Biomedical subjects

D G Jagelman

Publications and source records attributed to D G Jagelman.

At least 37 records · Page 2Linked to original sources

PubMed1992

Noncytotoxic drug therapy for intra-abdominal desmoid tumor in patients with familial adenomatous polyposis.

Forty of 416 patients with familial adenomatous polyposis were noted to have intra-abdominal desmoid tumors, and a subgroup of 16 were treated with noncytotoxic drug therapy. Drugs used were sulindac (14 patients), sulindac plus tamoxifen (3 patients), indomethacin (4 patients), tamoxifen (4 patients), progesterone (DEPO-PROVERA; Upjohn Co., Kalamazoo, MI) (2 patients), and testolactone (1 patient). Therapy with these drugs for continuous periods of six months or more resulted in three complete and seven partial remissions. When treated patients were compared with untreated patients (n = 12), there were significant benefits for the treated group, both in reduction of desmoid size and in improvement of symptoms, despite the inherent selection bias against this. Sulindac was the only drug used in enough patients to permit independent evaluation of its effect, with one complete and seven partial reductions of tumor size. Some patients had a delayed response to sulindac, with tumor shrinkage occurring after an initial period of tumor enlargement. When using sulindac for the treatment of desmoid tumors, this phenomenon should be considered.

Abdominal Neoplasms↗

PubMed1992

Laparoscopic total abdominal colectomy. A prospective trial.

The aim of this study was to prospectively assess the impact of laparoscopy upon the outcome of total abdominal colectomy (TAC). Specifically, patients underwent standard laparotomy with TAC and ileoproctostomy (TAC + IP), TAC and ileoanal reservoir (TAC + IAR), laparoscopically assisted TAC + IP (L-TAC + IP), or laparoscopically assisted TAC + IAR (L-TAC + IAR). Parameters studied included the length of surgery, length of ileus, length of hospitalization, morbidity, and mortality. Five patients underwent standard TAC (Group I), and five underwent L-TAC (Group II). Group I consisted of five patients of a mean age of 32 (range, 24-51) years who had mucosal ulcerative colitis (n = 1), familial adenomatous polyposis (n = 3), or colonic inertia (n = 1). Group II consisted of five patients of a mean age of 33 (range, 17-43) years who had mucosal ulcerative colitis (n = 1), familial adenomatous polyposis (n = 3), or colonic inertia (n = 1). This preliminary prospective study indicates that laparoscopically assisted TAC is feasible. L-TAC resulted in a slightly longer length of ileus and length of hospitalization; these differences were not statistically significant. Moreover, the length of time required for the laparoscopic procedures was 35 percent longer than for the open procedures. Although these results may improve as more cases are performed, dramatic differences in rates of postoperative recovery have not yet been realized. In conclusion, L-TAC, while technically feasible, dose not appear to offer any immediately recognizable benefits to the patient as compared with standard laparotomy.

Adolescent↗

PubMed1992

Gastroduodenal polyps in patients with familial adenomatous polyposis.

A review of the endoscopy reports and pathology results from esophagogastroduodenoscopy (EGD) of all patients with familial adenomatous polyposis (FAP) undergoing such an examination was performed. Two hundred forty-seven patients were identified, with an overall prevalence of duodenal adenomas of 66 percent and of fundic gland polyps of 61 percent. Analysis of our more recent experience (1986 to 1990) shows the prevalence to be 88 percent and 84 percent, respectively. A normal-appearing papilla was adenomatous in 50 percent of cases. No case of periampullary carcinoma developed in patients under surveillance. Routine EGD is indicated for patients with FAP. Duodenal adenomas and fundic gland polyps will occur in the majority of patients.

Adenomatous Polyposis Coli↗

PubMed1992

Retained mucosa after double-stapled ileal reservoir and ileoanal anastomosis.

