A Comparative In Vitro and In Vivo Study of Osteogenicity by Using Two Biomaterials and Two Human Mesenchymal Stem Cell Subtypes.

Background: Bone repair induced by stem cells and biomaterials may represent an alternative to autologous bone grafting. Mesenchymal stromal/stem cells (MSCs), easily accessible in every human, are prototypical cells that can be tested, alone or with a biomaterial, for creating new osteoblasts. The aim of this study was to compare the efficiency of two biomaterials-biphasic calcium phosphate (BCP) and bioactive glass (BG)-when loaded with either adult bone marrow mesenchymal stem cells (BMMSCs) or newborn nasal ecto-mesenchymal stem cells (NE-MSCs), the latter being collected for further repair of lip cleft-associated bone loss. Materials and Methods: BMMSCs were collected from two adults and NE-MSCs from two newborn infants. An in vitro study was performed in order to determine the best experimental conditions for adhesion, viability, proliferation and osteoblastic differentiation on BCP or BG granules. Bone-associated morphological changes and gene expression modifications were quantified using histological and molecular techniques. The in vivo study was based on the subcutaneous implantation in nude mice of the biomaterials, loaded or not with one of the two cell types. Eight weeks after, bone formation was assessed using histological and electron microscopy techniques. Results: Both cell types-BMMSC and NE-MSC-display the typical stem cell surface markers-CD73+, CD90+, CD105+, nestin - and exhibit the MSC-associated osteogenic, chondrogenic and adipogenic multipotency. NE-MSCs produce less collagen and alkaline phosphatase than BMMSCs. At the transcript level, NE-MSCs express more abundantly three genes coding for bone sialoprotein, osteocalcin and osteopontin while BMMSCs produce extra copies of RunX2. BMMSCs and NE-MSCs adhere and survive on BCP and BG. In vivo experiments reveal that bone formation is only observed with BMMSCs transplanted on BCP biomaterial. Conclusion: Although belonging to the same superfamily of mesenchymal stem cells, BMMSCs and NE-MSCs exhibit striking differences, in vitro and in vivo. For future clinical applications, the association of BMMSCs with BCP biomaterial seems to be the most promising.

[Ultrasound scan of a fetus with facial cleft must be done from the lip to the uvula]. / L'examen échographique d'un fœtus porteur d'une fente faciale doit se faire depuis la lèvre supérieure jusqu'à la luette.

Facial cleft are the most frequent craniofacial anomalies with an incidence of one for 1000 births, all births combined, and require specialized multidisciplinary care. Since 2005, the systematic realization of two ultrasound views (nose-lip and profile) is recommended for the exploration of the fetal face in the 2nd trimester of pregnancy. Application of these recommendations should allow screening of the majority of cleft lip and palate. However, cleft palates, without labiomaxillary involvement, are currently largely underdiagnosed at prenatal ultrasound, although they can be associated with a syndromic diagnosis in up to 30% of cases. The aim of this work is to describe, from embryology to surgical consultation, the complete ultrasound examination of a fetus with a classic facial cleft.

A thoracic parasternal granular cell tumor in a child: About one case and review of the literature, discussion about treatment guidelines, is complete resection compulsory?

Granular cell tumor is a well known soft tissue tumor, very rare in children; we describe here the first case of GCT in this particular location in a child. The diagnostic is easily done with histopathology. The treatment is based on the complete resection, no other validated treatment exists. We reviewed the literature to find out if it would be safe to consider a simple follow-up after partial resection of the tumor.

[Keloid scars (part II): Treatment and prevention]. / Les cicatrices chéloïdes (deuxième partie) : arsenal et stratégie thérapeutique.

Keloids scars are a dysregulated response to cutaneous wound healing and can be associated with substantial physical and psychological distress. Unfortunately, they occur when surgical excision is performed. While the pathogenesis of keloids continues to be investigated, numerous treatment options exist. But there is still no ideal treatment. Their management needs association of treatment and long-term follow-up to observe and manage recurrence. In this second part, we propose a strategy for management of keloids scars.

[A nasal congenital malformation not published in the literature: About 5 cases]. / Une malformation nasale congénitale non retrouvée dans la littérature : à propos de 5 cas.

