility of colostomy/ileostomy (very rare)a | 0.1–1 % |
Pancreatitis/pancreatic injury/pancreatic cyst/pancreatic fistulaa | 0.1–1 % |
Aspiration pneumonitis | 0.1–1 % |
Wound dehiscence | 0.1–1 % |
Incisional hernia formation (delayed heavy lifting/straining) | 0.1–1 % |
T-tube-related complications (if used) | |
T-tube cholangiogram | |
Dye reaction/cholangitis/pancreatitis/radiation exposure | <0.1 % |
Blockage of T-tube | 0.1–1 % |
Dislodgment of T-tube | 1–5 % |
Persistent biliary fistula (after removal; cholangio-cutaneous) | 0.1–1 % |
Less serious complications | |
Pain/tenderness | |
Acute (<4 weeks) | 5–20 % |
Chronic (>4 weeks) | <0.1 % |
Seroma/lymphocele formation | 0.1–1 % |
Muscle weakness (atrophy due to denervation esp subcostal incision) | 1–5 % |
Paralytic ileusa | 50–80 % |
Nasogastric tubea | 1–5 % |
Blood transfusion | <0.1 % |
Wound drain tube(s)a | 1–5 % |
Wound scarring (poor cosmesis/wound deformity) | 1–5 % |
Perspective
See Table 3.7. Early major complications are related to failure to drain bile, bleeding, bile leakage, and infection, and later complications include biliary stenosis and bile duct malignancy (for cysts) and recurrent cholangitis and liver (cirrhosis) fibrosis (for those with biliary atresia (BA)). Minor complications are common and usually resolve without sequelae. Coexisting congenital disorders or anomalies, e.g., cardiac, especially left atrial isomerism, may predispose to higher risks of complications and failures. When these patterns of disease coexist with biliary atresia, they are known as syndromal forms of BA. When these syndromal forms occur, they have a far worse prognosis than the idiopathic form. Longer-term survival is dependent on disease progression, recurrent infections, cholangiocarcinoma (in choledochal cysts), and the inevitable ongoing fibrosis of the liver in biliary atresia. This leads eventually to liver failure in every patient with BA. Liver transplantation will typically be required for biliary atresia in the long term. So, all patients would die without transplant at some stage, 2/3 before 5 years of age. The best units internationally can obtain 40–45 % of patients out to 5 years without transplant, but if not transplanted, all eventually succumb to liver failure. Transplant can be carried out down to 5 Kg body weight and possibly less, depending on the patient, disease, and unit. Without transplant all of these patients will go into liver failure at some age and die.
For choledochal cysts, there is a high rate of stenosis and cholelithiasis usually occurring 10–20 years after the initial surgery. This may even require liver transplantation. For the Kasai procedure, death will be inevitable without liver transplant at varying times (however, the success for the Kasai procedure is only about 5 years or so of adequate drainage).
Major Complications
For biliary atresia, one of the main complications is failure of adequate biliary drainage and persistent jaundice, with continued liver fibrosis/cirrhosis no matter whether they get drainage or not. Only the relative rates change. Fibrosis is faster in those that are not drained, so that synthetic function fails earlier, with subsequent liver cirrhosis, also due to progressive biliary fibrosis. Paradoxically, in those that drain well, there is a higher incidence of subsequent bacterial cholangitis, which can be very difficult to treat, accompanied by cessation of bile drainage, and may become chronic leading to permanent loss of drainage and liver failure. If there is no anatomical path for drainage (e.g., failed Kasai), then there is no path for bacteria, and cholangitis does not occur. Bile leakage may occur and can lead to bile ascites or fistula formation, surprisingly easily controlled and usually stops within a week or two. Venous bleeding can be catastrophic during the procedure and difficult to control and maintain flow. Portal hypertension with variceal bleeding often occurs after a Kasai procedure and often presents a significant risk to the patient. Cirrhosis with fibrosis of the liver and biliary system with excretory failure is a serious problem. Bleeding may be severe, arising from either arterial or venous injury (more likely in the adult patient as, in the child, the arterial vessels are very small). However, bleeding usually stops, but the liver parenchyma can be devascularized, the extent of which depends on the level of injury.
