Posterior auricular artery helix root free flap-part I: radio-anatomical study.

Full-thickness defects of the distal nose are an ongoing surgical challenge. Among the available techniques, pre-auricular chondrocutaneous free flaps based on the superficial temporal artery (STA) have good aesthetic and functional outcomes. However they often require autologous venous grafts. The aim of this radio-anatomical study was to evaluate the feasibility of the helix root free flap based on the posterior auricular artery (PAA). Vascular lengths, diameters, and networks were investigated in flaps harvested from cadavers. The perfusion of the flaps was studied by injecting patent blue. Some flaps were also injected with contrast agent and studied by angiography and computed tomography. Ten flaps from seven fresh cadavers were dissected and analysed. The mean length of the PAA was 114.2 mm and the mean diameter was 2.2 mm. Perfusion was investigated in six flaps and considered good in three of these. The study results demonstrate the feasibility of PAA-based helix root free flaps. This alternative technique provides an 11 cm pedicle with vessels of appropriate calibre, facilitating any potential microsurgery. The scar is mostly hidden behind the ear. This PAA-based helix root free flap could be a reliable and promising single-stage procedure to repair complex defects of the alae nasi.

A new approach of crenellated fronto-orbital advancement for the management of trigonocephaly: Technical note.

Trigonocephaly is a craniosynostosis characterized by a premature fusion of the metopic suture associating a characteristic triangular head shape, with a frontal medial crest and hypotelorism. Various techniques have been described for its surgical treatment which is usually performed during the first year of life. However, there might be cases with a late referral, after the age of one year. One of the technical issues with a fronto-orbital advancement surgery in a child over one year of age is that there is a significant risk of persistent residual bone defects. This article describes a surgical technique of crenellated fronto-orbital advancement for correcting trigonocephaly in children over one year of age, allowing to reduce residual bone defects.

Could fetal reduction induce facial cleft? Report of a case.

We describe a paramedian cleft of the lower lip that cannot be explained by embryological development in a child with only one predisposing factor, which was fetal reduction for a multiple pregnancy. To the best of our knowledge, there has been no report of a cleft that has been induced by the reduction of a multifetal pregnancy.

Para commissural cleft of the lower lip: A first case described in the literature.

We described the first case of para-commissural cleft of the lower lip in a 2 year-old child. He is from a trichorionic triamniotic triplet pregnancy after ovarian stimulation. He has no history except a selective fetal reduction of the mother. We correct this cleft of the lower lip with a double plasty of Malek. The functional and aesthetic result is satisfactory. This particular cleft cannot be explained by the embryological theory. We favor two hypothesis to explain this anomaly: a vascular origin with the stapedial artery and the selective fetal reduction.

[Paracommissural cleft of the lower lip: Therapeutic strategy]. / Fente labiale inférieure para-commissurale : stratégie thérapeutique.

We report the first case described in the literature of lateral, paracommissural cleft of the lower lip in a 2-year-old child. This anomaly caused labial incompetence requiring surgical correction. Different surgical techniques have been described for the treatment of the cleft of the upper lip. We studied each of them, analyzed the advantages and disadvantages of these techniques by inverting them and adapting them for the lower lip. We opted for the double "Z" plasty of Malek because the difference in height between the cleft side and the healthy side was important. In addition, this plasty hides part of the scar in the labiomental groove. The functional and aesthetic result at 6 months after surgery is satisfactory.

Unusual cystic lesion of the eyebrow: A case report of malignant chondroid syringoma.