A study was undertaken to assess the incidence of inflammation and dysplasia in retained mucosa after double-stapled ileoanal reservoir (IAR) for mucosal ulcerative colitis (MUC). Between September 1988 and February 1992, 56 patients with MUC underwent an IAR. Forty-five patients had a double-stapled IAR (DS-IAR), seven patients had a transanal pursestring stapled IAR (PS-IAR), and four patients had a PS-IAR with mucosectomy. Distal donuts obtained from the stapled IAR were submitted for pathologic review in 55 patients. Nine patients had only small bowel, connective tissue, and/or muscle noted on review. Mucosa was qualified as squamous epithelium (SE), transitional epithelium (TE), or columnar epithelium (CE). All samples were examined for evidence of inflammation and dysplasia. Four patients had SE only, one patient had TE, and 18 had CE. In addition, three patients had SE and CE, seven patients had SE and TE, two patients had CE and TE, and nine patients had all three types. The distance from the dentate line to the anastomosis ranged from 0 to 2.5 cm (mean, 1 cm). In 19 patients (35 percent), the distal donut revealed MUC. Of these 19 patients, six had persistent MUC (43 percent) at the time of subsequent biopsy. An additional four patients had MUC evident on follow-up biopsy but not on distal donuts; two of these four patients had no mucosa in their distal donuts. Only one of the patients with evidence of MUC on donuts and/or biopsy experienced any symptoms referable to active MUC (1.8 percent). None of the specimens examined had any evidence of dysplasia. In 31 patients, no MUC was present in the initial donuts or follow-up biopsies. Although the double-stapled technique appears safe, periodic monitoring is suggested.

Adolescent↗

PubMed1992

Retinal pigment epithelium lesions as a biomarker of disease in patients with familial adenomatous polyposis. A follow-up report.

BACKGROUND: The sensitivity of retinal pigment epithelium (RPE) lesions as a predictive congenital marker for the development of familial adenomatous polyposis (FAP) is evaluated. METHODS: In a prospective study, 34 patients at 50% risk of inheriting FAP were examined. Based on the presence or absence of four or more RPE lesions, patients were categorized as those who had inherited or those who lacked the FAP genes. All patients received dilated fundus examinations with binocular indirect ophthalmoscopy and all RPE lesions were documented with fundus photography. All patients underwent annual sigmoidoscopy to determine the presence or absence of polyps. RESULTS: A 3-year follow-up analysis showed that 8 of 14 patients who were positive for RPE lesions later developed polyps. Of the 20 patients considered negative for FAP based on normal fundus examination, none has developed polyps. CONCLUSION: The authors urge all patient at risk of inheriting FAP to undergo dilated fundus examination with binocular indirect ophthalmoscopy and wide-angle fundus photography at the earliest age possible. All patients with 4 or more RPE lesions should undergo annual sigmoidoscopic examinations beginning before 10 years of age.

Adenomatous Polyposis Coli↗

PubMed1991

Colectomy for constipation: physiologic investigation is the key to success.

The results of total abdominal colectomy (TAC) with ileorectal anastomosis as a treatment for colonic inertia (CI) were prospectively assessed. One hundred sixty-three patients were evaluated for chronic constipation between July 1988 and November 1990. Patients underwent pancolonic transit times, anorectal manometry, cinedefecography (CD), and electromyography (EMG). CI was defined as diffuse marker delay on transit study without evidence of puborectalis contraction on CD or EMG. Sixteen patients (10 percent; 15 females and 1 male) with a mean age of 45 years (range, 24-75 years) with CI underwent TAC. Preoperative bowel frequency ranged from three per week to one per month; all 16 patients evacuated only with high doses of laxatives, enemas, or both. TAC was performed with no postoperative mortality or major morbidity; three patients were readmitted four times for successful conservative treatment of partial small bowel obstruction. At a mean follow-up of 15 months (range, 2-35 months), these 16 patients reported a mean frequency of spontaneous bowel evacuations of 3.5 per day (range, one to six per day). Patient satisfaction with the operation was "excellent" or "good" in 15 cases (94 percent). Thorough preoperative physiologic evaluation permits the selection of a small group of patients with CI who may benefit tremendously from TAC.

Adult↗

PubMed1991

Neurophysiologic assessment of the anal sphincters.

