Correction of lip and nasal deformities in bilateral cleft operated with Le Mesurier procedure.

INTRODUCTION: Bilateral cleft lip surgery recently evolved with the generalization of cheilorhinoplasty replacing cheiloplasty alone. The aim of this study was to analyze lip and nasal deformities of bilateral cleft lip operated on with the Le Mesurier procedure, and then to evaluate possible secondary surgical solutions used in our Cleft Center. PATIENTS AND METHODS: We retrospectively reviewed a total of 39 patients born between 1991 and 2009 with a bilateral cleft all type included (isolated lip, lip and alveolar, associated or not with a cleft palate), all treated at the Caen University Hospital with a Le Mesurier procedure for the primary cleft lip closure. We studied population characteristics, the number of secondary surgical procedures for lip and nasal deformities. We analysed therapeutic approaches of the different secondary surgical procedures used and their morphological results. RESULTS: 85% of patients needed at least one secondary surgical procedure, 28% two procedures, 15% three procedures and 8% four or more procedures. Median age at secondary surgery was 6,8. 79% had a "scar repositioning", 33% managed the vermilion, 18% a restoration of the volume of the lip, 12% a philtral tissue sacrifice, 9% a procedure on Cupid's bow, 24% a surgery for nasal tip projection, 45% a columella lengthening and 58% a reposition of the alar base. DISCUSSION: Le Mesurier procedure for bilateral clefts leads to deformities that require secondary surgical procedures. We propose an algorithm to manage loop scar in this procedure.

Hallermann Streiff syndrome: Cranio-facial manifestations systematic review and report of two cases.

Hallermann Streiff syndrome (HSS) is a rare congenital abnormality with about 200 case reports in the literature. Its etiology is unknown although it may be due to a sporadic mutation. Diagnosis is based on the association of craniofacial malformation, dental abnormalities, hypotrichosis, atrophy of the skin, proportionate nanism, congenital cataract and bilateral microphtalmos. Cranio-facial deformities are the main signs detected and the most easily recognizable. We report cranio-facial and oral signs from a systemic literature review, and illustrate our findings with two of our patients diagnosed with HSS. Common cranio-facial manifestations are craniofacial malformation with a « parrot beak ¼ nose, micrognathia, aprominent skull, sutures closing anomaly, malocclusion, dental anomalies, eyebrows and eyelash lack and atrophy of the nose skin. Knowledge of these signs should allow for early diagnosis and adequate treatment and follow up.

Labiomental sensation after segmental mandibulectomy and inferior alveolar nerve preservation for osteoradionecrosis.

Advanced mandibular osteoradionecrosis requires a segmental mandibulectomy with reconstruction using a free fibular flap. The conservation of labiomental sensation by lateralization of the inferior alveolar nerve is unusual during a segmental mandibulectomy. A protocol for the evaluation of labiomental sensation has been created for the clinical follow-up of operated patients. The patients included were patients with mandibular osteoradionecrosis who underwent a segmental mandibulectomy with reconstruction by free fibular flap and whose pre-operative labiomental sensation was preserved. All patients were followed-up by the same examiner and operated on by the same surgeon. The neuro-sensitive examination analyses the different forms of sensation at 1 week and at 12 months postoperatively. Between May 2017 and May 2018, 3 consecutive patients were assessed. The results of the labiomental sensitive evaluation using our evaluation protocol attest to the preservation of labiomental sensation. The operating time was increased by an average of 35 min per surgical procedure. The conservation and re-routing of the inferior alveolar nerve in segmental mandibulectomy with fibula free flap reconstruction in patients with osteoradionecrosis allows for the preservation of labiomental sensation, which improves the quality of life of patients. Our protocol can be used in all surgical procedure that affect sensibility.

Management of non-syndromic craniosynostoses in France in 2015: A national survey.

PURPOSE: Craniosynostoses are managed by surgical and anaesthetic teams in specialist centres. Despite the availability of international guidelines, the perioperative management of craniosynostoses remains highly variable between centres. The aim of our study was to describe the different protocols for the management of non-syndromic craniosynostoses in France in 2015. MATERIALS AND METHODS: This descriptive study consisted of a survey about the protocols of pre-operative, per-operative and post-operative management of craniosynostoses. The survey was sent to the departments in French university hospitals that perform this surgery. RESULTS: Nineteen departments out of twenty replied to the survey. Sixteen departments organised multi-disciplinary meetings. The most frequent preoperative imaging requested was a Computed Tomography. More than half of the centres organised a follow-up until early adulthood. CONCLUSION: This study showed a great variability in the management of craniosynostoses. A recommendation from the study is to establish a scientific committee of practitioners in order to establish a standardised protocol. In addition, this study showed the need to create a specific section in the French rare diseases database (CeMaRa) for craniosynostoses.

