Introduction

Inflammatory bowel disease is a chronic intestinal disease of unknown etiology that takes several different forms including Crohn's disease and ulcerative colitis. Typically the inflammation in Crohn's disease is panenteric and full-thickness while in ulcerative colitis it is usually limited to colorectal mucosa. These archetypal diseases may sometimes be difficult to distinguish because they may have similar presenting features, especially when there is colonic involvement and both are associated with similar extra-intestinal manifestations. Histologically, Crohn's disease is characterized by apthous ulceration and cobblestoning with rectal sparing while ulcerative colitis is manifested by crypt abscesses and depletion of goblet cells with continuous inflammation always involving the rectum. However, in as many as 10% or more of patients, the histology may be indeterminate. The surgical treatment for Crohn's disease and ulcerative colitis and the outcomes vary considerably, and these are the subjects of this chapter.

Crohn's disease

Epidemiology

Crohn's disease is more common than ulcerative colitis, occurring in 11 cases per 100000 population per year, versus 2.3 cases of ulcerative colitis per 100000 population per year in the pediatric age group. The incidence of Crohn's disease is age specific, occurring in 2.5 cases per 100000 population per year in persons less than 15 years of age compared to 16 cases per 100 000 population per year in persons 15–19 years of age. Males and females are equally affected and Crohn's disease occurs more commonly in the Caucasian and Jewish populations compared to other ethnic groups.

Introduction

With the exception of mumps, salivary gland disease is relatively uncommon in childhood. However, the range of conditions that can occur are the same as in the adult population, although the relative incidence is very different.

Careful history-taking and detailed examination are critical. Details of onset, duration, periodicity, size, and location of any swelling and the character of any salivary secretions must be recorded. The child must be examined both intraorally and extraorally. Deep lobe parotid tumors may present with swelling in the tonsillar fossa and no external swelling. Swellings of the tail of the parotid or of the submandibular glands are easily confused with cervical lymphadenopathy.

In the majority of salivary diseases in children, imaging of the affected gland is an essential supplement to the history and examination. Bearing in mind that patient compliance cannot be relied upon, and that radiation exposure should be reduced as far as possible, ultrasonography (US) is the method of choice for the initial imaging of most conditions, including inflammatory diseases, obstruction due to calculi, and masses within the glands. Although sialography is the preferred technique for the evaluation of chronic or recurrent inflammation, it is difficult to undertake in the young child. Computed tomography (CT) or magnetic resonance imaging (MRI) are essential in the evaluation of neoplastic disease, although the child may require sedation or even general anesthesia in order to obtain good images.

What are haemorrhoids and where do they occur?

They are caused by the engorgement of the internal haemorrhoidal venous plexus, which normally forms part of the anal cushions and may be internal (above the dentate line) or external (below the dentate line). They are normally found at 3, 7 and 11 o'clock in the lithotomy position.

What symptoms may be present?

Painless bleeding, discharge and irritation are common but large haemorrhoids may thrombose and infarct causing severe pain.

How are haemorrhoids classified?

1st degree These bleed but do not prolapse

2nd degree Prolapse on straining but reduce spontaneously

3rd degree Prolapse on straining and only reduce with manual assistance

4th degree Prolapsed and irreducible and may strangulate

Why should haemorrhoids be investigated?

Serious bowel pathology, for example a carcinoma or inflammatory bowel disease, must be excluded. Rectal examination, proctoscopy and sigmoidoscopy should therefore be performed in all cases.

What is the initial management of haemorrhoids?

Conservative treatments such as:

• Dietary advise (with increased fibre)

• Topical applicants

• Reduction of stool straining

• Laxatives if constipated

What treatment modalities are available in the outpatients department?

For 1st and 2nd degree haemorrhoids the following can be used:

• Injection sclerotherapy: About 23 ml of sclerosant (usually phenol in oil) is injected into the submucosal pedicle causing elevation of the submucosa and tissue necrosis.

