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.

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.