Publications by authors named "Natacha Kadlub"

Objectives: The nasometer is the most widely used tool for objective assessment of phonation in both research and clinical practice. French standards have been validated in cases of total cleft lip and palate. The objective of this research is to propose a second validation study on velopalatal clefts.

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Purpose: Patients with syndromic hemifacial microsomia (SHFM) are at risk of obstructive sleep apnea (OSA). The aim of the study was to describe the prevalence of OSA and its management, especially in patients with Goldenhar syndrome (GS).

Methods: The respiratory polygraphies and clinical management of 15 patients, aged 2 to 23 years, evaluated at a national reference center, were analyzed.

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Background: Achondroplasia is the most frequent FGFR3-related chondrodysplasia, leading to rhizomelic dwarfism, craniofacial anomalies, stenosis of the foramen magnum, and sleep apnea. Craniofacial growth and its correlation with obstructive sleep apnea syndrome has not been assessed in achondroplasia. In this study, we provide a multimodal analysis of craniofacial growth and anatomo-functional correlations between craniofacial features and the severity of obstructive sleep apnea syndrome.

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Objective: The aim of this systematic review was to determine if there exists an efficacious drug treatment for cherubism, based on published studies.

Methods: This systematic review included observational case studies reporting pharmacological management of cherubism. We developed specific search strategies for PubMed (including Medline), ScienceDirect, Web of Science.

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: Durable reconstruction of critical size bone defects is still a surgical challenge despite the availability of numerous autologous and substitute bone options. In this paper, we have investigated the possibility of creating a living bone allograft, using the perfusion/decellularization/recellularization (PDR) technique, which was applied to an original model of vascularized porcine bone graft. : 11 porcine bone forelimbs, including radius and ulna, were harvested along with their vasculature including the interosseous artery and then decellularized using a sequential detergent perfusion protocol.

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Introduction: Large bone defects are challenging for surgeons. Available reimplanted bone substitutes can't properly restore optimal function along and long term osteointegration of the bone graft. Bone substitute based on the perfusion-decellularization technique seem to be interesting in order to overcome these limitations.

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The human fascia lata (HFL) is used widely in reconstructive surgery in indications other than fracture repair. The goal of this study was to compare microscopic, molecular, and mechanical properties of HFL and periosteum (HP) from a bone tissue engineering perspective. Cadaveric HP and HFL ( = 4 each) microscopic morphology was characterized using histology and immunohistochemistry (IHC), and the extracellular matrix (ECM) ultrastructure assessed by means of scanning electron microscopy (SEM).

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Introduction: To support mother with antenatal diagnosis of cleft lip/palate (CL/P), we implement an antenatal breastfeeding workshop to promote breastfeeding and its continuation. The aim of this study was to evaluate patient satisfaction after this workshop and efficiency of this workshop on breastfeeding rates and duration.

Materials And Methods: We conducted a prospective study from February 2018 to April 2022.

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Background: Velopharyngeal insufficiency persists in 15 to 30% of children with cleft palate, despite early velar surgery. Pharyngoplasty using a superior pedicle flap is the most common secondary surgery to treat velopharyngeal insufficiency. This study aims to identify the criteria leading to indicate velopharyngoplasty in 3 groups of age.

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Introduction: The head and neck (HN) are the most frequent sites of pediatric rhabdomyosarcoma (RMS). Alveolar RMS (ARMS) represents ~20% of all RMS cases and frequently spread to lymph nodes (LNs). The aim was to report locoregional control, event-free survival (EFS), and overall survival (OS), according to clinical and pathological features, LN staging, and treatment modalities.

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This study aimed to analyze surgical procedures for head and neck Ewing sarcoma (HNES) with regard to oncological, functional, and esthetic outcomes. A blinded multidisciplinary retrospective chart review of operated French HNES patients (Euro-EWING 99 trial, 1999-2014) was performed to assess patient/tumor characteristics, treatment details, and outcomes. Primary surgery without reconstruction was undertaken in 13 patients (emergency context/misdiagnosis).

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Objectives: Nevoid Basal Cell Carcinoma Syndrome (NBCS) is a rare genetic condition affecting multiple organs including the maxillofacial and dental region. The surgical removal of the odontogenic keratocystic tumors (OKT), the high rate of recurrence leads to a iatrogenic tooth loss requiring dental care. The aim of this study is therefore to describe the dental and orthodontic management, and to assess the impact of surgery on facial growth and oral development.

