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"What you see is not what you get". A plea to remove a 'normal' appendix during diagnostic laparoscopy.

BACKGROUND: Diagnostic laparoscopy (DL) is a well established alternative option to coeliotomy for suspected appendicitis. When a 'normal' appendix is found, appendectomy is often believe unnecessary. Little is known however about how normal a normal appearing appendix is. In this study we postulated that a normal appendix seen at DL, may show pathological indications at microscopy, and thus, to leave the appendix untouched may be unsafe. METHODS: A retrospective review of data from 48 patients which, in a five year period (1995-1999), had virtually normal appendices removed as completion of DL for lower abdominal pain. RESULTS: No procedure-related drawback and no subsequent complications were recorded. Symptoms subsided in all the patients. Mean hospital stay was 2.1 days. When receiving specimens, incidence of pathologic changes of the appendix were observed in 58 percent of the cases (n = 28). The negative predictive value of DL was 41 percent. CONCLUSIONS: Due to the consistently false negative rate of DL, and the low morbidity rate for laparoscopic appendectomy, we support incidental appendectomy in patients with lower abdominal pain.

Abdominal Pain↗

Intussusception of the appendix. A report of four cases and review of the literature.

The clinical and pathologic features of four cases of intussusception of the appendix are reported and the literature is reviewed. All patients had vague abdominal symptoms. The diagnosis of intussusception of the appendix was not made preoperatively in any of these cases. All four patients were females who ranged from 37 to 70 years of age (mean age, 46 years). Examination of the surgical specimens showed tow appendixes that had completely inverted, one with a polyp attached at the base of the appendix forming the intussusceptum and the other with inversion of the appendiceal tip. Three cases were associated with endometriosis and one with a tubulovillous adenoma. Radiologically and endoscopically, the intussuscepted appendix may mimic a neoplastic lesion. Since intussusception may be caused by both benign and malignant conditions, appropriate management will depend on the associated cause.

Adult↗

Diverticular disease of the appendix.

A retrospective study of diverticular disease of the appendix was made in 3,343 consecutive instances of appendectomies. A 2 per cent incidence of diverticular disease was found. These instances were classified into four morphologic types: 1, acute diverticulitis; 2, acute appendicitis with acute diverticulitis; 3, acute appendicitis with diverticulum, and 4, appendix with diverticulum. Types 1, 2 and 3 were divided into subgroups with or without perforation. The elements of clinical behavior in each group were examined in detail. Diverticulitis of the appendix is presented as a clinically variant form of the inflamed appendix. Some followed the pattern of typical acute appendicitis. However, most were distinctive at a later age of onset, longer interval of disease, fewer or absent symptoms of the gastrointestinal tract, failure of typical abdominal pain progression, delay in surgical treatment and a remarkably high incidence of perforation. In a chi-square analysis of 56 patients with acute diverticulitis of the appendix compared with 2,503 patients with acute appendicitis, more than a fourfold incidence of perforation in acute diverticulitis was significant beyond the 0.001 level. These findings of variant behavior and high incidence of perforation are cautionary features of this frequently overlooked disease.

Acute Disease↗

[The chronically altered appendix in children. Cooperative work of the Austrian Society for Pediatric Surgery].

In this multicentric study the anamnestic data and parents questionaires of 2-29 children were evaluated, whose appendix vermiformis at the time of operation showed no signs of inflammation. 31,1% macroscopically showed cicatrisation or fasciation, in 13,7% other enteral or gynaecological (1%) findings were present. In 1194 of the cases of patho-histological examination of the appendix had been made of which 36.4% were negative, 37.7% showed cicatrisation, 15,0% oxyuriasis and 10,4% coproliths. In the discussion of the significance of the chronically altered non-inflamed appendix we compared patients with and without macroscopical or microscopical alterations. We came to the conclusion that the probability-index as to sex, duration of symptoms, complexity of symptoms, incidence of postoperative well-being, proved the chronically altered appendix not to be an illness per se, but the result of spontaneously arrested inflammation. Since the rate of children admitted with a perforated appendix is high (15-20%) in comparison with the rate of complications after removal of non-inflamed appendices (2,9%), we believe that according to the diagnostical problems the principle can be maintained: in dubio pro operatione.

