Introduction

Thoracic organ transplantation is an important treatment option in children with acquired or congenital cardiopulmonary disease. In recent years, there has been considerable improvement in early outcomes following thoracic organ transplantation in children and 1-year survival is similar now to that in adults. Approximately 250 pediatric heart transplants and 60 lung transplants are performed annually in the United States. Thoracic organ transplantation in neonates and infants has been limited by donor availability, and the number of thoracic organ transplants performed each year in children has plateaued. Complications such as acute and chronic rejection, graft coronary artery disease (CAD) or bronchiolitis obliterans, as well as those of immunosuppression, pose serious threats to the long-term success of thoracic organ transplantation in children. Despite these potential impediments, life expectancy and quality of life for patients following transplantation exceed that for patients with end-stage cardiopulmonary disease who are managed medically. This chapter focuses on the clinical aspects of heart and lung transplantation in infants and children including indications, preoperative evaluation, postoperative course and management, complications and long-term outcomes.

Indications

Heart transplantation

As published by the Registry for the International Society for Heart and Lung Transplantation (ISHLT) in the Seventh Official Report in May 2004, the number of pediatric heart transplants has remained relatively constant over the last 10 years (Fig. 67.1). The most common indication for heart transplantation in the neonatal population remains complex congenital heart disease for which no reasonable corrective or palliative surgical therapy is available.

What are the indications for limb amputation? Which is the commonest in the UK?

• Lower limb ischaemia secondary to atherosclerosis and/or diabetes is by far the commonest indication for amputation in the UK

• Trauma which is the commonest indication in the Third World

• Infection

• Malignancy

• Congenital deformity

What types of lower limb amputation do you know?

• Hip disarticulation or hindquarter amputation

• Transfemoral or above knee amputation (AKA)

• Through knee amputation

• Transtibial or below knee amputation (BKA)

• Through ankle or Syme's amputation

• Partial foot amputations

What pre-operative measures should be taken?

This is an entirely multidisciplinary process. All the members of the team (physiotherapy, occupational therapy (OT), rehabilitation specialist, prosthetic specialist, nursing staff, psychologists as well as the surgeon) must be involved and if possible meet the patient prior to surgery.

The level of amputation must be decided upon. This depends on the patients “rehabilitation potential”, degree of tissue compromise, and severity and pattern of the vascular disease.

Finally a careful anaesthetic assessment is made, bearing in mind that these patients may have atherosclerosis affecting their renal, coronary and cerebral vasculture as well as other co-morbid factors such as hypertension and diabetes. They may even be heavy smokers with associated pulmonary compromise.

Name two common techniques used for a BKA

1. Long posterior flap (the Burgess and Romano technique)

2. Skew flap

How do you perform a BKA?

Position This is supine with the affected leg in a knee-flexed position.

Introduction

The epispadias/exstrophy complex encompasses a spectrum of rare midline defects in the pelvis and genitalia. Classic bladder exstrophy is a severe congenital malformation affecting approximately 1 in 40000 live births. Although the exact etiology remains unknown, current evidence seems to implicate poor mesenchymal ingrowth with subsequent deleterious effects on the timing and position of rupture of the cloacal membrane.

The birth of a child with bladder exstrophy marks the start of a lifelong journey. The severity of the defect promises an extensive surgical undertaking that starts most frequently during the first days of life and often extends through adolescence. Components of surgical correction include: closure of the bladder, posterior urethra, pelvis and abdominal wall at birth, genital reconstruction, and some form of continence procedure. The approach pioneered at the senior author's institution addresses these components in a modern, staged fashion. Alternatively, there are also proponents of complete primary closure, which attempts to address all components in a single operation. However, recent data from the American Academy of Pediatrics reveals inferior continence rates when this technique is used. Longer follow-up is needed to ascertain the applicability of this repair, as the associated complications are not insignificant.

With respect to outcomes, the goals of reconstruction in the exstrophy patient are urinary continence and satisfactory genital cosmesis and function. Although most children prefer to achieve continence with voiding per urethra, some will accomplish this same outcome with intermittent catheterization.

What are some of the common risk factors for peptic ulcer disease?

• Helicobacter pylori infection (in 90% of duodenal ulcer, DU)

• Alcohol abuse

• Non-steroidal anti-inflammatory drugs (NSAIDs)

• Steroids

• Old age

What pre-operative measures must be undertaken in the management of a patient with a perforated peptic ulcer?