• Rubber band ligation: During proctoscopy the haemorrhoid is identified and grasped using forceps. A band is applied at the base to strangulate the pile. This later sloughs off (this can also be used in 3rd degree haemorrhoids).

Institutions which provide the basis for contemporary children's specialty units and pediatric clinical care first developed in the nineteenth and early twentieth centuries. These reflected efforts to care for urban populations of children brought together by the forces of industrialization and were often the work of altruistic individuals, typically but not exclusively, well-to-do women, who were motivated by compassion for children. Their primary mission was often to provide an acceptable level of charity care to poor children in their communities. This is, in fact, still the case for many major children's hospitals worldwide.

The concept that children are unique patients with particular developmental, physiological and psychological needs, for whom a demonstrably better outcome could be obtained with specialized medical care came more recently. With particular regard to children's surgical care, events detailed in Chapter 1 by Dr. Hendren (North America) and Dr. O'Donnell (Europe/UK) gave rise to what we recognize as contemporary pediatric surgery by the mid twentieth century. Specialization and credentialing were progressively driven by the view that the quality of care for children was improved by having practitioners fully dedicated to children, by providing focused professional training for individuals interested in childhood disease, and by creating a children's specific environment for the delivery of that care. The wider medical community has not been free of controversy over these points. Perceived economic threat and fear of professional or institutional loss remain powerful forces even today when we discuss issues like regionalization or consolidation of certain services.

Chest wall deformities fall into two categories; those in which there is overgrowth of the ribs and cartilages causing either a depression (excavatum) or protrusion (carinatum) of the anterior chest wall, and those in which there is an absence (agenesis), deficiency (atresia), or failure of closure (dysplasia) of the various chest wall structures.

Pectus excavatum is by far the most common chest wall anomaly and occurs with a frequency of 1 in 1000 individuals (Fig. 11.1). Pectus carinatum is much less common, with about 10 pectus excavatum cases for each pectus carinatum in a large series, and usually presents during the pubertal growth spurt (Fig. 11.2).

The abnormalities in which there is partial agenesis or failure of fusion (ectopia cordis, Cantrell's pentalogy) are fortunately very rare because many of these conditions are incompatible with life (Fig. 11.3).

In Poland's syndrome, there is variable absence of breast tissue, pectoralis major muscle, pectoralis minor muscle, and ribs. Some patients lack only one structure and others lack all of them and have only skin covering the thoracic cavity (Fig. 11.4).

Pectus excavatum

Pathophysiology and symptoms

Anatomically, the depression of pectus excavatum may be mild, moderate, or severe, and it may be localized or diffuse, symmetric or asymmetric. Asymptomatic cases may be treated with an exercise and posture program, whereas anatomically severe cases may be symptomatic and require surgical correction.

The second edition of this book attempts to bring together and analyze what we currently know about the long-term effects of conditions and operative procedures in pediatric surgery and urology. The subject of long-term outcomes has been relatively neglected in the past. However, the realization that there is an ever-expanding cohort of children reaching maturity with a variable legacy from childhood surgical problems has prompted a more critical and detailed study of outcomes with the aim of optimizing current surgical practice. Encouragingly, all major international pediatric surgical meetings now include data on long-term outcomes. Furthermore, in the decade since the first edition of this text was conceived, the quality and relevance of the data are substantially more robust. Much more is now known about long-term function and quality of life. Data on outcomes provide a barometer for healthcare, indicating its efficacy, safety, acceptability, and sometimes its cost-effectiveness. Such data will be used increasingly to shape public policy and guide surgical practice. Perhaps, more than in any other field of surgery, it is necessary for pediatric surgeons and urologists to look critically at long-term outcomes and the effect these have on the quality of life of their patients and their families.

By inviting contributions from leading experts around the world including the USA, Europe, Australia, and Japan, we have collected together critical analyses of the literature, framed within the context of a wealth of institutional and personal experience. We are grateful to our many authors for their outstanding efforts.

