Post-viral idiopathic purpura fulminans is associated with inherited thrombophilia and anti-cardiolipin antibodies.

Introduction: Idiopathic purpura fulminans (IPF) is a rare and severe coagulation disorder, associated with transient anti-protein S (anti-PS) antibodies in the context of post-viral infection such as varicella. Anti-protein S antibodies are frequently found in the context of varicella, in contrast with the rarity of IPF. Other factors such as anti-phospholipid antibodies (APL) and inherited thrombophilia may be associated with severe vascular complication. Method: This is an ancillary study of a French multicenter retrospective series and systematic review of literature. We analyzed patients who were tested for inherited thrombophilia, namely antithrombin, protein C, protein S deficiency; prothrombin gene G20210A polymorphism (FII:G20210A),Factor V R506Q polymorphism (FV:R506Q); and/or for APL (lupus anticoagulant (LA), anti-cardiolipin antibodies (ACL), or anti-beta 2-GPI antibodies (Aß2GP1). Results: Among the 25 patients tested for inherited thrombophilia, 7 (28%) had positive results. Three had FV R506Q, two FII:G20210A, one compound heterozygote FV:R506Q associated to FII:G20210A, and one protein C deficiency. APL testing was performed in 32 patients. It was positive in 19 patients (59%): 17 ACL (53%), 5 LA (16%), 4 Aß2GP1 (13%). The risk of severe complications was not associated with presence of inherited thrombophilia or APL presence, with RR: 0.8 [95% CI: 0.37-1.71], p = 1 and RR: 0.7 [95% CI: 0.33-1.51], p = 0.39, respectively. We found a high prevalence of inherited thrombophilia or APL in a population of patients with IPF. However, we do not find an association with the occurrence of severe vascular complications or venous thromboembolism.

Characterization of the very young child's palatal vault growth pattern: how do its size and shape evolve?

AIM: This study aimed to characterise the palatal vault evolution during the first years of life, both in terms of shape and size. MATERIALS: The study sample was composed of 168 healthy children aged less than 4 years. Twenty-one measurements of distances and 6 angles were taken from 7 fixed landmarks set on the palatal vaults 3D surfaces reconstructed from CT-scans. To analyse only the shape evolution, the "sizefree" log-shape ratio of those measurements were computed and the global shape of the palatal vault and their transversal curve were plotted. Statistical analyses were performed to highlight the shape and size differences separately. CONCLUSION: The shape and size evolution of the palatal vault during the first years of life was not only correlated with deciduous dentition development. We assumed that the progressive orofacial muscles activation and tongue movements in the oral cavity may also explain these results as they induced strains on the palatal vault, warping it in various ways.

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.

Fetal median sacral artery anatomy study by micro-CT imaging.

PURPOSE: The median sacral artery (MSA) is the termination of the dorsal aorta, which undergoes a complex regression and remodeling process during embryo and fetal development. The MSA contributes to the pelvic vascularization and may be injured during pelvic surgery. The embryological steps of MSA development, anastomosis formation and anatomical variations are linked, but not fully understood. METHODS: The pelvic vascularization and more precisely the MSA of a human fetus at 22 weeks of gestation (GW) were studied using micro-CT imaging. Image treatment included arterial segmentations and 3D visualization. RESULTS: At 22 GW, the MSA was a well-developed straight artery in front of the sacrum and was longer than the abdominal aorta. Anastomoses between the MSA and the internal pudendal arteries and the superior rectal artery were detected. No evidence was found for the existence of a coccygeal glomus with arteriovenous anastomosis. CONCLUSIONS: Micro-CT imaging and 3D visualization helped us understand the MSA central role in pelvic vascularization through the ilio-aortic anastomotic system. It is essential to know this anastomotic network to treat pathological conditions, such as sacrococcygeal teratomas and parasitic ischiopagus twins (for instance, fetus in fetu and twin-reversed arterial perfusion sequence).

Three-dimensional computer-assisted dissection of pancreatic lymphatic anatomy on human fetuses: a step toward automatic image alignment.