AIM OF STUDY: Five cases of the same congenital malformation of the nose, affecting the nasal dorsum and the supra-tip, were supported in our university plastic surgery center. This malformation has not been described in the literature known to the authors. The aim of this study is to analyze this nasal deformity. PATIENTS AND METHODS: Five children presented this congenital deformity between 1994 and 2014. The patients were examined and the malformation precisely described. Genetic and histological examinations were carried on. The diagnosis and treatment of this pathology were discussed. RESULTS: This malformation associated 4 anomalies: hypertrophy of soft tissue of the dorsum located in the middle third of the nasal bridge, deformed alar cartilages turned back downward and outside, advanced support default and median skin brand similar to a scar. These patients showed no other abnormalities of the midline or respiratory disorders. No genetic disorder was found for these five patients, and no histological arguments were found. Three patients were operated, one until adulthood with a satisfying cosmetic result. CONCLUSION: Bibliographic research has not allowed us to make an accurate diagnosis of this malformation that appears to be non-syndromic and to have a genetic origin. Our therapeutic approach became more clear and it now seems legitimate to propose early excision of fat mass to prevent alar deformations, associated with a cortico-cancellous graft, which in our experience grows with age, to support the tip.

[Palpebral malformations in children]. / La paupière malformative chez l'enfant.

Palpebral malformations can be isolated or associated with a craniofacial disorder. Their assessment is based on clinical examination; additional investigations are mainly done to characterize craniofacial syndromes. In case of extrapalpebral lesions or complex craniofacial pathology, genetic testing must be performed. Some isolated malformations will only need a simple follow-up; others must have specific treatment, undertaken following a precise timing, taking into account child and anatomical structures' growth and the possible consequences of the malformation on the eye and child's sight (degree of urgency). When dealing with these malformations, there are two main risks to be taken into consideration: corneal irritation due to lagophtalmos and amblyopia owing to visual axis obstruction, anisometropia or strabismus.

[Skin tumors in children]. / Les tumeurs cutanées de l'enfant.

The presence of a congenital or acquired cutaneous lesion is a frequent reason for consultation in pediatric plastic surgery unit. The management of these lesions requires a good knowledge of specific diagnoses in children. This step is sometimes difficult because of the multiplicity of possible diagnosis. Some skin tumors may be the external sign of a general disease or an underlying malformation; those can change the overall prognosis and management and require to be properly identified. The decision of surgical excision depends on various criteria, including diagnosis but also the reconstruction possibilities. The timing of surgical treatment depends on the medical emergency of the tumor resection (benign tumor, spontaneously regressive tumor, risk of degeneration into malignancy), on the cosmetic and psychological impact but also on the growth or learning steps in child life. This article first provides an aid in the diagnosis of the most common or more characteristic skin tumors. The algorithm is principally based on the pigmentation aspect of the tumor. The age and conditions of the surgical management are specified for each type of tumor. Cutaneous hemangiomas and vascular malformations, and congenital cysts and fistulas are not reported in this article.

[The complications of skin expansion in paediatrics: Diagnostic, taking over and prevention]. / Les complications de l'expansion cutanée en pédiatrie : diagnostic, prise en charge et prévention.

Skin expansion is a difficult and long process in which can occur more or less serious complications. Overall complications rates describe in the literature vary between 13 and 37%. We can categorize them in major complications, which can lead to a failure maybe even an aggravation of the anterior status, and in minor complications that do not compromise the expansion process but can alter it. The main major complications are infection, skin suffering and necrosis which can lead to prosthesis exposition, leaks and technical problems with equipment dysfunctions that may cause difficulties or a failure of the inflations. The main minor complications are hematomas, seromas, valve or tube exposition, pains with paraesthesias caused by neighbouring organs compression, pathologic and unsightly scares and can lead to an important psychological impact. These complications can be due to a precarious skin's state, a material dysfunction or unpredictable technical problems but also by an inappropriate preoperative indication or planning. The emerging of a complication, however, is not synonymous to a failure of the procedure; a satisfactory reconstruction may still be obtained in 75% of all cases. The purpose of this article is to help to identify the situations at risk of complications in order to prevent, detect and treat them early.

[Autologous fat grafting in children]. / Les particularités du lipofilling chez l'enfant.

