Primary cheilorhinoseptoplasty using the Talmant protocol in unilateral complete cleft lip: functional and aesthetic results on nasal correction and comparison with the Tennison-Malek protocol.

Many surgical procedures used to treat patients with unilateral complete cleft lip do not include a complete primary rhinoseptoplasty, which is delayed until the end of growth as part of secondary surgery. Primary cheilorhinoseptoplasty using the Talmant technique has been performed at Lapeyronie University Hospital, Montpellier for 15 years. This retrospective study evaluated and compared the functional and aesthetic results obtained in such patients at 4-6 years after surgery with those obtained without primary rhinoseptoplasty in patients undergoing the Tennison-Malek technique. This study included a cohort of 60 children who presented a unilateral complete cleft lip: 29 were operated on with the Tennison-Malek technique and 31 with the Talmant technique. Three functional and 14 aesthetic criteria were evaluated. All functional criteria showed better results in the Talmant group: nocturnal nasal breathing (P < 0.001), incidence of closed rhinolalia (P = 0.0019), and presence of asymmetric nasal breathing (P < 0.001). Concerning the aesthetic results, all criteria evaluated on the frontal and basal photographs of each patient showed significantly better results in the Talmant technique group. The rate of satisfactory results was> 75% for 12 of the 14 criteria in the Talmant group, compared with none of the 14 criteria in the Tennison-Malek group.

Partial hybrid cranial vault remodeling in late correction of scaphocephaly and revision surgery. A monocentric retrospective study of nine consecutive cases.

There is currently no consensus on the surgical attitude to be adopted for late management of sagittal synostosis or for revision scaphocephaly surgeries without prolapse of the cerebellar tonsils. We present here a monocentric retrospective study of nine patients operated consecutively by a fixed expansion method of the cranial vault which may be associated with a fronto-orbital remodeling. The procedure consists in the realization of multiple parietal tongue-in-groove osteotomies fixed by resorbable plates. Simultaneous fronto-orbital remodeling have been performed when needed. No intraoperative complication was noted. The average operating time was 141min. Six patients (66.7%) had a blood transfusion during the perioperative period. The average hospital stay was 4.8days. With a mean follow-up of 26,7months, no surgical revision was noted. In all patients with clinical or ophthalmologic signs of intracranial hypertension, we highlighted a disappearance of signs within 4months. No protective helmet has been used. The craniofacial remodeling was judged very good by the family and the surgical team. Multiple tongue-in-groove tenons remolding cranioplasty associated or not with a fronto-orbital advancement is a safe technique. It seems to us to be a good alternative to floating or fixed bi-parietal or bi-parieto-frontal cranial flaps because of the very large increase in endocranial volume and the possibility of physiologically and aesthetically remodeling the parietal and fronto-orbital regions.

[Congenital cysts and fistulae in children]. / Kystes et fistules congénitaux de l'enfant.

Cysts and fistulae of the face and neck in children are formed before birth and correspond to the persistence of embryonic remnants that occur due to coalescence defects of embryonic buds or due to epidermal inclusion. They represent the most common pathological malformation of the face and neck. They may be separated according to their location into laterocervical cysts and fistulas or median cysts and fistulas. Their discovery may occur prematurely at birth or later during growth or in adulthood. Their treatment always requires surgical excision, which must be complete in order to prevent recurrences.

Bilateral idiopathic calf muscle hypertrophy: an exceptional cause of unsightly leg curvature.

The authors present the management of a young female patient who presented with longstanding bilateral calf muscle hypertrophy, with no known cause. Taking into account the patient's wishes and the fact that the hypertrophy was mainly located in the posteromedial compartment, we chose to carry out a subtotal bilateral resection of medial gastrocnemius muscles. This procedure was performed with an harmonic scalpel, permitting a excellent cosmetic result while avoiding complications or functional impairment. After a reviewing of the commonly used techniques, the authors discuss the chosen surgical approach taking into account its clinical particularity.

Mandibular effects of maxillary distraction osteogenesis in cleft lip and palate.