One hundred twenty consecutive patients with either fecal incontinence (60 patients), chronic constipation (41 patients), or idiopathic intractable pelvic pain (19 patients) were prospectively assessed. Patients underwent concentric needle electromyography (EMG), bilateral pudendal nerve terminal motor latency evaluation, anorectal manometry, and cinedefecography. The most common EMG finding in patients with fecal incontinence was decreased recruitment of motor units with squeezing and polyphasic motor unit potentials; these are consistent with an injury pattern. The most common EMG finding in the constipated patients was paradoxical puborectalis contraction. This latter abnormality was also a frequent finding in patients with rectal pain, as was prolongation of pudendal nerve latency. Paradoxical puborectalis contraction was diagnosed more frequently with EMG than with cinedefecography. Inter-examination correlation was best in the incontinent group between EMG and manometry. Cinedefecography had poor correlation with EMG in all patient groups but was valuable in the detection of additional pathology such as rectoanal intussusception and anterior rectocele. Electromyography including pudendal nerve terminal motor latency assessment is a valuable adjunct in the evaluation of disorders of evacuation. The information it yields is complementary to that offered by more routine physiologic examinations.

Anal Canal↗

PubMed1991

Does metoclopramide reduce the length of ileus after colorectal surgery? A prospective randomized trial.

Between August 1988 and September 1989, 100 consecutive patients who underwent elective abdominal colorectal surgical procedures were prospectively randomized to receive or not to receive metoclopramide. Metoclopramide was intravenously administered every 8 hours from the completion of surgery until a solid food diet was able to be tolerated. Seven patients were not included in the final tabulations because of one death, one small bowel obstruction requiring laparotomy, one anastomotic leak requiring laparotomy, and four protocol violations. Ninety-three patients, 37 men and 56 women (mean age, 59.5; range, 14-89 years) underwent 40 segmental colectomies, 13 total abdominal colectomies, 8 abdominoperineal resections, 8 ileoanal pouch procedures, 7 small bowel resections, and 17 other colorectal procedures. The 40 patients who received postoperative metoclopramide were in Group 1, and the 53 who did not were in Group 2. The mean length of time between laparotomy and commencement of oral fluid and oral solid intake in Groups 1 and 2 were 3.5 and 4.8 days, and 3.5 and 5.0 days, respectively. These differences were not statistically significant (P greater than 0.05). Prolonged ileus was seen in seven patients in Group 1 and in eight patients in Group 2. These differences were also not statistically significant (P greater than 0.05). Prolonged ileus was defined as the need for nasogastric tube reinsertion or discontinuation of oral intake. We conclude that metoclopramide does not significantly alter the course of postoperative ileus.

Adolescent↗

PubMed1991

The double-stapled ileal reservoir and ileoanal anastomosis. A prospective review of sphincter function and clinical outcome.

Fifteen consecutive patients (nine males and six females) who underwent construction of a double-stapled ileoanal reservoir (DS-IAR) were prospectively evaluated. Mean and maximal resting pressures preoperatively, before ileostomy closure, and at 12 months, were 53 and 84 mm Hg, 39 and 62 mm Hg, and 62 and 81 mm Hg. Mean and maximal squeeze pressures at those same time periods were 96 and 153 mm Hg, 111 and 173 mm Hg, and 95 and 168 mm Hg. There were no significant decreases in either resting or squeeze pressure between preoperative values and those obtained 12 months after surgery. However, the length of the high pressure zone decreased from 3.8 cm preoperatively to 2.3 cm at 12 months. This reflects the sacrifice of the cephalad 1.5 cm of the internal anal sphincter necessary to effect this anastomosis at a mean of 1.4 cm from the dentate line. However, this maneuver did not result in poor continence. Eleven patients whose ileostomies were closed for a mean of 9 months, ranging from 3 to 15 months, were evaluated regarding functional outcome. Only one patient had any incontinence and this patient had incomplete circular-stapled tissue rings, which necessitated transanal suture repair of the anastomotic defect. Similarly, three of the four patients who sometimes or rarely use a pad at night had transanal-suture reinforcement. Ten of the 11 patients never wear a pad during the day. No pelvic or perianal sepsis occurred. Stratified squamous epithelium was found in 6 of the 13 distal stapler "donuts" that were examined. In addition, 10 patients underwent biopsy of the tissue immediately caudad to the circular staple line at the time of ileostomy closure; in five, only stratified squamous epithelium was noted. The DS-IAR is associated with excellent objective physiologic and subjective functional results.

Adenomatous Polyposis Coli↗

PubMed1991

Systemic cytotoxic chemotherapy and radiation therapy for desmoid in familial adenomatous polyposis.

