Clinical features

Advanced cases may demonstrate malnutrition and abdominal distension. Extra-abdominal manifestations include erythema nodosum, pyoderma gangrenosum, arthritis, uveitis and sclerosing cholangitis.

Physical findings in mild cases are few or absent. Severe cases demonstrate weight loss, anaemia, dehydration, abdominal distension and tenderness.

Complications of ulcerative colitis

Investigations

Treatment

Clinical findings

A high incidence of anal lesions is seen. Sigmoidoscopy and biopsy or colonoscopy confirm the histological diagnosis. Colonoscopy will demonstrate segmental disease and the presence of fissures and fistulas in the bowel. Medical treatment is similar to ulcerative colitis but colectomy may be required, particularly for bowel obstruction; pouch or continent ileostomy is not performed due to risk of anastomotic breakdown and fistulas.

Surgical procedures for colitis

See Figure 9.2a, b and c.

Figure 9.2 (a) Colectomy and ileorectal anastomosis. (b) The continent ileostomy. (c) Ileal pouch with ileo-anal anastomosis.

Epidemiology and pathology

Diverticular disease comprises outpouching of the mucosa through the bowel wall associated with an increased intraluminal pressure. The most common site is the sigmoid colon. Diverticular disease is rare under the age of 35 years, but reaches a prevalence of 50% among those in their eighth or ninth decade. It is common in Western countries and rare in China, India and Africa. Lack of roughage in the diet appears to be the most common risk factor.

Inspissated faeces may accumulate within the narrow neck of a diverticulum and inflammation of the sac may lead to perforation, leading to a diverticular abscess or peritonitis. Fistula formation may occur to adjacent organs, for example the bladder. Diverticular disease may be asymptomatic. Acute diverticulitis presents as lower abdominal pain localising to the left iliac fossa. Changes in bowel habit and abdominal distension may occur.

Chronic diverticular disease may cause lower abdominal pain, alternating constipation and diarrhoea with excessive flatus. Findings in the chronic case may be minimal. Acute diverticulitis (p. 91) has been called left-sided appendicitis in terms of physical findings. A perforated diverticulum may cause generalised peritonitis, or become walled by omentum and small bowel to form an abscess surrounded by inflamed tissues: this is termed a diverticular mass.

Investigations

Full blood count, colonoscopy (electively) and CT scanning are the usual methods of confirming the diagnosis. Coexistent carcinoma of the colon is not uncommon, and it may be difficult to distinguish a diverticular mass from a cancer.

Treatment

Figure 9.3 Barium enema in a patient with diverticular disease.

Table 9.1 Upper versus lower gastrointestinal bleeds

Diverticular mass/abscess/fistula (Table 9.2)

The surgery required for such problems can be complex and difficult. Antibiotics may help reduce some of the inflammation preoperatively. Simple defunctioning ileostomy may be very helpful. Exclusion of cancer is mandatory.

Table 9.2 Hinchey grading system: classification of perforations of colon due to diverticular disease (and current management strategy)

Familial adenomatous polyposis

This is an important autosomal dominant condition. Multiple polyps develop in the colon and small bowel between the ages of 13 and 30 years. Progression through the cancer sequence is inevitable. A family history is often present. Rectal bleeding is the most common finding. Total colectomy is essential because of the universal progression to cancer. Ileo-anal pouch reconstruction is the treatment of choice for younger patients.

Hereditary non-polyposis colorectal cancer (HNPCC)

HNPCC is a hereditary syndrome, caused when a person inherits a mutation in one of five genes. People with HNPCC have a high risk of developing colorectal cancer (up to 80%); 2–5% of all colorectal cancers are attributed to HNPCC, or ‘Lynch Syndrome’. HNPCC also puts patients at risk of other forms of cancer (e.g. endometrium, ureter or renal pelvis).

