Efficacy of antireflux surgery in children with or without neurological impairment: a systematic review.

BACKGROUND: Antireflux surgery is commonly performed in children, yet evidence for its efficacy is limited. The aim of this review was to determine the effect of antireflux surgery with regard to objective measures of quality of life (QoL) and value of upper gastrointestinal investigations in neurologically normal (NN) and neurologically impaired (NI) children. METHODS: A systematic review was conducted of articles reporting children undergoing antireflux surgery in whom preoperative and postoperative objective testing was performed. Primarily, Embase, CINAHL, MEDLINE and PubMed were searched from inception to April 2019. Methodological Index for Non-randomized Studies (MINORS) criteria were used to assess article quality. RESULTS: Of 789 articles, 14 met the eligibility criteria, 12 prospective observational and 2 retrospective studies. The median MINORS score was 59·4 (i.q.r. 39 to 62·5) per cent. Seven studies reported assessment of validated QoL measures before and after antireflux surgery in 148 children. Follow-up ranged from 1 to 180 months. All studies confirmed significant improvements in QoL measures among NN and NI children at all follow-up points. Eleven studies reported on preoperative and postoperative investigations in between 416 and 440 children children. Follow-up ranged from 0·5 to 180 months. Nine studies confirmed improvements in gastro-oesophageal reflux using 24-h oesophageal pH monitoring with or without manometry, but conflicting results were identified for four studies reporting gastric emptying. No studies reported fluoroscopy or endoscopy adequately. CONCLUSION: Based on the results of studies of low-to-moderate quality, antireflux surgery improved QoL and reduced oesophageal acid exposure in NN and NI children in the short and medium term. Although antireflux surgery is a common elective operation, the lack of rigorous preoperative and postoperative evaluation(s) in the majority of patient-reported studies is striking.

ANTECEDENTES: La cirugía antirreflujo (antireflux surgery, ARS) se realiza con frecuencia en niños, aunque la evidencia sobre su eficacia es limitada. Nos propusimos determinar la eficacia de la ARS con respecto a medidas objetivas de calidad de vida (quality of life, QoL) y la utilidad de las exploraciones del tracto gastrointestinal superior en niños con funciones neurológicas normales (neurologically normal, NN) y con discapacidad neurológica (neurologically impaired, NI). MÉTODOS: Se llevó a cabo una revisión sistemática (de acuerdo con la normativa PRISMA) de artículos que describiesen series de niños sometidos a ARS en los que se realizaron pruebas objetivas preoperatorias y postoperatorias. Principalmente, se efectuó una búsqueda en EMBASE, CINAHL, Medline y Pubmed desde un comienzo hasta 04/19. La calidad de los artículos se evaluó siguiendo los criterios MINORS. RESULTADOS: De 789 artículos, 14 reunían los criterios de elegibilidad - 12 estudios observacionales prospectivos y 2 estudios retrospectivos. La mediana de la puntuación MINORS fue de 59,4 % (rango intercuartílico 23,4%). Siete estudios describieron la evaluación de medidas de QoL validadas antes y después de ARS en 148 niños. El seguimiento varió de 1 a 180 meses. Todos los estudios confirmaron mejorías significativas en medidas de QoL en los niños NN y NI en todos los puntos del seguimiento. Once estudios describieron sobre las investigaciones preoperatorias y postoperatorias en 507 niños. El seguimiento oscilaba de 0,5 a 180 meses. Nueve estudios confirmaron mejorías en el reflujo gastroesofágico utilizando la monitorización del pH esofágico de 24 horas +/- manometría, pero los resultados eran contradictorios en 3 estudios que evaluaron el vaciamiento gástrico. Ningún estudio describió de forma adecuada los datos de la fluoroscopia o endoscopia. CONCLUSIÓN: De los estudios identificados de baja o moderada calidad, en un seguimiento a corto y medio plazo, la ARS mejora la QoL y reduce la exposición del esófago al ácido en niños NN y niños NI. A pesar de que la ARS es una cirugía electiva habitual, es llamativa la falta de evaluaciones preoperatorias y postoperatorias rigurosas en la mayoría de los estudios de pacientes.

Perianal rhabdomyomatous mesenchymal hamartoma.

A rhabdomyomatous mesenchymal hamartoma (RMH) is a rare benign soft tissue tumour found in the face and neck region of children. A three-month-old male infant was referred to our unit with a polypoid anal lesion that had been present from birth. Histopathological examination of the excised sample showed haphazard arrangement of mature striated skeletal muscle in among nerves and blood vessels, and immunohistochemistry confirmed the lesion to be a RMH. There was no evidence of recurrence during the follow-up period. This case emphasises two points: the unique histological characteristics associated with RMH and how this allows distinction from other differential diagnoses, and the consideration of RMH as a diagnosis in perianal lesions.

Complete androgen insensitivity syndrome associated with bilateral Sertoli cell adenomas and paratesticular leiomyomas: case report and review of the literature.

BACKGROUND: Complete androgen insensitivity syndrome (CAIS) is a rare and usually unexpected cause of primary amenorrhoea that results from receptor resistance to androgens, producing a female phenotype in genetically male patients. CASE: A 16-year-old girl was diagnosed with CAIS after investigations for primary amenorrhoea. Her left inguinal gonad and the right intra-abdominal gonad were resected and histopathology revealed the presence of dysgenetic multinodular testes with absence of germ cells, significant hyperplasia of Sertoli cells (Sertoli cell adenoma) and presence of paratesticular leiomyomas. CONCLUSION: Although the risk of gonadal tumour development is considered to be low, a variety of tumours have been described in association with CAIS, but this is the first report of development of bilateral paratesticular leiomyomas developing concurrently with Sertoli cell adenomas.

Splenic artery aneurysm in a cardiac transplant patient: a case report.

We present the case of a 61-year-old woman who underwent repair of a symptomatic 4-cm splenic artery aneurysm. This patient had received heart transplantation for hypertrophic congestive cardiomyopathy 5 years before this event. She was under immunosuppression therapy with cyclosporine, prednisone and azathioprine. The aneurysm was ligated both proximally and distally without removal of the spleen. To our knowledge, this is the first reported case of an isolated splenic artery aneurysm in the heart transplant population. We discuss the potential role of immunosuppressive agents and hormonal factors in the development of this extremely rare occurrence.