Ultrasound findings in infantile systemic hyalinosis

To describe clinical and ultrasound findings in a patient with infantile systemic hyalinosis (ISH). A 5-month-old boy was evaluated of joint contractures. In addition to clinical and laboratory investigations, an ultrasound of his joints was done and compared to a child with similar age. On examination, a short neck, gingival hyperplasia and papular rash were noted. Joint examination showed painful passive movement, reduced range of motion, and joint contractures in knees, elbows, and small joints of the hands, without any evidence of synovial thickness. Ultrasound of the affected joints showed irregular cortical surface of MCPs and PIP, the presence of osteophytes and bone erosions, increased synovial fluid without evidence of synovial hyperplasia. This is the first report to show evidence of US findings in ISH. Ultrasound findings may help to distinguish ISH from JIA in early stages.

Microsurgical anatomy of the safe entry zones on the anterolateral brainstem related to surgical approaches to cavernous malformations

OBJECTIVE: To study the microanatomy of the brainstem related to the different safe entry zones used to approach intrinsic brainstem lesions. METHODS: Ten formalin-fixed and frozen brainstem specimens (20 sides) were analyzed. The white fiber dissection technique was used to study the intrinsic microsurgical anatomy as related to safe entry zones on the brainstem surface. Three anatomic landmarks on the anterolateral brainstem surface were selected: lateral mesencephalic sulcus, peritrigeminal area, and olivary body. Ten other specimens were used to study the axial sections of the inferior olivary nucleus. The clinical application of these anatomic nuances is presented. RESULTS: The lateral mesencephalic sulcus has a length of 7.4 to 13.3 mm (mean, 9.6 mm) and can be dissected safely in depths up to 4.9 to 11.7 mm (mean, 8.02 mm). In the peritrigeminal area, the distance of the fifth cranial nerve to the pyramidal tract is 3.1 to 5.7 mm (mean, 4.64 mm). The dissection may be performed 9.5 to 13.1 mm (mean, 11.2 mm) deeper, to the nucleus of the fifth cranial nerve. The inferior olivary nucleus provides safe access to lesions located up to 4.7 to 6.9 mm (mean, 5.52 mm) in the anterolateral aspect of the medulla. Clinical results confirm that these entry zones constitute surgical routes through which the brainstem may be safely approached. CONCLUSION: The white fiber dissection technique is a valuable tool for understanding the three-dimensional disposition of the anatomic structures. The lateral mesencephalic sulcus, the peritrigeminal area, and the inferior olivary nucleus provide surgical spaces and delineate the relatively safe alleys where the brainstem can be approached without injuring important neural structures.

Enterohormonal changes after digestive adaptation: Five-year results of a surgical proposal to treat obesity and associated diseases

Background Recent physiological knowledge allows the design of bariatric procedures that aim at neuroendocrine changes instead of at restriction and malabsorption. Digestive adaptation is a surgical technique for obesity based in this rationale. Methods The technique includes a sleeve gastrectomy, an omentectomy and a jejunectomy that leaves initial jejunum and small bowel totaling at least 3 m (still within normal variation of adult human bowel length). Fasting ghrelin and resistin and fasting and postprandial GLP-1 and PYY were measured pre- and postoperatively. Results Patients: 228 patients with initial body mass index (BMI) varying from 35 to 51 kg/m(2); follow-up: I to 5 years; average EBMIL% was 79.7% in the first year; 77.7% in the second year; 71.6% in the third year; 68.9% in the fourth year. Patients present early satiety and major improvement in presurgical comorbidities, especially diabetes. Fasting ghrelin and resistin were significantly reduced (P<0.05); GLP-1 and PYY response to food ingestion was enhanced (P<0.05). Surgical complications (4.4%) were resolved without sequela and without mortality. There was neither diarrhea nor detected malabsorption. Conclusions Based on physiological and supported by evolutionary data, this procedure creates a proportionally reduced gastrointestinal (GI) tract that amplifies postprandial neuroendocrine responses. It leaves basic GI functions unharmed. It reduces production of ghrelin and resistin and takes more nutrients to be absorbed distally enhancing GLP-1 and PYY secretion. Diabetes was improved significantly without duodenal exclusion. The patients do not present symptoms nor need nutritional support or drug medication because of the procedure, which is safe to perform.

