Generalized Pustular Psoriasis (Von Zumbusch Type) Following Renal Transplant: A Case Report.

Generalized pustular psoriasis appears as an uncommon variant form of psoriasis consisting of widespread pustules on an erythematous background (von Zumbusch). A 39-year-old male patient with a history of plaque psoriasis since the age of 9 who had an acute relapse of generalized pustular psoriasis 12 days following a successful renal transplantation is presented. Despite administered immunosuppression for transplantation, the addition of cyclosporine and methotrexate did not reverse the ongoing process of disease and the patient died on the 57th posttransplant day due to multiorgan failure subsequent to severe bone marrow suppression.

Successful Living-Related Renal Allograft in a Recipient With Factor V Leiden Deficiency: A Case Report.

Thrombophilia due to activated protein C resistance (Leiden mutation) is the most common inherited thrombophilic disorder with 5% incidence in whites. Renal transplant of these patients entails a risk of vascular thrombosis soon after the transplant; and acute rejection episodes and graft loss within the first year. We present a case of a successful living-related renal transplant in man with a recent history of repeat episodes of vascular access thrombosis attributed to inherited thrombophilia (heterozygosity for factor V mutation Q506 and homozygosity for mutation T677 for methylene-tetrahydrofolate reductase). Transplant recipient was administered anticoagulation therapy with low molecular weight heparin pre- and postoperatively. No thrombotic or hemorrhagic events occurred posttransplant. A high suspicion of thrombophilic disorders in patients with end-stage renal disease with vascular access thrombotic events should be screened further to prevent failure of a subsequent renal transplant. Inherited thrombophilic disorders may not exclude living-related kidney transplant provided that anticoagulation therapy is admin-istered perioperatively.

Fournier's gangrene due to perioperative iatrogenic colon perforation in a renal transplant recipient.

Fournier's gangrene is not a common cause of morbidity in renal transplant recipients, but, if it occurs, it is difficult to treat because of the immunosuppression and associated increased mortality rate. We describe the case of a male patient who underwent renal transplantation with complicated post-operative course, resulting in cecum perforation (thermal injury due to cautery use during transplantation) requiring exploratory laparotomy and cecostomy. A few days later, he developed Fournier's gangrene and urgent radical surgical debridement of the scrotum was performed, along with aggressive antibiotic regimen and the immunosuppressive treatment was modified. Subsequently, the patient underwent scheduled cecostomy closure (right hemicolectomy), while the scrotum trauma healed with tertiary intention. Epidemiologic characteristics, clinical presentation, diagnostic workup, therapeutic options and morbidity-mortality rates of Fournier's gangrene are reviewed, emphasizing the role of immunosuppression in renal transplant recipients to disease development.

Successful endovascular treatment of transplant intrarenal artery stenosis in renal transplant recipients: Two case reports.

Transplant renal artery stenosis (TRAS) is a relatively rare complication after renal transplantation. The site of the surgical anastomosis is most commonly involved, but sites both proximal and distal to the anastomosis may occur, as well. Angioplasty is the gold standard for the treatment of the stenosis, especially for intrarenal lesions. We report two cases of intrarenal TRAS and successful management with angioplasty without stent placement. Both patients were male, 44 and 55 years old respectively, and they presented with elevated blood pressure or serum creatinine within three months after transplantation. Subsequently, they have undergone angioplasty balloon dilatation with normalization of blood pressure and serum creatinine returning to baseline level. Percutaneous transluminal balloon renal angioplasty is a safe and effective method for the treatment of the intrarenal TRAS.

Emergency surgery for obstructing colorectal malignancy: prognostic and risk factors.