Maxillary distraction osteogenesis (DO) is a reliable treatment for severe maxillary deficiency in cleft lip and palate (CLP). The objective was to analyze its long-term effects on the mandible. A retrospective study of 24 CLP treated with maxillary DO using the Polley and Figueroa technique was done; patients were followed for more than 4 years. Preoperative (T0), 6-12 months postoperative (T1), and ≥ 4 years postoperative (T2) cephalometric radiographs were evaluated. A classical cephalometric analysis was used to assess treatment stability, and a Procrustes superimposition method was used to assess local changes in the shape of the mandible. The mean age of patients at T0 was 15.4 ± 4.1 years. SNA increased at T1 and T2 (P < 0.001), with no significant relapse between T1 and T2, indicating stability at 1 year after treatment (T0 = 72.4 ± 5.3°; T1 = 81.3 ± 6.2°; T2 = 79.9 ± 6.1°). SNB, facial angle, gonial angle, and symphyseal angle remained stable. Long-term analysis of the mandible demonstrated a minimal counter-clockwise rotation of the body (mandibular plane = -0.2 ± 3.2°) and ramus (-0.6 ± 4.3°). Maxillary DO in CLP had no significant effect on the shape or rotation of the mandible. The maxillary advancement remained stable after 1 year.

[Management of plexiform neurofibroma isolated in childhood: four patients]. / Prise en charge du neurofibrome plexiforme isolé de l'enfant : à propos de quatre observations.

Plexiform neurofibroma is a rare and benign tumor often associated with type 1 neurofibromatosis (NF1) or Von Recklinghausen's disease. Present in one third of cases of NF1, there are isolated forms where the diagnosis should remain a diagnosis of exclusion. We report four cases of isolated plexiform neurofibromas found in children from topographies and we discuss the pretherapeutic assessment, the surgical management and the long-term follow-up.

Accuracy of prenatal three-dimensional ultrasound in the diagnosis of cleft hard palate when cleft lip is present.

OBJECTIVE: To investigate the accuracy of prenatal axial three-dimensional (3D) ultrasound in predicting the absence or presence of cleft palate in the presence of cleft lip. METHODS: Between March 2005 and January 2009, there were 81 cases with a prenatal two-dimensional (2D) ultrasound screening diagnosis of unilateral or bilateral cleft lip at 22-25 weeks of gestation referred to our tertiary care center. Of these, 79 fetuses were included in this prospective study and two were excluded. Axial 3D ultrasound imaging of the fetal palate was performed and the diagnoses were compared with clinical findings at delivery. The frequencies of intact and cleft palate, the degree of association between the prenatal predictions and postnatal findings and the probability of detection of cleft lip and palate were determined. RESULTS: Of 79 prenatal predictions, 77 (97%) were correct and the association between the prenatal predictions and postnatal findings was strong. The sensitivity for detection of cleft lip and palate within this high-risk population was 100% and the specificity was 90%. In one of the excluded cases, the palate could not be visualized due to a fetal prone position. There were chromosomal anomalies in 4% of cases and associated structural or growth anomalies in 23%, termination of pregnancy was carried out in 4% and intrauterine fetal demise occurred in 3%. CONCLUSION: Axial 3D ultrasound of the fetal palate has high accuracy in identifying prenatal cleft palate when cleft lip is diagnosed at mid-trimester 2D ultrasound screening.

Prenatal assessment of the normal fetal soft palate by three-dimensional ultrasound examination: is there an objective technique?

OBJECTIVES: To describe a three-dimensional (3D) ultrasound technique for assessing the fetal soft palate. METHODS: A prospective study conducted from April to December 2006 included 87 fetuses in a low-risk population. Fetal ultrasound scans were performed between 21 and 25 weeks of gestation to determine the normal 3D ultrasound view of the fetal soft tissues of the palate. The sonographers used a 30 degrees-inclined axial 3D view of the fetal palate. Ultrasound images obtained in this view were compared with fetopathological specimens of the same gestational age by two observers, both pediatric surgeons. Each observer indicated whether they thought that the uvula or the velum could be detected, and the differences in responses between the observers were assessed. RESULTS: The frequencies of detection of the uvula and velum of each observer varied between 80% and 90%. The observers showed a significant difference in judging the visualization of the uvula, but not in judging the velum. CONCLUSIONS: A 30 degrees -inclined axial 3D ultrasound view seems to be effective in assessing the integrity of the fetal soft palate.

Use of Integra in a paediatric upper extremity degloving injury.