Endoscopic Anatomy of the Pterygopalatine Fossa and the Transpterygoid Approach: Development of a Surgical Instruction Model

Introduction: The pterygopalatine fossa (PPF) is a narrow space located between the posterior wall of the antrum and the pterygoid plates. Surgical access to the PPF is difficult because of its protected position and its complex neurovascular anatomy. Endonasal approaches using rod lens endoscopes, however, provide better visualization of this area and are associated with less morbidity than external approaches. Our aim was to develop a simple anatomical model using cadaveric specimens injected with intravascular colored silicone to demonstrate the endoscopic anatomy of the PPF. This model could be used for surgical instruction of the transpterygoid approach. Methods: We dissected six PPF in three cadaveric specimens prepared with intravascular injection of colored material using two different injection techniques. An endoscopic endonasal approach, including a wide nasoantral window and removal of the posterior antrum wall, provided access to the PPF. Results: We produced our best anatomical model injecting colored silicone via the common carotid artery. We found that, using an endoscopic approach, a retrograde dissection of the sphenopalatine artery helped to identify the internal maxillary artery (IMA) and its branches. Neural structures were identified deeper to the vascular elements. Notable anatomical landmarks for the endoscopic surgeon are the vidian nerve and its canal that leads to the petrous portion of the internal carotid artery (ICA), and the foramen rotundum, and V2 that leads to Meckel`s cave in the middle cranial fossa. These two nerves, vidian and V2, are separated by a pyramidal shaped bone and its apex marks the ICA. Conclusion: Our anatomical model provides the means to learn the endoscopic anatomy of the PPF and may be used for the simulation of surgical techniques. An endoscopic endonasal approach provides adequate exposure to all anatomical structures within the PPF. These structures may be used as landmarks to identify and control deeper neurovascular structures. The significance is that an anatomical model facilitates learning the surgical anatomy and the acquisition of surgical skills. A dissection superficial to the vascular structures preserves the neural elements. These nerves and their bony foramina, such as the vidian nerve and V2, are critical anatomical landmarks to identify and control the ICA at the skull base.

Neoskin development in the fetus with the use of a three-layer graft: an animal model for in utero closure of large skin defects

Objective: To assess the ability of a three-layer graft in the closuse of large fetal skin defects. Methods: Ovine fetuses underwent a large (4 x 3 cm) full-thickness skin defect over the lumbar region at 105 days` gestation (term = 140 days). A bilaminar artificial skin was placed over a cellulose interface to cover the defect (3-layer graft). The skin was partially reapproximated with a continuous nylon suture. Pregnancy was allowed to continue and the surgical site was submitted to histopathological analysis at different post-operative intervals. Results: Seven fetuses underwent surgery. One maternal/fetal death occurred, and the remaining 6 fetuses were analyzed. Artificial skin adherence to the wound edges was observed in cases that remained in utero for at least 15 days. Neoskin was present beneath the silicone layer of the bilaminar artificial skin. Conclusions: Our study shows that neoskin can develop in the fetus using a 3-layer graft, including epidermal growth beneath the silicone layer of the bilaminar skin graft. These findings suggest that the fetus is able to reepithelialise even large skin defects. Further experience is necessary to assess the quality of this repair.

Sonographic Evaluation of the Abductor Mechanism After Total Hip Arthroplasty

Objective. The purpose of this series was to determine the frequency of abductor mechanism avulsion by sonography after total hip arthroplasty with the Hardinge approach (J Bone Joint Surg Br 1982; 64:17-19) and its relationship to the presence of insufficiency of this musculature in the postoperative period. Methods. Thirty-four consecutive patients were prospectively accessed in the postoperative period of hip arthroplasty by the Trendelenburg test, hip sonography, and abductor muscle electromyography. In patients who were found to have clinical insufficiency of the abductor musculature, we also measured the femoral offset in the preoperative and postoperative radiographs. Hip sonography was performed by an experienced musculoskeletal radiologist blinded to the other tests, and the tendons of the gluteus medius and gluteus minimus were visualized on longitudinal and transverse sections with a 7- to 10-MHz linear transducer. Results. Eight patients presented clinical insufficiency of the abductor musculature as detected by the Trendelenburg test. Four of these 8 patients with abductor insufficiency presented tendinous avulsion detected by sonography. One of the 4 patients with abductor insufficiency and normal sonographic findings had a decrease in the femoral offset caused by the arthroplasty itself. Two patients presented electromyographic changes of the abductor musculature, with no tendinous avulsion detected by sonography and no abductor insufficiency. Conclusions. We concluded that in patients undergoing total hip arthroplasty by the Hardinge approach in whom insufficiency of the abductor musculature develops, sonography is an interesting method of investigation because it identified the cause of this problem in most of our patients.

