Ebstein anomaly is a rare congenital heart malformation typically involving the tricuspid valve and the right ventricle that has a wide range of anatomical and pathophysiological presentations. Various surgical repair techniques for Ebstein anomaly have been reported because of its near-infinite anatomical variability. Cone repair for Ebstein anomaly can achieve nearly anatomical reconstruction of the tricuspid valve with promising outcomes.In this article, the surgical techniques for cone repair in adult patients with Ebstein anomaly are described in detail, and clinical experiences and technically challenging cases are presented.

PURPOSE: To evaluate the efficacy of focal verteporfin photodynamic therapy (PDT) in patients diagnosed with chronic central serous chorioretinopathy (CSC).METHODS: This study enrolled 52 eyes of 52 patients with chronic CSC who had received verteporfin PDT. The laser spot size of 26 eyes covering only the localized hyperfluorescent area in indocyanine green angiography was classified as focal PDT. The PDT spot size of the other 26 eyes covered the total area of retinal pigment epithelial detachment including the leaking point and was defined as conventional PDT. The central subfield thickness and subfoveal choroidal thickness were measured using Heidelberg Spectralis optical coherence tomography before PDT and at months 1, 3, 6, and 12 after PDT.RESULTS: The mean spot size of the PDT was 1,995 µm in the focal group and 2,995 µm in the conventional group. Central subfield thickness steadily decreased in both groups. The mean baseline subfoveal choroidal thickness for the two groups was 334.95 and 348.35 µm, respectively, with no significant difference (p = 0.602). Subfoveal choroidal thickness decreased significantly to 304.20 µm at 1 month, 284.85 µm at 3 months, 271.60 µm at 6 months, and 265.95 µm at 12 months in the focal group (p < 0.001, p < 0.001, p < 0.001, and p < 0.001, respectively, compared with baseline). In the conventional group, subfoveal choroidal thickness decreased significantly to 318.75, 300, 284, and 272 µm at 1, 3, 6, and 12 months, respectively (p < 0.001, p < 0.001, p < 0.001 and p < 0.001 compared with baseline). There were no significant differences between the two groups in subfoveal choroidal thickness based on PDT spot size at 1, 3, 6, and 12 months (p = 0.633, p = 0.625, p = 0.676, and p =0.755, respectively).CONCLUSIONS: Focal verteporfin PDT for CSC significantly decreased the subretinal fluid and sufoveal choroidal thickness to the same extent as conventional PDT.
Angiography ; Central Serous Chorioretinopathy ; Choroid ; Humans ; Indocyanine Green ; Photochemotherapy ; Retinal Detachment ; Subretinal Fluid ; Tomography, Optical Coherence

BACKGROUND: Pathologic diagnosis of central nervous system (CNS) neoplasms is made by comparing light microscopic, immunohistochemical, and molecular cytogenetic findings with clinicoradiologic observations. Intraoperative frozen cytology smears can improve the diagnostic accuracy for CNS neoplasms. Here, we evaluate the diagnostic value of cytology in frozen diagnoses of CNS neoplasms. METHODS: Cases were selected from patients undergoing both frozen cytology and frozen sections. Diagnostic accuracy was evaluated. RESULTS: Four hundred and fifty-four cases were included in this retrospective single-center review study covering a span of 10 years. Five discrepant cases (1.1%) were found after excluding 53 deferred cases (31 cases of tentative diagnosis, 22 cases of inadequate frozen sampling). A total of 346 cases of complete concordance and 50 cases of partial concordance were classified as not discordant cases in the present study. Diagnostic accuracy of intraoperative frozen diagnosis was 87.2%, and the accuracy was 98.8% after excluding deferred cases. Discrepancies between frozen and permanent diagnoses (n = 5, 1.1%) were found in cases of nonrepresentative sampling (n = 2) and misinterpretation (n = 3). High concordance was observed more frequently in meningeal tumors (97/98, 99%), metastatic brain tumors (51/52, 98.1%), pituitary adenomas (86/89, 96.6%), schwannomas (45/47, 95.8%), high-grade astrocytic tumors (47/58, 81%), low grade astrocytic tumors (10/13, 76.9%), non-neoplastic lesions (23/36, 63.9%), in decreasing frequency. CONCLUSIONS: Using intraoperative cytology and frozen sections of CNS tumors is a highly accurate diagnostic ancillary method, providing subtyping of CNS neoplasms, especially in frequently encountered entities.
Brain Neoplasms ; Central Nervous System Neoplasms ; Central Nervous System ; Cytogenetics ; Diagnosis ; Frozen Sections ; Humans ; Meningeal Neoplasms ; Methods ; Neurilemmoma ; Pituitary Neoplasms ; Retrospective Studies

