Hypospadias management in children with anorectal malformation: a multidisciplinary single center experience.

Anorectal malformation (ARM) is often associated to other congenital malformations, requiring a tailored management. Hypospadias' treatment in ARM is poorly described. Aim of study is to describe our experience in ARM-hypospadias patients especially in relation to occult spinal dysraphism (OSD). ARM patients treated from 1999 to 2022 were retrospectively reviewed, selecting male with hypospadias. Clinical data, hypospadias's severity, ARM sub-type (Group A: perineal fistula; Group B: urethral fistula, bladder fistula, no fistula), OSD, other associated malformations, NLUTD were evaluated. Exclusion criteria: uncomplete data. Among 395 ARMs, 222 were males, 22 (10%) had hypospadias. Two patients were excluded. Group A: 8 patients, Group-B: 12. Hypospadias: proximal 9 patients, distal 11. Neuro-urological evaluation was performed before hypospadias repair. Eleven patients (55%) had OSD. Four OSD patients presented NLUTD and underwent detethering and CIC (two via cystostomy button, two via appendicostomy); two of them had hypospadias repaired. All proximal hypospadias underwent two stages of surgery. Distal hypospadias was corrected in 4/11 cases. Hypospadias is quite common in ARM patients and its surgical management must be scheduled considering the possible OSD and NLUTD, with the possible need for intermittent catheterization. Complexity of ARM and hypospadias appears to be related to each other.

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.

Avaliação dos efeitos a longo prazo da acupuntura e exercícios terapêuticos em ombro congelado de pacientes com acidente cerebral vascular / Evaluation of long term effects of acupuncture and therapeutic exercises on frozen shoulder in stroke patients

Objetivo do estudo: Determinar como a acupuntura e a terapia com exercícios afetam a função motora e espasmodicidade das mãos de pacientes com acidente cerebral vascular 6 meses após eles terem recebido este tipo de terapia. Métodos: Estudo clínico prospectivo, aleatório e cego de 20 pacientes, com idades entre 60-70 anos, na fase crônica de 6 meses após o AVC. Os sujeitos do estudo foram divididos em dois grupos: Grupo A, o qual foi tratado com acupuntura e terapia de exercícios (AP-ET) 6 meses atrás e Grupo B, o qual foi tratado apenas com terapia de exercícios (ET) 6 meses atrás. A avaliação incluiu uma entrevista ampla e a administração dos estágios de Brunnstrom, a Medida de Independência Funcional (MIF), a Escala Modificada de Ashworth (MAS), o Teste de Funcionalidade das Extremidades Superiores (UEFT), o Registro de Atividade Motora (MAL), a Série de Movimentos ativos e passivos (ROM, pROM), o teste de FUGL-Meyer da função da extremidade superior (FMA), o Questionário de Croft sobre Incapacidade do Ombro (CSDQ) e a Escala Analógica Visual (VAS) de dor. O Teste-t de Gosset foi usado para uma análise estatística. Resultados: As análises mostraram uma diferença estatisticamente significativa nos SCORES do pós-tratamento em comparação com 6 meses após o tratamento com cada grupo de estudo, para todos os parâmetros examinados (p<0.01 em todos os casos) exceto pelo MAS.

Aim of the study: To determine how acupuncture and exercise therapy affect motor function and spasticity on the hands of stroke patients 6 months after they had received this kind of therapy. Methods: Prospective, randomized, single blind clinical study of 20 patients, age 60-70 years, in the chronic phase 6 months after stroke. Subjects were divided into two groups: Group A, which was treated with acupuncture and exercise therapy (AP-ET) 6 months ago and Group B, which was treated with exercise therapy (ET) alone 6 months ago. the assessment included a comprehensive interview and administration of the Brunnstrom?s stages, Functional Independence Measure (FIM), Modified Ashworth Scale (MAS), Upper Extremity Function Test (UEFT), Motor Activity Log (MAL), active and passive Range of Motion (aROM, pROM), Fugl-Meyer test of upper extremity function (FMA), Croft Shoulder Disability Questionnaire (CSDQ) and Visual Analogue Scale (VAS) of pain. The Gosset t-Test was used for the statistical analysis. Results: Analyses showed a statistically significant difference in the post-treatment SCORES as compared to 6 months after treatment within each study group for all parameters examined (p<0.01 in all cases) except for MAS. Analyses showed a statistically significant difference in some parameters in the ET group as compared to the AP-ET group with greater mean values recorded in the AP-ET group for MAS and CSDQ. All other parameters showed no statistical differences between the two different therapy groups 6 months after the therapy. Conclusion: The results confirm the hypothesis that acupuncture and exercise therapy are useful in the treatment of frozen shoulder in stroke patients and that their effects are still present after 6 months of therapy, however, given the small patient population, further studies are needed to verify these results.