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Background: Macrostomia or lateral cleft lip is a rare congenital deformity. In this article we describe a surgical technique of macrostomia repair developed. The objective of this article is to assess the results of our surgical technique and to validate a method for macrostomia surgical result evaluation.

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Article Synopsis
  • - The study investigates the impact of the Interregional Pediatric Multicentric Tumor Board (IPMTB) on managing pediatric head and neck tumors in the Ile-de-France region of France, focusing on cases discussed from 2013 to 2018.
  • - A total of 679 cases, mostly malignant tumors (71%), were reviewed, with proposals made by the IPMTB being followed in 86% of instances, highlighting a significant change in treatment approaches during discussions.
  • - The IPMTB enhances collaboration among medical specialties, improves adherence to modern treatment protocols, and facilitates knowledge sharing for the management of rare tumors in pediatric patients.
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Purpose: Objective structured clinical examinations (OSCE) evaluate clinical reasoning, communication skills, and interpersonal behavior during medical education. In France, clinical training has long relied on bedside clinical practice in academic hospitals. The need for a simulated teaching environment has recently emerged, due to the increasing number of students admitted to medical schools, and the necessity of objectively evaluating practical skills.

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Article Synopsis
  • Cherubism is a rare genetic condition caused by a mutation in the SH3BP2 gene that affects the jaw bones, making them soft and filled with certain types of cells.
  • Scientists found a 6-year-old girl with a severe form of cherubism who showed not only jaw problems but also low bone density and signs of inflammation in her body.
  • If it turns out that cherubism can affect the whole skeleton, it could make treatment easier for patients, potentially using new medicines that target their entire body instead of just the jaw area.
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Knowledge of mandibular periosteum mechanical properties is fundamental for understanding its role in craniofacial growth, in trauma and bone regeneration. There is a lack in the literature regarding mechanical behavior of the human periosteum, including both experimental and modeling aspects. The proposed study involves tensile tests of periosteum samples from different locations including two locations of human mandibular periosteum: lingual and vestibular, compared with samples from various locations of the calvarial periosteum.

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The use of the Washio retroauricular flap for nasal reconstruction has been infrequently covered in recent literature, particularly concerning the pediatric population. A retrospective study was conducted between 2014 and 2018 and included all pediatric patients who underwent a Washio retroauricular flap procedure for nasal reconstruction operated on by the same surgeon at a referral center for pediatric plastic and maxillofacial surgery. The mean age at the time of the first stage of the Washio procedure was just under 8 years of age (range: 6 years 3 months-8 years 10 months).

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Article Synopsis
  • Large congenital melanocytic nevi (lCMN) are traditionally managed through repeated surgeries, with limited medical treatments available.
  • This study involved 17 patients with NRAS-mutated lCMN and explored the effects of MEK and Akt inhibitors on nevocyte activity, showing promising results in reducing cell viability and proliferation.
  • Ultimately, the research suggests that intradermal injection of these inhibitors could potentially serve as a new treatment option to reduce the need for surgeries in lCMN cases.
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Introduction: Transverse mandibular deficiency has been traditionally managed by orthodontic compensation. However, without resolving the underlying skeletal hypoplasia it leads to high relapse rates. Few studies have reviewed the long-term experience and potential complications of mandibular symphyseal distraction osteogenesis (MSDO) as an alternative treatment method.

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Pierre Robin sequence (PRS) may lead to life-threatening respiratory and feeding disorders. With the aim to analyse the association of the severities of retrognathia and glossoptosis with those of respiratory and feeding disorders, we retrospectively studied a series of 50 infants with retrognathia, glossoptosis, cleft palate, and airway obstruction. The patients were managed from birth to at least 6 years of age by a single pediatric team at the Armand Trousseau Hospital in Paris within a 12 years period (2000-2012).

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Article Synopsis
  • This study looked at French kids with head and neck Ewing's sarcomas, focusing on their age, tumor types, and how they were treated.
  • 47 patients were reviewed, most of them had small bone tumors and almost none had the disease spread to other parts of the body.
  • The research found that having experienced doctors and teams discussing treatment plans can help in giving these patients the best chance for recovery.
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Background: Cherubism is a rare autosomal dominant disorder of the jaws caused by mutation of the SH3BP2 gene. The bone is replaced by a fibrous granuloma containing multinucleated giant cells. Cells of the cherubism granuloma have never been systematically analyzed.

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