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Crohn's disease of the appendix.

Although appendectomy is often the first surgical procedure in patients with Crohn's disease (CD), only few data exist on appendiceal CD. The aim of this study was to analyze appendices of CD patients undergoing surgery. We analyzed 149 specimens from consecutive patients with CD who underwent primary right ileocolonic or (sub)total colonic resection. The appendix was present in 90 resection specimens. Histologic findings were compared with those of 180 age and sex matched controls. Thirty-six appendices showed histologic signs of CD (40%): A transmural inflammation was found in 24 cases (67%), and a mucosal inflammation in 12 cases (33%). Histologic hallmarks of mucosal involvement were focal or discontinuous inflammation with crypt distortion and a histiocytic and lymphocytic predominant inflammation. Furthermore, epitheloid granulomas and erosions or ulcers with abundant histiocytes at the lesions base were observed. In comparison to controls luminal obliteration was significantly overrepresented in CD whereas acute phlegmonous appendicitis was underrepresented ( P<0.01). Patients with CD of the appendix showed a more widespread colonic involvement than those without ( P<0.01). This study shows that CD of the appendix exhibits specific histologic features that allow a differentiation from non-CD-associated appendicitis. CD-associated appendicitis is a frequent event, probably signifying a more widespread colonic involvement.

Adolescent↗

Sliding appendiceal inguinal hernia with a congenital fibrovascular band connecting the appendix vermiformis to the right testis.

It is not uncommon to find the appendix vermiformis within a hernia sac; however, sliding appendiceal inguinal hernia is rare. A 9-month-old boy with an incarcerated right scrotal hernia is presented in this case report. Although the hernia was reduced through a conservative approach, appendix vermiformis remained in the hernia sac because of its attachment to the upper pole of the right testis. Exploratory surgery during the inguinal hernia repair revealed a connecting band that extended from the appendix vermiformis into the scrotum and attached to the right testicle. Histologic examination showed that the band was congenital. After reduction of an incarcerated hernia, the persistence of a thickened or a cord-like structure is a warning for the presence of a sliding hernia. We suggest that this uncommon developmental anomaly is likely to cause the processus vaginalis to remain patent, thus facilitating hernia formation.

Appendix↗

Reconstruction of bladder and urethra using ileocecal segment and appendix in patients with exstrophy-epispadias complex: the first report of a new surgical approach.

OBJECTIVE: We aimed to describe our experience in administering an innovative surgical technique to treat pediatric cases of exstrophy-epispadias complex. MATERIAL AND METHODS: Between 1995 and 2004, seven consecutive patients (six males) with exstrophy-epispadias complex were treated using ileocecal segment for bladder augmentation and appendix for urethral reconstruction. In a single-stage operation, the exstrophied bladder was dissected- and a segment of cecum, ascending colon, terminal ileum, and the corresponding appendix were isolated. Using the opened colon to augment the bladder, the ileal segment was fashioned to skin as temporary stoma, and the appendix was laid in the urethral lumen as the neourethra following urethral demucosation. This technique was used as a secondary surgery in one case and as a primary surgery in six neonates. Evaluation of the urinary tract status was performed by cystograms and ultrasonograms. RESULTS: Renal function was saved in all cases and continence was achieved by clean intermittent catheterization every two hours either via the neourethra (n = 6) or through the temporary stoma (n = 1). The patients did not experience any metabolic complications in their follow-ups. Moreover, no one had vesicourethral reflux, dehiscence, or fistula. CONCLUSIONS: The technique was deemed safe with acceptable outcomes even when secondary repair of previously failed operation was intended. Experiencing the technique in larger cohorts as well as longer follow-ups might be necessary to assess probable long-term complications.

Appendix↗

Rabbit IgH sequences in appendix germinal centers: VH diversification by gene conversion-like and hypermutation mechanisms.

Although the rabbit IgH locus contains approximately 100 VH genes, the majority of B cells rearrange VH1. To produce a primary repertoire containing a sufficient number of protective antibodies, rearranged VH1-DH-JH sequences may diversify within rabbit B cells in an organ that functions like a chicken bursa, sheep ileal Peyer's patch, or both. It was suggested many years ago that the rabbit appendix could be a bursal equivalent. To reexamine this possibility, we analyzed rearranged heavy chain variable region sequences in B cells from light and dark zones of appendix germinal centers from 6-week-old rabbits. Our findings indicate that antibody diversification occurs by gene conversion-like and somatic hypermutation mechanisms in appendix germinal centers of young rabbits.

Amino Acid Sequence↗

Free microvascular transfer of the vermiform appendix for creation of a tracheo-oesophageal fistula: A new method of voice reconstruction.

