Successful surgical management of non-perforating acute appendicitis with septic disseminated intravascular coagulation: A case report and review of the literature.

INTRODUCTION: Perforating appendicitis and abscess-forming appendicitis may cause septic disseminated intravascular coagulation (DIC). However, non-perforating acute appendicitis with septic DIC is extremely rare. PRESENTATION OF CASE: A 67-year-old man was referred to our hospital one day after starting oral antibiotic treatment for acute appendicitis. Physical examination revealed only slight spontaneous abdominal pain without tenderness and peritoneal irritation. Contrast-enhanced computed tomography demonstrated an enlarged appendix (10 mm in diameter) without fecalith, ascites, intraperitoneal free air, and abscess. There was no evidence of perforating appendicitis. Laboratory analysis revealed septic DIC. The patient was diagnosed with non-perforating acute appendicitis with septic DIC. The patient was distressed regarding whether he should be treated conservatively with an antibiotics-first strategy or undergo an appendectomy. Ultimately, a laparoscopic appendectomy was performed. Histopathological examination showed non-perforating gangrenous appendicitis. He required DIC therapy for 2 days postoperatively. He was discharged on postoperative day 9, and remained in good health 1 month after surgery. DISCUSSION: There is no absolute index of conversion to surgery with an antibiotics-first strategy of appendicitis treatment. Judging the limit of conservative treatment and determining the best moment to perform surgery is a critically important matter for patients with acute appendicitis. CONCLUSIONS: The incidence of conservative treatment preceding an antibiotics-first strategy for acute appendicitis is increasing. However, it is thought that appendectomy should be performed when acute appendicitis is complicated with septic DIC, even if it is a non-perforating appendicitis in which improvement with conservative treatment is anticipated.

Appendiceal neurofibroma with low-grade appendiceal mucinous neoplasm in neurofibromatosis type 1 patient: A case report.

INTRODUCTION: Neurofibromas are a characteristic of the autosomal dominant disorder Neurofibromatosis type 1 (NF1), also known as von Recklinghausen's disease. Appendiceal neurofibromas are extremely rare, and low-grade appendiceal mucinous neoplasms (LAMNs) have not previously been reported in NF1. PRESENTATION OF CASE: A 62-year-old man with NF1 was scheduled for elective surgical treatment of an asymptomatic, enlarged and diffusely thickened appendix that remained after curative antimicrobial treatment of acute appendicitis. Laboratory analysis revealed all normal. Colonoscopy showed thickened appendiceal mucosa projecting into the cecum. A sample of the mucosa was found to be pathologically benign. The patient was preoperatively diagnosed with treated acute appendicitis with chronic appendiceal inflammation versus appendiceal neoplasms. Laparoscopic cecectomy was performed. Multiple neurofibromas were observed in the muscle layer, submucosa, and mucosa of the appendix on histopathological examination. Immunohistochemical examination showed positive staining for S-100. Pathologically, the patient was diagnosed with appendiceal neurofibroma consistent with NF1 with LAMNs. His postoperative course was unremarkable. He was discharged on post-operative day 3 and remained in good health 7 month after surgery. CONCLUSIONS: Appendiceal neurofibromas are often preoperatively diagnosed as appendicitis. Appendiceal neurofibromas should be considered in patients with NF1 who are suspected of having appendicitis.

Intestinal obstruction caused by low-grade appendiceal mucinous neoplasm: A case report and review of the literature.

INTRODUCTION: Intestinal obstructions due to appendiceal pathology are rare. Obstructions caused by low-grade appendiceal mucinous neoplasms (LAMNs) are rarer still. PRESENTATION OF CASE: A 79-year-old woman was referred to our hospital for mechanical small intestinal obstruction. The patient had undergone prior left oophorectomy via a lower abdominal incision. Physical examination revealed abdominal distension and slight tenderness. Laboratory analysis was unremarkable. Contrast enhanced computed tomography (CT) demonstrated dilation of an obstructed small intestinal loop without evidence of strangulation. There appeared to be a low density mass measuring 3.0 × 1.5 cm with a potential twist in the mesentery near the transition point in the small intestine. The patient was diagnosed with mechanical small intestinal obstruction and was treated conservatively with nasogastric tube decompression for one week. After no clinical improvement, we elected to surgically explore her. Intraoperative findings revealed that the ileum was compressed by the appendix, which had a cystic mass on its tip. There was no evidence of intestinal ischemia. Laparotomy appendectomy alone was performed. Pathology revealed a LAMN measuring 3.0 × 1.5 cm. She remains disease-free with 18 months of postoperative follow-up. DISCUSSION: Intestinal obstruction due to external compression by an appendiceal mass is rare, and is often difficult to preoperatively diagnose with CT. CONCLUSIONS: The etiology of small intestinal obstruction with a transition point in the right lower quadrant of abdomen includes an appendiceal mass on the broad differential for this common problem.

