External biliary fistula.

External biliary fistulas, once common, are now rare: before the present report of 4 cases only 27 cases have been reported in the English literature since 1900. Review of the records of four patients with external biliary fistula confirmed its occurrence in patients over 50 years of age and the variable site for operning of the fistulous tract. Cholecystectomy provided successful treatment in three of the four patients but the fourth was too ill to undergo an operation; in general, definitive treatment is cholecystectomy, together with excision of the fistulous tract if this takes a direct path through the abdominal wall from the gallbladder, or curettage if the course is devious.     

儿童胃肠间质瘤治疗进展

胃肠道间质瘤(Gastrointestinal stromal tumors, GIST)是最常见的间叶源性肿瘤。最新统计结果显示,其全球发病率约5~19/106。成人患者临床表现常无特异性,患者多有腹痛、血便、腹胀等症状。GIST主要发生在中老年群体,在儿科极为罕见,因此儿童患者经常被漏诊或误诊。随着近年来基因检测的广泛应用,以及有关儿童GIST研究的深入,发现它代表着一个不同于成人GIST的临床疾病。成人GIST当前主要治疗方式以手术为主,同时根据患者病情应用靶向药物辅助治疗。然而对于儿科患者,一些用于成人肿瘤的治疗方案难以达到理想的治疗效果,甚至会造成不良后果。儿童胃肠道间质瘤的独特生物学特性,决定了它需要独特的治疗方案,以达到最佳的临床疗效。本综述旨在研讨最新文献,提供不同的儿童胃肠道间质瘤的治疗方式。     

Group B streptococcus mycotic aneurysm of the abdominal aorta: report of a case and review of the literature

Mycotic aneurysm of the aorta is an uncommon condition, and Group B Streptococcus (GBS) is exceedingly rare in this setting. We present the first reported case of a GBS-infected abdominal aortic aneurysm (AAA) in North America. Key clinical and imaging findings and pathologic correlation are highlighted. A relevant review of the literature is discussed, which will bring the reader up to date with this specific disease entity.     

有上腹部手术史的腹腔镜胆囊切除术探讨

目的:探讨有上腹部手术史的腹腔镜胆囊切除术(LC)的临床经验。方法:对87例有上腹部手术史的腹腔镜胆囊切除术的临床资料进行回顾性分析。结果:84例顺利完成腹腔镜手术,3例中转开腹手术,其中因气腹建立困难1例,胆囊三角区解剖不清2例,所有病人恢复顺利,行LC术后2～4d出院,中转开腹5～7d出院。结论:只要术前充分准备和评估,灵活选择穿刺孔,有效的腹腔粘连松解,术者具备高超的腔镜操作技术和丰富的胆道手术经验,有上腹部手术史的LC术是安全,可行的。     

Neonatal appendicitis mimicking intestinal duplication: a case report

ABSTRACT: INTRODUCTION: Acute appendicitis is a common disease in older children but rare in neonates. CASE PRESENTATION: We report the case of a 2-day-old Asian baby who suffered from neonatal appendicitis mimicking intestinal duplication. Laparoscopic appendectomy was successfully performed after the trans-umbilical division of adhesions, and the postoperative course was uneventful. CONCLUSION: There are few reports describing abdominal masses caused by appendicitis mimicking intestinal duplication. The laparoscopic approach for neonatal appendicitis is considered to be a safe and useful therapeutic modality with good cosmetic results.     

Presentation of two patients with malignant granulosa cell tumors, with a review of the literature

ABSTRACT: Granulosa cell tumors (GCTs) of the ovary account for 2 to 5 of ovarian malignancies. We present two patients with malignant ovarian adult GCT. In one patient, a combination of bleomycin, etoposide, and cisplatin was effective after initial surgery for malignant GCT. In the other, an aromatase inhibitor was effective for recurrent malignant GCT. We also review the literature for further management of this tumor. Because GCT of the ovary is rare, it will be necessary to elucidate the clinical phenotype and establish treatment protocols by accumulating and analyzing more patients.     