For choledochal cyst, biliary stricture formation may occur at any stage postoperatively and necessitate further surgery. Recurrent attacks of hyperamylasemia (raised lipase also) and pancreatitis are not infrequent after choledochal cyst surgery and are usually mild and can be managed nonsurgically in the majority of cases. Severe pancreatitis is relatively rare. Cholangiocarcinoma in the remaining choledochal cyst is not uncommon, often many years later.
Wound infection, peritonitis, or abscess formation may lead to multisystem organ failure and death, but the incidence of these complications is surprisingly small for the first operation. A second exploration, after sudden cessation of bile drainage, is now rarely used and multiple operations virtually never. With repeat operations, the complication rates increase, and the complications of subsequent liver transplantation are also increased, so that repeat surgery is employed less and less. These procedures carry a significant short- and longer-term risk of further complications and mortality.
Consent and Risk Reduction
Main Points to Explain
GA risk
Pain/discomfort
Bleeding/hematoma*
Infection (local/systemic)*
Nutritional deficiency
Risk of other abdominal organ injury*
Recurrent biliary stricture
Liver failure and transplantation
Later malignancy
Possible further surgery*
Risks without surgery*
Multisystem organ failure
Death*
*Dependent on pathology, comorbidities, and surgery performed
Surgery for Congenital Duodenal Obstruction
Description
General anesthesia is used. Congenital duodenal obstruction is due to either duodenal atresia (75 %) or stenosis (25 %) resulting in third-trimester polyhydramnios, duodenal dilatation, and early vomiting. Duodenal atresia or stenoses due to malrotation (with Ladd’s bands and/or volvulus), annular pancreas, and duodenal web represent the main underlying causes.
Duodenal atresia is often diagnosed antenatally. The diagnosis usually occurs late in the pregnancy so that termination is no longer possible. Approximately 30 % of those diagnosed antenatally will have a chromosomal abnormality (nearly all trisomy 21), and many duodenal atresia patients will have other abnormalities (even if they do not have a chromosomal abnormality). As a result, prenatal counseling can be complex. Duodenal atresias can present surprisingly late after birth, often after several days. The over-distended stomach empties itself immediately after birth, and the baby can then apparently tolerate small feeds for a considerable time while the stomach refills. Atresia is corrected by a duodenoduodenostomy, where the distal duodenum is rotated and flipped over the area of the obstruction to be joined to the proximal duodenum. This avoids dissecting into the area where the bile and pancreatic ducts enter the duodenum (nearly always close to the level of the obstruction).
Duodenal stenosis can vary, and the degree of obstruction determines the timing and severity of onset of symptoms, after birth. Occasionally, a patient with duodenal stenosis may not present for several years. The aim of surgery is either to open the obstructed area or to bypass it. A duodenal web is usually corrected by a longitudinal duodenotomy with incision or fulguration of the antimesenteric portion of the web (again to avoid damage to the bile ducts which can run either on surface of the web or usually closer to the mesenteric border). Once a web has been divided, the duodenum is closed transversely.
Anatomical Points
The anatomy of the duodenum is determined by the type of congenital defect present and whether malrotation is also a factor. The ampulla of Vater may be injured in the surgery, largely depending on the proximity to the site of obstruction and surgery, as the bile ducts open above or below the level of obstruction, or on the actual web when present. Concurrent cardiac anomalies may cause cardiac failure postoperatively and increase mortality. Other anomalies are present frequently in association with atresia or stenosis including Down’s syndrome (trisomy 21).