Descriptive findings on the morphology of dendritic spines in the rat medial amygdala

The rat posterodorsal medial amygdala (MePD) is a brain area in which gonadal hormones induce notable plastic effects in the density of dendritic spines. Dendritic spines are post-synaptic specializations whose shape and spacing change neuronal excitability. Our aim was to obtain new data on the dendritic spines morphology and density from MePD neurons using the carbocyanine dye Dil under confocal microscopy. In adult male rats, the dendritic spine density of the medial branches of the left MePD (mean +/- SD) was 1.15 +/- 0.67 spines/dendritic mu m. From the total sampled, approximately 53% of the spines were classified as thin, 22.5% as ""mushroom-like"", and 21.5% as stubby/wide. Other spine shapes (3%) included those ramified, with a filopodium-like or a gemule appearance, and others with a protruding spinule. Additional experiment joining Dil and synaptophysin (a pre-synaptic protein) labeling suggested synaptic sites on dendritic shafts and spines. Dendritic spines showed synaptophysin puncta close to their head and neck, although some spines had no evident labeled puncta on them or, conversely, multiple puncta appeared upon one spine. These results advance previous light microscopy results by revealing features and complexities of the dendritic spines at the same time that give new insight on the possible synaptic organization of the adult rat MePD. (C) 2010 Elsevier Ireland Ltd. All rights reserved.

Utility of Ictal Single Photon Emission Computed Tomography in Mesial Temporal Lobe Epilepsy With Hippocampal Atrophy: A Randomized Trial

BACKGROUND: The development of newer diagnostic technologies has reduced the need for invasive electroencephalographic (EEG) studies in identifying the epileptogenic zone, especially in adult patients with mesial temporal lobe epilepsy and hippocampal sclerosis (MTLE-HS). OBJECTIVE: To evaluate ictal single photon emission computed tomography (SPECT) in the evaluation and treatment of patients with MTLE-HS. METHODS: MTLE patients were randomly assigned to those with (SPECT, n = 124) and without ictal SPECT (non-SPECT, n = 116) in an intent-to-treat protocol. Primary end points were the proportion of patients with invasive EEG studies, and those offered surgery. Secondary end points were the length of hospital stay and the proportion of patients with secondarily generalized seizures (SGS) during video-EEG, postsurgical seizure outcome, and hospital cost. RESULTS: The proportion of patients offered surgery was similar in the SPECT (85%) and non-SPECT groups (81%), as well as the proportion that had invasive EEG studies (27% vs 23%). The mean duration of hospital stay was 1 day longer for the SPECT group (P < 0.001). SGS occurred in 51% of the SPECT and 26% of the non-SPECT group (P < 0.001). The cost of the presurgical evaluation was 35% higher for the SPECT compared with the non-SPECT group (P < 0.001). The proportion of patients seizure-free after surgery was similar in the SPECT (59%) compared with non-SPECT group (54%). CONCLUSION: Ictal-SPECT did not add localizing value beyond what was provided by EEG-video telemetry and structural MRI that altered the surgical decision and outcome for MTLE-HS patients. Ictal-SPECT increased hospital stay was associated with increased costs and a higher chance of SGS during video-EEG monitoring. These findings support the notion that a protocol including ictal SPECT is equivalent to one without SPECT in the presurgical evaluation of adult patients with MTLE-HS.

Pediatric epilepsy surgery and sudden unexpected death epilepsy: the contribution of a Brazilian epilepsy surgery program

Individuals with epilepsy are at higher risk of death than those from the general population, and sudden unexpected death in epilepsy (SUDEP) is the most important direct epilepsy-related cause of death. Epilepsies in the pediatric group are more frequently associated with known potentially risk factors for SUDEP, and a treatment resulting in an improved seizure control may also decrease mortality. The aim of this study is to identify the incidence of SUDEP in a group of operated-on children and adolescents. We analyzed 267 patients up to 18 years old, with medically intractable epilepsy submitted to surgery. We considered the age at surgery, the seizure type, the pathological findings, and the seizure outcome. Data were prospectively collected, according to the protocols of our institution`s ethics committee. The percentage of boys was 58.05. A good outcome was achieved in 72.6% of the cases and a bad outcome in 27.4%. Nine patients died during follow-up, six from clinical complications, and one from SUDEP. All patients who died during the long-term follow-up had persisted with refractory postoperative seizures. The patient who died from SUDEP died during a generalized tonic-clonic seizure. Of the patients, 72.6% had excellent postoperative outcome, and one patient died of SUDEP. All patients who died had had disabling seizures` persistence. The surgical treatment of epilepsy in children and adolescents is an efficient therapy for the medically intractable symptomatic epilepsies and also for the reduction of mortality and SUDEP risks.

Mucoepidermoid Carcinoma of the Lung Arising at the Primary Site of A Bronchogenic Cyst: Clinical, Cytogenetic, and Molecular Findings

Primary lung tumors are rare in children, and mucoepidermoid carcinoma (MEC) represents less than 10% of them. Additionally, MEC arising from bronchogenic cysts (BC) is particularly unusual. We describe the clinical and genetic findings on a MEC occurring within a previous location of a BC in an adolescent. This particular association has not been previously reported. The lesion revealed normal karyotype without the typical t(11;19)(q21;p13) translocation. Cyclin D1 overexpression (165-fold increase) was demonstrated by real-time PCR although FISH assessment showed normal hybridization at 11q13. Information on these unusual clinical presentations may present relevant insight on tumorigenesis of infrequent pediatric pulmonary tumors. Pediatr Blood Cancer 2011;56:311-313. (C) 2010 Wiley-Liss, Inc.