INTRODUCTION: Malignant chondroid syringomas, also known as cutaneous malignant mixed tumors, are rare neoplasms that most frequently occur on the torso or extremities of women. Here, we present an illustrated case of a facial malignant chondroid syringoma. MATERIALS AND METHODS: A 32-year-old female patient with no notable medical history presented with an approximately 1cm-wide, painless, palpably-mobile subcutaneous nodule, suggestive of a sebaceous cyst, just above the middle third of the right eyebrow. The nodule had grown steadily over six months. She had no palpable cervical lymphadenopathies. RESULTS: Anatomic pathology of the enucleated nodule found an adnexal sudoriparous tumor measuring 6×10mm and indicative of a malignant chondroid syringoma. Cervicofacial computed tomography and positron emission tomography scans showed no near or distant lymph node involvement. A second intervention for wide excision around the original enucleation lesion (+1cm) was validated in a multidisciplinary, cancerology-dermatology consultation. The eyebrow was reconstructed with a temporally-harvested fasciocutaneous island flap. DISCUSSION: Malignant chondroid syringomas are very rare and thus no standardized treatment has been established for them. Only 12 craniofacial localizations have been described to date. Radiation therapy and chemotherapy have not been shown effective for this malignancy, leaving only wide excision as a therapeutic option. A high and sustained (as much as 20 years after the initial diagnosis) risk of recurrence or metastasis necessitates prolonged patient follow-up.

Minimally invasive versus standard approach in LeFort 1 osteotomy in patients with history of cleft lip and palate.

OBJECTIVE: Compare literature-reported efficiency and complications of the standard maxillary advancement surgery with those of a minimally invasive mucosal approach in patients with CL/P requiring Le Fort 1 osteotomy. DESIGN: Meta-analysis vs. retrospective analysis of 18 consecutive cases. SETTING: Department of maxillofacial surgery at a tertiary-level public general hospital. PARTICIPANTS: The meta-analysis encompassed Medline, Embase and Cochrane, years 1990 to 2014, inclusive. The local series concerned all squeletally mature adolescents with non-syndromic CL/P who underwent orthognathic surgery between 30 April 2004 and 27 January 2012. INTERVENTIONS: Minimally invasive approach and perioperative orthodontics including intermaxillary fixation for 3 months after surgery. MAIN OUTCOME MEASURE(S): Assessment of complications. Standard lateral cephalograms were taken before surgery, then <1 week and 12 months after surgery. Delaire's cephalometric analysis was performed and the position of the maxilla was recorded. RESULTS: There were no significant differences between the literature and our series regarding sex and type of deformity (P=0.634 and 0.779, respectively). The mean horizontal and vertical relapse rates were 0.61 and 1.17mm (vs. 1.29 and 1.48mm in the meta-analysis) and the overall complication rate was 22.2% (vs. 12.76% but P=0.271). There was a significant difference regarding the palatal fistula rate (0 here vs. 21.43% in meta-analysis, P=0.028). CONCLUSIONS: The minimally invasive approach showed trends toward less relapse and less complications than conventional approaches. This technique seems adapted to the management of patients with CL/P sequelae. Other benefiting groups are underway.

Prognostic impact of Bacillus Calmette-Guérin interruption at the time of induction and consolidation.

CONTEXT: Intravesical Bacillus Calmette-Guérin (BCG) is a cause of bladder and systemic toxicity that is difficult to prevent and is responsible for treatment drop out in bladder cancer patients. More recently, BCG shortage has become the main cause of incomplete treatment. AIMS: The aim of this study was to examine the impact on long-term prognosis of bladder cancer patients following discontinuation of BCG instillations. SETTINGS AND DESIGN: In this retrospective study, data were examined from 333 consecutive nonmuscle invasive bladder cancer patients treated from 2005 to 2015 by transurethral resection (TUR) and had undergone adjuvant BCG therapy after TUR. SUBJECTS AND METHODS: Rate of complete cure, the reason for the interruption, toxicity, and the associations between discontinuance of BCG therapy, tumor characteristics, association with carcinoma in situ and tumor recurrence or progression were analyzed. STATISTICAL ANALYSIS USED: Recurrence and progression-free survival rate curves were estimated using the Kaplan-Meier method and were compared using the log-rank test. Univariate and multivariate analyses were performed using the Cox proportional hazards model. Differences among groups were considered as statistically significant when P < 0.05. RESULTS: Overall, 303 patients were eligible for analysis. Median follow up was 36 (confidence interval: 7-120) months. A total of 55 (18.1%) had <6 installations (Group I); 87 (28.7%) completed induction and 1-year maintenance (Group III); and 161 (53.1%) completed the induction course, but not the 1-year maintenance (Group II). Grade III-IV toxicity rates were significantly higher in Group I than Group II and III. Interruption for BCG shortage was the main cause of interrupting BCG in Group II. Multivariate analysis showed that discontinuation of BCG induction therapy was an independent predictor for tumor recurrence (P < 0.001) and 1-year BCG maintenance therapy for tumor progression (P = 0.005). CONCLUSIONS: Discontinuation of BCG therapy has a significantly deleterious effect on tumor recurrence and progression rates. Although BCG toxicity is a major cause of drop out, BCG shortage became a major cause of discontinuation. All effort must be done today to restore normal production of BCG worldwide.