Adolescent↗

[Pathologic appendix findings in gynecologic operations].

We report our experiences on three cases with pathologic findings of the appendix vermiformis diagnosed during gynecological operations including mucocele, carcinoid and adenocarcinoma. Mucocele of the appendix is a descriptive term for an abnormal mucous accumulation distending the appendiceal lumen, which can be complicated by pseudomyxoma peritonei. Carcinoid tumours arise from the neuroendocrine system and present a characteristic morphologic picture. Predilicted sites are the appendix and the small bowel. Adenocarcinomas of the appendix that are not associated with mucoceles have a clinical course resembling large bowel cancer. Patients with pathological findings of the appendix may be asymptomatic or present with a palpable right lower quadrant mass and/or pain. We discuss differential diagnosis and present a spectrum of histologic findings.

Adult↗

A rare laparoscopic diagnosis in acute abdominal pain: torsion of epiploic appendix.

Torsion of an epiploic appendix is seldom diagnosed preoperatively because of the difficult differential diagnosis with other diseases causing acute abdominal pain, particularly appendicitis. The classic McBurney approach overlooks many infarcted epiploic appendixes. The use of diagnostic laparoscopy in acute abdominal pain can show an infarcted epiploic appendix to be at the origin of pain. Four patients were admitted for acute abdominal pain and moderate tenderness in lower quadrants and were operated on for suspected acute appendicitis. In all cases laparoscopy found the torsion of an epiploic appendix, which was resected, and a normal cecal appendix. Postoperative course was uneventful. In case of acute abdominal pain in the lower quadrants of suspected appendiceal origin, laparoscopy should be routinely performed not only in women but also in men to avoid overlooking other intraperitoneal diseases that can be the cause of symptoms.

Abdomen, Acute↗

The primary site of CD4- 8- B220+ alphabeta T cells in lpr mice: the appendix in normal mice.

There have been no reports on an abundance of CD4- 8- B220+ alphabeta T cells, seen in autoimmune mice carrying the lpr gene (abnormal Fas gene), in any immune organs of normal mice. We herein report, however, that such alphabeta T cells were abundant at intraepithelial sites of the appendix in normal mice. They lacked the expression of NK1.1 Ags (C57BL/6 mice), but had the morphology of granular lymphocytes and contained forbidden T cell clones in the minor lymphocyte-stimulating antigen (Mls) system (C3H/He mice with Mls-1b2a). In other words, many properties of intraepithelial T cells in the appendix resembled those ascribed to abnormal alphabeta T cells, which expand in the lymph nodes and spleen of lpr mice. In the case of lpr mice, CD4- 8- B220+ alphabeta T cells first expanded in the appendix and then extended to other organs. CD4- 8- B220+ alphabeta T cells seemed to originate in situ from c-kit+ stem cells in the appendix. These results suggest that the appendix is one of the primary sites in which CD4- 8- B220+ alphabeta T cells exist, and that these cells carry many primordial properties as prototype T cells.

Animals↗

Appendiceal mucinous cystadenoma presenting as "porcelain" appendix with myxoglobulosis--a rare cause of a right lower quadrant mass.

Mucinous cystadenoma is a rare tumour of the vermiform appendix and is associated with cystic dilatation of the appendix, to which the more general term of mucocoele has been applied. Mucocoele of the appendix is only a descriptive term for abnormal mucus accumulation causing distension of the appendiceal lumen, irrespective of the underlying cause. Pre-operative diagnosis of appendix mucocoele, though infrequently made, is important as some of these lesions may be malignant, and also is essential in order to avoid the risk of rupture at surgery with subsequent development of pseudomyxoma peritonei. The appearances of mucocoele of the appendix and its uncommon variant of myxoglobulosis on plain radiograph, ultrasound and barium study are presented, together with review of the literature.