It is an autosomal dominant condition, so family history is crucial in detecting potential cases, guided by the Amsterdam II criteria: at least three members of the family has a diagnosed HNPCC-associated cancer, one of the three must be a first-degree relative, at least two successive generations should be affected and at least one must have been diagnosed before age 50. Screening for colorectal cancer in this group is essential and should start at an early age (after 20 years).

Aetiology and pathology

A diet rich in animal fat is a major risk factor. This may predispose gut bacteria to convert bile salts to carcinogens. Adenomatous polyps, genetic factors and inflammatory bowel disease are other well-known risk factors. The distribution of colorectal cancer is most common in the rectum and sigmoid. Up to 5% of patients have synchronous cancers and 75% of patients have coexistent benign adenomas. Tumours are classified as polypoid, ulcerative, annular or a combination.

Spread may occur by direct local invasion, by lymphatic, haematogenous or transcoelomic routes, and by direct implantation of exfoliated cells.

Staging

A conventional method of staging is the Dukes classification (Fig. 9.4):

Figure 9.4 The Dukes staging of colonic carcinoma.

Many centres now use the TNM staging system as a basis for further decisions about adjuvant radio- or chemotherapy (Box 9.7).

Screening

Increased numbers of Dukes stage A cancers can be detected by screening patients with either a known increased risk of cancer or first-degree relatives who have had polyps or cancers (Amsterdam criteria). Faecal occult blood estimations have also been shown to detect earlier cases of stage A disease. In the UK a national screening programme for colorectal cancer was started in 2007. Thus far the number of Duke’s A tumours has increased from 10% in the symptomatic group to 50% in the screened group.

Clinical features

The most important of these are shown in Box 9.8.

Right-sided tumours have non-specific complaints (e.g. malaise, weight loss, vague abdominal pain and occasionally a mass in the abdomen). Iron-deficiency anaemia is a common presentation. Intestinal obstruction is unusual for carcinomas of the right side, due to the liquid nature of the faeces.

Left-sided tumours are more likely to present with obstructive symptoms due to the solid stool and decreased calibre of bowel. Colicky abdominal pain and a change in bowel habit, which is usually loose or more frequent stool or alternating constipation and diarrhoea, occur. Mucus is another common symptom.

Rectal tumours present with rectal bleeding, mucus discharge and tenesmus. Advanced tumours may spread locally, causing faecal incontinence, back pain and urinary infections due to fistulas. Physical findings are often absent but a mass may be palpable per abdomen or rectally. Associated signs of disseminated disease include weight loss, ascites and hepatomegaly.

Investigations

All patients should have a rectal examination and a sigmoidoscopy and biopsy. Full visualisation of the bowel requires colonoscopy or CT colonography if colonoscopy cannot be completed. Once diagnosed, patients with colonic tumours are additionally imaged with CT of the chest, abdomen and pelvis for staging purposes. Patients with rectal cancer will have an additional MRI of the pelvis to better delineate whether the mesorectal envelope is threatened. If it is then preoperative adjuvant radiotherapy/neo-adjuvant chemoradiotherapy will be considered.

Treatment

Colonic carcinoma is treated by surgery (Fig. 9.5) following preoperative staging with CT scans, colonoscopy to exclude metachronous lesions and general fitness for a general anaesthetic. Bowel preparation is usually mechanical with Picolax or Klean-Prep. Colostomy is avoided.

Figure 9.5 Surgical management of colectomy. Shaded areas are resected: (a) right hemi-colectomy, (b) left hemi-colectomy, (c) sigmoid colectomy.

Rectal carcinomas present different preoperative problems and many patients are entered into trials of adjuvant radio/chemoradiotherapy (e.g. the CRO7 trial comparing preoperative radiotherapy and selective postoperative chemoradiotherapy for rectal cancer). Radiotherapy may be either a short course (5 days) or a long course, depending on stage of tumour, usually assessed by MRI of the rectum. Rectal cancers are surgically treated by anterior resection or abdominoperineal excision (Fig. 9.6). Complications of colorectal surgery are listed in Box 9.9.