Forty-two of 416 familial adenomatous polyposis (FAP) patients in the FAP registry at the Cleveland Clinic had desmoid tumors. The role of cytotoxic chemotherapy and radiation therapy in the management of these patients was investigated. Eight intra-abdominal desmoid tumors were treated by systemic cytotoxic chemotherapy. Two had complete remission, and one had partial remission. Five patients died as a result of the desmoid tumor or late complications of chemotherapy. Three intra-abdominal desmoids were treated by radiation therapy with no response. Neither cytotoxic chemotherapy nor radiation therapy is recommended as a first-choice treatment for intra-abdominal desmoid tumors in patients with FAP.

Adenomatous Polyposis Coli↗

PubMed1991

The role of sphincteroplasty for fecal incontinence reevaluated: a prospective physiologic and functional review.

Sixteen female patients (mean age 54.1 years; range 34-74 years) with a 9.8-year (range 1-25 years) history of incontinence to solid stool underwent overlapping sphincteroplasty with internal sphincter imbrication without fecal diversion. All patients were prospectively evaluated with preoperative anorectal manometry, electromyography, and pudendal nerve motor latency assessment, postoperative anorectal manometry, and preoperative and postoperative functional evaluation. Mean and maximal resting pressures increased from 30 mm Hg and 49 mm Hg preoperatively to 40 mm Hg and 57 mm Hg, respectively, postoperatively. Likewise, mean and maximal squeeze pressures increased from 27 mm Hg and 48 mm Hg preoperatively to 39 mm Hg and 73 mm Hg, respectively, postoperatively (P less than 0.01). Furthermore, anal canal high pressure zone length was increased by sphincteroplasty from a mean of 0.9 cm (range 0-3 cm) to a mean of 2.1 cm (range 1-4 cm). These objective physiologic improvements correlated well with subjective functional improvement. Subjectively, functional outcome was rated by patients as excellent in 38 percent, good in 38 percent, fair in 19 percent, and poor in only 5 percent of cases. Overlapping sphincteroplasty with internal sphincter imbrication improves both the anal sphincter physiologic profile and fecal continence.

Adult↗

PubMed1991

Ileorectal anastomosis--familial adenomatous polyposis.

Familial adenomatous polyposis is a generalized growth disorder. The predominant cause of death is, however, colorectal cancer in the untreated patient. Prophylactic colectomy, whether it be proctocolectomy and ileostomy, colectomy with ileorectal anastomosis, or colectomy with an ileoanal pouch procedure, will dramatically reduce the mortality from large bowel cancer. It is important, however, to realize that because of the generalized nature of this condition and the possibility of developing extracolonic malignancy, the surgical approach chosen should not be considered one that will necessarily cure the disease. Colectomy with ileorectal anastomosis still leaves the potential for rectal cancer. However, death from rectal cancer is distinctly uncommon. The risk of death following ileorectal anastomosis from rectal cancer is actually less than the risk of dying from cancer of the duodenum or from desmoid tumors. Both of these lesions are unpredictable in patients with familial adenomatous polyposis, and usually occur following the patient's prophylactic colectomy. With this low incidence of death from rectal cancer following ileorectal anastomosis it would seem to be debatable whether the complexities of the ileoanal pouch procedure together with its longer recovery and higher complication rate are justified in the routine management of young people with an early diagnosis of familial adenomatous polyposis.

Adenomatous Polyposis Coli↗

PubMed1991

Extra-colonic manifestations of familial adenomatous polyposis.

Familial adenomatous polyposis (FAP) is a genetic disease created by an abnormality of chromosome number five resulting in a generalized cellular growth disorder. The growth abnormality created by the genetic defect dictates the development of benign and malignant lesions in different organs of the body. Even the term familial adenomatous polyposis does not fully describe the defect because lesions other than polyps may occur in these patients. The major organ affected is the large intestine. Adenomas occur at an earlier age than normally seen in the general population, grow more numerously and more rapidly, and become malignant at an inordinately young age.

Adenomatous Polyposis Coli↗

PubMed1990

Evaluation of polymorphic genetic markers for linkage to the familial adenomatous polyposis locus on chromosome 5.