PURPOSE: Pancreatic cancer is the fourth cause of death by cancer worldwide. Lymph node (LN) involvement is known to be the main prognostic factor. However, lymphatic anatomy is complex and only partially characterized. The aim of the study was to study the pancreatic lymphatic system using computer-assisted anatomic dissection (CAAD) technique and also to update CAAD technique by automatizing slice alignment. METHODS: We dissected three human fetuses aged from 18 to 34 WA. 5-µm serial sections of duodeno-pancreas and spleen blocks were stained (hematoxylin-eosin, hematoxylin of Mayer and Masson trichrome), scanned, aligned and modeled in three dimensions. RESULTS: We observed a rich, diffuse but not systematized lymphatic network in the peri-pancreatic region. There was an equal distribution of LNs between the cephalic and body-tail portions. The lymphatic vascularization appeared in continuity from the celiac trunk to the distal ends of its hepatic and splenic arterial branches parallel to the nerve ramifications of the celiac plexus. We also observed a continuity between the drainage of the pancreatic head and the para-aortic region posteriorly. CONCLUSION: In view of the wealth of peri-pancreatic LNs, the number of LNs to harvest could be increased to improve nodal staging and prognostic evaluation. Pancreatic anatomy as described does not seem to be compatible with the sentinel LN procedure in pancreatic surgery. Finally, we are now able to offer an alternative to manual alignment with a semi-automated alignment.

Distal lower extremity coverage by distally based sural flaps: Methods to increase their vascular reliability.

INTRODUCTION: Although using distally based sural flaps is old and common, the described harvesting methods vary and often depend on unclear vascular anatomical data. We wanted, through a review of the existing harvesting techniques and our clinical experience, to synthesize these aspects in order to provide an optimal, safe and customized use of distally based sural flaps. PATIENTS AND METHODS: Fifty-eight sural flaps were performed in 53.4-year-old patients in average, presenting in 81% of the cases one or more vascular comorbidities. The mean cover surface was of 30cm2. The flaps were mainly fasciocutaneous. The tunneling of the pedicle was performed in 34.5% of the cases. A skin blade was preserved above the pedicle in 37.9% of flaps. The short saphenous vein was anastomosed in 27.6% of the cases at the recipient site and ligatured distally in 19% of the cases. The mean follow-up was 18.9months. RESULTS: In 31% of cases, the skin paddle presented an obvious venous congestion during flap inset successfully treated with an anastomosis or a distal ligature of the short saphenous vein. We noticed in six other flaps (10.3%) a postoperative venous congestion having led to a skin paddle partial necrosis, of which five (8.6%) were treated without compromising the quality of the reconstruction. One patient required a coverage using another flap. CONCLUSION: When the microsurgery is not required or possible, the distally based sural flaps represent a safe level of the reconstructive armamentarium. Managing the pedicle's dissection and the venous drainage needs to be discussed case-by-case and adapted to peroperatory observations. The anastomosis or ligation of the small saphenous vein, as the existence of a thin skin blade under the skin paddle appear to increase vascular reliability of these flaps.

[The congenital melanocytic nevi of the face in child: What's new?]. / Les nævus mélanocytaires de la face chez l'enfant : quoi de neuf ?

Congenital melanocytic nevi of the face are a frequent reason for consultation in paediatric plastic surgery. Usually of small size, they raise a complex problem of reconstruction when they are large and giant. The indication of excision is generally stated on aesthetic criteria whereas the risk of melanoma is especially important in the giant nevi. Simple suture, full thickness skin graft and expanded skin flaps are the techniques of choice. The treatment must be carried out precociously, follow a surgical planning, respect the aesthetic units of the face and the periorificial areas, adapt to the age of the child and bring psychological benefit to the child.

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.

[Geometrical growth models of the fetal forebrain, cerebellum, brainstem and change of the cranial base angles during fetal period]. / Modèles géométriques de croissance du cerveau, cervelet, tronc cérébral et modification des angles de la base du crâne au cours de la période fœtale.

INTRODUCTION: Brain growth plays likely an important role for the skull growth. In the fetus, there exists an heterochrony for the growth of supratentorial (forebrain) and infratentorial regions (brainstem and cerebellum). The aim of the study was thus to model geometrically the growth of these two regions and to compare it with the inflection of the base of skull. MATERIAL AND METHODS: Brain growth measurements were performed from midsagittal photographs of fetal brains obtained from an Anatomical Atlas over a period from 10 to 40 amenorrhea weeks (AW). After countouring and pointing anatomical and geometrical landmarks, we have developed a linear growth model based on principal component analysis (PCA). Besides, the variation of the sphenoidal and clivo-foraminal angles was studied from anatomical midsagittal slices of fetal heads sampled over a period from 16 to 39 AW. RESULTS: The PCA model brings to light the radial expansion of the forebrain growth (first component) associated with an inferior and posterior rotation of the occipital lobe. The growth of the infratentoriel region presents an inferior and posterior expansion associated with a second component corresponding to inferior and anterior expansions. From the 17 AW, appears an heterochrony between the supra- and infratentorial growths and an inversion of the ratio between the infra- and supratentorial dimensions after 30 AW. The sphenoidal and clivo-foraminal angles decrease slightly until 25 AW, and then increase quickly until the 39 AW. CONCLUSIONS: The growth of brain is accompanied by morphological change between the compartments supra- and infratentoriel but also on the level of the base of skull. The possible interactions will be discussed.