Figure 9.6 Surgical management of rectal carcinoma. (a) Anterior resection of the rectum. (b) Abdominoperineal excision of the rectum and anus. Shaded areas are resected.

Advanced disease

Local recurrence remains a serious problem for the patient and is most unlikely to be resectable. Palliation is the rule of treatment. Hepatic metastases, if they are solitary or less than four in number, can be resected, which has resulted in some improvement in survival. Multiple hepatic secondaries are treated palliatively by chemotherapy or radiofrequency ablation.

Haemorrhoids

These are engorgements of the haemorrhoidal venous plexuses with redundancy of their coverings. The anal cushions may remain in their usual position in the anal canal (first degree), descend to involve the skin of the distal anal canal so that they prolapse on defecation but reduce spontaneously (second degree), or become of such a size that they are always partly outside the anal canal (third degree). Classical positions are the left lateral, right posterior and right anterior positions, although secondary haemorrhoids can occur in between these anatomical sites. The diagnosis of haemorrhoids is shown in Table 9.4. Constipation and straining at the time of defecation may be a factor in the development of haemorrhoids. In women, pregnancy is a common risk factor.

Table 9.4 Conditions related to (and which may be confused with) haemorrhoids

Clinical features

Bleeding – usually at defecation – bright red in nature, dripping or spurting into the toilet pan or onto toilet paper may occur afterwards. This is painless. Minor faecal soiling or mucus leak is common along with pruritus, occasional discomfort and palpable swelling. Pain only occurs upon thrombosis or the development of coexistent anal fissure. On examination, haemorrhoids vary from a slight increase in the normal anal cushion size, visible only at proctoscopy, to large third degree internal/external haemorrhoids apparent on inspection of the anal verge.

Treatment

Reassurance that this is a benign condition is very helpful to alleviate the fear of more sinister pathology. Regulation of the bowels, particularly relief of constipation by an increased intake of fluids, fruit, vegetables and bulking agents, and the avoidance of prolonged straining at stool are often recommended. Proprietary medications and suppositories may also be helpful for the relief of pruritus.

Several treatments are available. Injection sclerotherapy and Barron banding (with or without the use of suction) are treatments used in the outpatient department. Cases requiring general anaesthesia include standard haemorrhoidectomy, THD and, more recently, stapled haemorrhoidopexy. The latter are generally reserved for resistant cases or third-degree haemorrhoids. Manual dilatation of the anus is rarely used for fear of irreversible damage to the anal sphincter. Injection sclerotherapy may need to be repeated. Barron-band ligation (Fig. 9.7) has a serious complication of pain and haemorrhage after the application of the bands. Cryotherapy and lateral anal sphincterotomy are rarely used for the treatment of haemorrhoids today. Haemorrhoidectomy aims to excise as much redundant epithelium and vasculature as possible and may be carried out by a variety of methods.

Figure 9.7 Band ligation for haemorrhoids. (a) The haemorrhoid is grasped (or held by suction). (b) The haemorrhoid is drawn further within the instrument (and thus within the elastic band held outside). (c) The strangulated haemorrhoid with the elastic band at its base (which necroses and is later shed, sometimes accompanied by bleeding).

Thrombosed haemorrhoid

Thrombosis may develop within haemorrhoidal veins and commonly presents as a surgical emergency. The episode is best managed conservatively with the application of ice, non-constipating analgesics and lubricant laxatives. Resolution takes place over the following 7–10 days. Urgent haemorrhoidectomy is occasionally used but runs the risk of postoperative anal stenosis.

Perianal haematoma

This condition, also known as thrombosed external pile, is a discrete, painful swelling in the perianal margin that looks like a small blackcurrant. There is often a history of straining 2 or 3 days prior to the episode of acute rectal pain. If severe, the haematoma may be deroofed under local anaesthetic with immediate relief.