Introduction

Cleft lip and palate is a complex congenital anomaly affecting the development of the face, oro-pharynx and nose, resulting in a disturbance of appearance and function. Often multiple treatment outcomes need to be considered for an individual patient due to the wide range of severity of clefts and the nature of the special structures involved. In general, long-term outcomes from cleft lip and palate surgery include an assessment of facial appearance, speech, facial growth, dental development, hearing and psychological well-being. Consequently, final outcomes may need to be assessed many years after the original surgery, usually during early adult life.

It is well recognized that different surgical protocols for primary cleft repair can have a major influence on long-term outcomes. Protocols may differ with regard to technique used, timing of surgery, the sequence of repair, or the possible use of presurgical orthopedic alignment of the maxillary segments. Whilst the severity of the cleft and the effectiveness of the surgery undoubtedly have a major influence on outcomes, other important factors may complicate the picture further, such as general developmental variations, syndromal associations, and socio-economic factors. Non-surgical interventions such as speech and language therapy, orthodontics and audiologic input also influence the final “surgical” outcomes. The technical skill of the surgeon is a further compounding influence – a skilled surgeon can partially compensate for a mediocre technique of repair achieving good results, whereas a poor surgeon may produce unacceptable results despite the application of an otherwise sound reconstructive technique.

What is the arterial and venous supply to the thyroid gland?

Arterial supply Superior and inferior thyroid arteries on both sides. The superior thyroid artery is a branch of the external carotid and the inferior thyroid artery is a branch of the thyrocervical trunk, which in turn is a branch of the subclavian artery. At times the single thyroid ima artery, which is a direct branch of the brachiocephalic trunk, may be present in the midline.

Venous supply This is via the superior, middle and inferior thyroid veins on both sides. The former two drain into the internal jugular veins whereas the inferior thyroid veins drain into the brachiocephalic veins.

What specific pre-operative measures are taken prior to a thyroidectomy for thyrotoxicosis?

• Patients must be made euthyroid

• Vocal cord assessment using indirect laryngoscopy

• Biochemical analysis of calcium metabolism pre-operatively

• β-blockers may also be considered in an unstable patient

• In large retro-sternal goitres a CT scan is performed to assess tracheal deviation and the extent of retro-sternal extension

• Iodine for 2 weeks pre-operatively can reduce the vascularity of the gland

What different types of thyroidectomy do you know?

• Hemithyroidectomy (lobectomy or isthmusectomy)

• Total thyroidectomy (subtotal, near total or total)

What is a subtotal thyroidectomy?

Subtotal thyroidectomy refers to the excision of the entire gland but leaving about 4 g of thyroid tissue on each side. It is thought that this will reduce the risk of damage to the recurrent laryngeal nerves as well as protecting the parathyroids. There is also enough thyroid tissue left behind to avoid post-operative thyroid hormone replacement.

William E. Ladd (1880–1967) is considered to be the father of pediatric surgery in North America. However, delving into records from the Massachusetts General Hospital (MGH), founded in 1821, discloses pediatric surgical cases cared for by surgeons 60 years before Ladd's time. A book published in 1839 by John C. Warren, who performed the first publicly demonstrated operation under general anesthesia at the MGH in 1846, included pediatric cases. A generation later, his son, J. Mason Warren, wrote a book after anesthesia was well established in which many more pediatric cases were described. When the Children's Hospital in Boston was founded in 1869 (Philadelphia Children's Hospital predated it in 1855), Benjamin Shaw, resident physician at the MGH wrote to the press, “Our existing institutions public and private provide adequately for hospital treatment of children.” He stated that 190 of 1264 (14%) admissions to the MGH in 1868 were children.

A recent book, The Children's Hospital of Boston, Built Better Than They Knew, by Clement A. Smith, describes a century of history at Children's. From 1882 to 1914 the most frequent surgical admission was for bone and joint tuberculosis. Early on there were no recognized cases of appendicitis, because Reginald Fitz at the MGH had not yet described that entity. In contrast there were 113 admissions for appendicitis from 1911 to 1913.