Lipofilling or fat grafting transfer is defined as a technique of filling soft tissue by autologous fat grafting. The basic principle of lipofilling is based on a harvest of adipose tissue, followed by a reinjection after treatment. Lipofilling main objective is a volume defect filling, but also improving cutaneous trophicity. Lipofilling specificities among children is mainly based on these indications. Complications of autologous fat grafting among children are the same as those in adults: we distinguish short-term complications (intraoperative and perioperative) and the medium and long-term complications. The harvesting of fat tissue is the main limiting factor of the technique, due to low percentage of body fat of children. Indications of lipofilling among children may be specific or similar to those in adults. There are two types of indications: cosmetic, in which the aim of lipofilling is correcting a defect density, acquired (iatrogenic, post-traumatic scar) or malformation (otomandibular dysplasia, craniosynostosis, Parry Romberg syndrom, Poland syndrom, pectus excavatum…). The aim of functional indications is correcting a velar insufficiency or lagophthalmos. In the paediatric sector, lipofilling has become an alternative to the conventional techniques, by its reliability, safety, reproducibility, and good results.

Added value of MRI for the prenatal diagnosis of isolated orofacial clefts and comparison with ultrasound.

OBJECTIVES: To evaluate the added value of fetal magnetic resonance imaging (MRI) in diagnosing and assessing isolated orofacial clefts and compare MRI with second-line diagnostic ultrasound. MATERIALS AND METHODS: In a two-year prospective bicenter study, fetuses with isolated orofacial clefts were reassessed using second-line diagnostic ultrasound and MRI. The results of second line ultrasound and those MRI were compared to each other. The gestational age at the time of ultrasound and MRI, and the final diagnosis for each of the imaging modalities were recorded. Finally, the results of second line ultrasound and those of MRI were compared to the results of neonatal clinical examination after delivery that served as standard of reference. RESULTS: Twenty-two women were included after informed consent was obtained. On average, diagnostic ultrasound was performed at 25.5weeks of gestation (range: 24-34weeks) and MRI at 29.5weeks of gestation (range: 27-34weeks). The results of ultrasound and those of MRI findings were strictly consistent in 20 women (20/22; 91%) but differed in 2 women (2/22; 9%). For all fetuses, the final radiological diagnosis was confirmed by clinical examination after delivery. CONCLUSION: If ultrasound examination proves technically challenging, fetal MRI can be used to obtain the same diagnostic information in 91% of cases and can help surgeons and interdisciplinary teams provide appropriate antenatal counseling.

[Median congenital fistula of the upper lip associated with Goldenhar syndrome: Report of a case]. / Fistule congénitale médiane de la lèvre supérieure associée à un syndrome de Goldenhar : à propos d'un cas.

AIM OF THE STUDY: We present the case of a patient with Goldenhar syndrome associated with congenital fistula in the middle of philtrum at the upper lip. PATIENTS AND METHODS: The patient was supported from birth for Goldenhar syndrome. Several procedures were performed in childhood to correct a macrostomia and mandibular hypoplasia right. At 14 years old, she has a rhinoplasty to correct a complex nasal malformation. During the procedure, a hole in the upper central incisor inter-space is found. It is extended by a fistula which runs through the front palate towards the vomer rail at the base of the partition. This fistula is blind behind the vomer and can be completely resected. Pathological examination of the resection reveals a squamous lining. RESULTS: The median fistula of the upper lip are extremely rare : less than 30 cases reported in the literature. The unusual run of the fistula and the recent appearance of a pituitary syndrome in this patient makes us look for a continuity between it and Rathke's pocket by computed tomography imaging type. CONCLUSION: The association of Goldenhar syndrome and median congenital fistula of the upper lip was never yet described in the literature. The association with hypopituitarism was suspected with the posterior extension of the malformation, not confirmed by the scanner, but strongly suspected jointly by the neurosurgical and plastic surgery team.

[The gingivo periosto plastic surgery with osseous substitute: Technique and first results]. / La gingivopériostoplastie avec substitut osseux : technique et premiers résultats.