One of the complications of permanent pacemaker implantation is unintended phrenic nerve stimulation. A 15-year-old boy with a permanent pacemaker presented with chest discomfort due to synchronous chest wall contraction with pacing beats. Even after reprogramming of the pacemaker, diaphragmatic stimulation persisted. Therefore, we performed thoracoscopic phrenic nerve insulation using a Gore-Tex patch to insulate the phrenic nerve from the wire. A minimally invasive approach using a thoracoscope is a feasible option for retractable phrenic nerve stimulation after pacemaker implantation.
Adolescent ; Humans ; Male ; Minimally Invasive Surgical Procedures ; Pacemaker, Artificial ; Phrenic Nerve* ; Polytetrafluoroethylene ; Thoracic Wall ; Thoracoscopes ; Thoracoscopy ; Thorax

Pulmonary arteriovenous fistula (PAVF) is a complication of the Glenn shunt. A 57-year-old tetralogy of Fallot (TOF) patient, who had undergone a Glenn shunt and TOF total correction, complained of dyspnea and cyanosis. PAVFs were present in the right lung, and right lung perfusion was nearly absent. After coil embolization, takedown of the Glenn shunt, and reconstruction of the right pulmonary artery, the patient's symptoms were relieved. Extrapulmonary radioisotope uptake caused by the PAVFs shown in lung perfusion scans decreased, and right lung perfusion increased gradually. Although the development and resolution of PAVFs after a Glenn shunt have been reported in the pediatric population, this may be the first report on this change in old age.
Arteriovenous Fistula* ; Cyanosis ; Dyspnea ; Embolization, Therapeutic ; Fontan Procedure ; Hepatopulmonary Syndrome ; Humans ; Lung ; Middle Aged* ; Perfusion ; Pulmonary Artery ; Tetralogy of Fallot*

Pectus arcuatum is a rare complex chest wall deformity. A 31-year-old female presented with a severely protruding upper sternum combined with a concave lower sternum. We planned a modified Ravitch-type operation. Through vertical mid-sternal incision, chondrectomies were performed from the second to fifth costal cartilages, saving the perichondrium. Horizontal osteotomy was performed in a wedge shape on the most protruding point, and followed by an additional partial osteotomy at the most concaved point. The harvested wedge-shape bone fragments were minced and re-implanted to the latter osteotomy site. The osteotomized sternum was fixed with multiple wirings. With chondrosternoplasty, a complex chest wall deformity can be corrected successfully.
Adult ; Bone Transplantation ; Congenital Abnormalities ; Costal Cartilage ; Female ; Funnel Chest ; Humans ; Osteotomy ; Pectus Carinatum ; Sternum ; Thoracic Wall