Pancreatitis in children is relatively rare but may be increasing in frequency. Two main patterns of disease exist in children: acute pancreatitis, which accounts for 75% of all cases, is abrupt in onset but usually self-limiting; and chronic pancreatitis, in which pancreatic inflammation and gland destruction are progressive.

Acute pancreatitis

Etiology

Although the pathophysiology and functional consequences of acute pancreatitis in children are identical to those observed in adults, etiology differs significantly (Table 40.1). In children, drugs and toxins (12%–18%) and systemic disease (12%–35%) are major causes of acute pancreatitis. Abdominal trauma is another important cause of acute pancreatitis, accounting for between 14% and 29% of cases; it may be related to blunt abdominal trauma, surgical trauma, or, in some instances, to child abuse. Up to 25% of cases are idiopathic, although this proportion is less in more recent series. Recurrent acute pancreatitis, characterized by repeated acute episodes, is associated with obstructive disease, such as pancreatic ductal abnormalities, biliary tract obstruction, duplication cyst and biliary lithiasis. Pancreas divisum is the most frequently encountered pancreatic malformation present in as many as 10%–15% of patients undergoing magnetic resonance cholangiopancreatography; this anomaly is associated with 2.5% of acute pancreatitis in children. Acute pancreatitis can occur within the first month of life, but most cases present around the age of 5 years and during adolescence.

Diagnosis

Diagnosis of acute pancreatitis can be difficult and outcome unpredictable at onset, as several local, regional and systemic insults can hamper the diagnosis and affect the prognosis.

How would you distinguish a direct from an indirect inguinal hernia?

Anatomically: A direct inguinal hernia protrudes through the posterior wall of the inguinal canal medial to the inferior epigastric artery, whereas an indirect hernia emerges from the deep inguinal ring lateral to it.

Clinically: An indirect inguinal hernia may be controlled by applying pressure on the abdominal wall at the site of the deep inguinal ring surface marking: 1.5 cm above the midpoint of the inguinal ligament (midpoint of a line drawn from the pubic tubercle to the anterior superior iliac spine).

Note: Not the midinguinal point (see Chapter 18, Femoral embolectomy).

What is the difference between an obstructed and a strangulated hernia?

An obstructed hernia contains obstructed but viable bowel, whereas a strangulated hernia may contain non-viable bowel since its venous drainage is compromised.

What pre-operative measures should be taken before the repair of an inguinal hernia?

Consider general versus local anaesthetic. The procedure under a local anaesthetic is generally not advised in the anxious patient and in the obese patient. It is contraindicated in suspected strangulated herniae (where a more extensive dissection may be required).

Consider an open versus laparoscopic repair. Laparoscopic repairs are generally chosen for bilateral or recurrent herniae.

Two units of blood should be cross-matched and perioperative intravenous antibiotics (e.g. Cefotaxime and Metronidazole) should be given in cases of strangulated herniae.

What is the surface marking of the parotid gland?

The superior margin can be marked on the side of the face by a curved line starting at the tragus of the ear running to the middle of the cheek, just below the zygomatic arch. From here a curved line drawn down to the angle of mandible marks the anterior border. The inferior border is between the angle of the mandible and the mastoid process.

What different types of parotidectomy do you know? When are they performed?

A parotidectomy is performed to excise a tumour of the parotid gland. A superficial parotidectomy is undertaken either as a primary diagnostic procedure or as a curative procedure when only the superficial lobe is involved in small and low-grade tumours. In cases where the deep lobe is also involved and/or the tumour is known to be malignant a conservative total parotidectomy must be performed. This refers to the excision of the entire gland with conservation of the facial nerve. Radical parotidectomy with excision of the nerve is undertaken in cases of adherent or infiltrative disease.

What pre-operative considerations may be undertaken prior to a parotidectomy?

• Clinical assessment of the facial nerve to identify any pre-operative functional deficit

• Local examination and imaging to identify involved lymph nodes

• CT or MRI scanning to assess the deep lobe.

• […]

Why are repair of flexor tendons in the hand challenging?

