Laparoscopic liver resection for a patient of hepatocellular carcinoma with von Willebrand disease: a case report.

BACKGROUND: The safety of laparoscopic hepatectomy for inherited coagulation disorders is unclear; however, the safety of open hepatectomy has been reported in several studies. Herein, we report the first case of a laparoscopic hepatectomy for a patient with von Willebrand Disease (VWD). CASE PRESENTATION: A 76-year-old male with a history of chronic hepatitis C and VWD type 2B was advised surgical resection of a 4 cm hepatocellular carcinoma in segment 7 of the liver. The patient was diagnosed with VWD in his 40 s due to gastrointestinal bleeding caused by gastric erosion. The von Willebrand factor (VWF) ristocetin cofactor activity was 30%, and VWF large multimer deficiency and increased ristocetin-induced platelet agglutination were observed. The preoperative platelet count was reduced to 3.5 × 104/µL; however, preoperative imaging findings had no evidence of liver cirrhosis, such as any collateral formations and splenomegaly. The indocyanine green retention rate at 15 min was 10%, and his Child-Pugh score was 5 (classification A). Perioperatively, VWF/factor VIII was administered in accordance with our institutional protocol. A laparoscopic partial hepatectomy of the right posterior segment was performed. The most bleeding during surgery occurred during the mobilization of the right lobe of the liver due to inflammatory adhesion between the retroperitoneum and the tumor. Bleeding during parenchymal transection was controlable. The duration of hepatic inflow occlusion was 65 min. The surgical duration was 349 min, and the estimated blood loss was 2150 ml. Four units of red blood cells and fresh frozen plasma were transfused at the initiation of parenchymal transection, and 10 units of platelets were transfused at the end of the parenchymal transection. On postoperative day 1, the transection surface drainage fluid became hemorrhagic, and emergency contrast-enhanced computed tomography showed extravasation in the greater omentum. Percutaneous transcatheter arterial embolization of the omental branch of the right gastroepiploic artery was performed. No further postoperative interventions were required. The patient was discharged on postoperative day 14. CONCLUSION: The indications for laparoscopic hepatectomy in patients with VWD should be carefully considered, and an open approach may still be the standard approach for patients with VWD.

A Rare Presentation of Lymph Node Metastasis of VIPoma After Three Years of Resection: A Case Report.

Vasoactive intestinal peptide-producing tumor of the pancreas (VIPoma) is one of the rarer subtypes of neuroendocrine tumor (NET) of the pancreas. It usually represents intractable diarrhea, weight loss, and electrolyte abnormalities secondary to diarrhea. The most common site of metastasis of VIPoma is the liver. Furthermore, lymph node metastasis (LNM) is rare, and no metachronous LNM with a resectable situation has been reported before. A 60-year-old male patient (height: 181 cm, body weight: 74 kg) with a history of operated pancreatic VIPoma three years ago was referred to our department due to the detection of lymphadenomegaly which was suggestive of lymph node metastasis by routine follow-up computed tomography (CT). Preoperative CT showed a lymph node on the left side of the abdominal aorta and caudal side of the left renal vein with a size of 1 cm. Lymphadenectomy was performed without significant complications and blood loss. This is the first report of metachronous LNM in a patient with operated VIPoma. Although much rarer than solid organ metastasis of VIPoma, LNM in these patients can also be seen synchronously and metachronously. Close follow-up and vigilance are key to preventing recurrence-related morbidity and mortality in these patients.

Is Endoscopic Sphincterotomy Sufficient in the Treatment of Sump Syndrome? A 25-Year Experience.

Background: Sump syndrome is one of the rare long-term complications of side-to-side choledochoduodenostomy (CD) leading to attacks of cholangitis due to accumulation of food and debris in the common bile duct distal to the anastomosis is one of the rare long-term complications after CD. Methods: Fifteen patients treated with the Sump syndrome in our institution between 1996 and 2023 were retrospectively evaluated for long-term outcome. Results: Endoscopic retrograde cholangiopancreatography (ERCP) with sphincterotomy and bile duct clearance was done in 11 patients, while four were subjected to revisional surgery in the form of a Roux-en-Y hepaticojejunostomy. No complications were recorded. There were 5 (38%) recurrences in a median follow-up period of 8 years (10 months-23 years). Of those, 3 patients were treated surgically and two with repeat ERCP. None of the patients developed any cholangiocarcinoma during follow-up. Conclusion: We conclude that although a high recurrence rate was observed, endoscopic treatment may be a valid approach in the treatment of Sump syndrome, with revisional surgery in the form of a Roux-en-Y hepaticojejunostomy as salvage therapy in recurrences.