A Survey of Zoonotic Pathogens Carried by Non-Indigenous Rodents at the Interface of the Wet Tropics of North Queensland, Australia.

In 1964, Brucella was isolated from rodents trapped in Wooroonooran National Park (WNP), in Northern Queensland, Australia. Genotyping of bacterial isolates in 2008 determined that they were a novel Brucella species. This study attempted to reisolate this species of Brucella from rodents living in the boundary area adjacent to WNP and to establish which endo- and ecto-parasites and bacterial agents were being carried by non-indigenous rodents at this interface. Seventy non-indigenous rodents were trapped [Mus musculus (52), Rattus rattus (17) and Rattus norvegicus (1)], euthanized and sampled on four properties adjacent to the WNP in July 2012. Organ pools were screened by culture for Salmonella, Leptospira and Brucella species, real-time PCR for Coxiella burnetii and conventional PCR for Leptospira. Collected ecto- and endo-parasites were identified using morphological criteria. The percentage of rodents carrying pathogens were Leptospira (40%), Salmonella choleraesuis ssp. arizonae (14.29%), ectoparasites (21.42%) and endoparasites (87%). Brucella and C. burnetii were not identified, and it was concluded that their prevalences were below 12%. Two rodent-specific helminthic species, namely Syphacia obvelata (2.86%) and Nippostrongylus brasiliensis (85.71%), were identified. The most prevalent ectoparasites belonged to Laelaps spp. (41.17%) followed by Polyplax spp. (23.53%), Hoplopleura spp. (17.65%), Ixodes holocyclus (17.64%) and Stephanocircus harrisoni (5.88%), respectively. These ectoparasites, except S. harrisoni, are known to transmit zoonotic pathogens such as Rickettsia spp. from rat to rat and could be transmitted to humans by other arthropods that bite humans. The high prevalence of pathogenic Leptospira species is of significant public health concern. This is the first known study of zoonotic agents carried by non-indigenous rodents living in the Australian wet-tropical forest interface.

A novel mutation of anti-Mullerian hormone gene in Persistent Mullerian Duct Syndrome presented with bilateral cryptorchidism: a case report.

BACKGROUND: We report a novel mutation in a case of Persistent Mullerian Duct Syndrome (PMDS). PMDS is characterized by the persistence of female reproductive organs derivatives in a 46,XY subject due to the failure of the Mullerian duct to regress in utero. To date, 53 different mutations of the anti-Mullerian hormone (AMH) gene, including the present one, have been identified. CLINICAL CASE: A 2-week-old male presented with bilateral cryptorchidism and normal male external genitalia. His karyotype was 46,XY. hCG stimulation test was normal. At age 1 year and 4 months, he underwent laparoscopic surgery which revealed a uterus and fallopian tubes. The anti-Mullerian hormone (AMH) level was undetectable (<0.01 ng/mL). Diagnosis of Persistent Mullerian Duct Syndrome, probably due to an AMH mutation, was made. GENETIC STUDIES: A unique homozygous T to G base substitution was found at position 2219, near the middle of the exon 5, changing codon CTG to CGG in anti-Mullerian hormone (AMH) gene. This mutation causes leucine to be converted to arginine at position 426 belonging to a (L)RA(L)LLLKALQ highly conserved sequence in the AMH gene. Both parents are heterozygous for the mutation. CONCLUSION: Persistent Mullerian Duct Syndrome (PMDS) is a rare cause of bilateral cryptorchidism, when in doubt the existence of Mullerian derivatives should be explored by laparoscopy. Assay of serum AMH helps to distinguish between mutations of AMH and AMH receptor. If serum AMH is very low or undetectable, sequencing of the AMH gene usually confirms the presence of a mutation.