This is because they are contained in well-lubricated synovial sheaths therefore:

• After lacerations they can readily retract a longer distance proximally.

• The repair must allow free gliding of the tendon within its sheath to ensure a normal function.

• Damage to the sheath and the various pulleys can result in bow stringing of the affected tendons reducing grip strength.

• Unsatisfactory post-repair rehabilitation may result in adhesions within the sheath resulting in inadequate function.

Describe the different zones of flexor tendon injuries in the hand

• Zone I Distal to the insertion of flexor digitorum superficialis (FDS) thus only contains the flexor digitorum profundus (FDP) tendon in the flexor sheath.

• Zone II This zone is from the beginning of the flexor sheath in the distal palm proximally to the start of Zone I distally. Zone II contains both the FDP and FDS tendons in the flexor sheath.

• Zone III This zone starts distal to the carpal tunnel and end at the beginning of Zone II thus containing all the tendons free from synovial sheath in the palm.

• Zone IV This is the carpal tunnel with the tendons lying under the flexor retinaculum.

• Zone V This refers to the tendons proximal to the carpal tunnel in the forearm.

Describe a technique for repair of a tendon

A core suture (Kessler repair) is used, ensuring that the knot is within the tendon as illustrated. This will reduce the bulk of the repair and allow easier gliding of the tendon. This is augmented by smaller circumferential sutures.

Introduction

As a result of major advances in all branches of pediatric care there have been considerable improvements in survival and physical health of children following pediatric surgery. Follow-up of pediatric surgical patients has, understandably, concentrated on physical or functional outcome, whereas interest in psychological and social sequelae is relatively recent. Conditions requiring pediatric surgery vary along a continuum from acute short-term problems requiring minor surgery, to those requiring major life-threatening surgery. This chapter is primarily concerned with the long-term psychological and social sequelae of conditions requiring major surgery.

The chapter is divided into four sections. The first focuses briefly on methodological issues in psychological research. The second addresses the effects of hospitalization and children's responses to hospitalization. The third section reviews studies of long-term psychological outcomes and the relationship to chronic health problems. Finally, issues arising from the previous sections are discussed.

Methodological issues

Study design

Good psychosocial adjustment in children has been described as being “reflected in behavior that is age-appropriate, normative and healthy and that follows a trajectory towards positive adult functioning”. The ideal way to assess long-term psychosocial outcomes is by prospective longitudinal studies. They are unique in their ability to determine the best causal relationship between early and later behavior, and they offer the possibility of studying whether specific risk factors lead to different outcomes, whether the effects are immediate or delayed, whether the effects persist over time and whether they are modified by intervening variables.

What two approaches do you know for performing an orchidectomy?

The two approaches for performing an orchidectomy are the scrotal or inguinal approaches.

Where do the lymphatics of the testis and the scrotal skin drain to respectively?

• The testis drains to the para-aortic lymph nodes at the level of L2.

• The scrotal skin drains via both the internal and external pudendal systems to the deep and superficial inguinal lymph nodes.

How does the above knowledge dictate the approach for orchidectomy for a testicular tumour?

As the scrotal skin and the testis have different blood supply and lymph drainage, an orchidectomy for a testicular tumour should be performed via an inguinal approach to avoid the possibility of tumour seeding to the deep and superficial inguinal systems.

What pre-operative measures must be taken prior to an orchidectomy?

• Informed consent (consider sperm banking)

• Clinical examination: tumour, other testis, hepatomegaly, lungs

• Tumour markers, liver and bone profile

• Chest X-ray (CXR)

• Scrotal ultrasound examining both testes

• Chest and abdominal CT scan (lungs, liver and para-aortic lymph nodes)

• Isotope bone scan

Briefly describe how you would perform an orchidectomy for testicular tumour

Position The patient is placed under a general anaesthetic and supine on the table. A routine preparation and draping of the groin is performed.

Incision and procedure A groin crease incision is made and is deepened into the inguinal canal by incising the external oblique aponeurosis (superficial inguinal ring). The spermatic cord is then identified and cross-clamped.