The contribution of telemedicine to humanitarian surgery.

Telemedicine enables us to push back the geographical and interactive boundaries of medicine. With a role in humanitarian missions, it is particularly pertinent at two key stages: the preparation phase, and at postoperative follow-up after the mission. It is our intention to describe our experience of telemedicine within a humanitarian context. Four teleconsultations were organized between departments of maxillofacial surgery in Caen (France) and in Bamako (Mali). 21 patients were assessed regarding their care. The preparation phase, taking place several weeks before the mission, allowed us to meet the patients preselected by Prof Traore. We were also able to review imaging, such as previous X-rays or preoperative CT scans. After discussion between the two teams, a decision on the coordination of patient care was reached, namely surgery performed by Prof Traore and the local team in Bamako, or surgery during the next mission to Ouagadougou. Several weeks after the mission, patients attended postoperative consultation by means of teleconsultation. This covered wound assessment, management of complications, and scheduling of follow-up surgery. The benefits of telemedicine in humanitarian projects are manifold: real-time exchange of specialist skills with Malian colleagues, collective therapeutic decisions, academic value, and anticipation of anesthetic and surgical needs before missions.

Impact of early intravelar veloplasty at six months on mandibular growth in patients with Pierre Robin Sequence.

Pierre Robin Sequence (PRS) combines mandible microretrognathia, asynchronism of the pharynx and tongue, glossoptosis and, in some cases, cleft palate. Its principal functional consequences are respiratory and feeding problems during the neonatal period. In this study, we focused on the impact of early closure of the cleft at six months on mandibular growth in patients with PRS. We performed a retrospective study of 15 patients followed for PRS and undergoing surgery performed by the same senior surgeon (HB) at our cleft center between 2005 and 2012. These patients underwent early closure of the cleft (at a mean age of 5.87 months) by intravelar veloplasty, as described by Sommerlad. Only one article with exploitable data analyzing facial and mandibular growth in a cephalometric study of children with PRS has been published. The children in this series, constituting the control group for our study, underwent veloplasty between the ages of 12 and 18 months, often accompanied by labioglossoplasty, and the cephalometric study was carried out between the ages of four and seven years. We compared this control group in which surgery was performed at 12-18 months with our series of children undergoing surgery at six months, in a cephalometric study based on teleradiographic profile measurements performed between the ages of four and seven years. We found that early closure of the cleft soft palate yielded results identical to those for the control group in terms of mandibular growth, without the need for labioglossopexy. Finally, early intravelar veloplasty led to early functional improvement in terms of speech and phonation.

Congenital Midline Upper Lip Sinuses: 3 Rare Cases.

Congenital sinuses of the upper lip are rare congenital malformations. There have been only 40 cases described in the literature. We report 3 cases of congenital midline upper lip sinus in Caucasian children. Two of those lesions were associated with other anomalies (complete cleft palate and hemifacial macrosomia or submucous cleft palate with bifid uvula). The pathophysiology remains unexplained yet. Congenital upper lip sinuses can be considered as possible microforms of cleft-lips. Associated anomalies are frequent and must be sought. The treatment is a full excision of the sinus tract and of the skin around the punctum in order to avoid risks of recurrence.

Management of Large Maxillomandibular Osteofibrous Dysplasia as Part of a Humanitarian Mission.

PURPOSE: Maxillomandibular ossifying fibroma is a benign tumor that affects young adults. Complete excision can allow satisfactory management with no recurrence. During a humanitarian mission, one is confronted with many types of damage from these fibromas. Their management requires wide resection (mandibulectomy interrupter or maxillectomy) and free flap reconstruction. However, technical conditions during a humanitarian mission might not allow the performance of a free flap reconstruction. How can such patients be managed? Should these patients receive a straightforward intervention performed on site during the mission or should they go to another country with a technical platform suitable for microsurgical reconstruction? PATIENTS AND METHODS: During a humanitarian mission in Ouagadougou, Burkina Faso, 6 patients with large ossifying fibromas traveled to France to undergo wide excision of the lesion and free flap reconstruction using the fibula. The Enfants du Noma paid for the travel and medical costs. RESULTS: No flap was lost. Four patients (67%) had local (disunity of scar or local infection) or general (malaria) complications that quickly resolved. CONCLUSIONS: Most teams agree that free flaps should not be performed during humanitarian missions, and only 1 German team practices in Sokoto, Nigeria. Therefore, medical travel is an attractive solution that allows optimal management and requires financial assistance from humanitarian organizations.