No abstract available.
Meningioma* ; Sclerosis*

PURPOSE: This study was aimed at comparing the rate of decrease in preoperative serum bilirubin after biliary drainage and the postoperative complication rate in patients with distal common bile duct (CBD) cancer. METHODS: Forty six patients who had undergone pancreaticoduodenectomy due to distal CBD cancer were included in this study. The patients were divided into the complicated and uncomplicated groups. For the patients who underwent preoperative biliary drainage, the preoperative bilirubin level and the rate of decrease in serum bilirubin were measured and compared between the two groups. RESULTS: The postoperative complication and mortality rates were 43.4% (20/46) and 6.5% (3/46), respectively. The complication rates were not different between the drainage and non-drainage groups (p=0.48). The preoperative serum bilirubin levels were also not different between the complicated and uncomplicated groups (p=0.214). In the uncomplicated group, the rate of decrease in the serum total bilirubin level tended to be higher than in the complicated group, but this was not statistically significant (mean: 0.82 vs 0.27 mg/dl/day, respectively, p=0.117). Patients with a higher preoperative bilirubin decrease rate showed a larger bilirubin decrease in the immediate postoperative period (from POD 1 to POD 7, r=0.371, p=0.05). CONCLUSION: The preoperative biliary drainage and the serum bilirubin level were not correlated with the postoperative complication rate. Patients with higher preoperative bilirubin decrease rates showed a larger bilirubin decrease in the immediate postoperative period, which can be interpreted as having a faster recovery of their liver function. Further study with a larger number of cases is required to see if the preoperative bilirubin decrease rate could be used as a prognostic indicator after pancreaticoduodenectomy.
Bilirubin* ; Common Bile Duct* ; Drainage* ; Humans ; Liver ; Mortality ; Pancreaticoduodenectomy ; Postoperative Complications* ; Postoperative Period

PURPOSE: This study reviews our experience with a step- by-step management approach of increasing aggressiveness and evaluates the treatment outcome for intraluminal hemorrhage. METHODS: The study group was comprised of patients who had experienced intraluminal hemorrhage after a low anterior resection with the double stapling technique from 1999 to 2003. The choice of management was selected according to our step-by-step management protocol, and the outcomes were evaluated for each step, lincluding mortality and complications. RESULTS: Nine patients (6 males and 3 females, mean age 55 years) were identified, the mean volume of packed RBC transfusion was 2 pints, and the mean distance of the anastomotic site from the anal verge was 6 cm. The median stapler size was 31 mm. The first step was cold saline irrigation and drainage; four of 9 patients were controlled. The second step was retention enema with topical hemostatics; one of remaining 5 patients stopped bleeding. The third step was colonoscopic hypertonic saline injection around the bleeding site with direct colonoscopic electrocauterization, two of remaining 4 patients were controlled. The last step was suturing the bleeding site through the anus, the remaining 2 patients stopped bleeding. One of the 9 patients developed leakage from the anastomotic site after the last step management, three of the 9 patients had long standing ileus, and one of the 9 patients developed acute renal failure after a massive transfusion. There were no postoperative deaths. CONCLUSIONS: It is safer and easier to control bleeding with step-by-step management system of increasing aggressiveness.
Acute Kidney Injury ; Anal Canal ; Drainage ; Enema ; Female ; Hemorrhage* ; Hemostatics ; Humans ; Ileus ; Male ; Mortality ; Rectal Neoplasms ; Treatment Outcome*

Surgical clip migration and subsequent stone formation in the common bile duct is a rare, but well-established complication after laparoscopic cholecystectomy. There are some suggestions about the mechanisms of the migration process, but the details are still unclear. We report here on a case in which common bile duct stones were formed around surgical clips, and other clips were found to have penetrated into the common hepatic duct, which we believe were in the process of migration after laparoscopic cholecystectomy. The patient required a laparotomy to retrieve the bile duct stones due to the distal bile duct stricture, and another laparotomy was necessary to remove the penetrating clips, which were deeply embedded in the bile duct wall. Although a variety of endoscopic and percutaneous interventional procedures are available in this era of modern medical technology, it is sometimes impractical to apply these procedures in such cases as ours, and exploratory laparotomy is sometimes required to correctly treat the patient. This case shows that the metallic surgical clips can penetrate into the intact bile duct wall through serial maceration, and we believe that careful application of clips may be the only way to prevent their migration after laparoscopic cholecystectomy.
Bile Ducts ; Cholecystectomy, Laparoscopic* ; Common Bile Duct ; Constriction, Pathologic ; Hepatic Duct, Common* ; Humans ; Laparotomy ; Surgical Instruments*