PURPOSE: Emergency surgery for colorectal malignant obstruction is thought to correlate with poor outcome. The main aim of our study was to identify possible factors that could predict obstruction, and risk factors of poor postoperative outcome. The second aim was to determine any differences between primary anastomosis and stoma creation in the obstruction population, especially in left-sided tumors. METHODS: A retrospective review of 212 patients who underwent surgery for colorectal malignancy between January 2008 and January 2013 was performed. Fifty-five patients (26%) underwent emergency surgery for completely obstructing colorectal carcinoma, and 157 (74%) underwent elective surgery. RESULTS: The groups were comparable for age, gender, ASA score, tumor location, tumor stage, lymph node metastasis and mortality. Advanced tumor stage was recorded as the only prognostic factor of obstruction (p=0.001). Postoperative mortality rate was 9.1% in the obstruction group and 6.4% in the elective group (p=0.498). Analysis didn't reveal any risk factors for poor early outcome in the obstruction group. All patients with right-sided obstructive cancer were treated with resection and primary anastomosis, while the same procedure was performed in almost 61% of operations for left-sided tumors with no anastomotic failure. CONCLUSIONS: Obstructive colorectal malignancy presents at a more advanced stage compared with non-obstructive cancer, with, interestingly, no statistically significant differences in postoperative mortality. Risk factors of poor early outcome couldn't be identified. Resection and primary restitution of continuity is the surgical approach of choice for right-sided obstructive cancers, but it can be, also, safely performed in left-sided cancers.

Splenic inflammatory pseudotumor-like follicular dendritic cell tumor.

Inflammatory pseudotumor of the spleen with expression of follicular dendritic cell markers is an extremely rare lesion with only a few cases reported previously. The present study reports on an inflammatory pseudotumor of the spleen 10 × 8 × 7 cm in size that was incidentally found in a 61-year-old man and increased gradually in size during a period of 3 months. Abdominal ultrasonography revealed a well-circumscribed splenic mass, and abdominal computed tomography confirmed the presence of a well-circumscribed isodense lesion in the splenic hilum with inhomogenous enhancement in the early-phase images and no enhancement on delayed-phase contrast-enhanced images. Magnetic resonance imaging of the abdomen showed a well-defined isodense tumor on T1-weighted images with mildly increased signal intensity on T2-weighted images, and this is only the second study that provides MRI findings of this entity. The patient underwent an uncomplicated open splenectomy for definitive histologic diagnosis. Under microscopic examination, the lesion was an admixture of lymphocytes, plasma cells and spindle cells. In situ hybridization analysis for Epstein-Barr virus (EBV) revealed that most of the spindle cells were positive for EBV, and immunochemistry showed the expression of the follicular dendritic cell markers CD21, CD35 and CD23 within the tumor. The diagnosis of inflammatory pseudotumor-like follicular dendritic cell tumor was established.

Intestinal neuronal dysplasia type B in adults: a controversial entity.

Intestinal neuronal dysplasia (IND) is a well-defined entity which raises controversy among authors, described as a congenital malformation of gastrointestinal innervation and caused by dysplastic embryonal development of the enteric nervous system. It is potentially associated with mild and chronic gastrointestinal motility disturbances. IND is rarely reported in adults and especially elderly patients. The present study reports on the case of a 71-year-old man suffering from longstanding idiopathic constipation and who was misdiagnosed for more than 60 years, despite several hospital admissions and a sigmoidectomy in the meantime. On the last admission, the patient presented with megacolon, abdominal pain and X-ray finding of bowel obstruction. Due to massive large bowel dilatation, an exploratory laparotomy failed to reveal any obvious mechanical cause, and a subtotal colectomy and Hartmann's procedure was performed. Bowel continuity was performed 3 months later. Analysis of full-thickness biopsies revealed enlarged myenteric and submucosal neurons as well as an increased number of giant cells and increased acetylcholinesterase activity in the mucosa. The diagnosis of IND was established. The main diagnostic criteria, the underlining pathophysiology and the recommended therapeutic approach of this rare entity are extensively reviewed.

Presacral ganglioneuroma: diagnostic considerations and therapeutic strategy.