Diagnostic ability of Ga-68 PSMA PET to detect dominant and non-dominant tumors, upgrading and adverse pathology in patients with PIRADS 4-5 index lesions undergoing radical prostatectomy.

BACKGROUND: To evaluate the additive role of Ga-68 PSMA PET as a primary staging tool in patients bearing prostate cancer in single PIRADS 4 or 5 index lesions. METHODS: Eighty-one biopsy-naive patients with preoperative mpMRI and Ga-68 PSMA PET who underwent radical prostatectomy (RP) were evaluated retrospectively. Forty-nine patients had PIRADS 4 and 32 had PIRADS 5 index lesions. The localization, grade, and volumetric properties of dominant (DT) and non-dominant tumors (NDT) in RP were compared to the index lesions of mpMRI and Ga-68 PSMA PET. RESULTS: The median age and PSA level were 62 (IQR; 59-69) years and 7 (IQR; 2-8) ng/ml, respectively. Ga-68 PSMA PET detected DTs in 100% of the patients including 13 patients in whom mpMR failed. In 45 patients an NDT was reported in RP. Ga-68 PSMA PET accurately detected NDT in 24 of 45 (53.3%) patients. Six patients (12.2%) in PIRADS 4 and 8 (25%) in PIRADS 5 group showed upgrading. In PIRADS 4, Ga-68 PSMA PET localized DT in all patients with upgraded tumors whereas mpMRI missed exact location in 2 of 6 (33.3%). In PIRADS 5 both mpMRI and Ga-68 PSMA PET accurately located all DTs. Overall detection rates of extracapsular extension (ECE) and seminal vesicle invasion (SVI) by mpMRI were 51.1% and 53.8%, respectively. Ga-68 PSMA PET detected ECE and SVI in 27.9% and 30.7%, respectively. When mpMRI and Ga-68 PSMA PET were used in combination detection rates of ECE and SVI increased to 65.1 and 61.5%. Ga-68 PSMA PET-detected six of ten patients with positive lymph nodes whereas mpMRI could not identify any. CONCLUSIONS: Ga-68 PSMA PET has a better diagnostic accuracy in detecting DT, NDT, upgrading, adverse pathology in patients with PIRADS 4 index lesions. However, mpMRI better predicted ECE and SVI than Ga-68 PSMA PET.

Two-year old girl with glial choristoma presented in a thyroglossal duct cyst.

BACKGROUND: Neuroglial choristomas are rare entities that are composed of differentiated neuroectodermal cells presenting where they do not belong. CASE: Here in this paper, we represent a two-year old patient with a very rare presentation of neuroglial choristoma which manifested itself within a thyroglossal duct cyst. In this paper we will also discuss pathogenesis, clinical manifestation, differential diagnosis and management of the neuroglial choristomas. CONCLUSION: In conclusion we believe this unique case may aid in understanding the pathophysiology, differential diagnosis, and management of this rare congenital anomaly.

A rare presentation of adrenal adenoma in infancy: isolated Cushing`s syndrome.

Adrenocortical tumors are rare in children. Most of these tumors present with endocrinological manifestations, majority of which with virilizing features alone or in combination with over production of other adrenal hormones. However, it is uncommon of an adrenocortical tumor to present solely with Cushing`s syndrome. In this paper we discuss the clinical presentation and management of a 5-month-old infant who had presented with Cushing`s syndrome due to a functioning adrenocortical adenoma without androgen and mineralocorticoid excess, and made a brief review on the clinical and histopathological characteristics of adrenocortical tumors.

Risk factors for febrile urinary tract infections in the first year after pediatric renal transplantation.

