A case of Erdheim-Chester disease with spinal cord compression and sphenoid sinus involvement.

Erdheim-Chester disease is a rare form of non-Langerhans cell histiocytosis. It is an inflammatory disorder associated with BRAF V600E mutation in 50% of cases. This multisystem disease is rarely associated with spinal involvement. Neurological involvement is an independent predictive factor of poor prognosis. The diagnosis is histopathological based on CD68-positive and CD1A-negative histiocytes. Treatment with interferon-alpha is an independent predictor of survival in Erdheim-Chester disease and vemurafenib has also been shown to be effective for BRAF V600E mutation. We report a clinical case of a 51-year-old patient with multiple and rare locations of Erdheim-Chester disease, particularly at the sphenoid sinus.

Is obstetric anal sphincter injury a risk factor for levator ani muscle avulsion in vaginal delivery?

OBJECTIVE: To analyze whether women who sustain obstetric anal sphincter injury (OASI) in their first vaginal delivery have a higher incidence of levator ani muscle (LAM) avulsion than do women without OASI. METHODS: We conducted a prospective observational cohort study of 80 primiparous women who gave birth at our tertiary obstetric unit between September 2011 and July 2013. The women were divided into two groups: women diagnosed with OASI during vaginal delivery (n = 40) and women without OASI (n = 40), matched by mode of delivery and newborn birth weight. Four-dimensional (4D) transperineal ultrasound was performed in all participants at 6-12 months after delivery to assess for integrity or avulsion of the LAM. Ultrasound images were evaluated by two observers who were blinded to group assignment and clinical data. Interobserver agreement was assessed to determine the reproducibility of 4D transperineal ultrasound for diagnosing LAM avulsion. RESULTS: We found no statistically significant differences between the two groups. The incidence of LAM avulsion was 24.3% in the OASI group and 34.2% in the control group (P = 0.448). Interobserver agreement in the diagnosis of LAM avulsion in the study population using 4D transperineal ultrasound was 89.5%, with a kappa index of 0.76. CONCLUSIONS: Our findings do not support OASI as a risk factor for LAM avulsion. Prospective studies in larger populations are needed to establish the biomechanical relationships of the pelvic floor structures involved in vaginal delivery. Copyright © 2015 ISUOG. Published by John Wiley & Sons Ltd.

Should we fear cement leakage during kyphoplasty in percutaneous traumatic spine surgery? A single experience with 76 consecutive cases.

OBJECTIVE: Although kyphoplasty is widely used to repair osteoporotic and pathologic vertebral fractures, balloon kyphoplasty and vertebral body stenting are new treatment options in cases of traumatic spinal injury. To our knowledge, there are no literature data on the incidence of cement leakage whereas these two percutaneous techniques are commonly used to repair non-pathologic fractures. The aim of this study was to evaluate and compare the clinical characteristics and the incidence of cement leakage associated with balloon kyphoplasty and vertebral body stenting in the percutaneous treatment of traumatic spinal injury. METHODS: A series of 76 consecutive kyphoplasties (50 with vertebral body stenting and 26 balloon kyphoplasties) were retrospectively reviewed. Preoperative and postoperative computed tomography scans were analyzed in order to detect cement leakage and grade it as minor, moderate or major. RESULTS: The overall leakage rate was 50%. None of the leakages gave rise to clinical symptoms. Although balloon kyphoplasty and vertebral body stenting did not differ in terms of the leakage rate, the latter technique was associated with a lower leakage volume. The Magerl type, fracture level and use of concomitant osteosynthesis did not appear to significantly influence the leakage rate. CONCLUSION: Vertebral body stenting can reduce the amount of cement leakage due to a better cohesion of the bone fragments after kyphosis correction and maintenance.

Unexpected ruptured aneurysm during posterior fossa surgery.

