Diagnosis and treatment of speech disorders in children with Moebius syndrome.

BACKGROUND: Moebius syndrome (MS) is characterized by congenital bilateral paralysis of the facial and abducens nerves. Clinical features include feeding problems, dysarthria, dysphagia, sialorrhea, strabismus, and lack of facial expression. Patients with MS frequently present with dysphagia during infancy. Further on during childhood a severe speech disorder is a common feature. However, articulation deficits in patients with MS are scarcely reported in the related scientific literature. OBJECTIVE: The aim of this study is to describe speech deviations, intelligibility and sialorrhea in patients with MS. MATERIAL AND METHODS: Eighty-seven patients with MS were prospectively studied. Age ranged from 4 to 18 years. A complete Speech and Language Pathology (SLP) evaluation was performed in all cases. The evaluation focused on articulation placement, sialorrhea and intelligibility of speech. RESULTS: Sialorrhea was detected in 23% of the patients. Abnormal articulation placement of bilabial phonemes was observed in 68% of the patients. Another 50% of the patients presented with articulation placement errors in other phonemes. Intelligibility was classified as adequate in 18% of the cases. Mildly affected intelligibility was found in 51% of the patients. Speech was considered moderately unintelligible in 20% of the cases. Unintelligible speech was found in 11% of the patients. CONCLUSIONS: From the results of this prospective study it can be concluded that a high percentage of patients with MS are at high risk of presenting with moderate to severe speech disorders. Thus, an early SLP intervention should be provided for this population in order to enhance speech development and reducing the risk of severe oral communication impairments.

Delayed Presentation of Vaginal Cuff Dehiscence after Robotic Hysterectomy for Gynecologic Cancer: A Case Series and Review of the Literature.

Background. Vaginal cuff dehiscence after hysterectomy has varying incidence according to surgical approach, with highest rates associated with laparoscopic surgery. Comparative data on timing of diagnosis describe a wide range of clinical presentation from weeks to years after hysterectomy. Limited reports have focused specifically on delayed presentation of vaginal cuff dehiscence. Cases. All cases of vaginal cuff dehiscence at our institution between 2005 and 2015 were collectively reviewed and three cases were identified of women who presented with cuff dehiscence greater than 180 days from index surgery. Diagnosis occurred at 342 to 461 days after operation. One patient presented with abdominal pain, a second case presented with vaginal discharge, and the third case lacked clinical symptoms altogether. Prior to diagnosis, one case received chemotherapy and external beam radiation for Stage IB1 cervical cancer and another case received external beam radiation alone for Stage II endometrioid adenocarcinoma. All cuffs were repaired vaginally with interrupted, early absorbable suture. Conclusion. Robotic total laparoscopic hysterectomy may be associated with increased risk of vaginal cuff dehiscence. Further studies are needed to determine risk factors and patient characteristics associated with delayed presentation of vaginal cuff dehiscence in robotic total hysterectomy as well as all surgical approaches.

Enfermedad de paget ósea: breve revisión de la literatura a propósito de un caso / Paget ́s bone disease: case report and brief literature review

La osteítis deformante o enfermedad de Paget es un trastorno óseo, crónico, poco frecuente en Chile, que puede afectar típicamente a pacientes después de los 55 años. Su etiología es probablemente multifactorial: actualmente se considera como posiblemente implicados factores ambientales y genéticos.La mayor parte de los pacientes son asintomáticos. Los diagnósticos habitualmente se realizan en base a hallazgos radiológicos. Cuando existen síntomas, suelen ser dolor y/o deformidad ósea. Pueden comprometerse uno o varios huesos.Ante su sospecha clínica, la exploración diagnóstica requiere de al menos el estudio con radiografías simples de las lesiones sospechosas, cintigrama óseo y parámetros de actividad metabólica ósea; recomendándose los niveles séricos de fosfatasas alca-linas, por su bajo costo y elevada disponibilidad.Las metástasis óseas condensantes son un importante diagnóstico diferencial a con-siderar.Sobre el tratamiento, se recomienda el uso de fármacos antirresortivos como terapia específica, y el uso de ortesis y terapia física.El seguimiento es clínico y con biomarcadores de recambio óseo.

Osteitis-deformans or Paget's disease is a chronic bone disorder uncommon in Chile, with a typical presentation after age 55. Its etiology is probably multifactorial: It is actualy considered that environmental and genetic factors are mainly involved.Most patients are asymptomatic. Diagnoses are usualy performed based on radio-logical findings. When symptoms exist, they are pain and/or bone deformity. One or several bones can be affects.If it ́s suspect, the diagnostic exploration requires at least the study with x-rays of the suspicious lesions, bone scintigraphy and parameters of bone metabolic activity, being recommended the serum levels of alkaline phosphatases, for its low cost and high availability.Condensing bone metatases have to be considered like an important differential di-agnosis.For the treatment, the use of antiresorptive drugs as specific therapy is recommend-ed, and the use of orthotics and physical therapy.Follow-up is made with clinical findings and with biomarkers of bone turnover.