• Intravenous fluid resuscitation

• Urinary catheterisation and hourly urine output monitoring

• Nasogastric intubation to decompress the stomach

• Central venous catheterisation (if the patient is haemodynamically unstable)

• Analgesia

• Intravenous proton pump inhibitors

• Intravenous antibiotics (e.g. Cefuroxime and Metronidazole)

• Blood investigations indicate a neutrophil leucocytosis (Pancreatitis must be excluded with an amylase)

• Erect CXR will reveal a pneumoperitoneum in only 80% of cases (Can also use a lateral decubitus film which is more sensitive)

Describe how you would repair a perforated DU

The aim is primarily to repair defect and to wash out the abdominal cavity.

Position This is supine the patient is prepared and draped as for a laparotomy.

Incision Upper midline through linea alba (see Chapter 24, Laparotomy).

Procedure The duodenum is isolated and the perforation identified. A patch of omentum is oversawn across the perforation using Vicryl sutures. The abdomen is irrigated with copious amounts of warm saline. A drain may be placed prior to closure.

Closure The abdomen is closed using mass closure technique as for a laparotomy.

What if there is a perforated gastric ulcer?

This will only be evident at operation and the treatment is the same as for DU. A biopsy of the ulcer should be taken for definitive pathological diagnosis prior to repair with an omental patch as there is a higher occurrence of malignant ulcer here.

In the early 1900s, an American surgeon named Ernest Amory Codman introduced the concept of “the end result idea” to encourage scrutiny of patient outcomes in hospitalized patients. Codman suggested that this approach be used to compare hospitals and surgeons and thus the era of benchmarking began. Controversy ensued as “the end result idea” threatened to shift focus from a system based on the privileges of seniority to a more objective one. Prompted by Codman's idea, the American College of Surgeons (ACS) developed a committee called the Standardization of Hospitals in the late 1920s that ultimately became the Joint Commission for the Accreditation of Hospitals. Let's jump ahead 60 years to the reports of Wennberg who reported remarkable differences in the rate of carotid endarterectomies, tonsillectomies, and hysterectomies in various geographic regions. These early reports ushered in the era of evidence-based medicine, defined as the integration of the current best evidence with clinical expertise and patient preferences used in making decisions regarding care. Trauma research focusing on outcome studies and evidence-based methodology is in its early stages and will be reviewed below.

Trauma systems

Optimal pediatric trauma care: pediatric vs. adult trauma centers

The debate regarding the optimal setting for pediatric trauma care delivery has existed for more than two decades and is unresolved at present. The Major Trauma Outcome Study and TRISS methodology have been used in several studies to highlight care of the pediatric trauma victim in adult trauma centers.

Introduction

Biliary atresia presents in the neonatal period, occurs with a frequency of between 1 in 8000 and 1 in 16000 live births and accounts for more than 50% of pediatric liver transplantation. The cause of the disease remains obscure but current evidence suggests that there is more than one etiologic factor. Both the intra- and extrahepatic bile ducts are affected and affected infants present with jaundice and pale stools within the first few weeks of life. The intrahepatic pathology, which has been likened to sclerosing cholangitis, is accompanied by an inflammatory sclerosing lesion of the extrahepatic bile ducts, which results in obstruction of the lumen and, in some cases, complete disappearance of segments of the biliary tract. Death from cirrhotic liver failure occurs within 2 years in untreated cases and biliary atresia represents the most frequent reason for liver transplantation in childhood.

Historical issues

Thomson3 published the first major review in 1892. He collected 49 cases from the literature and added a further case of his own. He recorded that “the children themselves are either jaundiced at birth, or they become so within the first week or so of life; otherwise they are healthy and well nourished.” He concluded that, whatever the etiology of the condition, it was characterized by a progressive destructive inflammatory lesion of the biliary tract.

Introduction

The “long-term” care and complications related to the population of hydrocephalic and myelomeningocele patients have been infrequently considered. Effective treatment, and even survival, for these patients began in the late 1950s, and was not widespread until the mid 1970s. The first effective hydrocephalic shunt was introduced in 1955. Routine treatment of all myelomeningocele patients did not begin until the late 1970s, after repudiation of “selection” criteria. Only now is the oldest generation of patients approaching their 40s, and the largest cohort of patients is barely into the young adult years. There has been little work published on the long-term medical and care issues for these patients.

It is clear in the care of these patients that the natural history of their disease does not include a decline in function. Caregivers and patients must recognize that a loss of function is either a new problem, such as the development of a syrinx leading to scoliosis, or the morbid sequela of a potentially treatable event, such as an infection leading to brain injury. There is some overlap between these two groups of patients. The majority of myelomeningocele patients also have hydrocephalus, and require management of the problems associated with hydrocephalus in addition to the unique issues involved with myelodysplasia.

