Endoscopy in colonic volvulus.

Flexible fiberoptic gastrointestinal endoscopy has greatly simplified the diagnosis and treatment of colonic volvulus. The management of 39 patients with colonic volvulus treated over 9 years was reviewed. Five per cent were treated with rectal tube decompression alone, 23% were treated with either sigmoidoscopic or colonoscopic reduction, and 26% were treated exclusively with operation. Endoscopic reduction was attempted in nearly half of the patients in preparation for operation. Recurrent volvulus occurred in 57% of patients initially treated with endoscopic reduction alone. Sigmoidoscopic examination did not confirm the diagnosis in 24% of instances in which it was used, although colonoscopy was always diagnostic. The overall mortality rate was 8%, but increased to 25% in patients with gangrene of the colon. Three patients who later proved to have gangrene of the colon had a normal initial sigmoidoscopic examination. Two of these patients died of intra-abdominal sepsis from a perforated colon. In five patients an accurate endoscopic diagnosis of gangrene prompted immediate exploration. None of these patients died. Endoscopy is a safe and effective diagnostic tool for the initial evaluation of patients with suspected colon volvulus. In addition, endoscopy may result in therapeutic decompression and may provide visual assessment of the viability of the bowel mucosa, thus assisting in the timing of appropriate operative treatment.     

Somatostatinomas, PPomas, neurotensinomas

We have reviewed data pertinent to three tumor syndromes that derive from overproduction of three GEP peptide hormones. The clinical syndrome of somatostatin excess remains well defined with diabetes, diarrhea, steatorrhea being predominant features. With the availability of assays and increasing awareness, more cases are being diagnosed in the intestine and these differ somewhat in their presentation with cholecystitis, GI bleeding, or a mass as the cardinal features. An unusual association with MEN II pheochromacytoma and neurofibromatosis is emerging. PPomas remain enigmatic. Although diarrhea is a feature, these tumors are usually silent and present with hypatomegally, abdominal pain, and jaundice because of the large size and malignant nature. Neurotensinomas remain rare and truly difficult to separate from the symptom complex produced by VIP excess. Edema, hypotension, cyanosis and flushing should alert one to the possibility of a neurotensin-secreting tumor.     

Operative timing and patient survival following distal splenorenal shunt

The importance of "operative timing" in cirrhotic patients with variceal hemorrhage is often underemphasized. To evaluate the effects of immediate versus delayed selective portasystemic decompression on hepatic function, operative mortality, and long-term patient survival, we reviewed the records of 77 patients who underwent distal splenorenal shunts (DSRS) over a 14-year period. A hepatic risk status score was calculated at the time of the index bleed (HRS1) or presentation and again just prior to operation (HRS2). Variables analyzed included age, sex, prior bleeding episodes, time from index bleed to operation, transfusion requirements, and etiology of cirrhosis. Operative mortality rates for immediate versus delayed DSRS were 46.2 per cent and 17 per cent, respectively. HRS improved significantly in elective DSRS patients from 1.46 to 1.30. Predictors of HRS2 included HRS1 and time in days from the index bleed to operation. The most important predictor of early survival for all patients after elective DSRS was the HRS2; however, for patients who underwent elective DSRS and survived, HRS1 was a better predictor of length of survival than HRS2. No other variable analyzed accurately predicted survival. We conclude that HRS can be expected to improve with supportive inhospital therapy; improved HRS at the time of operation is associated with decreased operative mortality; and the extent of liver disease as determined by HRS1 appears to be the chief determinant of long-term patient survival.     

Liposarcoma of the lower extremity

Twenty patients with primary liposarcoma of the lower extremity were treated over a 25-year period. The adequacy of the initial surgical procedure and histological grade of malignancy both influenced survival rates. In addition local recurrence was noted only in patients who had undergone "inadequate" initial excision. Inguinal lymph nodes were uninvolved by tumor in all cases. In general, patients with liposarcomas of myxoid and/or round cell type survived for long periods of time. However, even patients with myxoid lesions occasionally exhibited evidence of early blood-borne metastases. This study suggests an important relationship between an inadequate primary resection, local recurrence, and eventual retroperitoneal spread. In the majority of cases, this may have resulted from failure to control the primary distal extremity tumor, with subsequent contiguous spread of metastases into the ipsilateral retroperitoneal space. If feasible, radical soft part resection should be performed as the primary surgical therapy of these neoplasms. Tumors contiguous to the knee or ankle joint should be treated by primary amputation. Failure to control local disease may result in blood-borne dissemination or local spread along musculoaponeurotic planes to involve proximal groin or retroperitoneal space.     

