Twenty years of familial adenomatosis polyposis syndromes in the Singapore Polyposis Registry: an analysis of outcomes.

INTRODUCTION: The Singapore Polyposis Registry (SPR) was established in 1989 at the Singapore General Hospital. This initiative was aimed at providing a central registry service to facilitate identification, surveillance and management of families and individuals at high risk of colorectal cancer. The aim of the present study was to provide a comprehensive review of all patients with familial adenomatous polyposis (FAP) syndrome in the SPR. METHODS: All patients diagnosed with FAP in 1989-2009 were analysed. Data was extracted from a prospectively collected database. RESULTS: 122 patients from 88 families were analysed. The median age of this cohort was 29 (range 10-68) years. 97 percent of the cases were FAP and 3 percent were attenuated FAP. 92 patients tested positive for adenomatous polyposis coli gene. 42 percent of patients were diagnosed with colorectal cancer, of which 78 percent were diagnosed at an advanced stage. 73 percent of patients underwent restorative proctocolectomy and 21 percent had total colectomy. The median age at operation was 30 years. At median follow-up of 98 months, ten-year overall survival was 75.6 percent (95 percent confidence interval 67.0-84.2) and the median age at death was 40 years. For cancer cases, the overall recurrence was 13.5 percent. Recurrence and disease-free survival were not significant for the type of surgery performed (p-value is 0.486). CONCLUSION: The SPR plays an important and integral part in counselling patients and families with FAP. Improved surveillance programmes may be required to detect the development of cancers in these patients at an earlier stage.

Traumatic rectal perforation presenting as necrotising fasciitis of the lower limb.

Necrotising fasciitis is a life-threatening soft tissue infection that is associated with high mortality and morbidity. It has been described in the form of Fournier's gangrene following rectal perforations related to colorectal cancer. In these rare instances, spontaneous perforation of locally-advanced rectal carcinoma provides an entry point for bacterial seeding to the surrounding soft tissues, resulting in Gram-negative sepsis of the perineum. To our knowledge, necrotising fasciitis extending beyond the perineum due to rectal perforation has not been previously described. We report an unusual self-induced traumatic rectal perforation presenting with severe necrotising fasciitis of the lower limb in a 73-year-old Chinese man. Our patient was successfully treated with a multidisciplinary approach that involved a defunctioning colostomy as well as prompt and rigorous debridement of the affected limb. We also review the literature on the management of retroperitoneal rectal perforations and their sequela, as well as discuss the various surgical options commonly applied and their outcomes.

Laparoscopic high anterior resection with natural orifice specimen extraction (NOSE) for early rectal cancer.

Laparoscopic surgery for colorectal cancer requires an abdominal incision to extract the resected specimen. We describe a technique for laparoscopic resection of an early-stage upper rectal cancer in a 51-year-old man followed by transanal specimen delivery, hence avoiding the need for making any additional abdominal incisions for retrieval of the specimen. Pneumoperitoneum was created, followed by medial-tolateral mobilization of the sigmoid colon, and take down of the splenic flexure and division of the inferior mesenteric vessels laparoscopically. The upper rectum distal to the tumour and proximal colon was transected with a laparoscopic stapler. The specimen was retrieved transanally via an opening in the rectal stump. The proximal colon was then delivered transanally and the anvil of the circular stapler inserted before returning it to the pelvic cavity. The rectal stump was transected again just below the opening to close off the stump, and the colorectal anastomosis was then completed intracorporeally. The patient, a 51-year-old male (BMI 18.6 kg/m(2)) with a 2.5-cm, early-stage posterior rectal cancer 12 cm from the anal verge, underwent the above-described procedure. Postoperative recovery was uneventful. He resumed normal daily activities 1 week after surgery. Histology confirmed a T1N0 upper rectal cancer. In the effort to minimize surgical trauma and postoperative pain, natural orifice specimen extraction techniques have been attempted. This procedure may be applicable to benign tumours and early colorectal cancer, and serves as an intermediate step between laparoscopic and natural orifice surgery.

A prospective study assessing anal plug for containment of faecal soilage and incontinence.