Dermal substitutes such as Integra have been used in surgery since 1981. This product was developed to cover large skin burns but it may have a role in reconstructive upper limb surgery. In upper extremity avulsion, the choice of skin cover depends on many variables. Dermal substitute is an alternative when split-thickness skin grafting is appropriate. We present a case of severe upper limb avulsion, or 'degloving', in a child of 9 years old which was treated successfully with Integra. Integra is particulary useful in growing children to cover large skin losses.

[Management of chicken pox purpura fulminans: a pediatric case report]. / Prise en charge d'un purpura fulminans varicelleux: à propos d'un cas pédiatrique.

The authors report a case of a 4 years old girl who had presented a chicken-pox purpura fulminans. Lesions appeared 5 days after chicken-pox start and were quickly evoluted in cutaneous and sub-cutaneous necrosis on external side of thighs and behind side of right calf. A medical management was done with fresh plasma, blood, antithrombine 3, and fibrin. Specifics treatments were done: heparin and activated C protein. Surgical treatment was realised 5 weeks later. It consisted of clean necrosis areas and put a thin skin graft witch was took on the scalp. The evolution was fast good. The follow-up is 3 years without big esthetic and functional consequences. Some cases of this pathology were described in literature with serious lesions. The management should be multidisciplinary. Surgical treatment should be realised when lesions are stabilized. Scalp is a donor site for skin graft very interesting because of big quantity of skin and not esthetic consequence.

[Study of the morphologic variations of the scaphocephaly. Deduction for their systematisation]. / Etude des variations morphologiques des scaphocéphalies. Implication pour leur systématisation.

PURPOSE: The scaphocephaly is the most frequent craniosynostosis secondary to a premature synostosis of the sagittal suture. Despite this univocal pathogeny there are a great heterogeneity in the skull deformation and their systematisation stay imprecise. The aim of this study was to analyse the different morphology of the scaphocephaly by 3D CT-scan to clarify the different shapes. MATERIAL AND METHODS: This retrospective study was done from the morphometric analysis of forty-two 3D CT-scan (10 girls and 32 boys). The scaphocephaly was classified in two groups according to the systematisation of Stricker and Montaut: the sphenocephaly (large forehead) and the leptocephaly (narrow forehead). The variation of the calvaria shape was analysed and different index were calculated. RESULTS: There were 30 sphenocephaly and 12 leptocephaly. Excepted for the forehead deformation there was a morphometric difference of the occipital pole that was backward rotated in the leptocephaly. In the two groups there were retro-coronal constriction (40.5%) or pre-lambdoid constriction (9.5%) independently of the sagittal synostosis degree. CONCLUSIONS: The morphometric analysis shows that the deformation of the skull secondary to the premature synostosis of the sagittal suture can be different. The adaptative defect of the metopic suture can be to explain this difference. This deformation can be harmonious, or with a retro-coronal constriction resulting in a bitemporal narrowing, or with a pre-lambdoid constriction resulting in an occipital chignon. These results conduce to qualify the surgical correction to adapt at the different shapes.

Thoracoscopic repair of H-type tracheoesophageal fistula in the newborn: a technical case report.

H-type tracheoesophageal fistula (H-TEF) without esophageal atresia makes up 4% to 5% of esophageal congenital abnormalities. The authors present the thoracoscopic treatment of a 2.47-kg newborn boy with a fistula between the second and third thoracic vertebrae diagnosed by esophagography. Four trocars were used for fistula closure, and tracheal and esophageal suturing were accomplished without intraoperative incident. Five days after surgery, results of a barium swallow excluded anastomotic leaks. The chest tube was removed, and oral feeding was initiated.

Plagiocephaly: morphometry of skull base asymmetry.