Table 3.8
Surgery for congenital duodenal obstruction estimated frequency of complications, risks, and consequences
Complications, risks, and consequences | Estimated frequency |
|---|---|
Most significant/serious complications | |
Infectiona | |
Subcutaneous/wound | 1–5 % |
Intra-abdominal | 5–20 % |
Mediastinitis | <0.1 % |
Systemic (especially in those with chromosomal abnormalities +/− heart disease) | 5–20 % |
Bleeding and hematoma formationa | 1–5 % |
Pancreatic injury/leakage/damage to the bile ducts | 5–20 % |
Green bilious aspirates (universal for days or weeks after the surgery) | >80 % |
Duodenal leak at the anastomosis | 5–20 % |
Recurrence/persistence of duodenal obstruction (some degree of duodenal dysmotility will persist for life, so that GOR is very common) | 1–5 % |
Reflux esophagitis (GOR) | 20–50 % |
Multisystem failure (renal, pulmonary, cardiac failure) – overall (especially high in those with chromosomal abnormalities +/− heart disease)a | 1–5 % |
Small bowel obstruction (early or late)a [Anastomotic stenosis/adhesion formation] | 1–5 % |
Mortality – overall (depends on severity of other anomalies, rather than the duodenal obstruction itself) | 1–5 % |
Mortality without surgery (may be up to 100 %) (lethal chromosomal abnormalities (e.g., trisomy 18) may preclude surgery)a | >80 % |
Rare significant/serious problems | |
Liver injury | 0.1–1 % |
Subphrenic abscess | <0.1 % |
Complete anastomotic (duodenotomy) breakdown | 0.1–1 % |
Less serious complications | |
Pain/tenderness | |
Acute (<4 weeks) | 5–20 % |
Chronic (>4 weeks) | <0.1 % |
Intolerance of large meals (necessity for small frequent meals) | 20–50 % |
Delayed gastric emptying (Universal for up to several weeks and probably universal to some extent for life) | >80 % |
Wound scarring (poor cosmesis/wound deformity) | 1–5 % |
Nasogastric tubea (sometimes for weeks) | 50–80 % |
Perspective
See Table 3.8. Overall mortality is often more directly related to severe associated anomalies (chromosomal and congenital) rather than the duodenal obstruction itself. Those with lethal chromosomal abnormalities (e.g., trisomy 18) may be so severe as to preclude surgery. The major surgical complication is a leak at the anastomosis. Gastric emptying will seldom be normal, and the stomach takes weeks before it is emptying adequately. This may necessitate a naso-enteric tube. Duodenal obstruction is often seen within days of birth. Intravenous feeding may be required if the stomach will not empty postoperatively. Many complications are of a minor nature, and babies recover rapidly from surgery. Major complications are related to infection, perforation, recurrence of duodenal obstruction, leakage, and later complications of small bowel obstruction. Major infections, multisystem failure, and mortality are higher in those with significant congenital anomalies, often cardiac.
Major Complications
Anastomotic leakage is serious and occasionally not recognized early, leading to intra-abdominal infection and abscess formation. Systemic infection and multisystem failure may then ensue. Concurrent cardiac anomalies may cause cardiac failure and increase mortality. Bleeding is rare and is usually controlled at surgery. Wound infection may occur and may lead to an incisional hernia. Wound dehiscence is rare. Persistent duodenal obstruction can result from inadequate division of a duodenal web or from anastomotic stenosis or functionally from severe dysmotility. Severe gastroesophageal reflux can be prolonged and may require fundoplication. Small bowel obstruction may occur from postoperative adhesions, even many years later. Intravenous feeding may be required if the stomach will not empty postoperatively.