INTRODUCTION: Ortho dontico-surgical coverage of alveolar crack is essential in reason of its repercussions on facial growth and implementation of children's teeth set. We proposed to realize a premature gingivo periosto plastic surgery from 4 years old by osseous substitute to lower age of alveolar cracks closure and decrease the morbidity of patients. PATIENTS AND METHODS: We conducted a retrospective study over one year (January, 2012 to December, 2012), with six months postoperatively outcomes, on 23 cases of gingivo periosto plastic sugary with osseous substitute type glass by bone transplant at infantile plastic surgery service of Timone - Children teaching hospital of Marseille, France. RESULTS: We held 23 patients. Seventeen children, 12 boys and 5 girls presented unilateral cracks. Twenty patients required a quantity of glass bone under 1cc for the narrow cracks. Prevalence of the mucous cracks was low (4 cases on 23). Technique of gingivo periosto plastic surgery with osseous substitute is simple and our results are globally satisfactory. We observed less morbidity of the operating site. CONCLUSION: The first results of this study showed that gingivo periosto plastic surgery with osseous substitute glass bone is a simple, reliable and reproducible technique, with promising results. Reduction of site's morbidity by osseous transplant, accessibility of glass bone cost and simplicity of surgical gesture justified adoption of this technique.

[Management of oculo-palpebral consequences in facial paralysis]. / Prise en charge des atteintes orbito-palpébrales secondaires à une paralysie faciale.

Facial paralysis prognostic depends on eye lesion. In this pathology, lacrymal and palpebral functions will be modified: bad eye closure and leak of tears secretions. It can leads to corneal complications from keratitis to corneal abcedation and visual dysfonction. This chapter details different procedures and their indications to avoid this kind of complications.

Clinical presentation of PTEN mutations in childhood in the absence of family history of Cowden syndrome.

BACKGROUND: PTEN gene (MIM 601628) is a tumor suppressor gene implicated in PTEN hamartoma tumor syndromes (PHTS) including Cowden syndrome, Bannayan-Riley-Ruvalcaba syndrome, and Proteus-like syndrome. Bannayan-Riley-Ruvalcaba syndrome is considered as the pediatric form of PHTS. More recently, children presenting autism spectrum disorders with macrocephaly (ASD-M) have been reported. METHODS: We report clinical data from seven patients diagnosed in childhood with a PTEN germline mutation, excluding cases of familial Cowden syndrome. RESULTS: This study underlines the variability of phenotype associated with PTEN mutations diagnosed at pediatric age. Most of the patients did not fulfill usual criteria of Bannayan-Riley-Ruvalcaba syndrome or ASD-M. CONCLUSION: PTEN testing should be considered in any child presenting with severe macrocephaly (>+4SD) and another feature of PHTS.

[The growing nose]. / Le nez en croissance.

Children noses have special anatomical and functional characteristics. Early interventions performed before the end of the child's growth have been a main topic as they may cause adverse effects on the subsequent development and the function. This chapter describes the characteristics of the nasal pyramid and the septum at different stages of growth. Should one's approach be very cautious in children surgical indications, one should not hesitate opting for the treatment of congenital malposition or acquired when they jeopardize the nasal function. Considering possible procedures and specific conditions within pediatric field are discussed in this chapter.

[Current place of curettage in the management of giant congenital nevi: report of 29 patients]. / Place actuelle du curetage dans la prise en charge des nævi géants congénitaux: à propos de 29 cas.

SUBJECT: The management of giant nevi remains discussed to date. Curettage, early superficial technique, allows a lightening of the lesions. We wanted to define the current interest in its use, from results obtained after 20 years of experience. PATIENTS AND METHODS: Twenty-nine patients were treated by curettage between 1991 and 2011. Surgery consisting of excision of the superficial dermis was performed between the 1st and 7th week of life in an average of 1.8 procedures. The cosmetic result was judged subjectively. RESULTS: Healing was achieved in 7 days. We noted a few local complications but no general complications. The aesthetic results, with initial variable lightening, induced an overall parental satisfaction in 100 % of cases. A secondary heterogeneous and varying pigmentation was observed in all cases. CONCLUSION: To date, we believe that curettage is indicated for the treatment of giant nevi in some localization difficult to treat by conventional techniques (eyebrow, those associated with many satellite nevi) or those with a psychological impact too important to support a therapeutic delay.

