A fast, easy and reliable method for hamstrings graft size prediction in anterior cruciate ligament reconstruction.

PURPOSE: The aim of this study is to describe and validate a simple and reliable method to pre-operatively predict the size of the ACL graft in the double strand technique with autologous semitendinosus-gracilis tendons on the same MRI used for ACL rupture diagnosis. METHODS: The study included 92 patients, with a median age of 31 years (IQR 26-41 years), 73/92 (79%) of whom were males. All patients that underwent an ACL reconstruction with doubled ST + GT between 2017 and 2022 were counted in the study. RESULTS: Overall, the median predicted graft diameter from MR imaging was similar to the actual graft diameter with no significant differences (n.s.). Regarding the comparison between predicted and actual graft size, concordance was 78/92 (85%, 95% CI 76-91%), with κ = 0.797 which corresponds to a level of agreement defined as "Strong". Tendon sizes calculated on pre-operative MRI were evaluated both with intra-observer and inter-observer reliability demonstrating a statistically reproducible method. The predicted graft was then compared to the reported one with a statistically significant reliability found. CONCLUSION: This study can help the surgeons to perform a fast pre-operative planning of an ACL reconstruction for graft selection. If the planned graft with ST and GT is smaller than 8 mm, the clinician can decide to switch to a different type of graft or plan a different graft preparing technique and, therefore, reduce the risk of post-operative ligament re-rupture. The method proposed is reliable and reproducible. The major strength of the planning technique proposed is that it relies on data that are already available for the clinician before surgery, without the need of further analysis. LEVEL OF EVIDENCE: IV.

Secondary hemophagocytic lymphohystiocytosis in a Rubinstein Taybi syndrome patient.

Rubinstein-Taybi syndrome (RSTS) is an autosomal dominant disorder, caused by variants in CREBBP or EP300. Affected individuals present with distinctive craniofacial features, broad thumbs and/or halluces, intellectual disability and immunodeficiency. Here we report on one RSTS patient who experienced hemophagocytic lymphohystiocytosis (HLH) and disseminated herpes virus 1 ( HSV-1) disease. The clinical picture of RSTS is expanding to include autoinflammatory, autoimmune, and infectious complications. Prompt treatment of HLH and disseminated HSV-1 can lower the mortality rate of these life-threatening conditions.

Cerebral superb microvascular imaging in preterm neonates: in vivo evaluation of thalamic, striatal, and extrastriatal angioarchitecture.

PURPOSE: To explore the potential of superb microvascular imaging (SMI) in visualizing brain microvessels in preterm neonates of different gestational ages (GA). METHODS: In this retrospective, observational pilot study, 15 preterm newborns were equally divided into GA groups: extremely (GA < 28 weeks), very (28-31 weeks), and moderate to late (32-37 weeks) preterm. All patients underwent conventional transcranial ultrasounds during the first day of life following the American Institute of Ultrasound in Medicine practice guidelines. SMI was then performed; based on their SMI morphology and location, brain microvessels were classified as extrastriatal (cortical and medullary), striatal, or thalamic. Two examiners independently classified vessels as visible or invisible. To assess the association between vessel visibility and GA, binomial logistic regression analysis (separate for each microvessel group) was performed, taking visibility as a dependent variable and both examiners and GA as predictor variables. RESULTS: A statistically significant difference among GA groups was found in sex (P = 0.030), birth weight (P = 0.007), and Apgar score within 1 min after birth (P = 0.024). Microvascular visibility increased with GA for superficial vessels (P < 0.05 for both cortical and medullary), while striatal and thalamic vessels were visible in all neonates irrespective of their GA. CONCLUSIONS: SMI technology shows promise to assess brain microvasculature in preterm neonates, even potentially providing data on early brain development.

Precision-based exercise as a new therapeutic option for children and adolescents with haematological malignancies.

