What We Learned from Performing the Inverse Malek Procedure to Repair Bilateral Cleft Lips and Palates: A Single-Center Retrospective Study.

Background: This study reviews the surgical and functional outcomes of children diagnosed with a bilateral cleft lip and palate and treated by the same surgical team following specific surgical protocols 18 years after surgery and during the follow-up. Methods: Based on a single-center retrospective design, demographic and surgical data were gathered by the authors from international institutions. Most of the data were quantitative in nature, and descriptive statistical and non-parametric tests were employed for analysis. All children born with a bilateral cleft from 1982 to 2002 were considered. Children affected by a syndrome were excluded. Complications and speech results were the main items measured. Results: Thirty patients were selected; 73.3% were treated using the inverse Malek procedure, and 26.7% underwent a modified two-stage procedure. Seventy percent developed an oronasal fistula. An alveolar bone graft was performed in 83%, and 53.3% underwent Le Fort osteotomy. Thirty-six percent required a pharyngeal flap, with good speech results. The median number of times general anesthesia was used among all the interventions considered was 5.5 (4.25-6). Conclusions: This study presents the long-term results of using the inverse Malek procedure to treat children with a bilateral cleft lip and palate. It is shown that this is related to a high risk of developing a fistula, but has good long-term speech results.

Unilateral Cleft lip and Palate: Long-Term Results of the Malek Technique.

To review at 18 years-old the results of surgery and follow-up of children born in our hospital with unilateral cleft lip and palate (uCLP). They were operated at the time by the same surgeon, following the same primary surgical procedure (Malek).Retrospective cohort study.Tertiary Children's Hospital.All children born with uCLP between 1996 and 2001 and operated in our hospital. Syndromic children were excluded.Results of the primary surgery, ear-nose-throat interventions, maxillo-facial surgery and final phonatory results.Seventy-nine files of children born with a cleft were reviewed: 34 were taken into consideration for uCLP: 15 right and 19 left. They were operated in two stages, following the inverse Malek procedure. Sixty per cent had a fistula. Eighty-eight percent had grommets. Ninety-seven percent had an alveolar graft at a median age of nine (5-10) and 22% underwent a Le Fort osteotomy. Seven percent were operated for a pharyngeal flap, 29% for a secondary lip surgery at a mean age of 12.8 and 29% for a late rhinoplasty at a mean age of 14.8 years. A median of 5.7 multidisciplinary consultations was realized with a median number of general anesthesia of 7.1 (4-13).This retrospective study shows that the Malek procedure for children born with uCLP is related to a high risk of fistula but good long-term phonatory results. Twenty percent of children were operated for a Le Fort procedure and one-third for a secondary lip procedure and rhinoplasty.

Lymphatic malformations in children: retrospective review of surgical and interventional management.

PURPOSE: Lymphatic malformations (LMs) are classified as macrocystic, microcystic or mixed. Treatment depends on their characteristics: surgery, sclerotherapy, both combined, systemic treatment or observation. This study aims to analyze the surgical and interventional management of LMs in children over the last two decades in our university hospital. METHODS: Management of children born with LMs between 2000 and 2019 was reviewed. Parameters collected were: malformation characteristics, type of treatment, symptoms, imaging, timing of diagnosis and first treatment, number of interventions, recovery rate, complications and length of stay. RESULTS: Files of 48 children were reviewed: 27 with macrocystic and 21 with microcystic LMs. There was no statistically significant difference in type of treatment except for combined treatment, more performed in microcystic LMs (p = 0.04). Symptoms, imaging, timing of diagnosis and first treatment, number of interventions and complications were not statistically significant. Overall, the number of surgeries was lower than sclerotherapies (p = 0.04). Recovery rate after surgery was higher in macrocystic LMs (p = 0.01). Complications and length of stay were not statistically significant. CONCLUSION: A good rate of recovery was observed when surgery was performed, with no significant increase in complications and length of stay. A prospective study will be determinant to create a decisional algorithm for children with LMs.

Frontoethmoidal Encephalocele Correction Surgery in Benin: A Case Report.