Presacral ganglioneuroma is an extremely rare tumor of neural crest origin. To the best of our knowledge, less than 20 cases have been reported previously. The present study reports on a presacral ganglioneuroma, 10.5 × 8 × 4 cm in size, that was found incidentally in a 35-year-old man with prior history of diverticulitis. He was admitted to our hospital due to lower left abdominal pain. Abdominal computed tomography and magnetic resonance imaging confirmed the extension of the lesion from the S2 level to the coccyx. The mass had low signal intensity on T1-weighted images and heterogeneous high signal intensity on T2-weighted images with no intraspinal or rectal extension. T2-weighted images demonstrated a compartmentalized solid tumor with cystic components. Complete tumor resection with free surgical margins was achieved using an abdominal approach. The patient remains asymptomatic 2 years after surgery. We emphasize on clinical features, radiologic appearance and surgical treatment of this rare entity. The clinical and pathologic features of previously reported studies are also briefly reviewed.

Mixed large cell neuroendocrine carcinoma with squamous cell carcinoma of the rectum: Report of a rare case and review of the literature.

INTRODUCTION: Mixed large cell neuroendocrine neoplasms of the rectum are rare and aggressive neoplasms. Survival is poor due to the high rate of lymph node metastases and distant metastases at the time of diagnosis. PRESENTATION OF CASE: We report a case of a 50-year-old male patient with a mixed large cell neuroendocrine carcinoma with squamous cell carcinoma of the rectum located 8cm from the anal verge, treated with low anterior resection and total mesorectal excision with free surgical margins. There were lymph nodes metastases but no distant metastases at the time of diagnosis. The patient refused to receive adjuvant chemotherapy and died 6 months later due to liver failure as a result of multiple hepatic metastases. DISCUSSION: There are not known predisposing factors for the development of neuroendocrine rectal carcinoma. A neuroendocrine carcinoma of the rectum is a rare tumor with an incidence of less than 0.1% of all colorectal malignancies. The median survival ranges from 5 to 10.4 months in several studies and there are not sufficient data in bibliography about ideal adjuvant therapy after resection of mixed squamous large cell neuroendocrine carcinoma of the rectum. CONCLUSION: Low anterior resection and total mesorectal excision with free surgical margins in the presence of lymph nodes metastasis is not a sufficient treatment for rectal neuroendocrine carcinoma. More studies should be done in order to determine the ideal adjuvant treatment of these rare and aggressive tumors.

Sclerosing peritonitis presenting 2 years after renal transplantation in a former CAPD patient.

Sclerosing peritonitis (SP) is a rare but serious complication of peritoneal dialysis (PD), characterized by a fibrous peritoneal thickening. The etiology of this condition remains unknown but is likely to be multifactorial. Patients with SP almost invariably develop ultrafiltration and clearance failure. Although a number of pharmacologic drug treatment options have been tried with various results, surgical treatment and cessation of PD are almost always necessary and transfer to hemodialysis is the only practical option. Despite some evidence supporting the recovery of gastrointestinal function after renal transplantation in such patients, SP may very rarely appear much later after the cessation of PD and even after renal transplantation. We report an interesting case of a former PD patient who 2 years after renal transplantation presented with abdominal discomfort, vomiting, and malnutrition due to SP. Despite the initial conservative treatment, the symptoms persisted and a surgical treatment was decided upon. After that the patient recovered with no further complications. Although the appearance of SP after renal transplantation is extremely rare, it must be included in the differential diagnosis of every case of unexplained malnutrition and abdominal obstruction in a patient with a PD history.

Total parathyroidectomy without autotransplantation in dialysis patients and renal transplant recipients, long-term follow-up evaluation.

BACKGROUND: Persistent secondary hyperparathyroidism not responding to medication is treated successfully with surgical excision of parathyroid glands (total parathyroidectomy [PTX]). PTX without autotransplantation of parathyroid glands excludes the risk for recurrence of hyperparathyroidism. METHODS: During the years 2002 to 2005, 36 total parathyroidectomies were performed in 33 patients: 21 dialysis patients because of end-stage renal disease and 12 renal transplant recipients. RESULTS: PTX without autotransplantation was performed successfully in 33 patients, whereas 3 patients were reoperated for remaining parathyroid glands. Immediate improvement of clinical symptoms and a decrease of serum calcium and parathormone levels were observed after surgical procedures. Oral replacement treatment with vitamin D (1a-calcidiol) and calcium was commenced and long-term follow-up evaluation (23.5 +/- 7.6 mo) showed that calcium homeostasis was controlled adequately. CONCLUSIONS: PTX without autotransplantation is a safe and effective surgical procedure for the treatment of resistant secondary hyperparathyroidism with immediate response of clinical symptoms. Replacement treatment with vitamin D and calcium provides satisfactory coverage of individual needs.

