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Diversion colitis in children: an iatrogenic appendix vermiformis?

AIMS: Diversion colitis (DC) is a localized, relatively benign, iatrogenic condition which occurs in almost 100% of diverted colonic segments in patients who undergo ileostomy/colostomy for various reasons. The aim of this study was to establish histological features of DC in children. METHODS AND RESULTS: Twenty-three cases of DC following colostomy for Hirschsprung's disease in young children were analysed. The distinguishing features included prominent follicular lymphoid hyperplasia (100%), chronic mucosal inflammation (100%), accompanied by a variable degree of acute inflammation (78%) and Paneth cell metaplasia (26%). Less frequent histological findings were as follows: mild goblet cell depletion (22%), foci of cryptitis (13%), crypt abscesses (13%) and mild architectural distortion (22%). A previously unrecognized feature was the presence of mucosal aggregates of eosinophils, found in 43% of cases. A striking similarity between the normal appearance of the vermiform appendix and pathological features in DC was noted and the possible relationship between the two is discussed. CONCLUSIONS: Histological features of DC in children are very similar to those described in adults. They should help to distinguish it from ulcerative colitis and Hirschsprung's-associated enterocolitis in order to prevent inappropriate therapy and follow-up. There are many similarities between DC and the normal appendix vermiformis.

Appendix↗

Melanosis of the appendix: common in the paediatric age group.

AIMS: To investigate the incidence of melanosis of the vermiform appendix in a paediatric population, and look for correlations with season, apoptotic rate and inflammation as possible causes. METHODS AND RESULTS: We examined 300 appendices removed for abdominal pain and incidentally during other procedures over the period January 1996 to March 1999. Melanosis was present in 46% of adequate specimens with an even distribution between sexes and age groups. The two older age groups had a relatively higher incidence of severe involvement compared with the younger age group, although the difference was not statistically significant. We attempted to correlate the presence of melanosis with the season of the year and the peak incidences of paediatric gastrointestinal disease but could find no relationship. No statistically significant correlation was found between apoptotic rate and the presence of melanosis. CONCLUSIONS: The findings suggest melanosis of the appendix is relatively common. It is probably due to apoptosis associated with increased epithelial cell turnover due to many differing causes including infection, with purgative ingestion probably involved in only a minority of paediatric cases.

Adolescent↗

[Diverticulitis of the appendix vermiformis: ultrasonographic appearance].

AIM: To describe the sonographic findings in acute diverticulitis of the vermiform appendix. A case report and a review of literature are used to present the sonographic appearance, special clinical aspects and the pathogenesis of this rare entity. CONCLUSION: Acute diverticulitis of the vermiform appendix presents certain sonographic features, which allow preoperative diagnosis and differentiation from acute appendicitis and right colonic diverticulitis.

Acute Disease↗

[Ileocecal invagination of a mucocele of the appendix].

HISTORY AND CLINICAL FINDINGS: A 39-year-old patient was admitted because of pain in the right mid and lower abdomen of 2 weeks duration with vomiting and postprandial nausea. He complained of changing stool habits with diarrhoea and constipation. Clinically he showed a slight tenderness in the right lower abdomen. INVESTIGATIONS: Routine laboratory test were normal including CRP and WBC. Ultrasound showed a non-compressible mass with low intensity echoes, 3.6 x 4.6 cm in diameter in the right mid abdomen, slightly tender, surrounded by a circular, intestine-like structure. A gastrografin-swallow didn't reveal any obstacle in the proximal small intestine and was inconclusive further distal. A CT scan demonstrated a ileocoecal invagination extending in the colon ascendens to the right flexure with suspicion of a tumor. At the colonoscopy on the next day the invagination was not apparent any more, but a well-rounded tumour in the appendix region was seen. DIAGNOSIS, TREATMENT AND COURSE: Suspecting a coecal tumour with remitting ileocoecal invagination a ileocoecal resection was performed on the third day. The tumour proved to be a mass of mucus with parts of a villo-mucinous cystadenoma of the UMP type. Recovery was uneventful. CONCLUSION: Mucoceles of the appendix can cause ileocoecal invagination in the adult.