BACKGROUND: Voice rehabilitation following laryngectomy can take many forms. As its basic premise, vibrating air must be transferred to the mouth where articulation takes place and speech can be produced. It requires a source of air, a conduit for transfer, and a mechanism for prevention of regurgitation and aspiration. Creating a tracheo-oesophageal fistula and maintaining it with a vascularised appendix has been the intention of this report. METHODS: Three patients with an average age of 53 years underwent the procedure of free transfer of the appendix for voice restoration during the months of September 2004 through December 2004. All patients had laryngectomies and one had total cervical oesophageal reconstruction with a pedicled pectoralis major flap. Voice evaluation, swallowing function, and presence of aspiration were evaluated. RESULTS: All flaps survived without complications. The results of swallowing function were unaltered from preoperative levels. All flaps remained patent at an average follow-up period of 8 months. All three patients could produce loud voice, which was intelligible at a reasonable distance. Maximal phonation time was 4s in two patients and 5s in one patient. Voice rehabilitation using the free appendix flap can achieve a phonation time which is low and words and short phrases that are intelligible but limited so far to this level. The donor site morbidity is low and aspiration was not present. The results of this study indicate that this method may have a potential role in voice reconstruction but requires more experience and refinement of this technique.

Anastomosis, Surgical↗

Endometriosis and the appendix: a case series and comprehensive review of the literature.

OBJECTIVE: To report the prevalence of appendiceal disease in women with chronic pelvic pain undergoing laparoscopy for possible endometriosis, summarize the literature, and more accurately estimate the prevalence of endometriosis of the appendix. DESIGN: Prospective case series and literature review. SETTING: Academic research institute. PATIENT(S): One hundred thirty-three patients with chronic pelvic pain and possible endometriosis undergoing laparoscopy. INTERVENTION(S): History, physical exam, and abdominopelvic laparoscopy. Endometriosis and adhesions were excised using selective Nd:YAG contact laser trabeculoplasty and pathologically evaluated. Only patients with visible abnormalities involving the appendix were treated via concurrent laparoscopic appendectomy. MAIN OUTCOME MEASURE(S): Appendiceal abnormalities at laparoscopy. RESULT(S): Of 133 patients, 13 had a previous appendectomy with unknown pathology. Of the remaining 120 patients, 109 reported right lower quadrant pain. Of this subgroup, six patients had appendiceal pathology: four with pathology-confirmed endometriosis, one with Crohn's disease suspected at laparoscopy, and one with chronic appendicitis. The prevalence of appendiceal endometriosis in patients with biopsy-proven endometriosis (n = 97) or with right lower quadrant pain (n = 109) was 4.1% and 3.7%, respectively. This rate was similar to the 2.8% prevalence confirmed by literature review in patients with endometriosis but was much higher than that reported in all patients (0.4%). CONCLUSION(S): Appendiceal endometriosis, while relatively uncommon in patients with endometriosis, is rare in the general population. In patients with right lower quadrant or pelvic pain, the appendix should be inspected for endometriosis and evidence of nongynecologic disease.

Adult↗

Isolation of mesenchymal stem cells from human vermiform appendix.

BACKGROUND: Recent findings have shown that pluripotent stem cells exist in areas outside the bone marrow (BM). Moreover, it has been demonstrated that the appendix is important for the development of mucosal gut immunity, and hematopoietic progenitors have been isolated from animal and human appendices. MATERIALS AND METHODS: Non-inflamed appendices removed during laparotomy were processed and cultured until the appearance of adherent cells. Differentiations (performed under osteogenic, adipogenic, and myogenic conditions) were confirmed by immunohistochemistry and cytochemistry. Polymerase chain reaction and cytofluorimetric analyses were performed to evidence the presence of genes and protein specific lineages in appendix-derived mesenchymal stem cells (ADMCs). RESULTS: ADMCs were present in non-inflamed appendices. ADMCs under osteogenic conditions differentiated in osteoblasts and showed increased alkaline phosphatase expression; at the gene level, we observed the expression of Core binding factor alpha 1 (Cbfa1) and osteocalcin in osteogenic induced ADMCs. Under adipogenic conditions, lipidic drops in the cytoplasm, expression of lipoprotein lipase (LpL), and peroxisome proliferator-activated receptor gamma were observed; under myogenic conditions, myotubes expressing muscle specific proteins like desmin were formed. Myogenic regulatory factor 4 and MyoD were selectively induced in the ADMCs under myogenic conditions. CONCLUSIONS: This study shows for the first time that mesenchymal stem cells can be isolated from normal appendices obtained from a pediatric and adult age group (0-18 years of age). This finding not only may further knowledge of the maturation of the intestinal immunesystem but also could indicate a new physiological role of the human vermiform appendix.

