There is a spectrum of congenital anomalies of the lower urinary tract and pelvis covered by the term “exstrophy/epispadias.” At the worst end is cloacal exstrophy and at the most minor end is an epispadiac position of an otherwise normal urethra (Fig. 45.1). In males and females with an incompletely closed pubic ring the genitalia have a characteristic deformity called epispadias. The reconstruction of this anomaly is the same, regardless of the degree of bladder anomaly, although, especially in males, the system of bladder emptying may influence the surgical decisions. The extent of surgery required depends on the functional consequences of the deformity. Although chordee correction is commonly performed as a part of the reconstruction in infancy, further surgery after puberty is often needed to allow intercourse to take place.

Exstrophy is not, on its own, a lethal condition. The surgery and subsequent complications may be. In 1926 Dr. Charles Mayo, cofounder of the Clinic, published a paper on his experience of 66 cases of exstrophy. He recorded that 72% were still alive but he was not optimistic that the long-term results would be good, as most were still under 10 years old. Historically, 67% of patients would be dead by the age of 20, though his oldest survivor was 73. It was not until after the Second World War that a combination of therapeutic advances conferred a more normal lifespan on exstrophy patients.

Where do anorectal abscesses occur?

These abscess are usually caused by an infection in one of the anal glands in the intersphincteric space. As the infection worsens, there is the spread of pus, which may track to the skin causing a perianal abscess (most common), laterally through puborectalis causing an ischiorectal abscess, or more internally causing a supralevator abscess.

What are other causes of anorectal abscess?

Those not related to the anal glands and these are:

• Submucosal abscess from infected haemorrhoids or fissures

• Subcutaneous from an infected hair follicle

What is the usual organism responsible?

Gram-positive Staphylococcus aureus is the most common organism although occasionally bowel flora will cause an infection. If bowel flora is present there is a higher proportion of associated fistulae.

What is the surgical treatment?

Incision and drainage of the abscess under general anaesthetic is the treatment of choice.

How would you perform an incision and drainage of an anorectal abscess?

Pre-operatively The patient should be appropriately consented. There is no specific requirement for antibiotics unless the patient has cellulitis or is immunocompromised.

Position This is supine and in the lithotomy position. Rectal examination is then performed (proctoscopy/sigmoidoscopy) to search for an internal discharge or fistula opening (it is important not to probe in this acute setting as there is a risk of forming a fistula).

Incision A cruciate incision is made over the abscess at the point of maximal fluctuance.

Procedure A pus swab taken and sent for microscopy and sensitivities. The pus is drained out and loculations are broken down with a finger. Curettage is then performed to the wall of the abscess and any necrotic tissue is excised.

What are hydroceles? What causes of hydroceles do you know?

A hydrocele is a collection of serous fluid in the tunica vaginalis. This can be due to:

• Decrease absorption of the fluid due to damage to scrotal venous or lymphatic systems following groin or scrotal surgery

• Increased production due to leaky vessels secondary to a malignant or inflammatory process

• A patent processus vaginalis

• Trauma

What pre-operative measures must be taken?

It is important to identify any underlying testicular pathology, in particular a testicular tumour. This is done by meticulous history taking and physical examination. The testicle is generally behind the hydrocele and can be palpated through the hydrocele. Further investigation by way of an ultrasound scan can be diagnostic.

What are the options in the management of a hydrocele?

Conservative: This is to simply reassure the patient and may be an option if the hydrocele is asymptomatic, longstanding and after excluding any underlying pathology.

Aspiration: This is a quick and easy option that can be performed in the clinic but has a high incidence of recurrence.

Surgical excision: which can be either an extrusion procedure leaving the sac behind or using diathermy to excise the entire sac.

Name two surgical procedures used to repair a hydrocele? What is the main difference between the two?

Lord's and Jaboulay's procedures. In Jaboulay's the hydrocele sac is excised using diathermy and is associated with a greater degree of haematoma formation.