Children and adolescents with haematological malignancies (PedHM) are characterized by a severe loss of exercise ability during cancer treatment, lasting throughout their lives once healed and impacting their social inclusion prospects. The investigation of the effect of a precision-based exercise program on the connections between systems of the body in PedHM patients is the new frontier in clinical exercise physiology. This study is aimed at evaluating the effects of 11 weeks (3 times weekly) of combined training (cardiorespiratory, resistance, balance and flexibility) on the exercise intolerance in PedHM patients. Two-hundred twenty-six PedHM patients were recruited (47% F). High or medium frequency participation (HAd and MAd) was considered when a participant joined; > 65% or between 30% and < 64% of training sessions, respectively. The "up and down stairs'' test (TUDS), "6 min walking" test (6MWT), the "5 Repetition Maximum strength" leg extension and arm lateral raise test (5RM-LE and 5RM-ALR), flexibility (stand and reach), and balance (stabilometry), were performed and evaluated before and after training. The TUDS, the 5RM-LE and 5RM-ALR, and the flexibility exercises showed an increase in HAd and MAd groups (P < 0.05), while the 6MWT and balance tests showed improvement only in HAd group (P < 0.0001). These results support the ever-growing theory that, in the case of the treatment of PedHM, 'exercise is medicine' and it has the potential to increase the patient's chances of social inclusion.

The sacral chordoma margin.

OBJECTIVE: Aim of the manuscript is to discuss how to improve margins in sacral chordoma. BACKGROUND: Chordoma is a rare neoplasm, arising in half cases from the sacrum, with reported local failure in >50% after surgery. METHODS: A multidisciplinary meeting of the "Chordoma Global Consensus Group" was held in Milan in 2017, focusing on challenges in defining and achieving optimal margins in chordoma with respect to surgery, definitive particle radiation therapy (RT) and medical therapies. This review aims to report on the outcome of the consensus meeting and to provide a summary of the most recent evidence in this field. Possible new ways forward, including on-going international clinical studies, are discussed. RESULTS: En-bloc tumor-sacrum resection is the cornerstone of treatment of primary sacral chordoma, aiming to achieve negative microscopic margins. Radical definitive particle therapy seems to offer a similar outcome compared to surgery, although confirmation in comparative trials is lacking; besides there is still a certain degree of technical variability across institutions, corresponding to different fields of treatment and different tumor coverage. To address some of these questions, a prospective, randomized international study comparing surgery versus definitive high-dose RT is ongoing. Available data do not support the routine use of any medical therapy as (neo)adjuvant/cytoreductive treatment. CONCLUSION: Given the significant influence of margins status on local control in patients with primary localized sacral chordoma, the clear definition of adequate margins and a standard local approach across institutions for both surgery and particle RT is vital for improving the management of these patients.

Best practices for the management of local-regional recurrent chordoma: a position paper by the Chordoma Global Consensus Group.

Chordomas are rare, malignant bone tumors of the skull-base and axial skeleton. Until recently, there was no consensus among experts regarding appropriate clinical management of chordoma, resulting in inconsistent care and suboptimal outcomes for many patients. To address this shortcoming, the European Society of Medical Oncology (ESMO) and the Chordoma Foundation, the global chordoma patient advocacy group, convened a multi-disciplinary group of chordoma specialists to define by consensus evidence-based best practices for the optimal approach to chordoma. In January 2015, the first recommendations of this group were published, covering the management of primary and metastatic chordomas. Additional evidence and further discussion were needed to develop recommendations about the management of local-regional failures. Thus, ESMO and CF convened a second consensus group meeting in November 2015 to address the treatment of locally relapsed chordoma. This meeting involved over 60 specialists from Europe, the United States and Japan with expertise in treatment of patients with chordoma. The consensus achieved during that meeting is the subject of the present publication and complements the recommendations of the first position paper.

Long-term morbidity after multivisceral resection for retroperitoneal sarcoma.

BACKGROUND: More than 60 per cent of patients treated surgically for primary retroperitoneal sarcoma survive for at least 5 years. Extended surgical resection has been proposed for primary disease, but long-term morbidity data are lacking. A cross-sectional study was conducted to assess the long-term morbidity of patients undergoing surgery for retroperitoneal sarcoma. METHODS: Patients operated on between January 2002 and December 2011 were eligible for the study. Long-term morbidity was evaluated based on a semistructured clinical interview. Lower limb function was assessed by means of the Lower Extremity Functional Scale (LEFS), a self-report questionnaire with a total score ranging from 0 (low functioning) to 80 (high functioning). Pain was investigated by means of the Brief Pain Inventory--Short Form, with pain intensity scores reported on a scale from 0 (no pain) to 10 (worst pain). RESULTS: Some 243 patients underwent surgery, and 101 of 160 patients who were alive at the time of the investigation responded to the study invitation letter. Finally, 95 patients were enrolled in the study. Sensory impairment of the limbs was reported in 72 patients (76 per cent). The median LEFS score was 60 (i.q.r. 43-73). Mean scores for the pain intensity items varied from 1.23 to 2.68. In multivariable analysis, there was no difference in median levels of creatinine at survey between patients who did or did not undergo nephrectomy (difference between median values 13 (95 per cent c.i. -4 to 30) µmol/l; P = 0.170). CONCLUSION: Severe chronic pain and lower limb motor impairment after multivisceral resection for retroperitoneal sarcomas are rare. Long-term renal function is not significantly impaired when nephrectomy is performed.