Pancreatobiliary fistula associated with intraductal papillary mucinous carcinoma accompanying obstructive jaundice: A case report.

INTRODUCTION: Intraductal papillary mucinous neoplasms (IPMNs) occasionally involve formation of fistulas with other adjacent organs. Pancreatobiliary fistulas associated with IPMNs are rare, but affected patients often develop obstructive jaundice and cholangitis. PRESENTATION OF CASE: A 79-year-old man was referred to our hospital for evaluation of abnormal biliary enzymes. Contrast-enhanced computed tomography and endoscopic retrograde cholangiopancreatography demonstrated multiple cystic lesions with septa in the pancreatic head and fistulas between the cystic lesions and common bile duct. The clinical diagnosis was pancreatobiliary fistula associated with a mixed-type IPMN and accompanying obstructive jaundice. The patient underwent subtotal stomach-preserving pancreaticoduodenectomy. The resected specimen showed fistulas between the cystic lesions and common bile duct. Histopathological examination showed that the main and branch ducts of the pancreatic head were dilated and filled with mucus. The epithelia of the pancreatic ducts revealed papillary proliferation and an invasive adenocarcinoma arising from an intraductal neoplasm. Immunohistochemistry examination showed CDX2- and MUC2-positive reactions. The final diagnosis was an intraductal papillary mucinous carcinoma of the intestinal-type. The patient remained disease-free for 9 months postoperatively. DISCUSSION: The causes of death in patients who have pancreatobiliary fistulas associated with IPMNs without resection are cholangitis or hepatic insufficiency. Nonoperative treatment is limited for cases with obstructive jaundice. It is necessary to prevent obstructive jaundice and cholangitis due to a large quantity of mucinous material. CONCLUSIONS: Surgical resection should be considered, if possible, in patients with pancreatobiliary fistulas associated with IPMNs. A better prognosis is expected with prevention of obstructive jaundice or cholangitis.

Splenic hamartoma associated with thrombocytopenia: A case report.

INTRODUCTION: Hamartomas are rare, benign tumors of the spleen. Few cases of splenic hamartomas associated with thrombocytopenia have been reported. PRESENTATION OF CASE: An asymptomatic 64-year-old man with myelodysplastic syndrome was found to have a splenic tumor. Laboratory tests were significant for thrombocytopenia, with a platelet count of 7.8×104/µL. Ultrasonography showed splenomegaly (10.8×6.6cm), and a hypoechoic splenic mass (8.0×7.0cm). Color doppler ultrasound revealed blood flow within the mass, and the mass density was homogeneous on abdominal computed tomography (CT). Contrast-enhanced CT showed heterogeneous enhancement of the splenic mass during the arterial phase. Positron emission tomography (PET)-CT showed no significant fludeoxyglucose (FDG) accumulation within the mass. The differential diagnosis included splenic hamartoma, splenic hemangioma, splenomegaly associated with extramedullary hematopoiesis, and malignant tumor, including solitary splenic metastasis. A laparoscopic splenectomy was performed due to the possibility of malignancy, the presence of thrombocytopenia, and the risk of splenic rupture. The resected specimen showed a localized, well-demarcated, 8.0×7.0cm splenic mass. Histological examination revealed abnormal red pulp proliferation and the absence of normal splenic structures. The patient's post-operative course was uneventful. His platelet count improved on post-operative day 1 and he was discharged on post-operative day 9. He remained in good health with a normal platelet count one month after surgery. DISCUSSION: Making definitive preoperative diagnosis is difficult in splenic hamartomas. Surgery is necessary for diagnosis when malignancy cannot be ruled out. CONCLUSIONS: Surgery may also improve symptoms of hypersplenism, including thrombocytopenia.