Outcomes research differs in many ways from more traditionally performed clinical research. Whereas conventional “bench laboratory” experiments are designed to quantify short-term biological parameters for a given number of subjects, outcomes research employs strategies and methods to determine how disease and clinical interventions affect patient populations over time. Often, these studies are carried out in hopes of affecting public policy. In order to do so, study design must be rigorous, satisfying several criteria to ensure successful execution and valid results. For example, groups of equally distributed patients must be carefully selected, ethical and disease appropriate interventions must be implemented, and endpoints must be identified to accurately reflect how these study subjects are affected. At the same time, critical interpretation of results requires that potential biases be acknowledged and accounted for. Furthermore, as we will see, the assessment of children undergoing surgical treatment carries with it certain challenges unique to this population.

A brief history of outcomes research

From the earliest days of contemporary medical practice, observers have questioned the effects of various medical therapies. In fact, the ability to judge and assess the effects of clinical intervention is a necessary prerequisite to gauge the evolution of medical treatment. Codman was among the first physicians to stress the importance of formally assessing the results of clinical intervention. As a result, he is considered the father of Outcomes Research. An orthopedic surgeon in Boston during the 1920s and 1930s, Codman espoused his “End Result Idea.”

List some of the indications for a central venous cannula

• Measurement of right-heart filling pressure to guide fluid balance

• Administration of parenteral nutrition

• Administration of drugs that should be administered via a central vein (e.g. inotropes)

• Access for introduction of pulmonary artery catheter, pacing wires, etc.

• Establishing intravenous access if peripheral access is not possible

What different routes can be chosen for central vein access?

• Internal jugular vein

• Subclavian vein (this can be infra or supracalvicular)

• Femoral vein

• Peripheral veins (PICC lines)

Describe the course and relationships of the subclavian vein

The axillary vein becomes the subclavian vein at the level of the outer border of the first rib. It then proceeds medially, superior to the first rib and anterior to the scalenus anterior muscle. It remains behinds the clavicle and in close proximity to the dome of the pleura.

Describe the course and the relationships of the internal jugular vein

The internal jugular vein exits the base of the skull through the jugular foramen that corresponds to a point approximately one fingers breadth behind the lobe of the ear. It descends vertically and in its lower third lies behind the sternocleidomastoid muscle. It terminates at the medial end of the clavicle. This vein lies within the carotid sheath and in close proximity to the common carotid artery and the vagus nerve. The lower end of the internal jugular lies at the space between the sternal and clavicular heads of the sternocleidomastoid.

What different types of urinary catheters do you know?

• Latex catheters

• Silicone catheters (more suitable for long-term use)

• Three-way catheters used for irrigation of the bladder

• In/out catheters (single lumen) used for intermittent self-catheterisation (ISC)

• Beaked Coude catheter

How would you insert a male urethral catheter?

Position After explaining the procedure and obtaining consent the patient is placed supine and the genitals are cleaned using antiseptic solution. The foreskin must be retracted to clean the glans. Sterile drapes are used to cover the groin.

Procedure Local anaesthetic gel is inserted into the urethra through its meatal opening. The urethra should be held closed with firm pressure to allow the anaesthetic to take effect. The catheter is then inserted into the urethra and through the prostate and advanced as far as possible to ensure the bladder is reached. Once in the bladder urine will start to drain. A suitable catheter bag is connected to the catheter. The balloon is then inflated using an appropriate volume (10 ml if using an ordinary Foley catheter) of water.

Closure The patient is cleaned and the bag secured. A urinary sample is sent for microscopy and the residual volume of urine is noted and documented. Remember to pull back the foreskin to avoid the development of a paraphimosis.

How do you insert a suprapubic catheter?

Position After explaining the procedure and obtaining consent the patient is placed supine. It is important to examine the abdomen to ensure distension of the bladder. The lower abdomen is cleaned with an antiseptic solution and the area draped.

Lymphangiomas are benign abnormal collections of lymphatic vessels forming a mass composed of cystic spaces of variable size, which have the potential for local extension, thereby infiltrating surrounding structures. Lymphangiomas, cystic hygromas, and lymphangiomatosis can affect almost any area of the body where lymphatics are present, but predominate in the head, neck and axilla. Constituting 5.6% of all benign lesions of infancy and childhood, lymphangiomas have no predilection for sex or race. Lymphangiomas can also occur in the lung, gastrointestinal tract, spleen, liver and bone, and may be a manifestation of multifocal disease if found in these last three organs. Abdominal lymphangiomas are rare.