MRI findings of prostate stromal tumour of uncertain malignant potential: a case report

Prostatic stromal tumours are rare neoplasias that include benign, malignant and borderline lesions. Stromal tumour of uncertain malignant potential (STUMP) has been recently described and only a few reports exist in the literature. As a rare and distinct neoplasia, to date, there is no description of MRI findings of prostate STUMP. In this article, we describe the clinical and MRI features with histopathological correlation of a patient with prostate STUMP.

Masticatory muscle function three years after surgical correction of class III dentofacial deformity

Individuals with dentofacial deformities have masticatory muscle changes. The objective of the present study was to determine the effect of interdisciplinary treatment in patients with dentofacial deformities regarding electromyographic activity (EMG) of masticatory muscles three years after surgical correction. Thirteen patients with class III dentofacial deformities were studied, considered as group PI (before surgery) and group P3 (3 years to 3 years and 8 months after surgery). Fifteen individuals with no changes in facial morphology or dental occlusion were studied as controls. The participants underwent EMG examination of the temporal and masseter muscles during mastication and biting. Evaluation of the amplitude interval of EMG activity revealed a difference between P1 and P3 and no difference between P3 and the control group. In contrast, evaluation of root mean square revealed that, in general, P3 values were higher only when compared with PI and differed from the control group. There was an improvement in the EMG activity of the masticatory muscles, mainly observed in the masseter muscle, with values close to those of the control group in one of the analyses.

Masseter muscle thickness three years after surgical correction of class III dentofacial deformity

Objective: To analyse the effect of integrated orthodontic treatment, orthognathic surgery and orofacial myofunctional therapy on masseter muscle thickness in patients with class III dentofacial deformity three years after orthognathic surgery. Design: A longitudinal study was conducted on 13 patients with class III dentofacial deformities, denoted here as group P1 (before surgery) and group P3 (same patients 3 years to 3 years and 8 months after surgery). Fifteen individuals with no changes in facial morphology or dental occlusion were assigned to the control group (CG). Masseter muscle ultrasonography was performed in the resting and biting situations in the three groups. Data were analysed statistically by a mixed-effects linear model considering a level of significance of P < 0.05. Results: Significantly higher values (P < 0.01) of masseter muscle thickness (cm) were detected in group P3 (right rest: 0.82 +/- 0.16, left rest: 0.87 +/- 0.21, right bite: 1 +/- 0.22, left bite: 1.04 +/- 0.28) compared to group P1 (right rest: 0.63 +/- 0.19, left rest: 0.64 +/- 0.15, right bite: 0.87 +/- 0.16, left bite: 0.88 +/- 0.14). Between P3 and CG (right rest: 1.02 +/- 0.19, left rest: 1 +/- 0.19, right bite: 1.18 +/- 0.22, left bite: 1.16 +/- 0.22) there was a significant difference on the right side of the muscle (P < 0.05) in both situations and on the left side at rest. Conclusion: The proposed treatment resulted in improved masseter muscle thickness in patients with class III dentofacial deformity. (C) 2011 Elsevier Ltd. All rights reserved.

Adjunctive benefits of systemic etoricoxib in non-surgical treatment of aggressive periodontitis: Short-term evaluation

Background: This pilot study assessed the effect of short-duration treatment with etoricoxib as adjuvant therapy to scaling and root planing (SRP) on the clinical and radiographic parameters and prostaglandin E-2 (PGE(2)) levels in aggressive periodontitis. Methods: Subjects were randomly allocated to test or control treatment (n = 10 in each group) and submitted to SRP and treatment with etoricoxib, 120 mg/day, or placebo for 7 days. Probing depth, clinical attachment level (CAL), gingival recession, visible plaque index, bleeding on probing, linear distance (LD) from the cemento-enamel junction to the alveolar crest, and analysis of the gray levels were recorded before and 1 month after the therapies. The prostaglandin E-2 (PGE(2)) level in the gingival crevicular fluid (GCF) was measured by radioimmunoassay at the beginning of the study and 7 and 30 days after treatment. Results: No significant difference in the clinical parameters was observed between the groups at the end of the experimental period, although both groups presented significant improvement in all variables examined. There was a decrease in CAL from 5.54 +/- 0.47 mm to 3.59 +/- 0.53 mm in the test group and from 5.92 +/- 1.10 mmto 3.69 +/- 0.80 mm in the control group. A significant reduction in PGE(2) was found after 7 days of treatment. LD differed between the groups. Conclusion: Etoricoxib did not promote additional improvement in the clinical parameters; however, it produced an initial reduction in the PGE(2) levels in the GCF, which could be related to the discrete improvement in the bone condition.