Consent and Risk Reduction
Main Points to Explain
GA risk
Pain/discomfort
Bleeding/hematoma*
Infection (local/systemic)*
Reflux problems
Feeding problems
Duodenal leakage
Risk of other abdominal organ injury*
Possible further surgery*
Risks without surgery*
Multisystem organ failure
Death*
*Dependent on pathology, comorbidities, and surgery performed
Surgery for Midgut Obstructions and Inflammation: Malrotation, Volvulus, Jejunoileal Obstruction, and Meconium Ileus
Description
In any neonate with an intestinal obstruction, the objective of the operation is to define the problem and alleviate the obstruction. Resection of bowel may be required depending on the pathology and the degree of obstruction, ischemia, or necrosis present. For children, and especially neonates, a transverse muscle-cutting incision is typically used. There are few anatomical variations that affect the small bowel except for malrotation with Ladd’s bands and a Meckel’s diverticulum and other derivatives of the vitello intestinal duct. Malrotation is where the midgut loop fails to return to the abdominal cavity, with its normal rotation, between 10 and 12 weeks’ gestation. A Meckel’s diverticulum is a remnant of the vitellointestinal duct (the duct that runs from the yolk sac through the umbilicus to the apex of the midgut loop – where a Meckel’s is situated), which can give rise to other problems as well as the well-known Meckel’s diverticulum (see section “Surgery for Meckel’s Diverticulum and Vitellointestinal Remnants”). In addition, small intestine atresias and meconium ileus are causes of small bowel obstruction in the newborn.
Malrotation is any departure from the usual final adult positioning of the gut within the abdomen. If a patient presents with an intestinal obstruction and hypovolemic shock as a newborn, the degree of malrotation is usually complete, with the second, third, and fourth parts of the duodenum running vertically down just to the right side of the midline, with the cecum and ascending colon running up just next to it, but to the left of the midline. These, together with all of the intervening small bowel, make up the midgut loop of the fetus. Where there is a complete malrotation, the midgut loop is closely applied to a central thick cordlike “universal mesentery” that hangs free in the abdomen. This contains the blood supply to all of the midgut loop, with the proximal small bowel vessels going to the right and the distal small bowel and large bowel vessels going to the left. As a result, this pendulum-like arrangement is very liable to twist on itself at the upper point of attachment, as the midgut fills with food for the first time. The baby presents with pain (screaming), hypovolemic shock, green bile vomiting (with or without blood), and the passage of blood rectally. Venous gangrene of the whole of the midgut loop – middle of the first part of the duodenum to just short of the splenic flexure of the colon – may occur. In the older child (or even adult), the degree of malrotation is often not as marked causing intermittent attacks of obstruction, with central abdominal pain and green bile vomiting. There is often a history of several such attacks, which appear to be able to resolve, presumably by the midgut untwisting, until the diagnosis is eventually made. Nevertheless, surgery is often as an emergency, because of a midgut volvulus. This can occur at any age, and patients have presented well into their 40s and older. The treatment is essentially the same as in babies (see Ladd’s procedure below).
Anatomical Points
Malrotation
There are few anatomical variations that affect the small bowel except for Meckel’s diverticulum and malrotation with Ladd’s bands. A Meckel’s diverticulum is a remnant of the vitellointestinal duct, which can give rise to other lesions as well as the well-known Meckel’s diverticulum.
Malrotation can vary from complete failure to rotate to minor forms of maldescent of the cecum. In the fetus, the midgut loop is attached to the back wall of the abdomen at the middle of the second part of the duodenum and again just short of the splenic flexure. Between these two points, it is outside the abdomen in the umbilical cord. In the normal process of the midgut returning to the abdomen during the 10–12th week of gestation, the cecum and transverse colon rotate anterior to (over) the base of the small bowel mesentery with the cecum moving down to the RIF, as the duodenum rotates deep to it up into the LUQ. In that way, the root of the mesentery is attached diagonally across the whole of the abdomen on the longest attachment possible. If, as the intestine returns to the abdomen, it fails to rotate, then it simply hangs in the abdomen attached as it was in the fetus. Ladd’s bands represent the course the colon should have taken and therefore stretch across the duodenum.