Adolescent↗

[Crohn's disease of the appendix].

Crohn's disease limited to the appendix is uncommon. Clinically, it is likely to mimic acute appendicitis. It is more frequent in young people and definitive diagnosis is histological. We present a series of seven cases of Crohn's disease of the appendix that were treated in our surgery service over the past 12 years. The seven patients had pain in the lower right quadrant. In all patients, the preoperative diagnosis was acute appendicitis and appendectomy was performed. Histopathological evaluation was required for diagnosis. In the postoperative course, one patient was diagnosed with colonic Crohn's disease, and outcome was favorable with medical treatment. Although isolated Crohn's disease of the appendix is a rare entity, it should be considered in the preoperative differential diagnosis of patients with right lower quadrant pain and a protracted preoperative course mimicking acute appendicitis. Disease recurrence elsewhere in the alimentary tract is uncommon.

Adolescent↗

Autotransplantation of the vermiform appendix for ureteral substitution.

Ureteral substitution with vermiform appendix has been reported rarely. Herein is reported a case of a traumatic ureteropelvic disruption with a 7-cm long ureteral defect that was bridged successfully by appendix interposition for the first time in a child. The potential use of vermiform appendix to repair long ureteral defects provides an additional argument against incidental appendectomy.

Anastomosis, Surgical↗

A new technique of using the in situ appendix as a catheterizable stoma in continent urinary reservoirs.

A new technique of using the in situ appendix to construct a continent catheterizable stoma is described in a patient who underwent continent urinary diversion, and the technical aspects of this procedure are illustrated in detail. The appendix is remodeled by invaginating its base into the cecum for 1 cm., cecoplicating the middle portion for 2 cm. and bringing the distal end to the skin as a cutaneous stoma. This new technique demonstrates that the in situ appendix can be constructed successfully to provide continence without the need for isolating it from the cecum and implanting the distal end into the urinary reservoir as described in the Mitrofanoff technique.

Adult↗

Serotonin-immunoreactive cells in the lamina propria plexus of the appendix.

The lamina propria of the human appendix possesses a recently defined ganglionated neural plexus with neurosecretory-type component cells. In order to elucidate the functional role of this plexus, immunohistochemical light and electron microscopic studies were performed on 21 appendixes. By light microscopy, 18 specimens showed within the lamina propria neuron-like and neurosecretory-type cells that stained positively with lead hematoxylin and were positive for serotonin. The number of these cells varied from specimen to specimen. No correlation between the number of serotonin-immunoreactive intraepithelial enterochromaffin cells and the number of plexus cells could be established. Electron microscopic examination revealed identical pleomorphic neurosecretory granules within enterochromaffin cells and plexus cells. The site of serotonin storage in these granules was revealed by electron immunocytochemical methods. It is suggested that, under physiologic conditions, the lamina propria plexus has a modulatory role between the epithelium and the deeper mural enteric nervous system mediated by serotonin neurotransmission. Uncontrolled release of serotonin by the plexus may initiate inflammation and elicit pain related to the appendix.

Adolescent↗

Crohn's disease isolated to the appendix: truths and fallacies.

Twenty-five cases of Crohn's disease confined to the appendix were reported in eight hospitals in Israel during a 15-year period. Review of the histologic slides confirmed the diagnosis in 22 cases. Re-evaluation of these 22 patients included physical examination and radiologic studies of the small and large bowel. Rectosigmoidoscopy was performed in 16 patients. Signs and symptoms of Crohn's disease at other sites in the gastrointestinal tract did not occur during follow-up periods of two to 15 years (mean, 6.4 years) after appendectomy. This study and a review of the literature indicate that in most cases (93 per cent) Crohn's disease initially limited to the appendix is not a predictor of subsequent involvement of another portion of the bowel. It is concluded that the so-called Crohn's disease isolated to the appendix is a form of chronic granulomatous and follicular appendicitis of unknown etiology that is unrelated nosologically in the majority of the cases to Crohn's disease proper.

Adolescent↗

Diverticulosis of the vermiform appendix in patients with cystic fibrosis.

This paper presents a study of diverticulosis of the vermiform appendix in patients with cystic fibrosis. The records of two hospitals were reviewed. A total of 39 autopsy specimens and 18 surgical specimens were obtained. Diverticulosis of the appendix was found in 14 per cent (eight of 57) of all appendices. Two of these cases also showed diverticulitis. These results stand in contrast to the incidence of diverticulosis of the appendix in the general population, which has been measured at 1 to 2 per cent in various reviews.

Adolescent↗

[Actinomycosis of the appendix. Report of two cases].