BACKGROUND AND IMPORTANCE: Surgery is the recommended treatment for unique significant cerebellar metastasis, particularly in cases of hydrocephalus. Complications of posterior fossa surgery are associated with high risk of morbidity and mortality. We present a unique case of unexpected peroperative rupture of a cerebellar superior artery aneurysm during posterior fossa surgery. CLINICAL PRESENTATION: During posterior cranial fossa surgery, severe arterial bleeding occurred in front of the medulla oblongata. Immediate postoperative computed tomographic (CT) angiography revealed a fusiform aneurysm from a distal branch of the left superior cerebellar artery. CONCLUSION: To our knowledge, this is the first reported operative case of unexpected infratentorial ruptured aneurysm during posterior fossa surgery.

[Type II proatlantal artery with unusual origin of the occipital artery feeding of a type IV dural arteriovenous fistula: case report]. / Artère proatlantale de type II avec origine atypique d'une artère occipitale alimentant une fistule artérioveineuse durale de type IV : cas clinique.

CASE REPORT: We report the case of a 49 year-old woman, who presented with an intracranial haemorrhage. During cerebral angiography investigations, we observed an unusual vertebral artery that originated from the external carotid artery and an uncommon origin of the occipital artery from the vertebral artery feeding a dural arteriovenous fistula grade IV. This rare vertebral artery origin is probably the remnant of a type II proatlantal artery. In addition, the origin of the occipital artery evoked a vascular development disease including a dural arteriovenous fistula which may be the result. These multiple vascular abnormalities could be explained by embryologic disorders.

[Brown-Sequard syndrom complicating a C6 teardrop fracture associate with an occult vertebral artery dissection: case report]. / Syndrome de Brown-Séquard compliquant une fracture teardrop de C6 associée à une dissection d'une artère vertébrale asymptomatique : à propos d'un cas.

CASE REPORT: We report the case of a 27-year-old man, presenting a C6 tear-drop fracture associated to medullar contusion with a Brown-Sequard syndrome. An asymptomatic right vertebral artery dissection in its V2 portion was also discovered by the radiologic investigations. A surgical treatment and antiplatelet therapy were performed. The patient outcome was quite satisfactory.

[Subependymomas of lateral ventricle. Analysis of our series and review of literature]. / Les subépendymomes (astrocytomes subépendymaires) du ventricule latéral. Analyse de notre série et revue de la littérature.

Subependymoma is a benign lesion, slow-growing neoplasm, representing 0.2 to 0.7 % of intracranial tumors. They are often clinically silent, incidentally discovered at autopsy. These symptoms are related to big volume. They are attached to the septum pellucidum, leading to hydrocephalus by Monro foramen obstruction. Overall mean age at diagnosis is 39 years with more males than females. At CT-scan, subependymoma shows a slightly low attenuation compared to gray matter. There is no or mild enhancement following contrast injection. On MR T1-weighted imaging, subependymoma is isointense and hyperintense on MR T2-weighted imaging. Intramural calcifications and cystic components are noted in 20 to 30 % of patients. Peritumoral oedema is absent. Immunohistochemicals studies show intense positivity for S-100 and GFAP. The treatment is surgical with an excellent prognosis.

[Rare tumors of the lateral ventricle. Review of the literature]. / Les tumeurs rares du ventricule latéral. Revue de la littérature.

Metastases of lateral ventricle (LV) are attached to choroidal plexus. Primary source is cancer of kidney. Two thirds of patients are male. Oligodendrogliomas occur in young females. Signs of increased intracranial pressure are a constant feature. These tumors are found in anterior portion of the LV with severe enhancement and clumped calcifications. Treatment is surgical. Cavernomas have a preponderance of rapid growth with a bleeding revelation. Seizures are rare. Rebleeding is frequent and justifies a surgical treatment. Schwannomas are a rare entity in which the majority of patients are very young. MRI shows calcifications, cystic components and a strong enhancement. They are limited to sporadic cases, never associated with neurofibromatosis. Arachnoid cysts are located in the atrium and/or in the occipital horn. Patients are young (mean age < 40 years). MRI demonstrates an intracystic lesion with signal intensity similar to the CSF. Best treatment is an endoscopic fenestration. Epidermoid cysts occur in third decade. These pearly tumors appear isointense or a little hyperintense on T1-weighted imaging, very characteristic. They are enhanced after gadolinium injection and appear strongly hyperintense on T2-weighted imaging. An incomplete removal with a thorough long-term follow-up is necessary. Cavernomas of LV are hyperintense on T1- and T2-weighted imaging. They have a bleeding risk of 25 to 45%. Therefore, they must be operated.

