Takayuki Kuga

Pancreatoblastoma: Case report and review of treatment in the literature

A case of pancreatoblastoma arising from the body-to-tail of the pancreas in a 5-year-old boy is presented. The patient underwent exploratory laparotomy and, 11 days later, resection of the tumor (partial pancreatectomy, pyloroplasty, and splenectomy). Before resection, cyclophosphamide and vincristine were administered. Because of tumor spillage during resection, a combination of chemotherapy (administration of cyclophosphamide and adriamycin on that day) and postoperative radiotherapy was given. Nine months after resection, partial hepatectomy was performed for liver metastasis and consolidated by a more intensive chemotherapy regimen using cisplatin, adriamycin, vincristine, and cyclophosphamide. After completion of the chemotherapy, the patient had a 14-month uneventful course, and a locally recurrent tumor was treated by the fourth surgery (extirpation of the recurrent tumor, partial hepatectomy, partial colectomy, and partial gastrectomy) and intraoperative radiation. Thereafter, the boy has shown no evidence of disease at 3 years 8 months. The literature of pancreatoblastoma is reviewed from the therapeutic viewpoint.

A supernumerary ovary of the omentum with cystic change : Report of two cases and review of the literature

A supernumerary ovary is a rare gynecological anomaly. Particularly rare is the presence of cystic changes within the supernumerary ovary. We report two cases of neonates found to have a supernumerary ovary resembling an omental cyst. To the best of our knowledge, this report describes the first antenatal diagnosis of an omental cyst with a supernumerary ovary. To explain this unusual occurrence, it is suggested that an omental cyst becomes detached from the ovarian tissue and implants itself in the greater omentum, and that these supernumerary ovaries are of true embryologic origin, and not due to post‐surgical or post‐inflammatory implantation.

The occurrence of a strangulated ileus due to a traumatic transmesenteric hernia: Report of a case

We report herein the case of a 6-year-old boy in whom a strangulated ileus was caused by a traumatic transmesenteric hernia. The boy had fallen from his bicycle and suffered a severe blow to the abdomen. Abdominal pain and vomiting developed 10h after the accident and he was admitted to our hospital. Abdominal ultrasonogram and computed tomogram demonstrated ascites, intestinal wall thickening with fluid, and an engorged radiating mesenteric vasculature. Thus, an emergency laparotomy was performed which revealed bloody ascites, a strangulated ileus, and torsion with a transmesenteric hernia. The necrotic intestine was resected and an anastomosis was performed. Macroscopic and microscopic findings revealed a traumatic mesenteric rent. The unusual presentation of this case is discussed.

DETECTION OF TYPE III COLLAGEN FRAGMENTS IN SPECIMENS OF ABDOMINAL AORTIC ANEURYSMS

The purpose of this study was to analyze the collagens in aortic aneurysm walls and to investigate the mechanism of the formation of calcified abdominal aortic aneurysms (AAAs). Collagens were extracted from human aneurysmal aortic walls obtained during surgery, and from human nonaneurysmal aortic walls obtained at autopsy, using pepsinacetic acid digestion. Electrophoresis and immunoblotting were performed. Type III collagen was found to be reduced in the arteriosclerotic aneurysmal aortic walls. The α1 chain of type III collagen/α1 chain of type I collagen ratio was 0.35 ±0.11 in the aortic aneurysms and 0.68 ± 0.11 in the nonaneurysmal aortic walls (P=0.0111). All the calcified aneurysms were associated with type III collagen fragments having molecular mass of approximately 70 kDa and 30 kDa as estimated by sodium dodecyl sulfate-polyacrylamide gel electrophoresis. Thus, we concluded that AAAs with calcification may be caused by an abnormal degradation of type III collagen.

Successful management of a giant spinal arteriovenous malformation with multiple communications between primitive arterial and venous structures by embolization: Report of a case

A 47-year-old woman was admitted to our hospital with a giant spinal arteriovenous malformation (AVM) causing heart failure and thoracic myelopathy. Angiography revealed that the spinal AVM had multiple feeding vessels branching from the 5th through 12th intercostal arteries. The drainage vein flowed to the azygos vein and superior vena cava. The AVM destroyed the 7th thoracic vertebra. The cardiac output was 16.7l/min and the shunt ratio was 64% before treatment. Embolization with cyanoacrylate was performed because the operation was considered to be associated with a significant risk of paraplegia and organ ischemia. The cardiac output decreased to 11.6l/min and the shunt ratio was reduced to 32%. After embolization the patient demonstrated no symptoms of either heart failure or sensory deficits. During embolization, provocative tests using sodium amytal and lidocaine with magnetic stimulation were also performed. The above findings suggest that provocative tests and magnetic stimulation are useful to predict paraplegia, which could result from embolization while, in addition, embolization is considered to be a useful treatment for multiple shunt and nidus in this region.