OBJECTIVE: Faecal incontinence is a common and embarrassing problem for many individuals. Some patients remained symptomatic despite the availability of different treatments. There is a limited range of commercially available products designed to cope with faecal incontinence. The anal plug has been developed to contain the loss of stool. This study aimed to evaluate the use of anal plug in Asian patients with intractable faecal soilage and incontinence judged by clinical and functional outcomes. METHOD: A prospective study of consecutive patients with intractable faecal incontinence was carried out. Suitable patients tested the anal plug for 3 weeks. They completed a structured questionnaire on its use including the ASCRS quality of life questionnaire for faecal incontinence. RESULTS: Thirty patients, median age 63 (interquartile range 52-70) years, participated in the trial. Nineteen of 30 patients were comfortable wearing the plug, seven patients withdrew from the study because of discomfort, and four had tolerable discomfort and managed to complete the trial protocol. Patients who tolerated the plug found that it was highly successful in controlling faecal incontinence. Twenty-one of 30 patients wished to continue to use the plug regularly after the study. There was a trend toward improvement in quality of life scores during the study. CONCLUSION: The anal plug was effective in containing faecal incontinence and was well tolerated in the majority of patients selected for this treatment.

Young age influences treatment but not outcome of colon cancer.

BACKGROUND: Early age at onset is often considered a poor prognostic factor for colon cancer. The aim of this study was to determine the association between age, clinicopathologic features, adjuvant therapy, and outcomes following colon cancer resection. METHODS: A prospective database of 1,327 surgical stage I-III colon cancer patients operated on from 1990-2001 was evaluated, and patients grouped by age. RESULTS: Sixty-eight patients (5%) were diagnosed at age 40 (older). Younger patients were more likely to have left-sided tumors (66% vs 51%, P = .02), but no more likely to present with symptomatic lesions, more advanced tumors, or have worse pathologic features. Younger patients were noted to have more nodes retrieved in their surgical specimens than older patients (median 18 vs 14, P = .001), although the numbers of total colectomies were similar in both groups. Younger patients were also more likely to receive adjuvant chemotherapy, and this was most pronounced in the stage II cohort: 39% vs 14%, P = .003. With a median follow-up of 55 months, 5-year disease-specific survival (DSS) was similar in both study groups: 86% vs 87%, but 5-year overall survival (OS) was significantly higher in the younger patient cohort (84% vs 73%, P = .001). CONCLUSION: Younger patients undergoing complete resection of stage I-III colon cancer had DSS similar to older patients. However, younger patients had more nodes retrieved from their specimens and were more likely to receive adjuvant therapy, especially for node-negative disease. These factors may have contributed to their overall favorable outcome.

Prospective randomized crossover trial comparing fibre with lactulose in the treatment of idiopathic chronic constipation.

BACKGROUND: Fibre is often recommended as the first-choice treatment but its effects can be uneven. The aim of the study was to compare the clinical efficacy and tolerability of fibre versus lactulose in outpatients with chronic constipation. METHODS: In a prospective randomized crossover trial, patients were randomized to receive fibre or lactulose for four weeks. Between treatments, patients had at least one week free of laxatives. RESULTS: 50 patients, of median age 50 years (range, 18-85) were recruited and 39 patients completed the trial. Compared to fibre, lactulose resulted in significantly higher mean bowel frequency (7.3, 95% CI 5.7 to 8.9 vs. 5.5, 95% CI 4.4 to 6.5; p=0.001) and stool consistency score (3.4, 95% CI 3.1 to 3.7 vs. 2.9, 95% CI 2.5 to 3.3; p=0.018). Scores for ease of evacuation were similar. The frequencies of adverse effects were not significantly different, but greater in the lactulose group. Mean patients' recorded improvement score was significantly higher after taking lactulose than fibre (6.2, 95% CI 5.5 to 7.0 vs. 4.8, 95% CI 4.0 to 5.9; p=0.017). Of the 39 patients who completed the trial, 24 (61.5%) preferred lactulose and 14 (35.9%) preferred fibre. CONCLUSIONS: Lactulose had better efficacy than fibre for chronic constipation in ambulant patients, although both treatments were equally well tolerated in terms of adverse effects.

A missing tongue stud: an unusual appendicular foreign body.

Appendicitis caused by foreign bodies after body piercing is rare. Previous reports have described a variety of foreign bodies that were either found incidentally in the appendix or were felt to be responsible for appendicitis. We report a case where a patient known to have previously ingested a "tongue stud" after a tongue piercing presented with chronic right iliac fossa pain. The missing "tongue stud" had impacted in the appendix and was successfully removed by laparoscopic appendicectomy with resolution of patient's symptoms. We review the literature covering the clinical management of patients presenting with foreign bodies in the appendix.

Does gum chewing reduce postoperative ileus following open colectomy for left-sided colon and rectal cancer? A prospective randomized controlled trial.