Correlation between radiological assessment and histopathological diagnosis in retroperitoneal tumors: analysis of 291 consecutive patients at a tertiary reference sarcoma center.

OBJECTIVES: Aim of study was to assess the correlation between computed tomography scan (CT) findings and histopathology. MATERIAL AND METHODS: Data were collected on consecutive patients with suspected retroperitoneal sarcoma (RPS) referred to a tertiary sarcoma center. Patients underwent contrast enhanced multi-detector CT scans. Radiological features of lesions were classified according to the presence of a fatty (Group A) mass, or non-fatty (Group B) mass, both subdivided according to homogeneity and intralesional high-contrasted appearance. Radiological classification was compared with histopathological diagnosis. Sensitivity, specificity, positive/negative predictive value (PPV, NPV) were analyzed. RESULTS: Of 291 patients, 103/291 (35.4%) masses were classified in Group A and 188/291 (64.6%) in Group B. Diagnosis of mesenchymal tumor was obtained in 231/291 cases (79%) and non-mesenchymal tumor in 60/291 (21%). Sensitivity and specificity of Group A for liposarcoma were 76.7% and 92.0%; PPV and NPV were 86.4% and 85.6%. Sensitivity of Group B for a mesenchymal tumor was 55.4% and specificity was 0%; PPV and NPV were 68.1% and 0%. CONCLUSIONS: None of radiological criteria were sufficient to anticipate a specific diagnosis, with the only exception of well differentiated liposarcoma and angiomyolipoma. In a series of suspected RPS, 21% of the lesions were finally non-mesenchymal tumors.

Head and neck soft tissue sarcomas: prognostic factors and outcome in a series of patients treated at a single institution.

BACKGROUND: Head and neck soft tissue sarcomas (STS) represent a rare disease. PATIENTS AND METHODS: One hundred and sixty-seven patients underwent surgery at our institution with an eradicating intent between 1990 and 2010. Local recurrence (LR), distant metastasis (DM) and disease-specific mortality (DSM) incidence were studied along with clinicopathological prognostic factors. RESULTS: Ten-year crude cumulative incidence (CCI) of LR, DM and DSM were 19%, 11% and 26%, respectively (median follow-up 66 months). Independent prognostic factors for DSM were tumor size (P < 0.001) and grade (P = 0.032), while surgical margins obtained a border-line significance (0.070); LR was affected by the tumor size (P = 0.001), while DM only by grade (P = 0.047). The median survival after LR and DM were 14 months and 7 months, respectively. Tumors sited in the paranasal sinus and supraclavicular region had the worst survival. CONCLUSIONS: Head and neck represent a very critical anatomical site for STS. Achievement of local disease control appears to be crucial, since even LR could be a life-threatening event.

Frontline extended surgery is associated with improved survival in retroperitoneal low- to intermediate-grade soft tissue sarcomas.

BACKGROUND: The purpose of the study was to retrospectively reassess in our institutional series at a longer follow-up the value of a systematic attempt to carry out wide resections in retroperitoneal soft tissue sarcoma. PATIENTS AND METHODS: Three hundred and thirty-one consecutive patients surgically treated were analyzed. Since a shift toward a systematic more extended surgical approach took place starting from 2002, patients were divided in two groups according to the time of surgery. Overall survival (OS), crude cumulative incidence of local recurrence (LR) and distant metastases (DMs) were estimated. Cox model multivariate analysis was carried out. RESULTS: Five-year OS of patients operated in the recent period was 66%, compared with 48% for those operated in the previous period. This was associated with less LR (28% versus 49%), while the number of DMs was higher in the recent group (25% versus 12%). Beside the treatment period, the only independent determinant for survival was histological grade. CONCLUSIONS: The adoption of a policy of more liberal visceral en bloc resections was associated with a higher local control and OS. This benefit was evident in patients with grade I-II tumors, while DMs were a limiting factor in high-grade ones. New therapies are needed to control systemic disease as local surgery may improve local control.

Esthesioneuroblastoma metastatic to the trachea.