Mirror image malrotation, although thought to be rare, is now more frequently detected antenatally in association with left atrial isomerism, with situs inversus abdominus. The gut is the mirror image of normal, but is also frequently malrotated. A barium meal after birth confirms the situs inversus with the malrotation, and the patient then undergoes a mirror image Ladd’s procedure – often laparoscopically.
Table 3.9
Surgery for midgut obstructions and inflammation estimated frequency of complications, risks, and consequences
Complications, risks, and consequences | Estimated frequency |
|---|---|
Most significant/serious complications | |
Infectiona | 5–20 % |
Subcutaneous | 5–20 % |
Intra-abdominal/pelvic (peritonitis; abscess) (especially in the ELBW; premature) | 5–20 % |
Systemic sepsis (especially in the ELBW; premature) | 2–5 % |
Hepatic portal sepsis (rare) | <0.1 % |
Bleeding/hematoma formationa | |
Wound | 1–5 % |
Intra-abdominal | 0.1–1 % |
Small bowel obstruction (postoperative early or late)a [Adhesion formation] | 1–5 % |
Repeated further surgerya | 1–5 % |
Rare significant/serious problems | |
Perforation (spontaneous preoperative)a | 0.1–1 % |
Anastomotic leakagea | 0.1–1 % |
Fecal/enterocutaneous fistulaa (very rare) | <0.1 % |
Ureteric injury (very rare)a | <0.1 % |
Multisystem organ failure (renal, pulmonary, cardiac failure) | 0.1–1 % |
Mortalitya | 0.1–1 % |
Mortality without surgery (should surgery be refused)a | >80 % |
Less serious complications | |
Pain/tenderness | |
Acute (<4 weeks) | 5–20 % |
Chronic (>4 weeks) | <0.1 % |
Paralytic ileus (peritonitis from preoperative perforation) | 20–30 % |
Nerve parasthesia | 0.1–1 % |
Wound scarring (poor cosmesis/wound deformity)a | 1–5 % |
Nasogastric tubea | 1–5 % |
Wound drain tube(s)a | 1–5 % |
Malrotation: Complications of the Disease
Volvulus, usually of the entire midgut, occurs clockwise when viewed from in front. The vascular supply will be compromised to a varying degree and may cause venous then arterial infarction. In addition, the malrotation may be associated with duodenal obstruction from congenital bands running from the ascending colon across the front of the duodenum (Ladd’s bands). Volvulus occurs in the baby, while partial obstructions or volvulus can occur in the older child. If left too long, the patient will die of hypovolemic shock and/or venous gangrene of the whole of the midgut loop – middle of the second part of the duodenum to just short of the splenic flexure of the colon. The most significant complication is ischemic infarction of the midgut loop with subsequent death or years of parenteral nutrition followed by a gut transplant. With an established volvulus, death is inevitable without surgery.
In the premature it is very difficult to differentiate between necrotizing enterocolitis (see 46.10) and a midgut volvulus. Both have the same presentation, viz., hypovolemic shock and bowel obstruction. But, NEC is treated expectantly, initially, which would be disastrous where there is volvulus of the midgut loop. An ultrasound of the upper abdominal vasculature and the first few loops of bowel may well differentiate, whereas a plain X-ray may not. But, if there is any doubt, an emergency laparotomy should be performed without delay.
Malrotation: The Surgery and Its Complications
Ladd’s Procedure: The surgery for malrotation with or without volvulus is done as soon as possible to try to minimize gut loss. As for all obstructions to the small bowel in the neonate, an upper transverse muscle-cutting approach is used. Any volvulus is untwisted anticlockwise as you look at it. Ladd’s bands (fibrous bands running from the ascending colon across the duodenum) are divided, allowing the colon to be moved further to the left, exposing the root of the mesentery and the duodenum. The thick rope-like “universal mesentery” is splayed/teased open, so that the vessels to the duodenum and jejunum can be moved to the right, and the vessels to the terminal ileum, ascending colon, and transverse colon can be moved to the left. In this way the corresponding intestine can be moved with the vessels as far away from the midline as possible. Normal anatomy cannot be established as the ileocecal vessels that normally run down to the RIF now run a very short distance to the ascending colon, which is in the high midline. The most mobile parts of the rest of the small intestine are then placed back over the raw area created by splitting the mesentery, so that they will form adhesions to that area, hopefully preventing further twisting. The cecum is placed in the LIF and an appendectomy is usually carried out.