Urinary tract infection is the most common infectious complication following kidney transplant. Anatomic abnormalities, bladder dysfunction, a positive history of febrile urinary tract infection, and recipient age are reported risk factors. The aim of this study was to determine the risk factors for fUTI, which necessitated hospitalization in the first year after renal transplantation in our pediatric transplant population. A retrospective review of 195 pediatric patients who underwent kidney transplant between 2008 and 2017 from a single institution was performed. All patients admitted to the hospital with fUTI were marked for further analyses. The risk factors including age, gender, dialysis type, history of urologic disorders, and preoperative proteinuria for fUTI in the first year after kidney transplantation and graft survivals were investigated. Independent-sample t test and chi-square tests were used for univariate analysis. Exhaustive CHAID algorithm was used for multivariate analysis. The data of 115 male and 80 female patients were retracted. The mean ages of our cohort for males and females were 9.5 ± 5.1 and 10 ± 4.8 years, respectively. The age of the patients at transplant and their gender were found to be a statistically significant risk factors for developing fUTIs. Multivariate analysis showed that fUTI was common in female patients and a subgroup of male patients who had preoperative proteinuria, but no neurogenic bladder had higher risk compared with male patients without proteinuria. Patient surveillance and antibiotic prophylaxis algorithms can be developed to prevent febrile urinary tract infections seen after pediatric kidney transplantation in risky population.

Cardiovascular Effects of Continuous Positive Airway Pressure Treatment in Patients With Obstructive Sleep Apnea: A Meta-Analysis.

Continuous positive airway pressure (CPAP) is the first-line treatment of obstructive sleep apnea (OSA). Obstructive sleep apnea is a predictor of cardiovascular (CV) events. In this meta-analysis, we evaluated the effect of CPAP on left ventricular ejection fraction (LVEF), CV events, CV mortality, and all-cause mortality in patients with OSA. Nine articles (n = 9610 patients) were analyzed. Four different meta-analyses were performed: evaluation of LVEF, assessment of all-cause mortality, CV mortality, and CV events. Continuous positive airway pressure treatment was associated with a significant increase in LVEF (mean difference: 2.1%, 95% confidence interval [CI]: 0.8%-3.4%). There was a nonsignificant reduction in all-cause mortality (hazard ratio [HR]: 0.92, 95% CI: 0.73-1.15) but a significant reduction of 66% in the risk of CV mortality associated with the CPAP treatment (HR: 0.34, 95% CI: 0.17-0.68, P = .002). There was a nonsignificant reduction in the risk of CV events in the CPAP-treated patients (HR: 0.84, 95% CI: 0.60-1.18, P = .31). Our meta-analyses showed that CPAP treatment improves LVEF and could have a beneficial effect on CV mortality.

Erythropoietin mediates brain-vascular-kidney crosstalk and may be a treatment target for pulmonary and resistant essential hypertension.

Organ crosstalk pathways represent the next frontier for target-mining in molecular medicine for existing syndromes. Pulmonary hypertension and resistant essential hypertension are syndromes that have been proven elusive in etiology, and frequently refractory to first-line management. Underlying crosstalk mechanisms, not yet considered in these treatments, may hinder outcomes or unlock novel treatments. This review focuses systematically on erythropoietin, a synthesizable molecule, as a mediator of brain-kidney crosstalk. Insights gained from this review will be applied to cardiovascular diseases in a clinician-directed fashion.

The concept of crosstalk-directed embryological target mining and its application to essential hypertension treatment failures.

This review aims to introduce the novel concept of embryological target mining applied to interorgan crosstalk network genesis, and applies embryological target mining to multidrug-resistant essential hypertension (a prototype, complex, undertreated, multiorgan systemic syndrome) to uncover new treatment targets and critique why existing strategies fail. Briefly, interorgan crosstalk pathways represent the next frontier for target mining in molecular medicine. This is because stereotyped stepwise organogenesis presents a unique opportunity to infer interorgan crosstalk pathways that may be crucial to discovering novel treatment targets. Insights gained from this review will be applied to patient management in a clinician-directed fashion.

Atherosclerotic renal artery stenosis in the post-CORAL era part 2: new directions in Transcatheter Nephron Salvage following flawed revascularization trials.

Unlike endovascular therapeutic studies for atherosclerosis in many other vascular beds, major trials regarding endovascular renovascular revascularization have resulted in a stagnating equipoise. However, every major trial completed for this topic thus far has suffered from major methodological flaws that limit the validity and external generalizability of their results. Furthermore, certain patient populations who are known to benefit from renovascular revascularization may never be studied because they cannot be ethically withheld from life-saving treatment. Forthcoming percutaneous techniques may one day complement angioplasty and stenting in a burgeoning era of cellular modulation and endovascular-directed renal regeneration.