Esthesioneuroblastoma is a rare tumour, for which a multimodal approach, including a combination of surgery and radiation, appears to provide the best disease-free and overall survival. Well-known for its tendency for local recurrence and distant spreading by both lymphatic and haematogenous routes, the most common sites of metastases are lungs and bones, followed by liver, spleen, scalp, breast, adrenals and ovary. One single case of metastasis to the trachea has been reported in the literature. The case is reported here of a patient who developed metastatic esthesioneuroblastoma to the trachea 18 months after primary surgery and radiation therapy. The patient was treated by two subsequent N-YAG laser endoscopic resections and chemotherapy.

An unusual complication of gastric banding: recurrent small bowel obstruction caused by the connecting tube.

Laparoscopic adjustable gastric banding (LAGB) is a widely performed surgical procedure for morbid obesity. The application of this mini-invasive approach has given the benefits of shorter hospital stay, less postoperative pain and quicker functional recovery. LAGB complications are related either to the access-port, such as port-site infection or tubing disconnection, or to the band, such as band slippage, pouch dilatation, or intragastric migration. We report a case of recurrent small bowel obstruction caused by the connecting tube around a jejunal loop, in a woman who had under-gone LAGB 3 years before. The diagnosis was difficult to establish because the clinical history and examination were non-specific. A 3-dimensional CT scan was needed to explain the cause of the recurrent abdominal pain, and the small bowel loop was freed from the connecting tube at laparoscopy.

The prognostic value of the modified Glasgow Coma Scale in head trauma in dogs.

A clinical coma scale modified from the Glasgow Coma Scale used for humans has been suggested as a useful predictor of outcome in the head trauma patient. The objective of this study was to correlate the modified Glasgow Coma Scale (MGCS) score of dogs with head trauma with their probability of survival. Thirty-eight dogs with head trauma were selected and retrospectively evaluated. The information retrieved from the medical record of each dog included signalment, body weight, cause of head trauma, MGCS, presence of concurrent neck pain, and outcome (dead or alive) after 48 hours. Logistic regression was used to model survival in the 1st 48 hours as a function of MGCS, gender, weight, and calvarial fractures. The MGCS ranged from 5 to 18. Seven dogs died within 48 hours of the head trauma. The MGCS could predict the probability of survival in the 1st 48 hrs after head trauma with 50% probability in a patient with a score of 8. Gender, weight, and presence of skull fractures did not predict survival. In conclusion, the MGCS is a useful index for prediction of outcome in dogs with head trauma.

[Neurofibromatosis I: a clinical series and genetic correlations]. / Neurofibromatosi tipo 1. Casistica e correlazioni genetiche.

Type 1 neurofibromatosis represents 90% of all forms of neurofibromatosis. It is a disease with an incidence of 1:3000 neonates with a dominant autosomic form of transmission, but 50% of all cases are sporadic owing to new mutations. Diagnosis is mainly clinical and requires at least of the following signs: CAL, neurofibromas, glioma of the optic nerve, axillary lentigo, Lisch's nodules, bone lesions and the presence of the disease in at least one first-degree relation. These criteria may also be applied in children under 6 years old as is confirmed by the clinical series of 5 cases referred to our attention. The deletion of the long arm of chromosome 17 in the zone proximal to the centromere containing the gene of type 1 neurofibromatosis was observed in one case. The phenotype of this subject was correlated to another 5 cases reported in the literature.

[Demand of community health services in the periphery of a metropolitan area]. / Demanda de serviço de saúde comunitária na periferia de área metropolitana.

The content of 4,319 consultations in primary health care representing 7% of all consultations from March, 1985 to March, 1986 at the Murialdo Health Center, was analyzed. The health center is located in the outskirts of Porto Alegre, RS (Brazil). Woman represented 67% of the total demand. Most consultations were for children under 10 years of age (37%) and woman of childbearing age (21%). The first twenty reasons for the visit corresponded to 63% of the total, and the most common reason for visiting the health center was for renewal of prescriptions and/or ordering medication (9.3%). Of all the diagnoses, the first twenty accounted for 62% of the total number of patients and the most frequent were: hypertension (8.8%), upper respiratory tract infection (7.8%), and immunization (5.5%). Prescription and administration of medication were the most common actions performed. Referrals resulted in 7.3% of the visits. Of these, 5% were sent to other providers within the Murialdo service. Only 0.6% were referred for hospitalization. These findings, combined with others which describe aspects of morbimortality not easily measured in studies of spontaneous demand for medical services, should help in the planning of primary health care services and in the training of health personnel.