Adult↗

[Mucocele of the appendix. Incidental sonographic discovery and laparoscopic resection].

HISTORY AND ADMISSION FINDINGS: A 76-year-old currently asymptomatic man was admitted for routine sonography of the abdomen. 15 years he had had intermittently symptomatic cholecystolithiasis. 25 years before a colon contrast showed a normal coecum but no filling of the appendix. SUBSEQUENT INVESTIGATION: Sonographic examination showed the known cholecystolithisasis but additionally revealed as an incidental finding a tubular-cystic structure with a thin, echogenic wall and a hypoechogenic lumen. The lesion was located in the right upper quadrant, not compressible and arising from the coecum. Computed tomography confirmed a mucocele of the appendix without ascites and no inflammatory changes, peritoneal thickening or nodules. TREATMENT AND COURSE: Laparoscopic resection of the unruptured appendiceal mucocele together with cholestectomy was performed. On gross examination, the resected mucocele proved to be macroscopally an intact mass with a thin wall and full of white gelatinous material measuring 3 cm in diameter and 8 cm long. The histologic diagnosis was mucinous cystadenoma. The patient was discharged on the second postoperative day and recovered uneventfully. Because of the clear association of appendiceal cystadenoma with colorectal tumor a colonoscopy was performed which showed a normal colon. CONCLUSION: The differential diagnosis of a cystic mass in the right lower quadrant without previous appendectomy should include an appendiceal mucocele. If preoperatively there are no signs of malignancy, laparoscopic resection can be performed. In patients with histologic diagnosis of appendiceal cystadenoma: the colon should be examined to exclude synchronous colon tumors.

Aged↗

Sonographic appearance of an appendix carcinoma.

Malignant appendix tumours are rare entities. Especially adenocarcinomas, which only appear in about 10% of appendix tumours, are very seldom. Preoperative diagnosis is very difficult due to a lack of typical clinical signs and a clinical appearance mimicking perforated appendicitis. Nevertheless, sonography is able to show indirect signs and therefore it can provide the surgeon with more information for a better operative treatment.

Adenocarcinoma↗

[Differential diagnosis of cystic intra-abdominal lesions: mucocele of the appendix].

The sonographic appearance of a mucocele of the appendix in a 69-year-old woman is presented. The mucocele showed an atypical localisation in the right upper quadrant just below the gallbladder. This own observation is compared with other case reports in the literature. The value of ultrasound examinations in the diagnosis "mucocele of the appendix" is discussed.

Aged↗

[Preoperative diagnosis, pathologic anatomy and clinical significance of mucoceles of the appendix from the gynecologic viewpoint].

Owing to their proximity to the true pelvis and their topography, mucoceles of the appendix frequently cause clinical symptoms which call a gynecologic condition to mind. Sonographically, the structures of mucoceles are often similar to those of ovarian tumors. Within 12 months four patients with mucoceles underwent surgery at Heidelberg University Gynecological Clinic following diagnosis of a gynecologic disease (two tentative diagnoses of ovarian carcinoma, one of advanced ovarian carcinoma, one chance finding during Wertheim's operation). The problems of this rare condition are discussed with reference to the clinical findings, the intraoperative site, and the different morphology and maturity. Pathoanatomically and biologically, however, the clinical picture is not uniform. In mucoceles there is an accumulation of mucus with cystic dilatation of the lumen of the appendix. This is usually due to a tumor, which may be anything from a simple mucous membrane hyperplasia with extreme mucous formation, or an adenoma, to a mucilaginous adenocarcinoma. The rupture of a mucocele or metastasization of a carcinoma often lead to peritoneal mucous spread or peritoneal metastasization: in their patterns of distribution these are similar to a pseudomyxoma peritonei or a metastasizing ovarian carcinoma.

Aged↗

Radiological features of mucocele of the appendix.