[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.

[Mandibular asymmetry in plagiocephaly without synostosis]. / Etude de l'asymétrie mandibulaire dans les plagiocéphalies sans synostose.

SUBJECT: The number of plagiocephalies without synostosis (PWS) strongly increased in the last decade. Its impact on the mandible remains badly understood, the more so as there are various forms of PSS. The purpose of this study was to analyze mandible deformation according to the deformation of PWS. MATERIAL AND METHODS: The study was carried out retrospectively starting from three-dimensional scans of children presenting with a PWS. They were classified in 2 groups according to the deformation of the cranium. There were 51 frontal plagiocephalies (PF) and 19 occipital plagiocephalies (PO) for which the cephalic index was calculated. The position of the mandible compared to the base of cranium and its intrinsic asymmetry were analyzed (paired parametric Student test). RESULTS: The mandible was symmetrical in PO whereas it was asymmetrical for PF. This asymmetry was present at the level of the corpus and developed in 2 ways: that is to say asymmetry compensated for that of the base of the cranium (68.6%); or it worsened it (23.5%). The degree of brachycephaly was more important in PO than the PF with a cranial index of 0.93+/-0.07 and 0.85+/-0.07 respectively (P<0.05). In both cases the position of the mandibular condyle followed the asymmetry of the base of cranium and the asymmetry of the temporomandibular joint (TMJ) was secondary with that of the base of cranium. DISCUSSION: In addition to the asymmetry of TMJ position there was an intrinsic mandibular asymmetry in PF which was not found in PO. This asymmetry of the mandible was variable but in most cases it tended to compensate for the asymmetry of the base of the cranium which was more important in PF. The risk to have asymmetry of dental occlusion seems more important in PF than in PO, and an orthodontic follow-up appeared to be justified for these children.

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.

Sonographic assessment of normal fetal palate using three-dimensional imaging: a new technique.

OBJECTIVES: The aim of this study was to describe a novel three-dimensional (3D) ultrasound rendering technique to examine the normal fetal posterior palate and to assess its correspondence with the real fetal anatomy. METHODS: A prospective longitudinal study was conducted from January to October 2005 and included 100 fetuses in a low-risk population. Fetal ultrasound examinations were performed at 17, 22, 27 and 32 weeks' gestation to determine the normal 3D ultrasound view of the fetal palate at different gestational ages. The ultrasound scans were performed using the strict anterior axial plane of the starting reconstruction volume and the underside 3D view of the fetal palate. The 3D view of the fetal palate was compared with the normal anatomical view of the fetal palate obtained by surgical fetopathological examination of fetuses at the same gestational ages. The sonographic visualization rates of seven defined anatomical landmarks of the fetal palate were computed for each gestational age. The visualization rates across gestational ages were compared by use of the Cochrane Q test. The reliability of detection of each anatomical landmark across gestational ages was determined by Cronbach's Alpha. RESULTS: In all cases a 3D ultrasound view of the fetal maxilla and secondary palate was obtained at each period of gestation and corresponded well to the fetal anatomical specimens. The seven defined anatomical landmarks of the fetal palate were identified in 42-100% of cases. The visualization rates across gestational ages were significantly different in five of these anatomical landmarks. These differences can be explained by different developmental processes of these anatomical structures. The overall reliability of visualization across the gestational ages for the anatomical landmarks was medium to very high (0.73-0.96), except for the landmark interpalatal suture which was low (0.48). CONCLUSIONS: This technique of anterior axial 3D view reconstruction of the fetal palate seen by an underside view can provide unique diagnostic information on the integrity of the secondary palate. This innovative, simple and rapid technique may become the reference technique in ultrasound investigation of the fetal palate, and should be of value in diagnosing isolated secondary cleft palate or palatal involvement when cleft lip and alveolus are diagnosed.

[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.

[The human body and the computer as pedagogic tools for anatomy: review of the literature]. / Le corps humain et l'informatique comme outils pédagogiques de l'anatomie.

Since the first dissections, the human body has been the main tool for the teaching of anatomy in medical courses. For the last 30 years, university anatomy laboratory dissection has been brought into question and the total hours of anatomy teaching have decreased. In parallel, new technologies have progressed and become more competitive and more attractive than dissection. The aim of this review of the literature was to evaluate the use of the human body as a pedagogic tool compared to today's computer tools. Twenty comparative studies were reviewed. Their analysis showed that the human body remains the main tool in anatomy teaching even if anatomic demonstration (prosection) can replace dissection, and that the computer tools were complementary but not a substitute to dissection.