Effects of polymorphisms in the calpastatin and µ-calpain genes on meat tenderness in 3 French beef breeds.

The objectives of the study were to evaluate allelic frequencies and to test the association of polymorphisms in the calpastatin (CAST) and µ-calpain (CAPN1) genes with meat tenderness in 3 French beef breeds. A total of 1,114 Charolais, 1,254 Limousin, and 981 Blonde d'Aquitaine purebred young bulls were genotyped for 3 SNP in the CAST gene and 4 SNP in the CAPN1 gene. Two of these markers, 1 in each gene, can be found in Australian or American commercial genetic tests. Others have previously been reported in American studies or are newly evidenced SNP. The quantitative traits studied were Warner-Bratzler shear force and a tenderness score evaluated by trained sensory panels. All the SNP were informative in the 3 breeds. Associations of individual markers or haplotypes with traits were analyzed. The results differed in the 3 breeds. The G allele of a CAST marker (position 97574679 on Btau4.0) was found to exert a significant effect on the shear force (+0.18 phenotypic SD; RSD) and tenderness score (-0.22 RSD) in the Blonde d'Aquitaine breed. In the same breed, this marker was associated with another CAST SNP (position 97576054 on Btau4.0) such that the GA haplotype appeared to be associated with tougher meat. Two CAPN1 markers (positions 45221250 and 45241089 on Btau4.0) had a significant effect on both traits in the Charolais breed (from |0.11| to |0.25| RSD). In the same breed, these markers were associated with another CAPN1 SNP (position 45219395 on Btau4.0) such that the ACA and AGG haplotypes appeared to be associated with a tender meat and a tougher meat, respectively. Consequently, the present results indicate that the effects of the markers studied are breed-specific and cannot be extended to all Bos taurus breeds. Further studies are also required to identify other more appropriate markers for French beef breeds.

Pretransplant hyponatremia could be associated with a poor prognosis after liver transplantation.

INTRODUCTION: Predicting the prognosis of hepatic cirrhosis is the most accurate method to achieve a fair allocation among the liver transplant waiting list thereby reducing overall mortality rates. AIM: To study the survival rates of recipients who undergo liver transplantation in association with hyponatremia rates. METHODS: This retrospective study used a prospectively collected database. The characteristics of liver donors and recipients were: age (years), Model for End-stage Liver Disease (MELD), MELD Na score, recipient body mass index (kg/m(2)), warm ischemia time (minutes), cold ischemia time (minutes), intensive care unit time (days), hemocomponents transfused, recipient glycemia (mg/dL) and serum sodium (mEq/L), Child-Pugh-Turcotte classification (points), and survival time (months). We analyzed all 318 consecutive liver transplantations from February 1994 to May 2010 divided into two groups: A (Na > 130 mEq/L) and B (Na ≤ 130 mEq/L). The Kaplan-Meier method was used to evaluate survival rate and the Cox regression test to identify predictive factors. RESULTS: Hyponatremic patients displayed shorter survival (P = .04). The Cox regression for survival time showed that patients with low serum sodium values (group B) had: Child-Pugh scores with 1.13% plus risk of death for each point; cold ischemia time with 1.001% less risk of death for each minute; glycemia with 0.6% for each mg/dL; 0.66% use of cell-saver; plus a risk of death for each 100 mL plus 1.02% risk of death for each year of donor age. CONCLUSION: Hyponatremic cirrhotic patients show more advanced stages of disease compared to normonatremic cirrhotics. They usually display metabolic or cirrhotic decompensation and comorbidities. When these symptoms were associated with the use of extended criteria donors, increased cold ischemia time, and intraoperative bleeding, we observed lower survival rates.

The two mutations, Q204X and nt821, of the myostatin gene affect carcass and meat quality in young heterozygous bulls of French beef breeds.