Lymphangiomas should not be confused with primary lymphedema, a disorder in children and adolescents causing swelling in an extremity. This condition is associated with several genetic syndromes and in contrast to lymphangiomas has the potential for malignant transformation with time.

Gross observed that 65% of lymphangiomas were apparent at birth, and that 90% appeared by the end of the second year of life. The principal mode of presentation is that of a disfiguring, usually slow growing mass. Rapid enlargement, sometimes overnight, may occur following systemic infection or bleeding into the tumor.

Etiology and pathogenesis

The etiology of lymphangiomatous malformations is believed to be a developmental defect in lymphatic pathways which develop from the sixth week of gestation onwards, resulting in obstructed or inadequate efferent channels and proximal dilatation of afferent channels. The lymphatic system develops from five primitive sacs originating from the venous system.

Introduction

Gonadal tumors are rare in children and raise two main questions. First, is the tumor benign or malignant? The risk of malignancy is around 50% in boys and about 10% to 25% in girls (if both solid and cystic lesions are included). For benign tumors, a conservative surgical procedure (tumorectomy with gonadal preservation) should be attempted when possible. Malignant or potentially malignant tumors have an excellent prognosis provided they are properly treated by a surgeon and oncologist – current protocols using platinum and bleomycin are very effective, even for advanced-stage tumors. Second, how is future gonadal function best preserved? Gonadal function can be almost normal after treatment of a unilateral gonadal tumor, but in synchronous or metachronous bilateral tumors, gonadal function may be entirely destroyed. This has two major consequences: a lack of gamete production inducing sterility and a failure of sex hormone production which prevents the progression of natural puberty and the development of secondary sexual characteristics. Hormonal failure can be managed by lifelong hormonal substitution. However, sterility may have major psychological effects. Thus, gonadal function should be preserved if at all possible.

Advances in detection, diagnosis, minimally invasive management, and chemotherapy have all required changes in clinical practice, which is aimed at reducing morbidity without compromising oncological treatment.

Excluded from this chapter are the rarer problems of leukemia or lymphoma located in the ovary or testis, rare malignant paratesticular tumors (rhabdomyosarcoma or mesenchymal tumor), and rare soft tissue tumors (fibroma, leiomyoma, hemangioma).

Introduction

Genitoplasty has been the traditional treatment for various forms of ambiguous genitalia and genotypical abnormalities. These conditions represent a spectrum of clitorophallic size and form, as well as vaginal position, size, and location. In some cases, complete absence of the genitalia must also be considered. Careful consideration of anticipated gender and function outcomes must be weighed before choosing some form of feminizing genitoplasty. Through the years, techniques, timing, and materials for genitoplasty have evolved, whereas surgical goals have remained unchanged.

The immediate goal is to provide the external genitalia with an esthetic and feminine appearance. The long-term goals are to produce a functional vagina of sufficient size for comfortable sexual intercourse, to retain sexually sensitive tissue to allow orgasm and, if internal genitalia permit, to preserve fertility potential. The surgeon's intent is to facilitate the patient's positive psychosocial adjustment throughout the patient's life. Sometimes, a completely satisfactory solution is not possible.

Even though techniques have advanced through the years, these demanding repairs and reconstructions are not without significant complications, necessitating careful multidisciplinary follow-up. Though genitoplasty presents many immediate problems and considerations, the most challenging are the long-term surgical outcomes. Many surgeons consider creating presentable external female genitalia with a sizable vagina an adequate outcome, but cosmetic appearance and passive sexual function is only one measure of success. Because scant knowledge exists, little is known about how adults adjust to genitoplasty or whether the genitoplasty expresses their sexual preference.

List all the options available in providing cover to a skin wound

This is known as the reconstructive ladder starting with the simplest method and if not suitable moving on to the next (more complex) method aiming to achieve the best result.