A gene associated with the inherited syndrome, familial adenomatous polyposis (FAP), has been localized to the long arm of chromosome 5 near the 5q21-22 region, and markers that identify genetic polymorphisms near this locus are now available. The authors evaluated several of these markers for linkage to the FAP trait in 11 families entered in the Cleveland Clinic Polyposis Registry. The original probe that established linkage to the FAP locus (C11p11) has limited utility for family studies because of low heterozygosity and distance from the FAP gene. Other probes, however, should be useful for assessing FAP inheritance by restriction fragment length polymorphism analysis, for presymptomatic diagnosis of the disease.

Adenomatous Polyposis Coli↗

PubMed1990

Spontaneous mutation in familial adenomatous polyposis.

A retrospective review of the familial adenomatous polyposis registry at the Cleveland Clinic Foundation revealed an incidence of spontaneous mutation in familial adenomatous polyposis (FAP) of 22 percent of family kindreds. These patients were reviewed retrospectively and compared with the total FAP population followed at The Cleveland Clinic Foundation with respect to the onset of disease, the incidence of carcinoma in the resected colon, and incidence of extracolonic manifestations. Review of the characteristics and presentations of these patients suggested that these individuals may harbor a more severe form of FAP. This may be due, in part, to the delay in diagnosis and, therefore, a higher rate of development of colorectal carcinoma and possibly duodenal adenomas. There is also a demonstrable higher rate of extracolonic manifestations of FAP present in this subset of patients. When selecting the initial type of prophylactic colonic resection the surgeon should bear in mind the increased incidence of extracolonic manifestations of the disease in this group of patients and their potential for complications.

Adenomatous Polyposis Coli↗

PubMed1990

Predictive value of congenital hypertrophy of the retinal pigment epithelium as a clinical marker for familial adenomatous polyposis.

One hundred forty-eight members of 53 kindreds with familial adenomatous polyposis (FAP) were examined for congenital hypertrophy of the retinal pigment epithelium (CHRPE) and extracolonic manifestations (ECM) to assess the value of CHRPE as a predictive marker for FAP. Based on eye examination results, the families were divided into 2 groups. In a first group of 34 families, all 61 members diagnosed as having polyps and 13 of the 33 patients at risk had 4 or more lesions distributed in both eyes. By contrast, in a second group of 18 families, all 32 polyposis patients and all 18 members at risk had less than 4 lesions. Extra-colonic manifestations were present in 26 of 34 families in the first group and in 11 of 18 families in the second group. Data on one family with ambiguous ancestry were reviewed separately. The existence of 4 or more CHRPE lesions distributed in both eyes seems to be a congenital marker for FAP, present in 65.4 percent of families. When present in a family: 1) it is found in all diagnosed patients in that family, 2) can therefore be considered predictive for the development of polyps in other family members who carry the trait, and 3) if confirmed by longer follow-up, may possibly preclude members without the trait from further evaluation and surveillance.

Adenomatous Polyposis Coli↗

PubMed1990

Mortality in patients with familial adenomatous polyposis.

The authors identified 132 patients who died with a documented diagnosis of familial adenomatous polyposis (FAP). A review of the medical records, autopsy reports, and in-depth discussion with local physicians and well-informed family members was performed. It was impossible, even after the review, to ascertain the exact cause of death in 22 patients. In the remaining patients, the cause of death was as follows: metastatic colorectal carcinoma, 64 patients (58.2 percent), (colon, 49 [44.5 percent], rectal, 15 [13.6 percent]); desmoid tumors, 12 (10.9 percent); periampullary carcinoma, 9 (8.2 percent); brain tumors, 8 (7.3 percent); perioperative mortalities, 5 (4.5 percent); adrenal carcinoma, 1 (0.9 percent); and abdominal carcinomatosis, 1 (0.9 percent). Ten patients died of causes not related to FAP. The major causes of death in 36 patients who underwent prophylactic colectomy were desmoid tumor and periampullary malignancy. This finding underscores the importance of lifelong surveillance and periodic endoscopic evaluation in patients with FAP.

Abdominal Neoplasms↗

PubMed1990

Surgical alternatives for ulcerative colitis.

There is no medical cure of ulcerative colitis except for the removal of the entire large intestine, or at least its mucosa. It is unknown as to what percentage of patients with ulcerative colitis present for surgical correction, and the potential for surgery in turn usually is related to the extent of large intestine involved. This article explores new alternatives that are available so that proctocolectomy and ileostomy are no longer the sole option for patients with ulcerative colitis.

Anastomosis, Surgical↗