Hypoganglionosis in pregnancy: a case report

ABSTRACT: INTRODUCTION: We report a very rare case of isolated hypoganglionosis first diagnosed during early pregnancy, which should be discussed from an obstetric and a gastroenterological point of view. CASE PRESENTATION: A pregnant 18-year-old Caucasian woman presented at twelve weeks of gestation with lower abdominal pain, mild constipation and a large abdominal mass. Abdominal and pelvic magnetic resonance imaging demonstrated a megarectum and megasigmoid, and our patient was managed with medical therapy during her pregnancy, which occurred without major incidents. At the onset of labor, a fecaloma obstructing the pelvic outlet was detected, which required manual disimpaction. However, during the procedure a sudden continuous fetal bradycardia was detected. An emergency Cesarean section was performed but the fetus suffered hypoxic ischemic encephalopathy. One year after the delivery, our patient underwent a sigmoid resection. A histopathological analysis revealed a reduction of nerve cells in the myenteric and submucous plexus, suggesting hypoganglionosis. CONCLUSION: Although there are some reports of pregnancies complicated by megacolon, they are too few and too old to delineate guidelines for clinical orientation. In our article, we discuss several issues regarding the management of these rare intestinal innervation disorders during pregnancy that we believe will enhance their obstetric and gastroenterological management during pregnancy.     

多层螺旋CT检查与腹部X线平片对急性肠梗阻的诊断价值比较研究

目的:研究比较多层螺旋CT(MSCT)检查与腹部X线平片对急性肠梗阻(AIO)的诊断价值。方法:选择2016年1月到2018年4月间在蚌埠医学院附属阜阳医院接受手术治疗的200例AIO患者作为研究对象,对所有患者先常规予以腹部X线平片诊断,12h后再通过MSCT为患者实施诊断,对比两种方法的诊断结果、诊断体验效果以及漏诊率和误诊率。结果:MSCT的肠梗阻检出率为94.50%,明显较腹部X线平片的69.00%更高(P0.05)。MSCT所诊断的肠梗阻中,梗阻类型为绞窄型及梗阻病因为肠肿瘤者均占100.00%,较腹部X线平片的36.21%和54.26%明显更高(P0.05)。MSCT的诊断舒适度评分、图像清晰度评分较腹部X线平片明显更高,而操作复杂度评分较腹部X线平片明显更低(P0.05)。MSCT的漏诊率、误诊率分别为4.00%、1.50%,较腹部X线平片的22.00%、9.00%明显降低(P0.05)。结论:对于AIO患者,MSCT较腹部X线平片具有更高的诊断价值,诊断体验效果更好,漏诊率和误诊率偏低。     

Complete gallbladder torsion diagnosed with sequential computed tomography scans: a case report

ABSTRACT: INTRODUCTION: Torsion of the gallbladder is an extremely rare cause of acute abdomen, which commonly affects thin elderly women. A prompt surgical approach is necessary to avoid fatal complications associated with gangrene and perforation of the gallbladder. However, it is difficult to make a preoperative diagnosis using ordinary imaging modalities. CASE PRESENTATION: An 84-year-old Japanese woman was admitted to our hospital due to left lower abdominal pain. Her pain shifted suddenly to the right upper abdomen a half day after admission. Although her enlarged and wall-thickened gallbladder had been already seen at admission, it rotated approximately 180 degrees and deviated to the midline of her abdomen on the second computed tomography scan, which helped us to make a correct diagnosis of gallbladder torsion. The patient underwent an emergency operation (detorsion and cholecystectomy) and recovered without any complications. The gallbladder had necrosis due to torsion. CONCLUSION: Sequential diagnostic imaging might be helpful to make a preoperative diagnosis of gallbladder torsion when the gallbladder is enlarged and wall thickened but the patient does not present with typical clinical symptoms.     