This monograph will briefly review the pertinent pathology and initial treatment of these conditions. The more typical problems associated with each diagnosis will be discussed. The evolving longer-term issues affecting these patients will be highlighted.

Introduction

Posterior urethral valves are the commonest congenital obstruction of the lower urinary tract, comprising 10% of antenatally diagnosed uropathies with an incidence of up to 1 in 4000. This figure will rise if non-viable and terminated fetuses are included and as antenatal diagnosis is refined. Equally, there is a group of boys at the other end of the spectrum, whose lesser urethral obstruction produces no early urinary tract dilatation and who present late, even in adulthood with normal renal function and voiding difficulty. The interpretation of this latter group accounts for much of the variation in incidence and the different spectrum of severity found in different series of valve patients.

Posterior urethral obstruction more than any other urinary anomaly, has the capacity to affect the development and function of the whole urinary tract. Our understanding of this problem has evolved over the past decades and has included the identification, classification and treatment of the obstruction; recognition and management of the degrees and types of renal impairment that may be associated with it; the approach to associated vesicoureteric reflux and dilated upper urinary tracts; the function and development of the “valve bladder” (a central issue in our current thoughts about the long-term outcome of posterior urethral valves) and finally the impact on our practice of antenatal diagnosis and the opportunities that it offers not only in the identification but also in the early treatment of posterior urethral valves.

What is the aetiology of varicose veins?

The aetiology of varicose veins can be primary (95%) or secondary:

• Primary varicose veins are caused by incompetence of valves in the connecting veins between the deep and superficial venous systems. This leads to back flow of blood from the deep into the superficial veins leading to an increase in pressure and development of varicosities.

• Secondary varicose veins develop due to occlusion of the deep system (thrombosis) leading to diversion of venous flow to the superficial veins.

What are the common sites of venous incompetence?

• Sapheno-femoral junction between the greater saphenous vein and the femoral vein in the groin (90%)

• Sapheno-popliteal junction between the greater saphenous vein and the popliteal vein in the popliteal fossa

• Midthigh perforator

• 23 perforators in the calf

What is the surface marking of the greater saphenous vein?

The greater or long saphenous vein (LSV) starts at the midportion of the medial arch of the foot. It ascends anterior to the medial malleolus on the medial aspect of the lower leg. At the knee it passes about a hands breath behind the medial femoral condyle before passing towards the anteromedial thigh. It joins the femoral vein at the sapheno-femoral junction medial to the femoral artery (femoral pulse) about 3 cm below the inguinal ligament.

Gastrointestinal bleeding related to portal hypertension (PH) in children may be life threatening. Therapeutic alternatives used to relieve PH and reduce the risk of bleeding include various types of surgical and radiologic vascular procedures. Shunt surgery, which usually results in total diversion of portal blood flow, was introduced several decades ago. The long-term benefits and complications of this therapy have been critically reviewed in the previous edition of this book. New techniques developed during the 1990s. The transjugular intrahepatic porto-systemic stent shunt (TIPS) is now preferred to surgical shunts for treatment of PH in most cirrhotic patients. The splanchnic-to-left-portal vein bypass (Rex shunt), designed for definitive treatment of portal vein obstruction, results in restoration of physiologic intrahepatic portal blood flow. The less invasive techniques of percutaneous endoluminal dilatation or thrombectomy of the portal vein, with or without placement of a stent, have been used mainly as rescue therapies after failed shunts, TIPS or bypasses; a few attempts have been made to use them as a definitive treatment of portal vein obstruction.

Shunt surgery

Evolution of indications for shunt surgery

Different pathologic conditions cause portal hypertension in children and etiology must be considered when choosing a treatment for PH. In the case of extrahepatic portal obstruction (EHPO), which is either idiopathic or a complication of perinatal thrombosis of the portal vein, liver function is normal or near-normal, and for many years a shunt operation has been considered as the best method of providing lifetime protection against recurrence of bleeding, with an acceptable risk of complications.

What are the indications for insertion of a chest drain?

The main purpose of a chest drain is to remove or prevent the collection of pleural contents that result in ventilatory compromise. Some of the indications are listed below:

• Pneumothorax

• Haemothorax

• Pleural effusion (infective, malignant, congestive)

• Empyema

• Chest trauma (especially those with open or penetrating chest wounds)

• Chylothorax

• Post-operative (prophylactic)

• Prior to positive pressure ventilation

What surface makings help in deciding the site of chest drain insertion?

The safe site for a chest drain is within an imaginary triangle on the side of the chest wall, and, on the superior border of the rib that forms the inferior border of the chosen intercostal space.