INTRODUCTION: Primary encephalocele is a rare deformity that is challenging for the neurosurgeon. It requires a multidisciplinary team for adequate reconstructive surgery. CASE PRESENTATION: We report the case of a 6-month-old African boy who presented with a frontoethmoidal encephalocele; we present a technical description of the surgical procedure, using no implant. DISCUSSION/CONCLUSION: The postoperative evolution of the boy was uneventful, with a good clinical result at the follow-up.

Ultrasound-guided percutaneous endovenous laser treatment combined with sclerotherapy for the treatment of large intramuscular venous malformations.

BACKGROUND: Among vascular anomalies, congenital venous malformations (VMs) are the most common lesions. Treatment of VMs is sometimes difficult or cumbersome, depending on their size and tissue involvement. Surgery may lead to invasive and mutilating excisions, often allowing only partial removal, with an increased risk of recurrence. Sclerotherapy is a mainstream technique, resulting in endothelium destruction, fibrosis and subsequent shrinkage of the vascular lesion, also with a risk of recurrence. However, this technique may not be efficient in cases of large and infiltrating VMs. Endovenous thermal ablation has been found to be more effective than sclerotherapy for transmural vascular destruction and has therefore been employed in the treatment of VMs. METHODS: In this study we described a combined technique for the treatment of large intramuscular VMs in seven consecutive patients, associating endovenous laser ablation with sclerotherapy. The aim was to assess feasibility and safety of the procedure. RESULTS: We reported a high immediate technical success, clinical and radiological improvement, with no complications. CONCLUSIONS: The preliminary results presented herein show, that combining EVLA and sclerotherapy for the treatment of voluminous persistent intramuscular VM is safe and technically feasible. The combination of a wide direct intimal thermal damage with chemical sclerotherapy is the force of this approach. The small number of cases and the medium term follow up represent though a limitation.

[Advances in the treatment of complex venous malformation: endovenous laser]. / Nouveautés dans le traitement des malformations veineuses complexes : le laser endoveineux.

Congenital venous malformations (VMs) are the most common vascular abnormalities. Their treatment can be complex, depending on their size and surrounding tissues involvement. To date, sclerotherapy is considered the gold standard for the treatment of VMs. This technique, which aims to destroy the endothelium and thus cause fibrosis and retraction of the vascular lesion, is less effective in voluminous VMs. Endovenous thermal ablation is a widely validated treatment in the management of venous insufficiency, showing better efficacy than sclerotherapy in terms of trans-parietal vessel destruction. This approach has therefore also been described in the treatment of VMs. This technique has been introduced for the treatment of complex VMs at the Centre for Malformations and Rare Vascular Diseases of the CHUV.

Les malformations veineuses (MV) congénitales sont les anomalies vasculaires les plus fréquentes. Leur traitement peut être complexe en fonction de leur taille ou de l'atteinte des tissus avoisinants. À ce jour, la sclérothérapie est considérée comme le Gold Standard du traitement des MV. Cette technique, qui vise à détruire l'endothélium et à causer ainsi une fibrose et une rétraction de la lésion vasculaire, est moins efficace dans le cas des grandes MV. L'ablation thermique endoveineuse, largement validée dans le traitement de l'insuffisance veineuse, s'est révélée plus efficace que la sclérothérapie pour la destruction transpariétale d'un vaisseau. Cette approche a donc également été décrite dans le traitement des MV et la technique est actuellement employée pour le traitement des MV complexes au Centre des malformations et des maladies vasculaires rares du CHUV.

Is it better to reduce the intervals between pulsed dye laser treatments for port wine stains in children? Laser Doppler Imaging based study.

BACKGROUND: Pulsed Dye Laser (PDL) is the treatment of choice of Port Wine Stains (PWS). Laser Doppler Imaging (LDI) has been used to evaluate the effectiveness of this treatment. In a previous study, we demonstrated that LDI allows an objective evaluation. The purpose of this study is to investigate if reducing the delay between two laser sessions could improve the clinical outcome. METHOD: This prospective study was conducted from September 2015 to November 2017. Three Laser sessions were performed every month in twenty patients with PWS. The PWS response was assessed by LDI after each session and at the end of the third one. The present study was compared to the first one. RESULTS: The LDI confirmed the efficacy of PDL treatment with an average blanching rate of 26.7 %. The response is statistically significant after each session. When we compare both studies, there is an average decrease in vascularization of 0.42 for the first study and 0.50 for the present one. CONCLUSION: This study allows us to validate the use of LDI for the numerical evaluation of PDL effect on PWS in children. However, we cannot confirm that reducing the interval between laser sessions could improve therapeutic outcomes. LEVELS OF EVIDENCE: Treatment Study Level II (Prospective Comparative Study).