1. Simple dressing and allowing healing by secondary intention

2. Direct primary closure

3. Delayed primary closure

4. Skin graft

5. Local flap

6. Free flap

What are the two main types of skin graft? What are the advantages and disadvantages of each?

The two main types are full or partial thickness skin graft.

Partial-thickness grafts are available in larger sizes and can be used to cover large defects, as the donor site does not require closure. They are also meshed which improves their ability to “take” and to conform to irregular shaped defects. In the long term however they have a poorer cosmetic outcome. There may also be problems with the donor site during the healing process.

Full-thickness grafts have a better cosmetic outcome and match the surrounding skin better. There are fewer problems with the donor site as they are closed directly. Full-thickness grafts are available in small sizes however and cannot be used to cover very large defects. For example the full-thickness grafts used to cover skin defects in the fingers, after a Dupuytrens dermatofasciectomy. These grafts are taken from the volar forearm skin.

What are the causes of skin graft failure and what measures are undertaken to counteract them?

Infection Presence of a high bacterial load can lead to graft failure.

Thoracic surgery in childhood has changed considerably in the last 50 years. In particular, surgical indications and techniques have altered dramatically. At the beginning of the twentieth century, the main indications for thoracic surgery were related to infectious diseases, especially bronchiectasis and tuberculosis, whereas congenital malformations now constitute the commonest reason for lung resection. Thoracic surgery has become more commonplace in younger patients, including neonates. Safe techniques have prompted the development of prophylactic surgery in asymptomatic patients and thoracoscopic techniques offer a minimally invasive approach.

The long-term consequences of pulmonary resection and thoracotomy are related to many different factors: the underlying disease process and its natural history; the effects of loss of parenchymal volume; and the physical sequelae of surgery on the chest wall. In addition, in younger patients there is the added dimension of the effects of somatic growth on future pulmonary function.

As in all branches of pediatric surgery, knowledge of long-term sequelae is limited because of the lack of studies following patients through to adulthood. The late consequences of thoracoscopic procedures and the relative merits of open surgery and thoracoscopic techniques are poorly understood. However, specific data are reported in two broad areas: the functional consequences of pulmonary resection and musculoskeletal abnormalities resulting from thoracotomy.

Lung development and compensatory lung growth

In order to understand the effects of lung resection during childhood, it is helpful to review the process of normal lung development. The human lung is an organ which continues to mature after birth.

Intractable constipation and fecal incontinence are socially embarrassing and are commonly associated with spinal cord anomalies, anorectal malformations, Hirschsprung's disease and sacral agenesis. Initial conservative management of chronic constipation and fecal incontinence involves daily enemas, diet modification, stool softeners, suppositories, laxatives, and biofeedback. However, these programs are more unpleasant and difficult to manage as children get older. In the pre-MACE era when conservative management failed, children either faced the future wearing pads for fecal soiling or were doomed to permanent diverting colostomy as a last resort. The MACE procedure has allowed patients to become continent and independent with improved self-esteem.

Approximately 50% of patients with a neuropathic bladder secondary to myelomeningocele, who require bladder reconstructive surgery to establish continence or create a safe bladder, will also suffer from a neuropathic bowel with resultant chronic constipation and/or fecal incontinence, that does not respond to conservative measures. Conversely, up to 50% of patients born with an anorectal malformation will have long-term fecal incontinence, and as many as 50% of these will also have a neuropathic bladder, many of whom will require bladder reconstructive surgery. It is logical that these coexisting lower urinary tract and bowel problems should be managed simultaneously and not in isolation; in the majority of cases definitive bowel management usually rests with the reconstructive urologist. It is within this setting of holistic care that the MACE procedure was conceived and developed.

Principles of the MACE procedure

The MACE procedure simply combines three well-established surgical principles:

1. (a) the Mitrofanoff principle of a continent catheterizable abdominal stoma and conduit

2. […]

What is idiopathic hypertrophic pyloric stenosis (IHPS)?

