Extrahepatic portal vein aneurysm: a case report and review of the literature.

Portal vein aneurysm is a rare clinical entity with only 42 published cases in the English literature. We present a 30-yr old woman who was incidentally diagnosed with an extrahepatic portal vein aneurysm during the investigation for dyspepsia. Expectant management with regular follow-up and surveillance imaging was adopted. This pathology is increasingly encountered with the frequent use of radiological imaging modalities in the work-up of abdominal disorders. Etiology, clinical significance and management strategies for extrahepatic portal vein aneurysms are discussed.

Spontaneous aortic dissection and rupture in a patient with neurofibromatosis.

Vascular involvement in neurofibromatosis type I is a well-recognized but rare feature of the disease. This case report is the first such describing acute aortic dissection and rupture in a patient who also had incidental aneurysms of his carotid and iliac arteries. A review of the literature about pathogenesis and management strategies is also presented.

Arterial reconstruction for lower limb ischemia.

The treatment of advanced atherosclerosis involving the lower extremities has undergone considerable evolution over the last several decades. Current strategies hinge on an appreciation of the natural history of disease, the overall health status of the patient, and an armamentarium of endovascular and open surgical reconstructive techniques that may be tailored to optimize outcome for the individual patient. Patients with aorto-iliac disease have a variety of available options with generally good results. For infrainguinal disease, surgical bypass using autogenous vein is the mainstay of interventional therapy and will remain so for the foreseeable future. Increasing medical and surgical challenges are presented by this population, particularly as aggressive medical treatment of risk factors leads to an ongoing decline in cardiovascular mortality, combined with a dramatic increase in the prevalence of diabetes. The future, which is now already at hand, will bring the application of genomics, with a potential for altering the progression of disease as well as extending the long-term benefits of reconstruction.

Autogenous composite vein bypass graft for infrainguinal arterial reconstruction.

PURPOSE: Lower extremity arterial reconstruction in the absence of adequate greater saphenous vein remains a challenging problem in contemporary vascular practice. The purpose of this review is to evaluate the long-term results of autogenous composite vein grafts used for infrainguinal arterial bypass grafting. METHODS: We retrospectively evaluated a prospective vascular registry and reviewed inpatient and office records. RESULTS: From June 1983 to September 1999, 165 autogenous composite vein infrainguinal bypass grafts were performed in 154 patients (87 men, 67 women; mean age, 69 years). The mean follow-up was 25 months (range, 3-147). Patients had the usual risk factors, including a 30% incidence of prior coronary bypass grafting. Forty-eight percent of bypass grafts were performed after failed previous reconstructions, and 90% were performed for limb salvage. The conduits were comprised of 2 segments (75%), 3 segments (23%), and 4 segments (2%). The distal anastomosis was at the popliteal level in 17% and the tibial/pedal level in 83%. The 30-day operative mortality rate was 1.8%. Perioperative graft failure (< 30 days) occurred in 18 bypass grafts (11%), resulting in early amputation (< 30 days) in 1.2%. The overall 5-year cumulative patency rates were 44% +/- 5% for primary patency, 63% +/- 5% for primary-assisted patency (PAP), and 65% +/- 5% for secondary patency (SP). A high revision rate for stenosis or thrombosis was required during follow-up to maintain patency of the grafts (27%). Limb salvage was 81% +/- 5% at 5 years. Primary reconstructions with composite vein fared significantly better than secondary reconstructions (SP 76% vs 54% at 5 years, P <.01). Arm vein composites showed superior patency compared with greater saphenous vein composites (SP 79% vs 61% at 5 years, P <.05). CONCLUSIONS: Infrainguinal reconstruction with autogenous composite vein results in durable graft patency and limb salvage rates in patients with few alternatives for revascularization. Intensive graft surveillance with aggressive graft revision is necessary to achieve these results.

Duplication of the vermiform appendix.

In this brief report, the authors present a case of duplication of the vermiform appendix with appendicitis occurring in both appendices and causing small bowel obstruction.

Peritoneal mucinous carcinomatosis after laparoscopic-assisted anterior resection for early rectal cancer: report of a case.

Minimally invasive colon surgery has been shown to be both technically feasible and a safe alternative to laparotomy. Its efficacy for the curative resection of colorectal cancer, however, remains controversial. Of major concern are the increasing reports of port-site recurrence after use of laparoscopic techniques in malignant disease. In this article a heretofore unreported complication of peritoneal mucinous carcinomatosis after laparoscopic-assisted anterior resection for early stage rectal cancer is presented. Isolated peritoneal recurrence is rare after curative resection of Stage I rectal cancer. The effect of pneumoperitoneum on tumor dissemination is discussed.

Regional distribution in human of a novel aortic collagen-associated microfibrillar protein.