Appendectomy↗

Epithelial noncarcinoid tumors and tumor-like lesions of the appendix. A clinicopathologic study of 184 patients with a multivariate analysis of prognostic factors.

BACKGROUND: The current understanding of appendiceal epithelial tumors is based on series composed of relatively small numbers of patients and on case reports. The aim of this study was to perform clinicopathologic correlation, particularly concerning prognosis, on a relatively large series of patients with epithelial tumors and tumor-like lesions of the human vermiform appendix. METHODS: One hundred eighty-four cases of simple mucoceles, hyperplastic polyps, adenomas, carcinomas, and mucinous tumors of uncertain malignant potential (UMP) were reviewed. Retrospective follow-up data were obtained in carcinoma and UMP tumor cases. RESULTS: Most neoplasms were of the mucinous type characteristic of the appendix, but a small fraction were nonmucinous and resembled neoplasms typical of the spectrum encountered in the colorectum. Both benign and malignant conditions in some cases showed acellular mucin dissecting the appendiceal wall and mucin outside the appendix. Immunostains were sometimes helpful in identifying epithelial cells within mucus, but hematoxylin and eosin and mucin stains were adequate in most cases. Two factors were significantly associated with survival by multivariate analysis: the presence of mucin outside the right lower quadrant of the abdomen and the presence of epithelial cells in the peritoneal cavity outside the appendix. CONCLUSIONS: The distribution of mucin within the abdomen and the presence of cells outside the visceral peritoneum of the appendix are important prognostic factors that should be recorded in these neoplasms. Tumors designated as UMP lesions behave in a benign or low grade fashion.

Adenocarcinoma↗

Primary adenocarcinoma of appendix, colonic type associated with perforating peritonitis in an elderly patient.

We report a case of colonic type adenocarcinoma of the appendix with perforating peritonitis in a 92-year-old man. The preoperative diagnosis was localized peritonitis due to acute appendicitis and emergency laparotomy was performed. A gray, hard tumor was palpated at the base of the appendix. Appendiceal cancer was suspected, and right hemicolectomy was performed. The histopathological diagnosis was moderately differentiated adenocarcinoma of the appendix. The tumor obstructed the orifice of the appendix, and this may have caused the perforation of the appendix. The patient had an uneventful postoperative course and there have been no signs of recurrence in the 2 years since the operation.

Adenocarcinoma↗

Androgen and oestrogen receptor status of the human appendix testis.

Since the human appendix testis is a Mullerian duct derivative it has been theorised that rising oestrogen levels in puberty result in enlargement of this organ and consequent predisposition to torsion. This study sought to establish the presence or absence of both androgen and oestrogen receptors in the human appendix testis. Bilateral appendix testis specimens surgically excised from ten patients undergoing scrotal exploration for acute scrotum were stained immunohistochemically for androgen and oestrogen receptors. These were examined by light microscopy. The human appendix testis was found to express both oestrogen and androgen receptors. Marked regional variation of androgen and oestrogen receptor positivity was demonstrated. The surface epithelium and some stromal cells were androgen receptor positive, whereas oestrogen receptors were confined to ductular invaginations, gland-like structures and some stromal cells. Rising levels of androgens and oestrogens in pubertal boys may account for enlargement and the predisposition of the human appendix testis for torsion since this structure contains receptors for both.

Adolescent↗

Leukaemia and lymphoma of the appendix presenting as acute appendicitis or acute abdomen. Four case reports with a review of the literature.