Entire stent grafting of the thoracoabdominal aorta in a renal transplant recipient subsequent to extra-anatomical bypasses of the main abdominal vessels.

We present a complex case of a renal transplant recipient with ruptured suprarenal abdominal aortic aneurysm who had previously undergone endovascular repair of descending thoracic and abdominal aortic aneurysm. This patient was treated successfully combining extra-anatomical bypasses of main abdominal arteries and subsequent endovascular stent grafting covering the entire thoracoabdominal aorta.

Total parathyroidectomy with autotransplantation in patients with renal hyperparathyroidism: indications and surgical approach.

OBJECTIVE: renal hyperparathyroidism (rH) is one of the most serious complications in long-term hemodialysis patients. the purpose of this retrospective study was the evaluation of the surgical indications, parathyroid histology, and the results of total parathyroidectomy with autotransplantation (Ptx+At) in patients with rH. DESIGN: the study included 36 patients with rH in whom Ptx+At was carried out at the First surgical Department of the Evangelismos Hospital over a 5-year period. Indications for Ptx+At were high levels of parathyroid hormone (PtH >800 ng/L) associated with hypercalcemia and/or hyperphosphatemia, that were refractory to medical treatment, as well as clinical effects of rH, including pruritus, bone and joints pain, muscle weakness, progression of soft tissue calcification, and spontaneous fractures. Ultrasonography, (99m)Tc sestamibi scan and computed tomography were used to evaluate the thyroid and parathyroids. RESULTS: there was no mortality related to surgery. Preoperative symptoms, serum PtH, serum alkaline phosphatace, hyperphosphatemia, and hypercalcemia were improved or normalised in most patients. recurrence was observed in one case; this autotransplanted patient required resection of transplanted tissue from his forearm. Hypoparathyroidism was not recorded. CONCLUSIONS: Ptx+At is a safe option for the treatment of rH that is accompanied by low morbidity, mortality, and recurrence rate. It is important to identify all parathyroid glands at surgery and to choose adequate parathyroid tissue for autograft.

Successful endovascular repair of ruptured abdominal aortic aneurysm in a renal transplant recipient.

A renal transplant recipient presented in the early post-transplantation period with rupture of an abdominal aortic aneurysm. The high mortality rate of the surgical repair of ruptured aneurysm in addition to the concern of preserving the renal graft prompted us to seek alternative approaches, such as repairing the aneurysm by means of endovascular techniques. The ruptured aneurysm was confirmed by performing computed tomography and digital angiography and thereafter was successfully repaired by endovascular stenting technique (Talent stent-graft), which seems to be a safe and effective method of preserving a renal graft.

Changes in health-related quality of life in Greek adult patients 1 year after successful renal transplantation.

OBJECTIVES: This study was undertaken to compare and to evaluate the health-related quality of life (HRQOL) in Greek adult transplant recipients before and 1 year after successful renal transplantation (RT) and to examine which parameters had the greatest effects on their HRQOL. The SF-36 survey score was used. MATERIALS AND METHODS: Eighty-five Greek hemodialysis patients underwent RT at the Transplant Unit of Evangelismos General Hospital of Athens, including 44 men and 41 women (mean age, 43.8 years; range, 21-59 years). Thirty-nine patients had received a kidney from a living-related donor, and 46 from a cadaver. The scale scores of a Greek version of the SF-36 survey were compared between the transplant and the hemodialysis patients. We also examined the relationships of the scale scores with the patients' age and the type of donor. RESULTS: According to the SF-36 health survey, transplant recipients had better results for general health perception (P