La cholécystite et la cholélithiase chez l'enfant

Cholecystitis and cholelithiasis in children would appear to be a rare surgical finding; however, the authors have encountered 15 cases. Although in approximately half the children with this condition the symptomatology is sufficient to draw attention to the biliary tract, in the other half the presence of periumbilical or right lower quadrant abdominal tenderness can easily be misleading. Only by considering the use of cholecystography will one be in a position to make a diagnosis of cholelithiasis in these latter cases.Among the possible causally associated factors in the adult, sex (female) and obesity alone may be applicable in children. Cholecystectomy with peroperative cholangiography is the treatment of choice, even though the occurrence of migratory calculi in the common bile duct is exceptional.     

Prospective controlled randomized trial on prevention of postoperative abdominal adhesions by Icodextrin 4% solution after laparotomic operation for small bowel obstruction caused by adherences [POPA study: Prevention of Postoperative Adhesions on behalf of the World Society of Emergency Surgery]

Background

In some randomized trials successful laparoscopic cholecystectomy for cholecystitis is associated with an earlier recovery and shorter hospital stay when compared with open cholecystectomy. Other studies did not confirm these results and showed that the potential advantages of laparoscopic cholecystectomy for cholecystitis can be offset by a high conversion rate to open surgery. Moreover in these studies a similar postoperative programme to optimize recovery comparing laparoscopic and open approaches was not standardized. These studies also do not report all eligible patients and are not double blinded.

Design

The present study project is a prospective, randomized investigation. The study will be performed in the Department of General, Emergency and Transplant Surgery St Orsola-Malpighi University Hospital (Bologna, Italy), a large teaching institutions, with the participation of all surgeons who accept to be involved in (and together with other selected centers). The patients will be divided in two groups: in the first group the patient will be submitted to laparoscopic cholecystectomy within 72 hours after the diagnosis while in the second group will be submitted to laparotomic cholecystectomy within 72 hours after the diagnosis.

Trial Registration

TRIAL REGISTRATION NUMBER ISRCTN27929536 – The ACTIVE (Acute Cholecystitis Trial Invasive Versus Endoscopic) study. A multicentre randomised, double-blind, controlled trial of laparoscopic versus open surgery for acute cholecystitis in adults.     

Clinical-epidemiological profile of children with schistosomal myeloradiculopathy attended at the Instituto Materno-Infantil de Pernambuco

The most critical phase of exposure to schistosomal infection is the infancy, because of the more frequent contact with contaminated water and the immaturity of the immune system. One of the most severe presentations of this parasitosis is the involvement of the spinal cord, which prognosis is largely dependent on early diagnosis and treatment. Reports on this clinical form of schistosomiasis in children are rare in the literature. We present here the clinical-epidemiological profile of schistosomal myeloradiculopathy (SMR) from ten children who were admitted at the Instituto Materno-Infantil de Pernambuco over a five-year period. They were evaluated according to an investigation protocol. Most of these patients presented an acute neurological picture which included as the main clinical manifestations: sphincteral disorders, low back and lower limbs pain, paresthesia, lower limbs muscle weakness and absence of deep tendon reflex, and impairment of the gait. The diagnosis was presumptive in the majority of the cases. This study emphasizes the importance of considering the diagnosis of SMR in pediatric patients coming from endemic areas who present a low cord syndrome, in order to start the appropriate therapy and avoid future complications.     

Paraneoplastic limbic encephalitis presenting as a neurological emergency: a case report

Introduction

Dentin dysplasia is a rare hereditary disturbance of dentin formation characterized by defective dentin development with clinically normal appearing crowns, severe hypermobility of teeth and spontaneous dental abscesses or cysts. Radiographic analysis shows obliteration of all pulp chambers, short, blunted and malformed or absent roots and peri-apical radiolucencies of non carious teeth.

Case presentation

We present a case of dentin dysplasia type I in a 12-year-old Iranian boy, and the clinical, radiographic and histopathologic findings of this condition and treatment are described.

Conclusions

There are still many inconclusive issues in the diagnosis and management of patients with dentin dysplasia. The diagnostic features of this rare disturbance will remain incompletely defined until additional cases have been described. Early diagnosis of the condition and initiation of effective regular dental treatments may help these patients to prevent or delay loss of dentition.     