Leukaemic and lymphomatous infiltration of the appendix is rare and even rarer is acute appendicitis as the initial manifestation. From our routine biopsy material we collected four cases of haematological malignancies presenting as acute appendicitis or acute abdomen, caused or accompanied by tumoral infiltration of the appendix. Appendicitis was the initial manifestation that allowed diagnosis of the underlying disease. The clinical histories and histological examinations of the appendices and of one autopsy are described. We report the first detailed description of acute myeloid leukaemia involving the appendix, and three cases of lymphomatous infiltration of the appendix presenting with appendicitis, and give an overview of the literature. In these days of budgetary cuts in national health services, where one may be tempted not to have seemingly commonplace cases of appendicitis histologically verified, our cases emphasize that careful histopathological examination of all appendectomy specimens should be mandatory. Despite the fact that leukaemia and lymphoma of the appendix are rare, our cases illustrate that these must be included in the differential diagnosis of acute appendicitis and that physicians and surgeons have to be aware of these conditions.

Abdomen, Acute↗

Goblet cell carcinoid of the appendix endoscopically diagnosed and examined with p53 immunostaining.

We report a case of a 62-year-old woman with goblet cell carcinoid of the appendix. She was admitted to our hospital in September 1994 after the discovery of liver tumors. After admission, a tumor in the right kidney and multiple tumors in the liver were found. She was diagnosed with renal cell cancer and metastasis to the liver and underwent excision of the kidney and enucleation of the largest liver tumor. Histological examination revealed that the liver tumor was a metastatic carcinoid tumor. As carcinoid tumors have frequently been found in the appendix, endoscopic examination was performed and a lesion was found in the appendix by colonoscopy. As predicted, the biopsy specimen was a carcinoid tumor, and she underwent an appendectomy. Histologically, the tumor was a goblet cell carcinoid. Goblet cell carcinoid is a rather rare neoplasm that has the histologic features of both carcinoids and adenocarcinoma. Forty-two cases of goblet cell carcinoid of the appendix have been reported thus far in Japan. However, few were diagnosed via endoscopic examination before surgical operation. We also carried out an immunohistochemical study with anti p53 antibody on the goblet cell carcinoid tumor of the appendix. Most tumor cells were strongly positive, while in three benign carcinoid tumors investigated simultaneously they were negative. These findings suggest that goblet cell carcinoid has an aggressive phenotype compared with benign carcinoid tumors.

Appendiceal Neoplasms↗

Laparoscopic appendectomy for Crohn's disease of the appendix presenting as acute appendicitis.

BACKGROUND: Crohn's disease confined to the appendix is rare but has been well described in the literature. It can mimic acute appendicitis clinically. After surgical treatment, recurrences of Crohn's disease are rare. We report the first case of treatment by laparoscopic appendectomy of Crohn's disease confined to the appendix. METHODS: A healthy 32-year old man presented with a week-long history of vague lower abdominal pain. Diagnostic work-up, which included CT, enteroclysis, and routine blood work, revealed a patent appendiceal lumen with an inflammatory mass in the right lower quadrant. RESULTS: Diagnostic laparoscopy revealed an inflamed appendix, and a laparoscopic appendectomy was performed, with frozen-section examination revealing Crohn's disease of the appendix. Two years after surgery, the patient has not had a recurrence of symptoms. CONCLUSIONS: Crohn's disease of the appendix can mimic acute appendicitis, although often with a more indolent course. The disease may be treated successfully by laparoscopic appendectomy, with good long-term results.

Acute Disease↗

Appendiceal colic in childhood: the role of inspissated casts of stool within the appendix.

Seventy pediatric patients with histories of recurrent crampy abdominal pain, right lower quadrant tenderness, and proven filling defects or distention of the appendix on radiographs, have been subjected to appendectomy. Inspissated fecal material was found within the appendix in 72.8% of such children. Fecal material was found in only 12.9% of 155 normal appendices and in 33.8% of 530 acutely inflamed appendices. It is postulated that inspissated fecal material acts as a foreign body of the appendix, leading to intermittent obstruction and distention of the appendix--appendiceal colic. An 86% correlation between the appendiceal filling defects seen on radiographs and the inspissated fecal material found within the appendix at operation has been noted. Ninety-six per cent of these 70 children had been relieved of their recurrent abdominal pain by appendectomy. Appendiceal colic characterized by crampy abdominal pain, right lower quadrant tenderness, and appendiceal filling defects appears to be a veritable and verifiable diagnosis.