Complications of regional anaesthesia.

Regional anaesthesia can marvellously dull the pain (and limit some other complications) of trauma, surgery and childbirth. But like all powerful techniques, it may have complications. Here the complications of regional anaesthesia are reviewed. The risks, presentation and the management of these complications are discussed in turn.

[Two immunoassays for antimullerian hormone measurement: analytical and clinical performances]. / Deux dosages de l'hormone antimüllérienne : performances analytiques et cliniques.

Today, serum antimullerian hormone (AMH) is considered as an interesting marker of fertility potential in women to determine follicular status and in men to evaluate testicular function. We study analytical and clinical characteristics of two AMH commercialized immunoassays: Immunotech and DSL methods. The detection limits (close to 0.13 ng/mL), functional sensitivities (close to 0.30 ng/mL) are equivalent, and imprecision results are acceptable for entirely manual assays. The Immunotech method is linear within the calibration range (up to 21 ng/mL) and the DSL method presents a lack of linearity making it accurate only up to 11 ng/mL (and not up to 14 ng/mL as it is indicated by the manufacturer). The two methods allow to measure human AMH, don't cross react with TGF-beta superfamily proteins and the DSL immunoassay recognize mouse (25%), rat (68%) and calf (100%) AMH. The comparison between the two methods (from 0.3 to 200 ng/mL) shows a good correlation (r = 0.979) with not statistically different results (p = 0.31). From a clinical point of view, the two methods allow the evaluation of follicular status in normo-ovulatory women and in women with polycystic ovary syndrome. Results are in agreement with studies showing that AMH serum concentration is strongly correlated with the number of antral follicles. In conclusion, the Immunotech method seems to be more efficient than the DSL method even if the two methods are suitable for clinical applications needing AMH measurements.

A visual pitfall: persistent Müllerian duct syndrome (PMDS).

Persistent Müllerian Duct Syndrome (PMDS) is a rare disorder of the anti-mullerian hormone (AMH) synthesis or receptor, which due to the visual contrast of normal masculine external genitalia and female internal genitalia can raise confusion, sometimes during surgery for cryptorchidism or hernia inguinalis. For an acute and accurate analysis of such a situation a thorough knowledge of gonadal embryology is mandatory. The diagnosis is made on finding Müllerian structures in an individual with complete virilization without signs of hypocortisolism or exposition to maternal androgens during foetal life. Karyotyping and gonadal biopsy provide additional information to confirm the diagnosis. As the risk of malignant transformation is not clear, orchidopexy is advised in patients with cryptorchidism, with lifelong palpatory follow-up. In case of urologic symptoms, surgical removal of the Müllerian remnants can be considered, with careful attention for the vulnerable ductus deferens. Despite optimal treatment the prognosis regarding fertility remain uncertain.

Variation in tobacco use profiles obtained from periodontal maintenance patients.

OBJECTIVES: To compare methods of tobacco use profiling in patients undergoing maintenance treatment for periodontitis. METHODS: The smoking status of 108 subjects attending a university-based specialist periodontics clinic was assessed by examination of referring records, patient-completed questionnaires, clinician-administered interviews and by salivary cotinine and expired-air carbon monoxide (ECO) measurement. RESULTS: Ten percent of the detected smokers (salivary cotinine>14.2ngml(-1)) reported to be non-smokers in the clinician-administered interview. 4.2% of the smokers detected by ECO analysis (ECO>8ppm) reported to be non-smokers in the clinician-administered interview. Systematic under reporting of tobacco use was also apparent in patient-completed questionnaires from the periodontics clinic and, to a larger degree, in the referring clinic records when compared to the clinician-administered interview. CONCLUSIONS: By biochemically analyzing recent cigarette smoke exposure, our data show that clinician assessment is the most accurate means by which to obtain anamnestic data concerning tobacco use. ECO analysis using portable CO monitors is inexpensive and simple with no requirement for laboratory support. The combination of ECO measurement and clinician-administered tobacco use interview represents a clinically applicable method to improve the detection of an important, periodontally susceptible population-regular smokers who deny smoking.