The clinical features of mucocele of the appendix are nonspecific, and a correct preoperative diagnosis based on abdominal pain, a palpable mass in the right lower quadrant, and conventional radiography is rare. The diagnosis of appendiceal mucocele has become easier with the utilisation of the recent imaging techniques ultrasonography and computed tomography. Six patients with mucocele of the appendix are presented, and the characteristic radiological features and differential diagnosis are discussed.

Adult↗

Torsion of vermiform appendix: value of ultrasonographic findings.

A case of torsion of the vermiform appendix is described in a five-year-old boy with a two-day history of right-sided abdominal pain. No associated abdominal tenderness was reported, no vomiting nor fever. The pain was localized in the same place for 24 hours and became more severe and constant. Blood film showed a normal white cell count. After two ultrasonographic examinations in the course of 24 hours, the sign of a distended intestinal loop became constant. With the diagnosis of mucous-producing appendicular lesions or appendicular torsion, a laparotomy was performed. The appendix was severely congested and gangrenous; there was a 360-degree anticlockwise twist in its base. The related literature is reviewed and the value of the ultrasound scanner images and the possible mechanism involved is discussed.

Appendix↗

Carcinoid tumors of the appendix in children--epidemiology, clinical aspects and procedure.

INTRODUCTION: Incidental detection of a carcinoid tumor of the appendix after appendectomy is often accompanied by uncertainty about the further procedure. The tumor frequency in our own patient population, the further course in these children and a practicable follow-up protocol have to be determined. PATIENTS AND METHODS: All appendectomies performed between January 1, 1982, and December 31, 1996, were retrospectively evaluated with regard to carcinoids, monitoring the clinical course, and follow-up of the patients involved. RESULTS: A total of 4747 appendectomies were performed, 8 children had a histologically manifest carcinoid (0.169%). All patients were symptom-free in the further course, no metastases or signs of a carcinoid were documented during a mean follow-up period of 6.6 years. CONCLUSION: The youngest patient with a metastasizing carcinoid tumor of the appendix reported in the literature was 19 years old. Nevertheless, all younger patients should undergo regular follow-ups; this is done in our department by serum serotonin and chromogranin A determination.

Adolescent↗

Activation-induced deaminase cloning, localization, and protein extraction from young VH-mutant rabbit appendix.

Studies in mouse, human, and chicken suggest that activation-induced deaminase (AID) is involved in three known processes leading to antibody diversification: somatic hypermutation, gene conversion, and class-switch recombination. Developing rabbit appendix provides a particularly good site for studying all three of these B cell maturation events. We report here successful cloning of rabbit AID and isolation of AID protein from rabbit appendix-cell nuclear and cytoplasmic extracts. We succeeded in identifying and locating AID protein in cells by immunohistochemical and immunofluorescent staining techniques and examined colocalization of AID and other molecules important for Ab diversification. This report extends our knowledge about AID to a mammalian species that uses gene conversion to diversify rearranged Ig genes. Although much work remains to understand fully the mechanism of action of AID and its association with other cellular components, the rabbit system now offers a particularly useful model for future studies of these dynamics.

Amino Acid Sequence↗

Role of appendix in the development of inflammatory bowel disease in TCR-alpha mutant mice.

