Mucin-producing hepatic cystic neoplasms: an uncommon but challenging disease often misdiagnosed and mismanaged.

Background: Mucin-producing hepatic cystic neoplasms (MHCN) are uncommon and potentially malignant.Methods: Nine MHCN were encountered in our centre for over 32 years. Patients' clinical, biological, radiological and pathological features were reviewed. Lesions were classified into Mucinous Cystic Neoplasms (MCN) and Intraductal Papillary Neoplasms of the Bile duct (IPNB) (WHO 2010 classification).Results: Five MCN and 4 IPNB were reviewed. Serum and intracystic tumour markers were insufficient to diagnose malignancy. Complications were encountered in five out of nine patients (56%), mean symptom duration was 26 months (range: 1-132). Three patients were mismanaged pre-referral. Radiological features enabled preoperative diagnosis in eight out of nine patients (89%). Greater tumour size, unilocular lesion and mural nodularity indicated malignancy. Radical tumour excision was achieved in eight patients. One IPNB patient was misdiagnosed and underwent unroofing. For 103 months median follow-up, five out of six patients with benign tumours were alive and disease-free, whereas the misdiagnosed IPNB recurred with fatal malignant transformation seven years later. Among the three patients with malignancies (median follow-up: 77 months), two IPNB died, one from cancer recurrence and one from unrelated causes, whereas the malignant MCN was alive and disease-free.Conclusions: Appropriate MHCN diagnosis is crucial, yet it is often misdiagnosed and mismanaged. The prognosis after complete excision is favourable.

Reproducibility of three-dimensional posterior cranial base angles using low-dose computed tomography.

OBJECTIVES: One of the key aspects of three-dimensional (3D) craniofacial cephalometry is the measurement of posterior cranial base angle as this area is deeply involved in craniofacial development. The purpose of our retrospective study was to define the best reproducible 3D posterior cranial base angles among five 3D angles transposed from 2D cephalometry (Cousin, BL1 of Ross and Ravosa, Bjork, Delaire, CBA4 of Liberman) and seven 3D angles based on physical anthropology studies and on new concepts (R1 to R7). The null hypothesis was that all 3D posterior cranial base angles were equally reproducible. MATERIAL AND METHODS: We used a preoperative low-dose computed tomography (CT) data from 20 adult patients undergoing orthognathic surgery after approval by local ethical committee. Two independent observers performed two series of 23 3D landmark identifications on 3D CT surface rendering of each patient using Maxilim software. Then, the same observers performed twice 3D cephalometric analyses (23 landmarks, 4 midpoints, 19 planes) that provided the automatic measurement of 12 posterior cranial base angles. RESULTS: Inter-observer correlation coefficient varied from 0.545 (Cousin) to 0.695 (CBA4 of Liberman) and from -0.177 (R2) to 0.827 (R4). CONCLUSIONS: The null hypothesis was rejected. The most reproducible angle was 3D angle R4 based on "basion," "superior optic" (right, left), and "crista galli inferior" landmarks. CLINICAL RELEVANCE: R4 angle might be used as reference 3D posterior cranial base angle in further clinical studies involving 3D cephalometry as a diagnostic tool for orthodontics and for orthognathic surgery.

Three-dimensional computed tomography cephalometric craniofacial analysis: experimental validation in vitro.

The development of three-dimensional (3D) cephalometric analysis is essential for the computer-assisted planning of orthognathic surgery. The aim of this study was to transform and adapt Delaire's two-dimensional cephalometric analysis into the third dimension; this transposition was then validated. The comparative advantage of using 3D computed tomography (CT) surface renderings over profile X-rays was analysed. Comparison was made of inter- and intra-observer reproducibility of the cephalometric measurements done on profile X-rays and on 3D CT surface renderings on the same 26 dry skulls. The accuracy was also tested of the measurements done on 3D CT surface renderings (ACRO 3D) in relation to those directly taken on dry skulls with the help of a 3D measuring instrument. Inter- and intra-observer reproducibility proved significantly superior (p<0.0001) following the 3D CT method. There were no significant differences in the accuracy of measurements between the ACRO 3D software and the 3D measuring instrument. The ACRO 3D software was confirmed as being a reliable tool for developing 3D CT cephalometric analyses. Further research may entail clinical validation of the 3D CT craniofacial cephalometric method of analysis.

High suspicion of bacillary angiomatosis in a kidney transplant recipient: a difficult way to diagnose--case report.

Bacillary angiomatosis is an infection caused by Bartonella, which has first been described in human immunodeficiency virus (HIV)-infected patients. We report an unusually located lesion, in a totally asymptomatic kidney transplant recipient. The diagnosis was strongly suggested based on the iconography and our histological analysis, but was not confirmed using polymerase chain reaction (PCR) and immunohistochemical studies. We illustrate our difficult way to the diagnosis as well as the course of the disease and our therapeutic strategy.

Successful treatment of Acanthamoeba rhinosinusitis in a patient with AIDS.