The aim of this study was to perform a morphometric analysis of the skull base to understand the mechanism of asymmetry between malformational and deformational plagiocephaly. Since 1988, we have carried out 102 CT scans with three-dimensional reconstruction of the endocranial side of the skull base. There were 20 cases of malformational plagiocephaly, consisting of 18 unicoronal synostoses (UCS) and two unilambdoid synostoses (ULS), and 82 cases of plagiocephaly without synostosis, made up of 60 cases of frontal plagiocephaly (FP) and 22 of occipital plagiocephaly (OP). The skull base asymmetry was analyzed in three ways: (1) the hemibases and the cranial fossa angles were compared with each other; (2) the structural asymmetry of the chondrocranium was calculated; and (3) the architectural asymmetry was measured in relation to the medio-labyrinthine plane. The skull base asymmetry of malformational plagiocephaly was located in the anterior and middle cranial fossae for UCS and in the posterior and middle cranial fossae for ULS. The asymmetry was the result of a localized structural anomaly of the chondrocranium. The asymmetry of deformational plagiocephaly corresponded to a distortion in relation to the medio-labyrinthine plane while the chondrocranium remained symmetric. The skull base asymmetry of malformational plagiocephaly is secondary to a localized malformation of the chondrocranium, and that of the deformational plagiocephaly is secondary to an architectural deformation.

[Clinico-radiological study of the skull deformation in the plagiocephaly without synostosis]. / Etude clinico-radiologique des déformations du crâne dans les plagiocéphalies sans synostose.

UNLABELLED: The aim of this study was to analyze the mechanism of the skull deformation observed in frontal and occipital plagiocephaly without synostosis. POPULATION AND METHODS: From 1987 to 2001, 96 plagiocephalies without synostosis, 72 males and 24 females, were examined. There were 72 Frontal Plagiocephalies (FP) and 24 Occipital Plagiocephalies (OP). The clinical examination of the neck muscles was performed to find out a torticolis with or without retraction. All patients underwent a 3D CT scan of the skull to exclude a craniostenosis. Cephalic Index (CI) was also calculated. RESULTS: The FP had a torticollis in 89.3% of cases associated with a muscle retraction (54.8%), a tonus asymmetry (16.6%), or in association (28.6%). The skull had a parallelepiped shape and the greater axis was oblique. The CI was 0.85. The OP had a torticollis in 50% of case associated with a retraction (28.6%), a tonus asymmetry (57.1%), or the association (14.3%). The skull had a square shape with an occipital flat and the CI was 0.95. DISCUSSION: The FP and OP are two deformations whose mechanism is different. The FP corresponds to a three plans skull asymmetry secondary to muscle traction on the base of the skull, and the OP corresponds to a posterior localized asymmetry of the skull secondary to compression. CONCLUSION: The traction and/or compression deformation start in utero or in the newborn period and the supine position is an aggravating positional factor.

[The secondary bone grafting in cleft lip and palate: technical notes and indications about 62 cases]. / La greffe osseuse secondaire dans les fentes labio-maxillo-palatines totales : modalités techniques et indications à propos de 62 greffes.

INTRODUCTION: Secondary bone grafting of the maxilla has become a well-accepted procedure in the surgical protocol for rehabilitation of patients with cleft lip and palate (CLP). The aim of this study is to review the surgical procedure and the indication of the secondary bone graft. MATERIALS AND METHODS: Sixty-two secondary bone graft were retrospectively reviewed from 1993 to 2000. There were 50 unilateral CLP and 12 bilateral CLP. The age at the time of bone grafting ranged from 9 years to 25.5 years with a mean at 14.34 +/- 2.9 years. The same operator performed a Gingivoperiosteoplasty (GPO) in all cases and the graft material was cancellous iliac bone in all cases. RESULTS: There were three indications of the bone graft and in each case the objective is different. First the interceptive bone graft in mixed dentition (50%): it was performed prior to the orthodontic treatment 12.8 +/- 2.3 years. The graft provides a bone support for teeth adjacent to the cleft. Second the stabilisation bone graft in permanent dentition (33.8%): it stabilize the orthodontic treatment and/or plan orthognathic surgery (15.2 +/- 1.6 years). Third the consolidation bone graft (16.2%): it was a late secondary bone graft after the complete growth (17,3 +/- 3,6 years) to correct the sequellae. It restored the maxilla continuity, stabilised the clefting teeth and prepared a prosthetic rehabilitation. CONCLUSION: The benefice of the secondary bone graft with GPO are numerous and is a part of the protocol treatment in CLP patients. A multidisciplinary follow up is necessary for the indication and timing of secondary bone grafting. The indication is subject to the clefting teeth, to the occlusal status and to the maxillary growth.

[Clinical management of cleft lip and palate in university hospital of Montpellier]. / Prise en charge des fentes labio-maxillo-palatines au centre hospitalier universitaire de Montpellier.