INTRODUCTION: We report two patients with actinomycosis of the appendix extending to the caecum and the ileum, and diagnosed postoperatively on histological analysis, the authors reviewed the literature. EXEGESIS: Actinomycosis of the appendix can be acute or chronic. Diagnosis may be obtained preoperatively on the analysis of aspiration or biopsy material under CT scan control. It is frequently done postoperatively on the analysis of surgical specimen. Actinomycosis can be treated with antibiotics only during six months if the diagnosis is made preoperative, by surgery followed by antibiotics during 6-12 months, according to the extension of the actinomycosis, if the diagnosis is made after surgery, and by a combination of a surgery and antibiotics in complex forms. CONCLUSION: Actinomycosis of the appendix is an infrequent pathology that merits to be known. With antibiotic therapy, we can avoid surgery or wide resections.

Actinomycosis↗

Double appendix.

A case of appendix duplication is described. A normal appendix was present in the usual position while a rudimentary second appendix was found to arise separately from the cecum.

Adult↗

[Primary adenocarcinoma of the appendix as differential diagnosis of advanced ovarian carcinoma].

Malignant tumours of the appendix are rare. They are usually carcinoid tumours that must be distinguished from extremely rare adenocarcinomas. Metastatic mucinous adenocarcinomas of the appendix are only reported as case histories. In clinical terms, the tumours usually manifest themselves as acute appendicitis, as ruptured appendicitis, as a tumour in the right lower abdominal quadrant or as a pelvic tumour, which are generally mistaken for an ovarian tumour with the same sonographic image. Advanced primary adenocarcinomas of the appendix with ovarian metastases cannot be distinguished intraoperatively from a FIGO III ovarian carcinoma. The pathologist makes the definitive diagnosis. These characteristics also apply to the case presented here. Surgical therapy of the isolated primary appendiceal carcinoma consists of a hemicolectomy--an appendectomy in favourable cases--and, in the case of a metastasised carcinoma, according to the guidelines for an advanced ovarian or colon carcinoma. The effect of chemotherapy is insufficiently documented.

Adenocarcinoma, Mucinous↗

Gangliocytic paraganglioma of the appendix.

AIMS: A case of gangliocytic paraganglioma is reported in the appendix which, to the best of our knowledge, is the first case at this particular site to be described in modern literature. METHODS AND RESULTS: A 47-year-old man with signs and symptoms of acute appendicitis underwent appendectomy. In the resected specimen a tumour with a diameter of 9 mm was found, which microscopically consisted of three different cell types: (a) epithelioid cells lying in a trabecular pattern and in formations reminiscent of 'Zellballen' as seen in paragangliomas (b) spindle cells and (c) ganglion-like cells. A diagnosis of 'gangliocytic paraganglioma' was made and confirmed by immunohistochemical and ultrastructural examination. CONCLUSION: Gangliocytic paragangliomas are rare tumours of uncertain histogenesis. More than 40 years ago a tumour in the appendix with features similar to our case was described by Masson as 'neuro-carcinoïde'. Concerning its origin, Masson, as well as other authors describing gangliocytic paragangliomas decades later, referred to the endodermal-neuroecto dermal complexes found by Van Campenhout. It is felt that the current finding of a gangliocytic paraganglioma in the appendix supports the hypothesis that gangliocytic paragangliomas arise from these embryonal structures.

Appendectomy↗

Primary epithelial neoplasms and other epithelial lesions of the appendix (excluding carcinoid tumors).

Epithelial tumors of the appendix range from low-grade mucosal-based tumors which, when confined to the appendix, have an excellent prognosis but, once outside the appendix, have a fair prognosis and often a prolonged disease course, to high grade invasive carcinomas that are rapidly fatal. Low grade mucinous neoplasms may rupture and spread to the peritoneum as pseudomyxoma peritonei, and the nomenclature of these tumors has been the subject of considerable disagreement among pathologists; the designation "low grade appendiceal mucinous neoplasm" has recently been proposed for reasons discussed herein. Demonstrating rupture of these neoplasms may require particularly diligent gross and microscopic evaluation as the rupture site often heals over leaving only subtle evidence of its presence. Invasive adenocarcinomas are often mucinous and may also spread to the peritoneum. Against this backdrop, the clinical and pathologic features of low grade appendiceal mucinous neoplasms and mucinous adenocarcinomas, as well as other types such as typical colorectal type and signet-ring cell type, are reviewed. In addition, emerging entities, serrated polyps and serrated adenomas, whose significance is only beginning to be understood, are considered. Retention cysts, hyperplastic polyps, and diffuse mucosal hyperplasia, although not necessarily neoplastic, are reviewed here as they may enter into the differential diagnosis of appendiceal mucinous neoplasms.

Appendiceal Neoplasms↗