We have reported two hypothetical proteins of human aorta, based on sequences cloned from a cDNA library constructed from mRNAs purified from the adventitia. These sequences have immunoglobulin-kappa (IgK)-like domains, and we have shown that microfibrils of the aortic adventia are immunoreactive with antibodies against IgK. The present study was performed to characterize more specifically the regional distribution in human of one of these proteins in particular and the distribution of matrix proteins with IgK-like motifs in general. An antibody was raised in rabbit against a synthetic peptide based on a unique sequence in one of the hypothetical proteins (NPSNRVTPQKNFP), which has not been reported in the sequence of any other known protein. This antibody and a rabbit anti-human IgK antibody were used as first antibodies in the staining reactions. A monoclonal mouse anti-smooth muscle cell alpha-actin antibody was also used. Immunoreactivity with the sequence-specific antibody was limited to the aorta and large vessels. Adventitial microfibrillar staining was more conspicuous in the abdominal aorta than in the thoracic aorta and in the internal iliac than in the external iliac artery. The immunoreactive protein was associated with fibroblasts and not smooth muscle cells. Immunoreactivity coated the collagen fibers diffusely, while elastin fibers were not stained. Further studies using antibodies against IgK demonstrated immunoreactivity of collagen and fibroblasts in a variety of tissues: spleen, ovary, testicle, cervix, prostate, skin, and breast (but not brain). Immunoglobulin motifs may be a feature of matrix proteins produced by fibroblasts in many tissues, but the first motif that we identified from a cDNA library of aortic adventitia appears to be specific to aorta and large vessels.

Ollier's disease: varus angulation at the lower femur and its management.

Attention is drawn to the high incidence of varus angulation in the lower femur in Ollier's disease; eight of a total of 14 patients with this condition have this deformity. There may be retardation or arrest of the medial portion of the lower femoral growth plate. One case demonstrates a bone bridge, a condition not previously described in Ollier's disease. The limb-length inequality and varus angulation require concurrent management by a variety of techniques, which are described. Three of the eight patients have reached skeletal maturity; the remainder provide useful information on the condition and are a stimulus for discussion of future management.

Experience with the Whipple procedure (pancreaticoduodenectomy) in a university-affiliated community hospital.

BACKGROUND: The purpose of this report is to review the current standards of the Whipple pancreaticoduodenectomy and show that excellent results are achievable in a low-volume, university-affiliated community hospital. METHODS: A case series of consecutive patients operated on during the period November 1981 to June 1996 was evaluated retrospectively. Medical records were abstracted for demographic data, clinical presentation, comorbid factors, pathological diagnosis and staging, operative records, perioperative mortality, morbidity, and length of stay. Postoperative follow-up data were obtained from telephone interviews and from the primary referring physicians. RESULTS: A total of 29 patients underwent a pancreaticoduodenectomy procedure during this 15-year period. Twenty-eight patients underwent the standard Whipple resection and 1 patient underwent an extended resection owing to the extent of the disease. The average age was 64 years (range 41 to 82). Comorbid diseases were present in 59% of cases. Jaundice was the main presenting complaint (62%), loss of weight and appetite was present in 34%. The most common indication for this procedure was malignant periampullary disease (83% of cases). Of patients with adenocarcinoma of the pancreas, 67% were stage I and 33% were stage III. The operation lasted an average of 5.5 hours (range 3.5 to 8 h). The mean operative blood loss was 1153 mL (range 250 to 4,000). The median length of stay was 11 days (range 7 to 81). There was 1 operative mortality (3%), and the overall major morbidity rate was 28%. Three patients required reoperation (10%), 2 for intraabdominal hemorrhage and 1 for delayed gastric emptying. The major morbidity was hemorrhage at the gastrojejunostomy site (14%); 2 cases were intraabdominal and 2 were intraluminal. Pancreaticojejunostomy leak occurred in 1 patient, resulting in a localized intraabdominal abscess. Delayed gastric emptying, defined as the need for nasogastric suctioning for more than 10 days postoperatively, occurred in only 1 patient. Overall, an oral diet was tolerated after a median of 6 days (range 4 to 61). Seventy-two percent of patients had no major complications at all, 17% had one major complication, and 10% had two or more major complications. Pancreatic insufficiency was the major long-term complication, developing in about 50% of patients. There were no biliary strictures. The median survival for patients with carcinoma of the pancreas was 21 months and the 5-year survival was 15%. CONCLUSIONS: The above study demonstrates that a complicated procedure like the Whipple pancreaticoduodenectomy can be performed with excellent results in a community hospital. The most important prerequisite is that the surgeon be adequately trained in the procedure. In low-volume hospitals, the case load should be restricted to a minimal number of trained surgeons in order to concentrate the experience.

Three-dimensional modeling and molecular evolution of Matrix Cell Adhesion Molecule #2: MAT-CAM 2.

Our research group has recently reported the partial amino acid sequence of an aortic adventitial collagen-associated fibrillar protein, which may be the target of an autoimmune response in patients with abdommal aortic aneurysms (AAA). Its apparent molecular weight is approximately 40 kDa, so we have named it aneurysm-associated antigenic protein-40 kDa (AAAP-40). It has similarities to microfibril-associated glycoprotein-36 kDa (MAGP-36), which was reported by Kobayashi et al. to have a tissue distribution limited to the aorta in pig; unlike other microfibrillar proteins which appear to distribute ubiquitously throughout the body with elastin. Accordingly, if AAAP-40 is the human homolog of MAGP-36, it would explain how an autoimmune reaction against this protein might have consequences more or less limited to the aorta and its branches.

Primary omental torsion in children.

Primary torsion of the omentum is an unusual cause of an acute abdomen and commonly mimics acute appendicitis. The following report of four obese children is supportive of obesity as a predisposing factor. The paucity of gastrointestinal symptoms, anorexia, nausea, vomiting, and the relatively long duration of symptoms, may increase the index of suspicion. In the majority of cases, the diagnosis is made intraoperatively by digital exploration through the muscle-splitting incision. The torsed omentum is easily delivered through the same incision, and excision results in complete recovery.