Management of labio-maxillo-palatine clefts has two major requirements: to constitute a multidisciplinary staff (surgical, phonological, orthodontical) intervening as soon as possible and determination of a precise therapeutical chronology not only for primary surgery but also for sequellae. Primary surgical protocol is in cases of total clefts these defined by Malek and Psaume; and for pure labial or incomplete clefts, we perform a neonatal surgery. Integration of interceptive correction of sequellae into thus protocol appears basic: correction of alveolar cleft by gingivoplasty (5 to 7 years) associated with secondary home-graft between 11 to 13 years; early nasal revision since 2 years for functional and aesthetic reasons. Early control of speech development, otologic problems and their management appears a very important point. Introduction of the concept of maxillary distraction appears to us a very important improvement for correcting orthognatic cases with major problems of squeletical growth. Recent introduction of the antenatal diagnosis introduces a new concept in psychological approach of these cases. It is necessary to establish a network for managing these cases since the antenatal period.

[Imaging of oto-mandibular dysplasias]. / Imagerie des dysplasies oto-mandibulaires.

The modern imaging opened a possibility of precise exploration of otomandibular dysplasias. The techniques of browsing by IRM and CT Scan and the software of image processing which is associated with these techniques (three-dimensional, superposition and removing of the anatomical structures) allow to carry out a true anatomical dissection of the whole of these malformative syndromes. The study of the skeletal anomalies gains by specifying the disorders of development of the various mandibular segments: temporomandibular joint, ramus and horizontal branch and also, all cranio-maxillar structures: orbital rim, cranial basis. Study of masticatory muscles shows an important damage correlated with skeletal troubles and performs a grading of morphological and functional gravity. This analytical study emphasizes some malformative axis: malar axis in cases of mandibulofacial dystosis, temporomandibular axis in hemifacial microsomia. Modern imaging can appreciate, by development of three-dimensional cephalometry, growth anomalies and quantification of post therapeutical results. At last usefulness of imaging appears in therapeutic modelisation and in the field of Computer-Aided Planning of surgical techniques of osteotomies and overall maxillaries distractions.

[Synovial sarcoma of the mandible in children. Apropos of a case]. / Synovialosarcome de la mandibule chez l'enfant. A propos d'une observation.

UNLABELLED: Synovialosarcoma is an aggressive malignant soft-tissue tumor. It's a mesenchymal tumor rare in the cephalic region in the children. Its occurs most frequently in the extremities, in the adolescents and young adults between 15 and 40 years. His treatment are principally a radical surgical excision. We report the case of a 10 year old boy who had a mandibular tumor developing in the first premolar area and invading the submandibular region. The histologic diagnosis was biphasic synovialosarcoma with epithelial predominance. The staging showed a stage II tumor (5 cm) of the submandibular region with invading the mouth floor and the mandible. After the failure of the polychemotherapy we had performed a radical surgical excision with a functional cervical lymphadenectomy. The tumor was excised in one piece with the horizontal part of the left hemimandible. The treatment was completed by radiotherapy. In a second time a reconstructive surgery was performed with a fibula free flap. The result at one year show a good local control and a perfect esthetic and functional result of the mandible. CONCLUSION: Synovialosarcoma is a very aggressive malignant soft-tissue tumor with a high metastatic risk. Management must be rapid as soon as the diagnosis is made. Surgical excision is the main treatment in association with the chemotherapy and radiotherapy.

[Congenital skull depression. Report of 2 cases]. / Dépression congénitale de la voûte crânienne. A propos de deux cas.

Congenital depression of the fetal skull is a rare lesion (1 to 2.5 per 10,000 births) resulting in an asymmetrical skull. This depression is caused by exaggerated or prolonged pressure applied to the fetal head in utero or during delivery. Two types of congenital skull depression have been described: deformity without fracture and depression with fracture, the more common form. Congenital skull depression is important because it may be associated with underlying brain injury, especially when the depression is associated with fracture. The simple permanent cosmetic deformity may also constitute a complication. CT scan can now rule out intracranial complications and in this case, a conservative management is reasonable treatment option. The observation period should not exceed six months. After this period, orthopedic or surgical management may be indicated because spontaneous elevation of depression is uncommon after six months. The authors report two cases of congenital skull depression with deformity without fracture. The pathogenesis and treatment are discussed.