[Eyelid approach and pediatric orbital tumors: indications and surgical technique. About three children]. / Voie trans-palpébrale et chirurgie des lésions intraorbitaires pédiatriques : indications et technique chirurgicale. À propos de trois enfants.

Interdisciplinary collaboration can offer unusual approaches for the treatment of orbital pathologies. The authors report their experience with the eyelid incision in treating three children with epidural orbitary hematoma, dermoid cyst of the upper outer quadrant and a dermoid cyst of the internal inferior quadrant. For all the children, removal was complete and cosmetic results satisfying. The authors discuss the indications and complications of this surgical approach.

[Breast morphology in Turner's syndrome. A prospective multicentre clinical study about 21 cases]. / Morphologie mammaire dans le syndrome de Turner. Étude clinique prospective multicentrique de 21 cas.

INTRODUCTION: The Turner's syndrome encompassed several conditions, of which monosomy X (absence of the entire sex chromosome X) is most common. It is a chromosomal abnormality in which all or part of the sex chromosomes X is absent. Typical females have two X chromosomes, but in Turner's syndrome, one of those sex chromosomes is missing or presents abnormalities. Patients show a shield shaped thorax with thick and bulging chest, breast hypotrophy and widely spaced nipples. The objective of this study was to characterize the breast abnormalities observed in Turnerian. PATIENTS AND METHODS: We describe a prospective multicentric study (August 2007-March 2008) on 21 nullipar patients, ranging from 16 to 35 years old. Six were monosomic and 14 were Turner mosaic (in this case the chromosome is missing in some cells but not others), 19 were treated with estrogens and progestatives. This study was achieved through the use of clinical examinations including body, waist, hips (BWH) measurements and photography. The statistical method involved a descriptive analysis, linear correlation calculations and student test. RESULTS: The breast morphology appears to be quite closed to that of the general woman population, but with average thorax volume more bulky mainly in the anteroposterior zone, and with more reduced breast volumes. No specific abnormalities in the chest development were observed. No differences in the hypotrophy, hypertrophy, and normal breast volume repartition were observed between monosomic and mosaic patients. The self-satisfaction index on the breast look is quite low, patients mainly complain about breast hypotrophy. Nevertheless, these results are not representative of the whole turnerians, since this study address only to volunteer patients and we cannot exclude possible distortions. CONCLUSIONS: In contrast to common beliefs, we don't have observed any increase of the average of the internipple space; this observation is in good agreement with the most recent published literature works, which report only an apparent increase of this intermamelonary distance versus the thoracic width (in front view), probably caused to an optical distortion effect.

[Role of the plastic surgeon in the management of ecthyma gangrenosum in children: clinical example]. / Rôle du chirurgien plasticien dans la prise en charge de l'ecthyma gangrenosum chez l'enfant : exemple clinique.

Ecthyma gangrenosum is a cutaneous infection, which result from a Pseudomonas aeruginosa septicemia, encountered in most of the case in immunocompromised people. Authors demonstrate the important role of the plastic surgeon in the diagnosis and therapeutic management of the disease in children. An eight-month-old infant has been hospitalized for acute leukaemia. She developed an extensive painful macule in the buttocks and perineal area in a septic context. A multidisciplinary management allowed to set up an adapted antibiotherapy, an early escharrotomy, a protection of the wound by digestive and urine derivation and a reconstruction with wound healing by second intention and split thickness skin graft, which lead to a good quality cure and wound healing at the end of 37 days of evolution. This case demonstrates the importance of the surgical management in the treatment of ecthyma gangrenosum. The wound healing associated with a split thickness skin graft seems to be the less invasive solution in a frail patient and the fastest to re-start the chemotherapy.

[Diagnosis and management of vascular anomalies]. / Diagnostic et prise en charge des anomalies vasculaires.

Vascular anomalies are a complex pathological group. They are especially difficult to study because of confusion in the terminology used. The classification developed by the International Society for the Study of Vascular Anomalies (ISSVA) in 1996 allows using a common scientific language. There are two groups of lesions: vascular tumor and vascular malformation. The management of these anomalies is difficult and must involve an interdisciplinary approach including specialists in plastic surgery, radiology, pediatry and dermatology. We propose a simplified approach for the management of these pathologies. This approach is coming from the experience of Marseille (France) multidisciplinary team.