Intestinal duplication in the tongue: embryological and radiological point of view.

Intestinal duplication in the tongue is a rare entity. Occurrence in the anterior part of the tongue is exceptional. We report an intestinal duplication in the tongue causing eating difficulties and discuss the accuracy of embryologic and histopathology knowledge as radiology. A transoral complete resection of the lesion was performed, without postoperative complications. There was no recurrence with a follow-up of 15 years.

Erratum to: Delayed Closure of Giant Omphaloceles in West Africa: Report of Five Cases.

[This corrects the article DOI: 10.1055/s-0037-1599796.].

Syndromic sebaceous nevus: current findings.

BACKGROUND: Sebaceous nevus is a congenital malformation of the skin that usually occurs on the scalp or face. Syndromic forms do rarely exist with associated cerebral and ocular malformations. The skin lesions are pale at birth and become irregular by puberty. In the adult patient, tumors (usually benign) develop from sebaceous nevus. Their surgical excision during childhood can give a better result in terms of the definitive scar. OBJECTIVES: The aim of this study is to analyze our cases of syndromic sebaceous hamartoma, perform a review of the existing literature, and propose guidelines for the therapeutic plan. METHODS: This is a retrospective study reviewing the cases of syndromic sebaceous nevus treated in the Department of Orthopedic Plastic Pediatric Surgery in Montpellier, France, and the Department of Pediatric Surgery in Lausanne, Switzerland, between 1994 and 2016. RESULTS: The files of six patients with syndromic sebaceous nevus were analyzed. The average age at the first consultation was 4 months. The location was craniofacial in all cases. Cerebral radiological imaging was performed on all patients; two showed abnormal findings. Four patients underwent ophthalmic examination, which all revealed abnormalities. Three patients had other associated malformations. Three patients presented with epilepsy or learning difficulties in the course of follow-up. CONCLUSION: All patients presenting with extensive sebaceous nevus of the craniofacial region should benefit from cerebral imagery and ophthalmic examination since there is a very high probability of associated abnormalities. The developmental problems encountered could not be definitively associated with the skin malformations.

[Rare vascular diseases, building dedicated multidisciplinary specialized center]. / Maladies vasculaires rares, mise en place d'un centre multidisciplinaire spécialisé au CHUV.

Rare Vascular Diseases (RVD) encompass different types of vessel involvement. Some cause a dilation, others a weakening or tortuosity of the arterial wall, others an obstruction or excessive calcification of arterial walls. Clinical pathway of patients with RVD to diagnosis is often long and complex. Thus, in order to allow early diagnosis and coordinated multidisciplinary management and follow-up, a specialized RVD centre has been set-up at the CHUV, following the framework of the national concept of rare diseases.

Les maladies vasculaires rares (MVR) englobent différents types d'atteintes des vaisseaux. Certaines engendrent une dilatation ou une tortuosité de la paroi artérielle, d'autres une fragilisation de la paroi, d'autres encore entraînent une obstruction du vaisseau, une calcification excessive des parois, ou des malformations vasculaires. Comme pour toutes les maladies rares, le parcours des patients vers un diagnostic est souvent long et complexe. Afin de permettre un diagnostic le plus précoce possible, ainsi qu'un suivi coordonné et une prise en charge multidisciplinaire médicale et sociale, un centre des MVR a été mis en place au CHUV, dans le cadre du concept national des maladies rares.

[The treatment of venous malformations in adult patients: the role of US-guided sclerotherapy]. / Traitement des malformations veineuses chez l'adulte : rôle de la sclérothérapie échoguidée.

Venous malformations are slow flow dysplastic lesions, constituted by a vascular nest without arterial or capillary connections, more or less independent of the normal venous anatomy and circulation. In certain cases a treatment is required for symptom relief or for natural complications management. The percutaneous sclerotherapy under ultrasound guidance is increasingly used as an effective and mini-invasive option, allowing obtaining very good results with minor side effects. Several substances have been used with different efficacy and side effects rate. We review the literature and present some cases.