T cell receptor-alpha mutant mice (TCR-alpha-/-), created by gene targeting of the TCR-alpha gene in embryonic stem cells, spontaneously develop inflammatory bowel disease (IBD) resembling human ulcerative colitis. Since gut-associated lymphoid tissue is likely to play an important role in the development of chronic intestinal inflammation, we examined the changes in the appendix lymphoid follicle (ALF) and Peyer's patches (PP) in these mice. We found the structure of the ALF to be remarkably similar to that of the PP in the small intestine; in both instances, lymphoid follicles covered by surface epithelium (dome-formation) were found. The amount of proliferation in the lymphoid follicles of the appendix estimated by in vivo incorporation of 5-bromo-2'deoxyuridine was more than two times that of PP in TCR-alpha-/- mice. ELISPOT assay showed an increase of IgA, IgG1, and IgG2a, but not IgM-secreting B cells in ALF of TCR-alpha-/- mice compared to TCR-alpha+/- control mice. Furthermore, TCR-alpha-/- mice revealed an increase of autoantibody-producing B cells against the cytoskeletal protein tropomyosin in ALF as compared to PP. When TCR-alpha-/- mice underwent appendectomy at a young age (3-5 wk), the number of mesenteric lymph nodes cells at 6-7 mo were markedly less than in the sham-operated TCR-alpha-/- mice. Furthermore, appendectomy at 1 mo of age suppressed the development of IBD, with only 3.3% of these mice developing IBD in the 6-7-mo period of observation. In contrast, approximately 80% of controls, including the sham-operated TCR-alpha-/- mice, developed IBD during this period. These results suggest that ALF, rather than PP, is the priming site of cells involved in the disease process and plays an important role in the development of IBD in TCR-alpha-/- mice.

Animals↗

Myxoglobulosis of the appendix.

Myxoglobulosis is a rare morphologic variant of appendiceal mucocele characterized by intraluminal mucinous globules of the appendix. Most reported cases have presented clinically as an acute abdomen or as an incidental laparotomy or autopsy finding. We report a case of myxoglobulosis in a 32-year-old man who presented with an extra-appendiceal mass following a 10-year symptomatic course. Laparotomy disclosed a pericecal collection of opaque, white globules originating from a perforated appendix walled off by fibrous adhesions. The globules exhibited some histologic and staining properties at variance with those described in previous reports. These findings suggest that myxoglobulosis may be more heterogeneous pathogenetically than the distinctive gross appearance of the lesion would indicate.

Adult↗

Endosalpingiosis of the vermiform appendix.

Endosalpingiosis within the muscularis propria of the distal vermiform appendix was discovered in an appendectomy specimen removed during exploratory laparotomy for inflammatory tubal disease. Endosalpingiosis has not been previously reported in the appendix. The pathogenesis and possible significance of this lesion are discussed.

Adult↗

Cecal apical changes with appendicitis: diagnosing appendicitis when the appendix is borderline abnormal or not seen.

In most cases, CT of appendicitis shows a distended appendix with periappendiceal inflammation. A distended appendix, however, is not always visualized. In these patients, identifying changes at the anatomic cecal apex can secure a diagnosis of appendicitis. Cecal apical changes with appendicitis include focal cecal apical thickening, the arrowhead sign, and the cecal bar. This pictorial essay describes and illustrates the spectrum of CT appearances of cecal apical changes that can be seen with appendicitis.

Adolescent↗

Intussusception of the appendix in a patient with cystic fibrosis.

This case report describes the clinical presentation and the radiographic, endoscopic, and pathologic findings in a patient with cystic fibrosis (CF) and intussusception of the appendix. This is the first time that intussusception of the appendix has been documented in a patient with CF. This disorder should be considered in the CF patient with cramping lower abdominal pain or rectal bleeding.

Adolescent↗

Free vascularized appendix transfer for reconstruction of penile urethras with severe fibrosis.

Despite the development of newer techniques with a free radial forearm tube flaps for phallus reconstruction, severe urethral strictures are still seen in such cases after irradiation or repeated infection because of the paucity of healthy, well-vascularized tissue. For urethral reconstruction in cases with poorly vascularized tissue as well as for total penile creation, a new technique involving a free vascularized appendix transfer combined with a radial forearm osteocutaneous flap was successfully used in two cases. The appendix provides a normal tube structure composed of a muscular tubular layer lined with mucosal epithelium. It has no hair and has rich vascularization. This results in little stricture at the junction with the original urethra, no occurrence of urethral stones, and possible postoperative enlargement of the diameter with changes in catheters. This method will allow a patient with severe fibrosis around the urethra to undergo one-stage phallus reconstruction with minimal complications.

Adult↗