Ovarian granulosa cell tumors express a functional membrane receptor for anti-Müllerian hormone in transgenic mice.

Anti-Müllerian hormone inhibits granulosa cell growth and function. Both anti-Müllerian hormone and its type II receptor are expressed in normal granulosa cells. We show by histologic and molecular analyses that ovarian tumors developing in transgenic mice, obtained by targeted oncogenesis using an anti-Müllerian hormone promoter-SV40 oncogene construct, are of granulosa-cell origin. Because tissue-specific, cell-surface molecules are of particular interest for the analysis and treatment of tumors, we examined the expression of anti-Müllerian hormone type II receptor in the ovaries of these transgenic mice. We demonstrate that the anti-Müllerian hormone type II receptor is expressed not only in normal ovarian follicles, but also in granulosa cell tumors. Using a cell line derived from one of these tumors, we show that the anti-Müllerian hormone type II receptor protein is present on the surface of tumor cells and binds anti-Müllerian hormone. Furthermore, we show that the anti-Müllerian hormone receptor is functional in the granulosa tumor cell line, with anti-Müllerian hormone treatment inducing selective activation of Smad1. In conclusion, in this study we present a new murine transgenic model of granulosa cell tumors of the ovary and, using this model, we demonstrate for the first time cell-surface expression of a highly tissue-specific molecule, anti-Müllerian hormone type II receptor, as well as the selective activation of Smad proteins by anti-Müllerian hormone, in granulosa tumor cells.

Differential and opposed transcriptional effects of protein fusions containing the VP16 activation domain.

Overexpression of strong transcriptional activators like herpes simplex virion protein 16 (VP16) may lead to non-specific inhibition of gene expression as a result of the titration of transcription factors. Here we report that a fusion between the homeoprotein Hoxa2 and the VP16 activation domain inhibits transcription from the strong promoter/enhancers of cytomegalovirus (CMV) and Rous sarcoma virus (RSV). A similar fusion involving a Hoxa2 mutant protein that is defective in DNA binding has no effect on the CMV promoter but increases, rather than inhibits, the RSV promoter activity. This suggests that depending on its ability to bind DNA, the VP16 activator can interact with different sets of cofactors, giving rise to distinct transcriptional effects.

[Positron emission tomography in tumor pathology]. / La tomographie par émission de positons en pathologie tumorale.

After a brief reminder concerning the PET technique and use of FDG, the author sets out to summarize the results of numerous PET centers in the world and in more detail, results of European units he visited. Leaving neurology and cardiology out, the most important works are dealing with tumoral pathology. The sensitivity of the technique is high for localizing tumoral tissue, evaluating lymphatic spread and detecting metastases. On the contrary, specificity is low as non tumoral lesions, especially inflammatory lesions, tuberculosis may pick up FDG though with less intensity. PET contributes to precise staging TNM of cancer, especially in case of pulmonary neoplasms. Results of clinical evaluation of bronchial carcinoma are presented. Other research programs show similar positive results for the staging of digestive tract neoplasm, primary or metastatic lymph node diseases, metastases from a quiescent, unknown primary tumor. The fact that the number of PET units is small explains the relatively poor dissemination of the method. It is rather obvious that the limitation of the number of PET units is due to economic reasons. Since 1990, many scientific evaluations of this technique conclude positively on PET's future. As it essentially is a functional method, the future developments of PET will largely depend on the discovery of new tracers.