Les malformations veineuses sont des lésions à flux lent, constituées d'un nid vasculaire sans connexion artérielle ni capillaire, plus ou moins indépendantes du réseau veineux normal. Dans certains cas, un traitement est indiqué pour en diminuer les symptômes ou pour gérer les éventuelles complications naturelles. Le traitement par sclérothérapie percutanée sous guidage échographique se révèle une option efficace et peu invasive, permettant de diminuer le volume et d'obtenir de très bons résultats avec moins d'effets secondaires. Plusieurs substances ont été utilisées, avec différents résultats en termes d'efficacité et d'effets secondaires. Nous présentons une revue de la littérature et quelques exemples.

[Velopharyngeal insufficiency in children]. / Insuffisance vélo-pharyngée chez l'enfant.

Velopharyngeal insufficiency (VPI) represents an incomplete closure between the soft palate and the posterior pharyngeal wall. Its etiology can be anatomical (cleft palate), neurologic, or iatrogenic (after adenoidectomy). The evaluation of a VPI begins with a through speech and language assessment and can be complemented by instrumental investigations. VPI treatment relies on its early identification, followed by a specific speech therapy management. Surgery is performed in case of no improvement with speech therapy or in case of an anatomical defect not allowing the child to improve. IVP management requires a multidisciplinary team.

L'insuffisance vélo-pharyngée (IVP) désigne un défaut d'occlusion entre le voile du palais et la paroi postérieure du pharynx. Son étiologie peut être d'ordres anatomique (fente palatine), neurologique ou iatrogène (adénoïdectomie). L'évaluation de l'IVP débute par un bilan phoniatrique et orthophonique détaillé et peut être complétée par des explorations instrumentales. Son traitement repose sur une identification précoce puis une prise en charge et un suivi orthophonique spécifiques. Une chirurgie est réalisée en cas d'absence d'amélioration ou de défaut anatomique ne permettant pas à l'enfant de progresser. L'IVP est une pathologie intéressant plus particulièrement l'enfant et sa prise en charge se fait par une équipe multidisciplinaire.

Delayed Closure of Giant Omphaloceles in West Africa: Report of Five Cases.

Giant omphalocele (GO) management is controversial and not easy. Conservative management at birth and delayed surgical closure is usually mandatory. Postponed surgery may be challenging and carry the risk of intensive care treatment. We report on five children who were treated in our department for GO between 2000 and 2010. Initially, the patients were managed conservatively in West Africa. Delayed closure of the ventral hernia was performed in Switzerland after patient transfer through a nongovernmental organization. Fascial closure was performed at the median age of 23 months. Median diameter of the hernias was 10 × 10 cm ranging from 10 × 8 cm to 24 × 15 cm. Four (80%) patients had associated anomalies. Three children needed mechanical ventilation in the intensive care unit after surgery. Median hospitalization was 19 days. Complications were seen in two patients. The follow-up showed no recurrence of ventral hernia. There was no mortality. This report shows that conservative management of a GO at birth with delayed closure of the ventral hernia after transferring the patients to a European center is a safe approach for West African children and avoids life-threatening procedures. Delayed closure of a GO may be nevertheless challenging everywhere.

[Vascular anomalies]. / Anomalies vasculaires: exemple du syndrome de Klippel-Trenaunay.

Vascular anomalies are rare conditions that could be observed at all ages. They are classified, according to their histology, in vascular tumors or vascular malformations. The general practitioner plays a significant role in diagnosis and patient management, diagnosis being suspected on clinical history. In case of vascular anomaly, ultrasound-Doppler assessment is helpful to characterize morphologic and hemodynamic changes of the lesion and permits to monitor the evolution and to detect complications. Further investigations are often necessary prior to multidisciplinary management. In this article, a brief overview of vascular anomalies, their multidisciplinary management and the exemple of Klippel-Trenaunay syndrome are presented.

Assessment of the role of LASER-Doppler in the treatment of port-wine stains in infants.