Laparoscopic Ladd’s Procedure: The procedure can now be performed laparoscopically, even in the presence of volvulus, and is being performed more and more commonly with time. Obtaining an adequate window to obtain an adequate view is often difficult when the gut is distended, so conversion to an open procedure is not uncommon when volvulus has occurred.
Complications of the Disease: If there is gangrenous bowel, then it has to be resected, hopefully, without losing the entire midgut loop, as this will be essentially lethal, without prolonged TPN, +/− SB transplantation. If surgery is delayed, then the patient may be irretrievable.
Complications of the Surgery: Short-term problems are survival of the child and the intestine and then leaking anastomoses. The dissection in the mesentery often injures lacteals, which will produce a temporary chylous ascites. A short-to medium-chain fat dietary substitute or parenteral nutrition for 4–6 weeks may be useful if prolonged ileus occurs.
Longer term, the deliberate placing of the mobile part of the small bowel into the raw dissected mesentery will lead to extensive adhesions. While this will diminish the tendency to a recurrence of any volvulus, it does increase the incidence of adhesive obstruction, so that there is a 20–30 % lifetime risk of an adhesive bowel obstruction (Murphy et al. 2006).
Jejunoileal Obstruction in the Newborn
Jejunoileal obstruction in the newborn is predominantly associated with an atresia probably caused by a complete interruption to the blood flow to the intestine in the fetus. This leads to loss of the intestine. Because the fetal bowel contains no organisms, the dead bowel disappears, and the two blind ends seal themselves. Intestinal atresias take various forms, from loss of the lumen only to a significant gap, or multiple atresias with small remaining segments – “a string of sausages” (Federici et al. 2003). Incomplete obstruction associated with stenosis is rare (but does occur in the duodenum). Jejunoileal obstructions are rarely associated with other congenital anomalies. Unless the atresia is associated with a significant or lethal loss of bowel, the long-term outlook is usually good. In those with a very proximal jejunal atresia, the degree of dilatation of the proximal bowel may be grotesque so that disparity between it and the distal bowel is a major issue.
Complications of the Disease
Jejunal Atresia
Proximal to a jejunal atresia, the obstructed intestine undergoes massive dilation and becomes inert (thought to be due to ischemic changes in the antimesenteric wall plus the back-pressure effect). These dramatic changes are easily detected on antenatal ultrasound. At birth, an X-ray will show relatively few grossly distended loops of bowel. As with all small bowel obstructions in the newborn, if left untreated, the proximal intestine will progressively distend and will eventually perforate or infarct with subsequent peritonitis and death.
The Surgery: In all small bowel atresias, the proximal intestine becomes distended, and the distal intestine becomes shrunken and unused. The more proximal the atresia, the more marked the upstream distension, giving rise to a gross disparity in size especially in proximal jejunal atresias, so that anastomoses are difficult. Where possible the most distended part of the intestine (the distal end of the proximal part) is resected, thereby presenting a less distended part to be anastomosed. Jejunal atresias are frequently close to the DJ flexure, limiting the surgeon’s ability to resect all of the distended bowel. Nevertheless, the most grossly dilated bowel is resected, where possible. The rest of the distended proximal intestine is tailored to a narrower tube (by discarding the floppy antimesenteric part) and is anastomosed with its end anastomosed to a longitudinal incision made into the antimesenteric border of the distal (contracted and unused) small bowel – the so-called end-to-back anastomosis used in all anastomosis of the small and large intestine where there has been a congenital small or large bowel obstruction. The end-to-back anastomosis allows a larger than normal proximal intestine to be anastomosed to a smaller than normal distal intestine. As for all intestinal anastomoses in the newborn, a single-layer interrupted absorbable suturing technique is usually used. In North America, however, stapling is often used especially for the tailoring procedure in jejunal atresia. Where there are multiple atresias, resection and multiple anastomoses may be required. Occasionally, a temporary proximal stoma may be used to protect multiple distal anastomoses while they seal and heal.