OBJECTIVE: Postoperative ileus is common after colorectal resection and can prolong hospital stay. Gum chewing, a type of sham feeding, may to stimulate gut motility via cephalic-vagal stimulation, and thereby reduce the length of ileus. This study aimed to determine whether gum chewing in the immediate postoperative period facilitated recovery from ileus following resection for left-sided colorectal cancer. METHODS: In a prospective randomized control trial, 38 patients undergoing open surgery for left-sided colorectal cancer were allocated to standard postoperative care (control group, n = 19) or to standard postoperative care plus the immediate use of chewing gum (treatment group, n = 19). RESULT: Control patients passed flatus by mean of 2.7 days (SD 1.0) and faeces by 3.9 days (SD 1.5); for the treatment group, this was 2.4 days (SD 1.0) and 3.2 days (SD 1.5) respectively, (NS, P = 0.56 and P = 0.38). Length of hospital stay was 11.1 days (SD 7.3) in control group and 9.4 days (SD 2.5) in the treatment group (NS, P = 0.75). CONCLUSION: The addition of gum chewing to a standardized postoperative regimen did not reduce the period of postoperative ileus or shorten length of stay following open surgery for left-sided colorectal cancer.

Meta-analysis of randomized clinical trials comparing drainage alone vs primary sphincter-cutting procedures for anorectal abscess-fistula.

BACKGROUND AND AIM: Concurrent definitive treatment of underlying fistulas from infected anal glands at the time when the anorectal abscesses are drained is controversial as this is associated with a higher incidence of faecal incontinence, failure and recurrence. This meta-analysis was conducted to determine the merits of drainage alone vs primary sphincter-cutting procedures (which includes fistulotomy and fistulectomy) for anorectal abscess-fistula. METHODS: Medline, Embase and Cochrane Central Register of Controlled Trials database searches identified all randomized controlled trials using the keywords: anorectal abscess, anal sepsis, drainage, fistulotomy, fistulectomy or surgery from 1966 to 2004. The outcome variables analysed were recurrence, faecal continence and wound-healing times. RESULTS: Five trials were considered suitable for the meta-analysis, with a total of 405 patients. Sphincter-cutting procedures for anorectal abscesses resulted in 83% reduction in recurrence rate [relative risk (RR) 0.17, 95% confidence interval (CI) 0.09-0.32, p<0.001]. However, there was a tendency to a higher risk of faecal incontinence to flatus and soiling when primary sphincter-cutting procedure was performed (RR 2.46, 95% CI 0.75-8.06, p=0.140). CONCLUSION: There is no conclusive evidence if simple drainage or sphincter-cutting procedure is better in the treatment of anorectal abscess-fistula.

Ileostomy carcinomas a review: the latent risk after colectomy for ulcerative colitis and familial adenomatous polyposis.

BACKGROUND: Ileostomy carcinoma after colectomy for ulcerative colitis and familial adenomatous polyposis is rare. METHODS: Forty-three case reports from the literature and a case of ours are reviewed. RESULTS: The risk of malignancy following ileostomy formation appears to be increased compared to the very low incidence of primary small bowel carcinoma. Chronic physical or chemical irritation of the stoma may predispose the ileal mucosa to colonic metaplasia with subsequent adenoma formation, dysplasia and invasive malignant change. This is particularly so where ileostomies are fashioned for familial adenomatous polyposis and ulcerative colitis. CONCLUSION: Routine surveillance of patients who have had an ileostomy for 15 years or longer may lead to earlier detection of this complication.

A modified technique using the Yankauer sucker and argon plasma coagulation for anorectal procedures.

Argon plasma coagulation (APC) is a useful and effective treatment for some anorectal conditions. We describe a modification of the APC instrumentation that aids the application of APC in such cases.

Vacuum-assisted closure in the management of the open abdomen: a report of a case and initial experiences.

In certain surgical patients undergoing laparotomy it may be best to leave the abdomen open as a laparostomy. Care of the resulting wound represents a considerable medical and nursing challenge. This article presents such a case and the authors' initial experience of managing laparostomy wounds with vacuum-assisted closure.

Prospective, randomized trial comparing diathermy excision and diathermy coagulation for symptomatic, prolapsed hemorrhoids.