The availability of genetic tests to detect different mutations in the myostatin gene allows the identification of heterozygous animals and would warrant the superiority of these animals for slaughter performance if this superiority is confirmed. Thus, 2 mutations of this gene, Q204X and nt821, were studied in 3 French beef breeds in the program Qualvigène. This work was done with 1,114 Charolais, 1,254 Limousin, and 981 Blonde d'Aquitaine young bulls from, respectively, 48, 36, and 30 sires and slaughtered from 2004 to 2006. In addition to the usual carcass traits recorded at slaughter (e.g., carcass yield, muscle score), carcass composition was estimated by weighing internal fat and dissecting the 6th rib. The muscle characteristic traits analyzed were lipid and collagen contents, muscle fiber section area, and pH. Regarding meat quality, sensory qualities of meat samples were evaluated by a taste panel, and Warner-Bratzler shear force was measured. Deoxyribonucleic acid was extracted from the blood samples of all calves, the blood samples of 78% of the dams, and the blood or semen samples of all the sires. Genotypes were determined for 2 disruptive mutations, Q204X and nt821. Analyses were conducted by breed. The superiority of carcass traits of calves carrying one copy of the mutated allele (Q204X or nt821) over noncarrier animals was approximately +1 SD in the Charolais and Limousin breeds but was not significant in the Blonde d'Aquitaine. In the Charolais breed, for which the frequency was the greatest (7%), young bulls carrying the Q204X mutation presented a carcass with less fat, less intramuscular fat and collagen contents, and a clearer and more tender meat than those of homozygous-normal cattle. The meat of these animals also had slightly less flavor. Also in the Charolais breed, 13 of 48 sires were heterozygous. For each sire, the substitution effect of the wild allele by the mutant allele was approximately +1 SD for carcass conformation and yield, showing that the estimate of the substitution effect was independent of family structure, as it ought to be for a causal mutation. These results illustrate the challenge of using genetic tests to detect animals with the genetic potential for greater grades of carcasses and meat quality.

Biomechanical and histologic alteration of facial recipient bone after reconstruction with autogenous bone grafts and alloplastic implants: a 1-year study.

Potential alteration of the underlying recipient bone resulting from a graft or implant has significant clinical relevance. The present study was designed to evaluate the biomechanical and histologic alteration of facial recipient bone with autogenous bone graft and alloplastic implants over a 1-year period. The bilateral arches of 15 rabbits were randomized between four groups: (1) control (n = 6), subperiosteal exposure of the zygomatic arch was made; (2) onlay (n = 12), bone graft was placed as an onlay to the zygomatic arch; (3) inlay (n = 6), bone graft was placed as an inlay within the zygomatic arch; (4) implant (n = 6), a stainless steel plate was placed as an onlay to the zygomatic arch. Animals were killed 1 year after grafting. In the onlay groups, all steel implants and half of the onlay bone grafts (n = 6) were separated from the zygomatic arch; the remaining onlay bone grafts (n = 6) were left on the zygomatic arch. Three-point breaking strength was measured through the center of the graft/implant site on the zygomatic arch, followed by histologic evaluation and histometric assessment of residual bone density. The findings demonstrated no difference in the breaking strength per unit bone area between the control zygomatic arch group and the onlay group in which the bone graft was left in place. Breaking strength of the zygomatic arch in the former two groups was significantly greater than that in either group in which the onlay bone graft or implant had been removed, and was also greater than the breaking strength in that group in which inlay bone had been placed (p < 0.05). Histologic assessment showed full-thickness conversion in architecture of the zygomatic arch from compact to woven bone beneath onlays of either autogenous bone graft or steel implant; histometric assessment demonstrated an accompanying decrease in bone density in the latter groups relative to the control zygoma (p < 0.05). We conclude that onlay autogenous bone graft and alloplastic implants to the facial skeleton induce transformation of both graft and recipient bone from compact to woven architecture, accompanied by a reduction in bone density. The biomechanical strength of recipient facial bone is significantly weakened if an onlay bone graft or implant is removed. Weakening occurs per unit area of remaining bone, and is therefore independent of any thinning that may occur within the recipient bone because of graft/implant placement. These findings may impact upon decisions to augment stress-bearing regions of the facial skeleton with bone graft or implants, particularly if the graft/implant may eventually require removal.