Impact of collagen subtype proportions in peritoneal tissues on inguinal hernia formation in adults and infants

Impaired collagen subtype proportions in the matrices results in the alteration of tissue tensile strength, and is an underlying cause of adult inguinal hernia formation; however, it remains unclear if this alteration is also responsible for infantile inguinal hernia formation. We correlated the ratios of collagen type I to type III between adults and infants with and those without an inguinal hernia, in an attempt to clarify the pathogenesis of this disorder. We extracted collagen from the hernial sacs of patients with an inguinal hernia, and from the normal peritoneum of patients without an inguinal hernia. After separation by electrophoresis, the collagen bands were quantified and we compared the ratios of collagen type I to type III between the patients with and those without an inguinal hernia. The ratio of collagen type I to type III was significantly lower in the adults with an inguinal hernia (n=8, 6.17±2.69) than in those without an inguinal hernia (n=8, 9.38±2.99, P=0.041), whereas the ratios were similar in infants with (n=10, 5.72±2.66) and those without an inguinal hernia (n=6, 5.60±1.88, P=0.925). Our results showed that tissue weakness was not involved in the pathogenesis of infantile inguinal hernia formation, confirming that simple herniotomy is adequate treatment.

Immunocytochemical analysis of cellular infiltrates in human appendicitis.

Abstract This study was conducted to determine the immunologic cellular composition in human ap-pendicitis and its association with the development of perforated appendicitis. Appendiceal specimens from 27 patients with acute appendicitis were immunostained to detect lymphocyte surface markers. Moreover, the lymphocyte surface markers of peripheral blood were analyzed by laser flow cytometry in 12 patients. Helper T lymphocytes (CD4) were present in all the patients, while B lymphocytes (CD19), natural killer (NK) cells (CD56), and cytotoxic T lymphocytes (CD8) were present in 7 (70%), 10 (100%), and 9 patients (90%) with perforated appendicitis, and in 12 (63.2%), 10 (58.8%), and 6 (54.5%) patients without perforation, respectively. There were significant differences between the patients with a perforated appendix and those without perforation, in the positivity rate for CD8 and CD56 cells (P < 0.05). The number of cells positive for CD56, being NK cells, in the blood from the patients with perforation was significantly lower than that in the blood from those without perforation (P < 0.05). The infiltration of a greater number of cytotoxic T lymphocytes and NK cells was observed in the appendices from patients with perforated appendicitis than in those from patients with nonperforated appendicitis.

The lack of type III collagen in a patient with aneurysms and an aortic dissection

We analyzed 79 consecutive patients with aneurysms and found a patient who lacked type III collagen. Collagen was extracted from the skin, and the lack of type III collagen was determined by means of sodium dodecyl sulfate-polyacrylamide gel electrophoresis (SDS-PAGE). Only 1 of the 79 patients was found to lack type III collagen. This patient was a 55-year-old man who had sustained an abdominal aortic aneurysm and aortic dissection. He did not show any of the typical clinical symptoms of Ehlers-Danlos syndrome type IV, such as hyperextensible skin and joints. He had none of the usual risk factors nor any clear family history of the syndrome. Furthermore, his collagen fibrils demonstrated a homogeneous appearance. This case may represent a new form of type III collagen deficiency.

High submucosal blood flow and low anastomotic tension prevent anastomotic leakage in rabbits

In many cases of long-gap congenital esophageal atresia, direct anastomosis is difficult. In these cases, the esophagus is first lengthened by myotomy before anastomosis. We determined the degree of submucosal blood flow and/or approximation force at the site of anastomosis in rabbits after (1) separation of the esophagus from the outer membrane, (2) 1 cm and 2 cm resection of the esophagus, and (3) circular or spiral myotomy of the esophagus after 2 cm resection. In the first experimental group, submucosal blood flow volume <115.2 ml/min/100 g resulted in anastomotic leakage. In the second experimental group, a 1 cm resected esophagus with an approximation force of 33.3 ± 8.2g did not result in leakage, while a 2 cm resected esophagus with an approximation force of 111.7±13.3 g resulted in leakage. It was found that leakage occurred when the approximation force was higher than 49.1 g even if submucosal blood flow volume was greater than 131.8 ml/min/100 g. In the third experiment, both circular and spiral myotomy reduced the approximation force. Although there was no difference in the changes in submucosal blood flow volume between the two types myotomy, circular myotomy was superior to spiral myotomy in the reduction of the approximation force at the site of anastomosis. We conclude that both approximation force and submucosal blood flow are important factors in preventing anastomotic leakage.

Langerhans cell histiocytosis with an anterior mediastinal mass involving calcification: confirmation by needle biopsy

al. [3] stressed the presence of primary CID rather than the other morphological features seen in the Hoyeraal-Hreidarsson syndrome. By contrast, the case of Aalfs et al. [ 1] had the characteristic facial dysmorphism but lacked the infection proneness. In the original article, Hoyeraal et al. [5] described two brothers with microcephaly, growth failure, cerebellar hypoplasia and thrombocytopenia one of whom did not show signs of primary immunodeficiency. Assuming that the siblings suffered from the same condition, this suggests that either recurrent infections are not always part of the syndrome, or that symptoms of CID can emerge at a later age. The patient of Berthet et al. [2] and ours deteriorated with infectious complications after the diagnosis of CID. If all these cases display variable features of the Hoyeraal-Hreidarsson syndrome (Table 1), the absence of cerebellar hypoplasia in our case points to the variability of this syndrome. The megaloblastic changes in hypoplastic marrow may be another distinctive feature of this syndrome [1, 5, 6, 8]. Kobrinsky et al. [7] reported seven children with haematopoietic dysplasia characterized by progressive hypoplasia and megaloblastic change, four of whom showed psychomotor retardation, short stature and recurrent infections.