Fissure in ano

Perianal sepsis

A variety of anal conditions present with suppuration, as shown in Table 9.5. The spread of sepsis begins in the intersphincteric space and may spread vertically, horizontally or circumferentially (Fig. 9.8). Five anatomical sites of abscess are classified into intersphincteric, perianal, intermuscular, supralevator and ischiorectal. The formation of a fistula occurs as a result of pus spreading along a track to emerge distal to the mucocutaneous junction, and an internal and external track is formed. Fistulas are classified according to their intersphincteric/trans-sphincteric nature. Other types of fistula include superficial, suprasphincteric and extrasphincteric (Fig. 9.9).

Table 9.5 Conditions associated with perianal sepsis

Figure 9.8 Spread of infection. (a) Vertical spread. (b) Horizontal spread.

Figure 9.9 Types of fistula. (a) Trans-sphincteric. (b) Suprasphincteric. (c) Extrasphincteric.

Pilonidal disease

This is common, and usually occurs in the natal cleft but can occur in the umbilicus and in the webs of the fingers. Aetiology is unclear. It presents as a sepsis resulting in pain, tenderness and discharge, often with a history of previous recurrent episodes. There are one or more openings in the midline or to either side with protruding tufts of hair. The management is usually surgical. Orthodox treatment is excision of the pits and the wound is left to heal by secondary intention. Post-treatment shaving of the skin is necessary for 6 months – failure to do so is the commonest cause of recurrence.

Hidradenitis suppurativa

The apocrine sweat glands, usually in the perineum, inguinal regions and axilla, become the site of a mixed bacterial infection. Obesity, poor hygiene and hormonal imbalance are contributory factors. Various degrees of sepsis occur, with tracks running widely in the subcutaneous tissue. In the perianal region, these may be confused with anal fistula. The treatment is to control the contributory factors and to lay open the septic areas. Long-term prophylactic antibiotics and occasionally plastic surgery procedures are necessary.

Pruritus ani

Pruritus ani is a common symptom which may be caused by inadequate hygiene, anal disorders, as part of a skin infection, local infection, infestation by parasites or an allergic reaction to pharmaceuticals or as part of a generalised pruritus in obstructive jaundice or in faecal incontinence due to a sphincter defect. Itching in the perianal skin is the initial symptom. If the causative factor persists, soreness and pain even on walking occur. The perianal skin commonly shows evidence of abrasion or the presence of one of the specific factors described.

The key to successful management is the accurate determination and treatment of the cause. Ointments for symptomatic management should only be used when a precise diagnosis has been made. Short courses of topical steroids may be given. Threadworms should be treated. Strict instruction in anal hygiene should be given to avoid faecal soiling.

Faecal incontinence

This may occur either as the inability to defer defecation for more than a few minutes or as passive incontinence defined as the loss of stool without the patient being immediately aware. Aetiology may include sphincter inadequacy due to large third-degree haemorrhoids, rectal prolapse or a large faecal mass in the rectum, or anal canal tumour. Damage to the sphincter from previous surgery or overstretching (previous surgery or unusual sexual practices) or obstetric injury (third-degree perineal tear) or loss of the motor innovation to the internal sphincter, e.g. diabetes, spina bifida or prolonged, complicated obstetric delivery, may all contribute. Loss of cerebrospinal regulation and fistula between rectum and vagina may also be factors.

Tumours of the anal canal

Perianal warts caused by the human papilloma virus are common, most often sexually transmitted by both sexes. They are common in HIV infection. Bowen’s disease, basal cell and squamous cell carcinomas of the anus have all been described. Their presentation is similar to all other anal diagnoses and a careful examination must be undertaken to establish the diagnosis. Perianal warts are treated by excision under a general anaesthetic, as are keratoacanthoma and Bowen’s disease. Basal cell carcinomas and squamous cell carcinomas may respond to either local excision or radiotherapy with or without chemotherapy.