Abdomen, Acute↗

[Torsion of the appendix testis].

The differential diagnosis of an acute scrotum includes spermatic cord torsion, torsion of a testicular appendage, torsion of spermatocele, epididymitis, orchitis, trauma, hernia, testicular segmental infraction and tumor. Among these, torsion of appendix testis could got dramatic improvement if accurately diagnosed and treated. 5 patients of torsion of appendix testis have been identified in our hospital in the recent 5 years. Ages ranged from 9 to 13 years old (mean age 11). The lesions were on the right side in 2 cases and left side in 3 cases. Duration of scrotal pain ranged from 2 to 7 days (mean of 4 days). The urine analyses were normal in all cases. The white blood counts were all within normal limits with mean of 5.72 x 10(3)/ul. No abnormality in passing urine or other infectious sign could be detected. Doppler ultrasonography or nuclear medicine testicular scan were performed selectively due to clinical availability. The result was equivocal and did not support a definite diagnosis. All 5 cases received surgical treatment. The necrotic testicular appendix was excised and reactive hydrocele treated. All the patients were discharged from the hospital the day after operation and recovered quickly. The pathological report revealed congestion and extensive hemorrhagic necrosis of the testicular appendix. Advances in technology have been helpful in improving the accuracy of diagnosis but technology is not infallible and an over reliance on it can also result in misdiagnosis. Various reports supporting the use of scrotal ultrasound in evaluating cases of acute scrotum pain. Ultrasonography has definitely helped in detecting scrotal pathology but its limitations need to be appreciated. Because of the difficulty of making an accurate diagnosis in acute scrotum, misdiagnosis and delayed operation offer result in disappointment of testis salvage rates. We have supported a policy of early scrotal exploration in any case suspicious of torsion of appendix testis.

Child↗

[Association of carcinoid tumor of the appendix and Crohn disease (case report and review of the literature)].

The authors describe the coexistence of the carcinoid of the appendix and Crohn's disease. In the case of their woman patient the carcinoid was identified with the examination of the resected ileoascendent part of the bowel resulting of the complication of the Crohn's disease. 10 similar association is known in the literature but none of the patients had the signs of the carcinoid syndrome. Subsequent adrenerg syndrome after an alimentary hypoglycemia (increased evacuation of the cathecolamines and their metabolites in the urine), food allergy (increased IgE type antibody to milk protein) or gastroenteropancreatic (GEP) neuroendocrine tumor (based on the result of the 111In-octreoscan and the increased 5-hydroxyindolaceticaciduria) equally suspected of the symptoms (palpitation, flush) manifested after the operation. They look for the connection between the genesis of the intestinal carcinoid and the Crohn's disease with working up the bibliography. Summing up the references with a view of the latest it can be stated that the carcinoid of the appendix occurs more than orders of magnitude in the samples from inflamed surrounding than the samples from autopsy (0.24%--820/338,000 inflamed appendix and 0.03%--19/53,430 appendix from autopsy). Consequently the inflammation create favourable condition for the development of the carcinoid of the appendix.

Adult↗

Mucocele of the appendix with hematuria.

Mucocele of the appendix, a nonspecific and descriptive term for an abnormal mucous accumulation within the appendiceal lumen, regardless of the underlying cause, is a rare clinical disease that is not usual as a consideration in the differential diagnosis of right lower quadrant lesions. The reported prevalence of mucocele of the appendix in appendectomy specimens is 0.2-0.3 percent. Hematuria due to mucocele of the appendix is extremely rare. Up to now, only few cases of such condition have been recorded in the literature. We describe a woman who experienced intermittent episodes of right lower quadrant pain and hematuria. Abdominal exploration incidentally displayed mucocele of the appendix. No evidence of other lesions was found. The patient was still in good health after operation. This case highlights mucocele of the appendix as a consideration in the differential diagnosis of right lower quadrant pain with hematuria.

Aged↗