The three sides of this triangle are marked anteriorly by the lateral border of the pectoralis major muscle, posteriorly by the anterior axillary line and inferiorly by the sixth rib.

What are the anatomical reasons for the site chosen in Question 2?

• Intercostal neurovascular bundles are at the inferior border of the rib and away from the site of tube insertion.

• The long thoracic nerve is marked by the midaxillary line and thus outside the above triangle.

Describe how you would insert an intercostal chest drain

Position The patient is placed supine at a 45-degree angle and tilted away from the side of the procedure. The shoulder is then abducted (resting hands on top of head) to allow better access to the chest wall.

Introduction

Choledochal cyst is a congenital dilatation of the common bile duct and has the potential to become malignant. It has a hereditary predisposition, which may explain the higher incidence seen in Asia, particularly in Japan, and its familial occurrence in siblings and twins. It was first reported by Douglas in 1852 and may or may not be associated with congenital dilatation of the intrahepatic bile ducts (Fig. 36.1). Choledochal cyst is almost always associated with an abnormal junction between the pancreatic and common bile duct, i.e., pancreaticobiliary malunion. This allows pancreatic secretions to reflux into the biliary tree, and bile to flow into the pancreatic duct, causing various pathologic changes in the biliary tract, pancreas, and liver.

Because concurrent abnormalities of the pancreatic duct and intrahepatic bile ducts are almost always present, the importance of cholangiography in the planning of surgical management cannot be overemphasized. If these anomalies go unnoticed, aberrant pancreatic anatomy might be damaged, causing serious postoperative morbidity. Internal drainage of the choledochal cyst, a procedure popular in the past, has been abandoned for more than a decade now because of a prohibitive incidence of postoperative complications such as recurrent cholangitis, cholelithiasis, and biliary duct cancer. Primary cyst excision with biliary reconstruction to avoid two-way reflux of bile and pancreatic secretions is now the standard surgical treatment of choice.

Surgery for choledochal cyst is generally successful and a satisfactory surgical outcome with low morbidity is expected in the short term.

Introduction

In former years, the clinical management of upper tract dilation was simple. Because most children with hydronephrosis presented with a mass or a clinical problem such as infection or pain, it was assumed that the cause was an obstruction. Surgical treatment was performed and the outcome was usually satisfactory. The advent of prenatal ultrasonography and the resultant identification of large numbers of asymptomatic upper tract dilation has forced physicians to alter their understanding of the significance of hydronephrosis and to better define obstruction. This is largely because dilation often improves or resolves spontaneously, which questions the necessity of surgical treatment. Whereas in the past only few natural history studies were available, a greater appreciation of the spontaneous resolution of prenatally detected hydronephrosis has been gleaned from multiple studies from various centers around the world.

Any analysis of long-term outcomes of upper tract dilation management must of necessity deal separately with the symptomatic and asymptomatic varieties and must also recognize that dilation involving the kidney alone is different from dilation affecting kidney and ureter together. Accordingly, this chapter will be divided into two sections. The first will deal with pure hydronephrosis and consider the child with dilated renal collecting systems without ureteral dilation. The second portion will examine hydroureteronephrosis and dilation of both the renal collecting system and ureter.

Dilation of the renal collecting system is easily diagnosed on radiographic imaging studies. However, not all upper tract dilation is caused by obstruction.

Esophageal replacement

Esophageal replacement is an infrequent procedure required after great effort has been put forth to save the native esophagus. The most frequent indications for esophageal replacement include long-gap esophageal atresia with or without tracheoesophageal fistula, and esophageal strictures most often due to caustic ingestion or secondary to gastroesophageal reflux disease. Although children with esophageal atresia, gastroesophageal reflux disease, and to a lesser degree, caustic ingestion, are commonly encountered in pediatric surgery, they rarely require esophageal replacement. Our ability to anastomose the two ends of the esophagus in infants with esophageal atresia has improved over the years with techniques such as circular myotomies, upper pouch flaps, and dilation or stretching of the upper pouch and distal esophagus prior to anastomosis. Caustic ingestions have become more infrequent with the changes in packaging of hazardous materials in the home. Peptic strictures due to acid reflux are on the decline due to our heightened awareness of the long-term damage caused by gastroesophageal reflux disease, and our improved medical armamentarium available to control acid reflux. In addition, novel procedures to save the strictured esophagus have been reported. These include stricturoplasty with placement of a vascularized colonic patch, and stenting of caustic esophageal strictures combined with steroid and acid reducing therapy. Despite these improvements, and our understanding that even a somewhat compromised native esophagus may be desirable when compared to the alternatives, there is a distinct subset of infants and children who will require an esophageal replacement.