Idiopathic scrotal calcinosis: a non-elucidated pathogenesis and its surgical treatment

Scrotal calcinosis (SC) is a rare, benign entity defined as the presence of multiple calcified nodules within the scrotal skin. In most cases, there are no associated symptoms. We report the case of 27-year-old man with a massive SC. Treatment was surgical with complete excision of the affected part of the scrotum wall. Histopathologically, there was no epithelial lining around the calcified nodules and no cystic structure. Therefore, our case was considered idiopathic SC.     

Chronic osteomyelitis of the clavicle

Osteomyelitis of the clavicle is an uncommon disease, but it should be considered in patients who present with pain, cellulitis, or drainage in the sternoclavicular area following head and neck surgery, irradiation, subclavian vein catheterization, or immunosuppression. An idiopathic presentation is possible. In contrast to primary osteomyelitis of the clavicle, which is occasionally seen in children, secondary osteomyelitis is quite rare. It is often mistaken for a fracture or a possible neoplasm on plain x-rays. Tomograms and CT scanning are confirmatory, and in early cases, technetium-99m bone scanning can be helpful. Treatment must include early, aggressive surgical debridement of all affected tissues, followed by wound coverage with a well-vascularized flap and perioperative antibiotics.     

Limb‐body wall defect: Experience of a reference service of fetal medicine from Southern Brazil

Background: Limb‐body wall defect is a rare condition characterized by a combination of large and complex defects of the ventral thorax and abdominal wall with craniofacial and limb anomalies. Methods: The aim of this study was to describe the experience of our fetal medicine service, a reference from Southern Brazil, with prenatally diagnosed patients with a limb‐body wall defect in a 3 years period. Only patients who fulfilled the criteria suggested by Hunter et al. (2011) were included in the study. Clinical data and results of radiological and cytogenetic evaluation were collected from their medical records. Results: Our sample was composed of 8 patients. Many of their mothers were younger than 25 years (50%) and in their first pregnancy (62.5%). It is noteworthy that one patient was referred due to suspected anencephaly and another due to a twin pregnancy with an embryonic sac. Craniofacial defects were verified in three patients (37.5%), thoracic/abdominal abnormalities in 6 (75%) and limb defects in eight (100%). Congenital heart defects were observed in five patients (62.5%). One of them presented a previously undescribed complex heart defect. Conclusion: The results disclosed that complementary exams, such as MRI and echocardiography, are important to better define the observed defects. Some of them, such as congenital heart defects, may be more common than previously reported. This definition is essential for the proper management of the pregnancy and genetic counseling of the family. The birth of these children must be planned with caution and for the prognosis a long survival possibility, despite unlikely and rare, must be considered. Birth Defects Research (Part A) 100:739–749, 2014. © 2014 Wiley Periodicals, Inc.     

Cytologic diagnosis of hemophagocytic lymphohistiocytosis in ascitic fluid: a case report

BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH), also known as macrophage activation syndrome, is a rare, fatal hematopoietic disease. Its cytologic features may be subtle because the abnormal histiocytes may not be recognized if one is not aware of this entity. We report a case of HLH involving the ascitic fluid. CASE REPORT: A 73-year-old man developed weakness, lethargy, decreased appetite and progressive shortness of breath after a cholecystectomy. Physical examination revealed hypotension, tachycardia and chest dullness with decreased breath sounds bilaterally. Radiologic examination revealed bilateral pleural effusions. The patient accumulated fluid in the peritoneal cavity, lungs, retroperitoneum and mediastinum. Bone marrow biopsy showed abundant histiocytes infiltrating the marrow cavity, and many of these histiocytes contained cellular debris. A diagnosis of HLH was therefore made. The abdominal paracentesis specimen contained many similar histiocytes exhibiting erythrophagocytosis and lymphophagocytosis. These abnormal histiocytes were positive for CD68 and negative for AE1/AE3, confirming the diagnosis of HLH. The patient died soon after from disseminated aspergillosis. CONCLUSION: HLH is cytologically characterized by the presence of abnormal histiocytes with ingested cellular debris. In serous effusions they should not be confused with mesothelial cells. Immunohistochemical studies may help confirm the diagnosis.     