PURPOSE: This study was designed to compare diathermy excision and diathermy coagulation in the treatment of symptomatic prolapsed piles. METHODS: Forty-five consecutive patients were randomly assigned to diathermy excision hemorrhoidectomy (Group A, n = 25) and diathermy coagulation (Group B, n = 20) under general anesthesia. RESULTS: The median duration of surgery was ten minutes for both groups. There was no statistical difference in the severity of postoperative pain at rest between the two groups, but Group A patients felt less pain during defecation on the third postoperative day (median, 5 (interquartile range, 3-7) vs. 8 (4-9); P = 0.04) and on the sixth postoperative day (median, 5 (interquartile range, 2-6) vs. 9 (5-10); P = 0.02). There was, however, no statistical difference in postoperative oral analgesics use and patients' satisfaction scores between the two groups. Complication rates were similar except that diathermy coagulation tended to leave some residual skin components of external hemorrhoid especially in very large prolapsed piles. Group A patients resumed work earlier (mean, 12 (range, 4-20) vs. 17 (11-21) days); however, this was not statistically significant ( P = 0.1). CONCLUSIONS: Diathermy coagulation of hemorrhoids is a simple technique and may be considered in suitable cases.

Potential role of glial cell line-derived neurotrophic factor receptors in Müller glial cells during light-induced retinal degeneration.

Glial cell line-derived neurotrophic factor (GDNF), neurturin (NTN) and their receptors (GFRalpha1, GFRalpha2 and Ret) play an important role in the survival of neurons in the central and peripheral nervous system. For example, GDNF as well as other trophic factors promotes photoreceptor survival during retinal degeneration. Recent studies have proposed that part of neurotophic rescue of photoreceptors may be indirect, mediated by interaction of the neurotrophic factors with other cell types, that in turn release secondary factors that act directly on photoreceptors. In the present study, we examined the GDNF receptor expression in control and light-damaged retina, and found that GFRalpha2 protein is upregulated in retina-specific Müller glial cells during photoreceptor degeneration. We also examined the effect of GDNF or NTN on cultured Müller cells. Exogenous GDNF increased brain-derived neurotrophic factor, basic fibroblast growth factor and GDNF, but not NTN mRNA production. On the other hand, NTN increased NTN, but not GDNF mRNA production in cultured Müller cells. These observations suggest that GDNF, NTN and their receptors are involved in the regulation of trophic factor production in retinal glial cells, and that functional glia-neuron network may utilize GDNF family for the protection of neural cells during retinal degeneration.

Bladder and sexual dysfunction following laparoscopically assisted and conventional open mesorectal resection for cancer.

BACKGROUND: Bladder and sexual dysfunction, secondary to pelvic nerve injury, are recognized complications of rectal resection. This study investigated the frequency of these complications following laparoscopically assisted and conventional open mesorectal resection for cancer. METHODS: A total of 170 patients with rectal cancer was identified from a previous randomized trial of laparoscopic versus open resection. A retrospective analysis of bladder and sexual function before and after operation was performed by means of postal questionnaires and telephone interviews. RESULTS: At the time of the study, 111 (65 per cent) of the 170 patients were alive, of whom 80 (72 per cent) responded. Of the responders, 40 patients had undergone laparoscopically assisted resection and 40 had had an open operation. No significant deterioration in bladder function following operation was observed, although two patients in the laparoscopic group required long-term intermittent self-catheterization. A significant difference in male, but not female, sexual function was noted, with seven of 15 sexually active men in the laparoscopic group reporting impotence or impaired ejaculation, compared with only one of 22 patients having an open operation (P = 0.004). All patients with bladder or sexual dysfunction in the laparoscopic group had resection of either bulky or low rectal cancers. CONCLUSION: Laparoscopically assisted rectal resection is associated with a higher rate of male sexual dysfunction, but not bladder dysfunction, compared with the open approach. This has implications, particularly for sexually active males with bulky or low rectal cancers, when deciding the best operative approach.

Unusual endolaryngeal extension of a large thyroglossal duct cyst.

INTRODUCTION: Thyroglossal duct cysts account for approximately 70% of congenital neck abnormalities. Hoarseness of voice and the extension of the cyst into the larynx are very uncommon. CLINICAL PICTURE: We present a patient with a long history of a large thyroglossal cyst who developed progressive hoarseness of voice. The cyst had protruded into the larynx producing the appearance of a laryngeal neoplasm. Computed tomography (CT) clearly demonstrated the nature of the cyst and its encroachment into the larynx. TREATMENT: This was safely excised with a Sistrunk procedure and a temporary tracheostomy to maintain a secure airway, in view of the possibility of postoperative supraglottic oedema. CONCLUSION: Endolaryngeal extension of the thyroglossal duct cyst is rare. Evaluation of unusual large midline neck masses should include a CT scan. The intralaryngeal component of the cyst can be easily dissected off from the neighbouring structures during dissection in the Sistrunk procedure. A tracheostomy may be needed.