Enterooxyntin release from isolated perfused canine jejunum

A humoral factor may mediate the intestinal phase of gastric acid secretion. An ex vivo perfused segment of canine jejunum maintained by an oxygenated asanguinous physiologic perfusate was used to test for release of an enterooxyntin (EO) in response to balloon distention at 30 mm Hg for 15 min. Gastric acid secretion in guinea pig fundic mucosa was determined indirectly by a quantitative cytochemical bioassay (CBA) of oxyntic cell hydroxyl ion production (HIP). An increase in the optical density (OD) caused by the cytochemical stain in the oxyntic cells reflects HIP, an index of acid secretion. Basal OD for segments with distention was 16.6 ± 0.53 and for those without 15.5 ± 0.68 (NS). Results are expressed as mean change of OD from basal (mean ΔOD ± SEM).

1.

		Time (min)
	10	30	60	120
Mean ΔOD, distention	4.9 ± 1.61	5.9 ± 1.11	7.2 ± 1.21	5.2 ± 0.91
Mean ΔOD, no distention	0.6 ± 1.0	0.1 ± 0.8	0.58 ± 1.0	0.7 ± 1.2

1

Significant stimulation of HIP ($\text{P < 0.02}$) by EO.

EO caused greater stimulation of HIP than gastrin or histamine. EO was heat stable. Trichloroacetic acid treatment decreased EO activity as did pronase digestion suggesting that EO is composed of one or more peptides. Conclusion: EO, an acid secretagogue, is a humoral agent probably composed of one or more peptides and is released by small bowel distention. Mechanical distention of the small bowel may be an important mechanism for the perpetuation of gastric acid secretion. The ex vivo perfused jejunal segment in conjunction with the CBA are ideal tools with which to study mechanisms of release of EO and the mechanism of action of EO on the oxyntic cell.     

Induction of immune tolerance in patients with hemophilia A and inhibitors treated with porcine VIIIC by home therapy

Home therapy with porcine factor VIIIC was safe and effective when administered to five hemophilic patients over periods of 8 1/2, 6, 4, 3 1/2, and 2 years. No significant transfusion reactions occurred. Before treatment with porcine factor VIIIC, all five had high-level, high- responding anti-human VIIIC inhibitors initially lacking anti-porcine factor VIIIC activity. Although specific anti-porcine VIIIC inhibitors arose in all patients, these were generally transient, and only one patient became refractory to treatment. We believe that porcine factor VIIIC is the treatment of choice in patients whose inhibitors do not cross-react. All five patients lost their original anti-human VIIIC inhibitors after starting treatment with porcine VIIIC, permitting the reintroduction of human VIIIC in three of them. There has been no recurrence of anti-human VIIIC inhibitor activity during 2 to 3 years of regular treatment with human VIIIC in these patients. This suggests that tolerance to human VIIIC has arisen as a result of treatment with porcine VIIIC. Porcine VIIIC may have a role in the desensitization of some factor VIIIC inhibitor patients.     

Diagnostic dilemmas in patients with cystic neoplasms of the pancreas

Summary Cystic neoplasms of the pancreas (CNP) are rare lesions that can be difficult to diagnose preoperatively. Twenty patients with cystic neoplasms of the pancreas including five microcystic adenomas, six benign mucinous cystic neoplasms, three malignant mucinous cystic neoplasms, two solid and papillary epithelial neoplasms, and four cystic neuroendocrine tumors were treated at a single institution between 1962 and 1987. The average duration of symptoms prior to diagnosis was 10 months. Five patients were asymptomatic. Forty percent of patients presented with an abdominal mass. Plain abdominal x-rays and UGI barium contrast studies were never diagnostic. Ultrasonography, computerized tomography (CT) and visceral angiography aided in the correct diagnosis in 28%, 36%, and 75% of patients studied, respectively. Overall a correct diagnosis was made preoperatively in only 35% of patients. Twelve of 13 patients were correctly diagnosed at laparotomy with intraoperative biopsy. Without biopsy the mass was misdiagnosed at laparotomy in five of six cases. CNP must be suspected inany patients who present with an upper abdominal mass with or without abdominal pain and no history of pancreatitis. CT may be diagnostic in up to one third of cases and should be obtained routinely to demonstrate the proximity of the lesion to other structures. Visceral angiography should also be obtained prior to operation. A generous incisional biopsy should be obtained of all pancreatic cysts that are not to be resected.     

Locus assignment of two alpha-globin structural mutants from the Caribbean basin: alpha Fort de France (alpha 45 Arg) and alpha Spanish Town (alpha 27 Val)

Two alpha-globin structural mutants were mapped to their encoding loci by in vitro translation of hybrid-selected alpha 1- and alpha 2-globin mRNA. The more highly expressed mutant, alpha Spanish Town (alpha 27Val), is encoded at the alpha 2 locus and the less expressed mutant, alpha Fort de France (alpha 45Arg), is encoded at the alpha 1 locus. These results further define the distribution of alpha-globin structural mutations within the alpha-globin gene cluster and substantiate the dominant role of the alpha 2-globin locus in alpha- globin expression.