[A case of advanced gastric cancer with multiple liver metastases completely responding long term to paclitaxel plus S-1 therapy].

A 69-year-old man was admitted to our hospital with complaints of loss of appetite, fatigue and dysphasia. Upper gastroscopy revealed advanced gastric cancer. Abdominal CT suggested liver metastases. At first we thought the liver metastases has been completely resected, but we found multiple liver metastases unexpectedly. So only total gastric resection and liver biopsy were performed. The pathological diagnosis was metastatic carcinoma. Paclitaxel (PTX) and S-1 combination chemotherapy was started after operation and was continued for 42 courses. A CT scan showed a complete response, and he has been well without tumor re-growth ever since. The combination of PTX and S-1 not only may be an effective regimen for gastric cancer with liver metastases, but also can be used without side effects for a long time.

Extensive primary repair of the thoracic aorta in Stanford type A acute aortic dissection by means of a synthetic vascular graft with a self-expandable stent.

OBJECTIVES: To minimize any residual false lumen when operating on patients with an acute type A aortic dissection, we tried to perform extensive primary repair of the thoracic aorta with the modified elephant trunk technique. The early and midterm results of these surgical interventions are reported and evaluated. METHODS: Among the acute type A aortic dissections with extensive false lumen encountered since December 1997, 19 consecutive patients, 15 DeBakey type I with the tear in the ascending, transverse, or both aortas, and 4 DeBakey type III-D with the tear located in the descending aorta, underwent insertion of a synthetic graft with a distally anchored stent in the descending thoracic aorta. The interpolation method was used as an introducer combined with total replacement of the aortic arch by using a synthetic branching graft with only a median sternotomy. RESULTS: One patient died, and 18 were discharged after full recovery. Postoperative computed tomographic scans showed that no residual false lumina were present proximal to the diaphragmatic level, and no false lumina were found in 10 patients. Two patients with acute ischemia of the right kidney caused by narrowing of the true lumen, as demonstrated by radiographic computed tomography, improved significantly after surgical intervention with restoration of blood flow in the true lumen. Paraplegia was not observed in any patient. CONCLUSIONS: In emergency operations for an acute type A aortic dissection, the operation is often limited to replacing the ascending aorta because priority is given to saving the patient's life. However, it is possible to perform extensive primary repair of the thoracic aorta with relative safety by interpolating a synthetic graft with a self-expandable stent.

Repair of the thoracic aorta by transaortic stent grafting (open stenting).

BACKGROUND: The late results of direct open stent grafting of the aortic arch for aortic arch repair have not been reported previously. METHODS: Between September 1997 and December 2000 19 patients underwent open stent grafting with carotid artery bypass for thoracic arteriosclerotic aneurysms (TAA) of the distal aortic arch. In addition, 21 patients underwent open stent grafting with total aortic arch replacement for Stanford type A acute aortic dissection and 7 patients underwent stenting with carotid bypass for Stanford type B chronic aortic dissection. RESULTS: The early mortality rate was 11% for TAA, 10% for type A dissection, and 0% for type B dissection. Whereas none of the TAA or type A dissection required a second operation on the thoracic aorta, 1 TAA patient died 6 months postoperatively after sudden aortic rupture and 1 type B patient required descending aortic replacement because of ulceration caused by the stent graft at 11 months postoperatively. On follow-up computed tomography scan, in TAA patients, true aneurysms excluded by the stent graft showed early thrombosis, but the absorption of thrombosed aneurysms started from 1 to 6 months postoperatively and gradually progressed. In patients with type B chronic dissection, the false lumen showed early thrombosis and the true lumen was dilated at the central portion of the graft, which might increase turbulent flow by interaction with the stent. In patients with type A acute dissection, the false lumen showed both early thrombosis and early absorption. CONCLUSIONS: Early and late results of open stenting are acceptable and follow-up computed tomography scan may be able to predict late results of open stenting.