This is a congenital condition that presents in infants at around 6 weeks of age. There is hypertrophy of the circular muscle of the pylorus causing gastric outflow obstruction. It is a familial condition and seen especially in the first-born males. IHPS is considered a surgical emergency as the infant is unable to feed and loses large quantities of acidic gastric fluid leading to dehydration, alkalosis and electrolyte imbalances.

What is the biochemical abnormality in an infant with pyloric stenosis?

Hypochloraemic metabolic alkalosis. The serum potassium and sodium may also be decreased. There may even be a paradoxical acid urine as the kidneys excrete hydrogen ions in order to preserve potassium.

How is IHPS diagnosed?

Classically there is a history of non-bilious projectile vomiting, dehydration and failure to thrive. The child remains hungry after each vomiting. Clinically the thickened pylorus can be palpated in the epigastrium as an olive-sized mass. The diagnosis can be further supported with a test feed and confirmed on ultrasound scanning.

What is the management of an infant with pyloric stenosis?

• Fluid resuscitation using 0.45% normal saline with the addition of potassium chloride to each bag

• NGT insertion and regular aspiration of the gastric contents

• Surgery (Ramstedt's pyloromyotomy) after the alkalosis is corrected (typically after HCO3 is below 25 mmol/l)

Describe the steps taken in performing a Ramstedt's pyloromyotomy

Position The infant is placed supine with whole abdomen prepared and draped.

Incision Usually a right upper quadrant cutting or muscle splitting incision is used (upper midline, umbilical and laparoscopic approaches may also be used).

There is a wide variety of conditions which affect the small bowel in children, from the newborn period to adolescence. Many of these, including abdominal wall defects, meconium ileus, Crohn's disease, and the general topic of short bowel syndrome, are discussed elsewhere in this text. This chapter will review the long-term outcomes associated with congenital duodenal obstruction (i.e., atresia and webs), jejunoileal atresia and webs, Meckel's diverticulum, intussusception, malrotation, and intestinal duplication. Most children recover from these problems and go on to live a normal healthy life. For this reason, relatively few studies exist documenting long-term outcomes. In this chapter the authors have used a combination of the existing literature, and their own institutional experience, to document outcome in an objective way.

Long-term problems associated with small bowel disorders can be classified into several categories:

• those specific to the disorder itself (e.g., motility disturbances associated with atresia or malrotation);

• those that are a result of surgical intervention (e.g., short bowel syndrome following massive resection, or peritoneal adhesions following laparotomy);

• those related to underlying disease processes or associated anomalies.

Complications and survival can also be divided into early and late time periods. Since both may influence long-term outcomes, some disease processes require a discussion of both early and late morbidity and mortality.

Congenital duodenal obstruction

Although infants with complete duodenal atresia present in the neonatal period, duodenal web or stenosis may present later during the first years of life. The obstruction is postampullary in approximately 80% of patients and preampullary in 20%.

During the past decade there have been advances in the understanding of gastrointestinal (GI) motility and sensory disorders in children. Newly validated diagnostic techniques can accurately diagnose previously misunderstood patients, and progress is being made in the treatment of enteric neuromuscular disorders. In this chapter pediatric GI motility disorders are discussed according to the anatomy of the GI tract.

Esophageal disorders

The esophagus includes three functional regions: the upper esophageal sphincter (UES), the esophageal body, and the lower esophageal sphincter (LES). The UES consists of striated muscle, which relaxes in response to swallowing. The esophageal body is lined by striated muscle in the proximal third, mixed striated and smooth muscle in the middle third, and smooth muscle in the lower third. Esophageal peristalsis is initiated by swallowing and is independent of intrinsic myoelectrical activity. The coordinated motor pattern of the esophagus is called primary peristalsis. Secondary peristalsis is usually induced by luminal distention or incomplete clearance of luminal contents by primary peristalsis. This is an important mechanism for the clearance of gastric contents during gastroesophageal reflux. The lower esophageal sphincter (LES), a band of smooth muscle located at the junction of the distal esophagus and gastric cardia, is tonically contracted except during swallowing when it relaxes momentarily to allow the food bolus to pass into the stomach. Inappropriate LES relaxation independent of swallowing is the mechanism responsible for the majority of gastroesophageal reflux episodes both in adults and children.