Complications of the Surgery: There are two common postoperative issues. The first is that the long tailoring suture line may leak, and/or stricture, and secondly the proximal intestine will take a long time to recover its motility after the prenatal ischemic damage (that caused the atresia in the first place) and the long-tailored anastomosis. Therefore, these babies often require IV nutrition for weeks.
Ileal Atresia
This is less common, but has its own unique problems. In one specific circumstance where there is a distal ileal atresia, the blood supply to the distal ileum is derived from an ascending branch of the ileocolic artery running back up the small bowel from the ileocecal junction. So, if there is a gap in the mesentery anywhere in the distal ileum supplied by this vessel, then the intestine is free to twist around the artery, and if it does, it creates the appearance of an “apple-peel atresia.” As with jejunal atresia, the proximal intestine will dilate, but not as markedly as for jejunal atresias.
The Surgery: Uncomplicated atresias can occur anywhere in between these two sites (the grossly distended proximal jejunal atresia and the distal ileum’s apple peel). Then, the surgery is far more straightforward; the most distended part of the proximal intestine is resected and an end-to-end anastomosis carried out. Recovery times are far shorter, but still take a few days. The apple-peel atresia’s tenuous blood supply makes it difficult to straighten out the intestine and reanastomose the ends, as the part that has been spiraled around the artery may lose its blood supply as it is straightened. Therefore, partial resection of this intestine is frequently necessary to ensure a good blood supply to both ends.
Long-term complications for jejunal and ileal atresias are few. Adhesive obstruction occurs in approximately 5 % and usually within 2 years of the surgery. Where there has been an abdominal wall defect, the adhesive obstruction rate is higher (van Eijck et al. 2008). Rarely the loss of intestine is critical. If it is, then long-term parenteral nutrition is required, while enteral feeding is gradually established. Hopefully, the residual gut gradually adapts to enable full enteral feeding. In the newborn, the gut is still growing and appears to be able to compensate for some of the loss of length, as well as being able to undergo mucosal adaptation.
If, however, the residual gut is not long enough to support life, then there are gut lengthening procedures (where the gut is split longitudinally and then reanastomosed end-to-end) or interposition procedures (that slow the transit time) that have been used. They have met with mixed success. The “multiple step” procedure is now gaining popularity (proceedings of the World Congress of Surgery, Adelaide, 2008). This is a procedure where there are multiple incisions made into the side of the gut for just over half of its circumference, and then those incisions are closed longitudinally. The gut is then lengthened rather like a paper chain. This procedure appears to increase transit time, and as the residual narrowed gut dilates, it increases the absorptive surface area as well. Initial results would suggest that this is more successful than the older procedures.
If the terminal ileum has to be resected, then vitamin B12 absorption will be poor. If the resection is carried out in the neonate, however, then passive absorption appears to compensate (Ooi et al. 1992) to a far greater extent than in the adult, so that serum levels of B12 are usually normal. The Schilling test will be abnormal. If the serum levels are normal, supplements are not necessary in childhood, but levels should be checked. When such an affected female becomes pregnant, however, the fetus requires folate and B12 at levels that cannot then be supplied by a normal diet for this mother, so that females who have lost the terminal ileum need to be aware of the extra requirement during pregnancy.
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