Etude Histologique et Microradiographique du Cartilage Hémal de la Vertebre de la Carpe, Cyprinus carpio L. (Pisces, Teleostei, Cyprinidae)

The abdominal vertebrae of the adult carp retain a bulk of cartilage at the basement of the haemapophyses. This cartilage has two opposite directions of differentiation. There is an enchondral ossification of the hypertrophic calcified cartilage in its distal area whereas its proximal area is calcifying without previous hypertrophy. The calcification of this proximal area (hyaline calcified cartilage) is permanent and shows typical rings and waves of Liesegang. The calcification of the cartilage of the hemapophyses is of a globular type. The hyaline calcified cartilage is not a metaplastic bone. Other studies, specially with electron microscope, will allow us to understand the innermost process of the different stages of calcification in the cartilage of the carp.     

Anesthetic implications of laparoscopic surgery

Minimally invasive therapy aims to minimize the trauma of any interventional process but still achieve a satisfactory therapeutic result. The development of "critical pathways," rapid mobilization and early feeding have contributed towards the goal of shorter hospital stay. This concept has been extended to include laparoscopic cholecystectomy and hernia repair. Reports have been published confirming the safety of same day discharge for the majority of patients. However, we would caution against overenthusiastic ambulatory laparoscopic cholecystectomy on the rational but unproven assumption that early discharge will lead to occasional delays in diagnosis and management of postoperative complications. Intraoperative complications of laparoscopic surgery are mostly due to traumatic injuries sustained during blind trocar insertion and physiologic changes associated with patient positioning and pneumoperitoneum creation. General anesthesia and controlled ventilation comprise the accepted anesthetic technique to reduce the increase in PaCO2. Investigators have recently documented the cardiorespiratory compromise associated with upper abdominal laparoscopic surgery, and particular emphasis is placed on careful perioperative monitoring of ASA III-IV patients during insufflation. Setting limits on the inflationary pressure is advised in these patients. Anesthesiologists must maintain a high index of suspicion for complications such as gas embolism, extraperitoneal insufflation and surgical emphysema, pneumothorax and pneumomediastinum. Postoperative nausea and vomiting are among the most common and distressing symptoms after laparoscopic surgery. A highly potent and selective 5-HT3 receptor antagonist, ondansetron, has proven to be an effective oral and IV prophylaxis against postoperative emesis in preliminary studies. Opioids remain an important component of the anesthesia technique, although the introduction of newer potent NSAIDs may diminish their use. A preoperative multimodal analgesic regimen involving skin infiltration with local anesthesia. NSAIDs to attenuate peripheral pain and opioids for central pain may reduce postoperative discomfort and expedite patient recovery/discharge. There is no conclusive evidence to demonstrate clinically significant effects of nitrous oxide on surgical conditions during laparoscopic cholecystectomy or on the incidence of postoperative emesis. Laparoscopic cholecystectomy has proven to be a major advance in the treatment of patients with symptomatic gallbladder disease.     

Failure of sequential therapy to eradicate Helicobacter pylori in previously treated subjects

BACKGROUND: Recently, studies in adults and subsequently in children have demonstrated a very high success rate for sequential therapy as a primary therapy when compared to traditional therapy regimens. METHODs: We report our experience with a sequential therapy regimen for the eradication of Helicobacter pylori in five infected children and a young adult, whose conventional therapy regimens had been unsuccessful. RESULTS: Five patients failed the sequential therapy. All of them experienced between two and four failures of traditional therapy prior to the sequential treatment protocol. The only patient who succeeded on the sequential therapy had just one previous failure. All of our patients who had failed sequential therapy achieved eradication of the bacteria with quadruple therapy. CONCLUSIONS: In view of our disappointing results, sequential therapy is unsuccessful as a therapy for children and young adults who have failed previous treatment regimens. At the present time, quadruple therapy is indicated for this group. Well-designed placebo-controlled trials are indicated to further characterize this group of patients.