BACKGROUND: Port-wine stains (PWS) are malformations of capillaries in 0.3% of newborn children. The treatment of choice is by pulsed dye LASER (PDL), and requires several sessions. The efficacy of this treatment is at present evaluated on the basis of clinical inspection and of digital photographs taken throughout the treatment. LASER-Doppler imaging (LDI) is a noninvasive method of imaging the perfusion of the tissues by the microcirculatory system (capillaries). The aim of this paper is to demonstrate that LDI allows a quantitative, numerical evaluation of the efficacy of the PDL treatment of PWS. METHOD: The PDL sessions were organized according to the usual scheme, every other month, from September 1, 2012, to September 30, 2013. LDI imaging was performed at the start and at the conclusion of the PDL treatment, and simultaneously on healthy skin in order to obtain reference values. The results evidenced by LDI were analyzed according to the "Wilcoxon signed-rank" test before and after each session, and in the intervals between the three PDL treatment sessions. RESULTS: Our prospective study is based on 20 new children. On average, the vascularization of the PWS was reduced by 56% after three laser sessions. Compared with healthy skin, initial vascularization of PWS was 62% higher than that of healthy skin at the start of treatment, and 6% higher after three sessions. During the 2 months between two sessions, vascularization of the capillary network increased by 27%. CONCLUSION: This study shows that LDI can demonstrate and measure the efficacy of PDL treatment of PWS in children. The figures obtained when measuring the results by LDI corroborate the clinical assessments and may allow us to refine, and perhaps even modify, our present use of PDL and thus improve the efficacy of the treatment.

Propranolol in infantile haemangioma: simplifying pretreatment monitoring.

BACKGROUND: Infantile haemangiomas (IHs) are very common vascular tumours. Propranolol is at present the first-line treatment for problematic and complicated haemangioma. In accordance with a Swiss protocol, children are monitored for 2 days at the start of the treatment to detect possible side effects of this drug. Our study advocates a simplification of the pretreatment monitoring process. METHODS: All children with a problematic and complicated haemangioma treated with propranolol between September 2009 and September 2012 were included in the study. All patients were hospitalised under constant nurse supervision for 48 hours at the start of the treatment and subjected to cardiac and blood measurements. The dosage of propranolol was 1 mg/kg/day on the first day and 2 mg/kg/day from the second day. Demographic data, clinical features, treatment outcome and complications were analysed. RESULTS: Twenty-nine infants were included in our study. Of these, 86.2% responded immediately to the treatment. There were no severe adverse reactions. Six patients presented transient side effects such as bradycardia, hypotension after the first dose and hypoglycaemia later. No side effects occurred after the second dose. Treatment was never interrupted. CONCLUSION: Propranolol (a ß-blocker) is a safe treatment for problematic IH. Side effects may occur after the first dose. A strict 48 hour monitoring in hospital is expensive and may be unnecessary as long as the contraindications for the drug are respected.

Management of multiple echinococcosis in childhood with albendazole and surgery.

BACKGROUND/PURPOSE: Multiple echinococcosis (ME) is a severe disease in childhood inaccessible to an initial radical surgical treatment. The aim of this study was to evaluate the efficacy of Albendazole in ME and to discuss the role of surgery in this pathology. METHODS: Eleven patients were included in a prospective study between 1996 and 2004. ME was defined by the presence of 10 or more cysts in the same organ. Albendazole was given as 10 mg/kg daily continuously. Treatment outcome was defined as cure, improvement, stabilization or deterioration. Surgery was discussed after 1 year of treatment. RESULTS: Our patients totalized 296 cysts located essentially on the liver (178 cysts) and the lungs (78 cysts). With exclusive Albendazole therapy, 57.7% pulmonary cysts and 96% peritoneal cysts were considered as cured. This rate was only 31.5% in hepatic localization. After surgical therapy, 67.4% of hepatic cysts were cured. No productive biliary fistula was observed. Two patients were operated laparoscpically. The total treatment duration ranged between 1 and 5 years. Parasitologic examination of operated cysts showed that 30% of them were viable even after 3 years of treatment. CONCLUSION: Albendazole has proven a strong efficacy in pulmonary and peritoneal localizations. A complementary surgical treatment is often necessary in hepatic localizations and it is facilitated with previous Albenazole therapy. Combination of ABZ and surgery seems to have encouraging results and must be applied for those patients. The high rate of viable cysts after medical therapy is problematic and must incite to develop new antihelminthic agents.