Long-term efficacy of glycopyrrolate on sialorrhea in Goldenhar syndrome: a case report.

BACKGROUND: Goldenhar syndrome (GS) is a rare congenital disease characterized by impaired development of different facial structures and deformations of the teeth structures. Sialorrhea, which can cause difficulties in breathing and language impairment, is very common in GS and often difficult to treat. This case report highlights the short- and long-term importance of the therapeutic choice - glycopyrronium in oral solution - for the treatment of sialorrhea in children with poly-malformative syndrome, complicated by outcomes of post-hemorrhagic hydrocephalus. CASE PRESENTATION: We report the case of a 6-year-old child with GS, carrying a percutaneous endoscopic gastrostomy after tracheostomy. The child also presented developmental dysfunction of oral motor skills of feeding, complicated by severe sialorrhea, related to the maxillo-facial dysmorphism. Sialorrhea caused several respiratory tract infections and led to an increase in the care burden. Both the inoculations of botulinum toxin and the treatment with scopolamine transdermal patch have shown mild and transient efficacy. The therapeutic choice of glycopyrronium in oral solution was the most suitable for this patient, leading to long-term sialorrhea control. CONCLUSIONS: This clinical experience represents the first long-term efficacy and tolerability evaluation in using glycopyrrolate oral solution in treating drooling in children with GS. The reduction of drooling over time and the lack of clinically relevant adverse events have contributed to the decrease of respiratory tract infections, the development of oral motor skills, and determining a positive psycho-social impact on the patient's quality of life and her family.

Morphologic and Aerodynamic Changes of Upper Airway in Pediatric Hemifacial Microsomia Patients Undergoing Distraction Osteogenesis.

Current studies on hemifacial microsomia (HFM) patients undergoing unilateral distraction osteogenesis (DO) mainly confined to description of facial morphology. This study was to investigate the effect of unilateral DO on upper airway in pediatric HFM patients using computational fluid dynamics. The investigators implemented the present retrospective study composed of 20 patients (age 9.5 ±â€Š2.5 years, 11 males, 9 females) with moderate HFM performed unilateral DO on the ramus of mandible. Computational fluid dynamics models of the upper airway were obtained based on the computed tomography data sets which were taken before and 6 months after distraction. Morphologic and aerodynamic parameters were respectively computed and compared at peak inspiration. Paired t-tests were used to compare the differences between the before and after parameters with the significance set at P < 0.05. The authors observed that after DO, oropharynx and nasopharynx were the major expanded regions, average pressure increased in all cross-sections, average velocity decreased significantly except in the superior border of the epiglottis plane, and the airflow resistance significantly reduced in the whole upper airway (P < 0.05). The results indicate that unilateral DO may expand the constricted oropharynx and nasopharynx, obtain a relatively symmetrical airway shape and decrease the airflow resistance which consequently reduce the workload necessary for breathing and facilitate inspiration.

One-Year Relapse of Mandibular Distraction for Hemifacial Microsomia Using Masseteric Botulinum Toxin Type A Injections.

BACKGROUND: The relapse of hemifacial microsomia was thought to be highly related to the soft tissue envelope around the mandible angle mainly composed by masseter and medial pterygoid. According to the reason, we tried to apply masseter injection of type A botulinum toxin to weaken the soft envelope tension on the early stage post mandible distraction in adult HFM patients. METHODS: Eight patients diagnosed with HFM were studied and randomly assigned to an experimental or control group. Patients in the experimental group were treated with DO, orthognathic surgeries, autologous fat grafting, and bilateral masseter muscle injection with type A botulinum toxin. The patients in control group were treated with the same procedures as the patients in experimental group except for masseter muscle injection with type A botulinum toxin. The recurrence rates of both groups were evaluated and analyzed after nearly 1 year of follow-up. RESULTS: The mean recurrence rate was 26.30% ±â€Š11.84% (range 7.62%-37.27%) in the 8 patients after 1-year follow-up. The relapse rate was 16.32% ±â€Š7.78% (7.62%-26.22%) in the experimental group and 36.28% ±â€Š1.03% (34.84%-37.27%) in the control group. There was a significant difference (P = 0.002) between the experimental group and the control group. CONCLUSIONS: The combination of DO, orthognathic surgeries, autologous fat particle transplantation, and masseter muscle type A botulinum toxin injection technique could be a comprehensive treatment plan for adult patients of HFM. Furthermore, masseter injection of type A botulinum toxin might be an alternative method to reduce the early recurrence rate of postoperative adult patients of HFM.

Precisión en la planificación quirúrgica 3D en un paciente con síndrome de Goldenhar / Precision in 3D surgical planning in a patient with Goldenhar syndrome

Objetivo. Presentar un caso clínico de una paciente con síndrome de Goldenhar en el cual se utilizó, para la planificación de la corrección de su asimetría facial, el software Materialise CMF® (Leuven, Bélgica). Caso clínico. aciente de sexo femenino de 27 años que padece síndrome de Goldenhar. Se le realizó cirugía ortognática para corregir su asimetría facial. Para el diagnóstico, planificación y simulación quirúrgica del caso se utilizó el software 3D Materialise CMF® (Leuven, Bélgica). Conclusión. El método de planificación tradicional no es preciso, y estas imprecisiones producen una acumulación de errores en todo el proceso. Estos conceptos cobran gran relevancia en los casos de deformidades asimétricas, en donde los métodos bidimensionales son insuficientes, dejando un gran margen para la intuición, para la habilidad del profesional y, por lo tanto, para el error. El método de planificación 3D facilita el estudio, la planificación y la transferencia de lo planificado a la cirugía, minimizando los errores y logrando la máxima precisión (AU)

Aim. Submit a case of a patient with Goldenhar syndrome. The Materialise CMF® (Leuven, Belgium) software was used for planning her facial asymmetry correction. Case report. Female patient aged 27 who suffers from Goldenhar syndrome. He underwent orthognathic surgery to correct facial asymmetry. For diagnosis, surgical planning and simulation case 3D Materialise CMF® software (Leuven, Belgium) was used. Conclusions. The traditional method of planning is not necessary, these inaccuracies are an accumulation of errors in the whole process. These concepts assume great importance in cases of asymmetric deformities where the 2-dimensional methods are insufficient, leaving much room for intuition, for the professional ability and therefore for error. The method of 3D planning facilitates the study, planning and transfer of what is thought to surgery, minimizing errors and achieving maximum accuracy (AU)

Manejo anestésico y de la vía aérea en un niño con síndrome de Goldenhar / Management of anaesthesia and the airway in a child with Goldenhar syndrome

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A case of Goldenhar syndrome associated with growth hormone deficiency.

We have experienced the case of a 10-year-old boy who had Goldenhar syndrome accompanied by growth hormone (GH) deficiency. His height increased after treatment with growth hormone was administered. We found no untoward effects of the hormone and we consider that treatment with GH is useful for patients who present with Goldenhar syndrome associated with growth hormone deficiency.