Acanthamoeba infection is a rare, difficult-to-treat, and often fatal, opportunistic parasitic infection in immunocompromised hosts, such as patients infected with HIV. We describe an aggressive nasal and sinus infection by Acanthamoeba spp. in a person with AIDS. The resolution of this Acanthamoeba infection was secondary to a multidisciplinary treatment approach involving a combination of surgery as well as high-dose amphotericin B plus 5-fluorocytosine. In the era of the HIV/AIDS pandemic, the present report underscores the need for early identification and prompt aggressive treatment to ensure successful management of this rare but potentially fatal opportunistic infection.

Acute localized diverticulitis: optimum management requires accurate staging.

Between 1977 and 1989, 151 patients were treated in our institution for acute sigmoid diverticulitis. Thirty-one patients were operated on for diffuse peritonitis, and were excluded from the study. One hundred twenty patients had localized disease. There were 59 men and 61 women, with a mean age of 60 years (range, 30 to 87 years). Thirteen were under 40 years of age. A "phlegmonous" diverticulitis (no pericolic abscess) was diagnosed in 78 cases (group I). A pericolic abscess was identified in 42 cases (group II). The medical treatment was successful in 97% of the patients of the group I. Only 15 patients required a delayed elective resection for recurrence or chronic complications, within the next 24 months. There were no operative deaths. All the other patients were doing well after a mean follow-up of 5 years (9-144 months), without any disease-related death. Patients presenting with a localized pericolic abscess (group II, n = 42) were initially treated either conservatively (n = 22) or by a more or less extensive drainage (n = 20). There were two deaths in the "conservative" group. Primary or delayed colonic resection was indicated in 34 cases because of uncontrolled sepsis, recurrence or secondary chronic complications. It is concluded that accurate classification of the disease is essential. If no peritonitis has developed, the presence of an abscess is the main determinant in both prognosis and treatment. Most patients who develop an acute phlegmonous diverticulitis do well with conservative treatment, and prophylactic resection is not indicated. Curative colectomy is reserved for patients developing persistent complications over the next few months.(ABSTRACT TRUNCATED AT 250 WORDS)

Elevation of serum thyroxine-binding globulin (but not of cortisol-binding globulin and sex hormone-binding globulin) associated with the progression of human immunodeficiency virus infection.

PURPOSE: In order to assess the relation of thyroid function tests to human immunodeficiency virus (HIV) infection, we determined the levels of serum thyroid hormones, serum binding proteins [thyroxine-binding globulin (TBG), cortisol-binding globulin (CBG), and sex hormone-binding globulin (SHBG)], and serum tumor necrosis factor (TNF) in HIV-seropositive subjects at different clinical stages. PATIENTS AND METHODS: Thirty-seven HIV-seropositive patients were studied: 7 at stage II, 13 at stage III, and 17 at stage IV (eight ambulatory and nine hospitalized) according to the Centers for Disease Control's criteria. RESULTS: As compared with stage II and stage III patients, stage IV patients had significantly higher mean TBG and total thyroxine (TT4) values, similar and normal total triiodothyronine (TT3) levels, and similar and abnormally low reverse triiodothyronine (rT3) concentrations. However, stage IV hospitalized patients had significantly lower TT3 values than stage IV ambulatory patients. In contrast to TBG, mean levels of CBG and SHBG were comparable in the three groups and within normal limits. For the whole population of HIV patients, there was a highly significant correlation between the CD4 lymphocyte count and TBG (r = -0.529, p less than 0.001) but not with CBG and SHBG levels. Finally, TNF values higher than 10 pg/mL were detected in six of the 17 stage IV patients and in only one of the 13 stage III patients (p = 0.059); elevated TNF levels correlated with a lower CD4 count (p less than 0.01) but not with serum TBG levels. CONCLUSION: The progression of HIV infection is associated with an elevation of serum TNF and TBG, but not of CBG or SHBG. HIV-infected patients have an unexpectedly normal TT3-low rT3 state.

Brucella endocarditis: the role of combined medical and surgical treatment.

Brucella endocarditis, although a rare complication of brucellosis, is the main cause of death related to this disease. This report describes a case of aortic endocarditis due to Brucella abortus in an elderly farmer with known aortic stenosis. Urgent valve replacement was performed because of progressive heart failure despite appropriate antimicrobial treatment. The infection was cured with trimethoprim-sulfamethoxazole and rifampin given for 3 months after surgery. A review of the literature reports on the 38 other cases of cured brucella endocarditis made clear the need for combined antimicrobial treatment and surgical valve replacement.

Treatment by aspiration of brain abscesses.

Sixteen patients, with a total of 18 abscesses, were treated by aspiration and systemic antibiotic therapy, to which antiepileptic prophylaxis and corticosteroids were added. Diagnosis of the abscesses and monitoring of their evolution relied principally upon computed tomography; this technique was also used per-operatively when aspirating small abscesses. Bacteriological examinations were positive in all our cases and a polymicrobial flora was found in half of these. One or, eventually, two aspirations within the same week were sufficient, in most cases, to promote healing. Nevertheless, in three patients, the abscesses, however sterilized, did not show any volume reduction, probably because of adhesions to the dura mater, of a large part of the abscess surface area. One patient died from concomitant cardiac disease and one patient remained seriously disabled. Epilepsy was observed as a sequela, in six patients. This study emphasizes the role of neuroradiological and bacteriological examinations, whilst surgery may be restricted, in most cases, to a simple aspiration.