RESUMO
Biliary atresia presents as an obliterative cholangiopathy with neonatal jaundice and pale stools. The disease exhibits aetiological heterogeneity with a multiplicity of potential causative factors, both developmental and environmental. A number of clinical variants making up a minority of all cases can be defined relatively precisely which match suggested aetiology better although in most it still remains speculative. These include the syndromic form (BASM), the cystic form and those associated with CMV IgM antibodies. We review not only the clinical evidence for a developmental or an immune-mediated aetiology perhaps triggered by perinatal viral exposure but also several other recently suggested concepts such as microchimerism, gene susceptibility and environmental toxins.
Assuntos
Ductos Biliares/crescimento & desenvolvimento , Atresia Biliar/epidemiologia , Atresia Biliar/etiologia , Quimerismo , Exposição Ambiental/efeitos adversos , Imunidade Humoral , Atresia Biliar/diagnóstico , Atresia Biliar/cirurgia , Biópsia , Citomegalovirus/imunologia , Citomegalovirus/isolamento & purificação , Predisposição Genética para Doença , Humanos , Imunoglobulina M/imunologia , Incidência , Lactente , Fígado/patologia , Testes de Função Hepática , Linfócitos T/imunologia , UltrassonografiaRESUMO
We report the case of a school-aged boy who presented with clinical features suggesting acute appendicitis. However, further imaging which included CT, demonstrated an inflammatory mass involving the transverse colon raising the suspicion of lymphoma. He then developed intestinal obstruction, and in view of the rapid progression of the disease, he was thought to have non-Hodgkin's lymphoma. He underwent an open excisional biopsy, which revealed a necroinflammatory process and no suggestion of lymphoma or an alternative malignancy or specific diagnosis. His steroid treatment was stopped, and he made a good recovery postoperatively. Positive COVID-19 antibodies, positive response to steroids, results and clinical features were consistent with paediatric inflammatory multisystem syndrome (PIMS-TS), with extensive investigation not offering an alternative diagnosis.While PIMS-TS is a relatively new entity, we believe that this case highlights the importance of it being considered a differential diagnosis of a child presenting with an inflammatory mass.
Assuntos
Neoplasias Abdominais , COVID-19 , COVID-19/complicações , Criança , Humanos , Masculino , SARS-CoV-2 , Síndrome de Resposta Inflamatória SistêmicaRESUMO
INTRODUCTION: Improved parental experience is related to improved mental and physical health outcomes for the infant. The COVID-19 pandemic abruptly impacted on healthcare delivery and services need information to shape how to manage the disruption and recovery. METHODS: Our aim was to develop a systematic process to capture parents' experience of their neonatal surgical healthcare journey during the pandemic. We identified relevant stakeholders and using semistructured interviews, we explored three key themes.(1) How to recruit and collect data from representative parents?(2) What questions should be asked?(3) How to disseminate results for service development? RESULTS: Responses indicated the need to involve 'difficult to access groups' (eg, first language not English, high social deprivation, low health literacy), defined the range of family and patient characteristics variables to be considered for representative responses (eg, antenatal diagnosis, disease complexity, number of siblings, single parent, parental health). The proposed questions were grouped into five main topics: information preadmission; in-patient experience; support during admission; the effect of COVID-19; discharge and posthospital experience. Recommendations for dissemination included local, regional and national fora as well as the need to feedback to participants about the changes made.Based on the analysis, we developed a semistructured interview which underwent cognitive testing, prepilot and pilot phase testing. DISCUSSION: This protocol is grounded in the views of relevant stakeholders to ensure it captures relevant information in a pragmatic but methodologically sound way. It will next be used to assess parental experience in a large neonatal surgical unit. We hope that the protocol could be adapted and used by other groups.
Assuntos
COVID-19 , Atenção à Saúde , Feminino , Humanos , Lactente , Recém-Nascido , Pandemias , Pais/psicologia , Gravidez , Pesquisa QualitativaRESUMO
AIM OF THE STUDY: We aimed to compare the management of pediatric benign ovarian tumors between an English center and three Egyptian institutions. MATERIALS AND METHODS: This was a retrospective review of all children presenting with benign ovarian tumors between January 2014 and January 2019. A standardized dataset was used to compare between both sides. RESULTS: Eighty-nine patients were included (54 English and 35 Egyptians). Median age at diagnosis in England was 13 years (2-16y), while in Egypt it was 7 years (9m-16y) with P =0.001. Mature teratomas or dermoid cysts were the most common findings in England and Egypt; 75.9% and 82.8% of cases, respectively. The presentation with an acute abdomen represented 27.8% of English and 28.6% of Egyptian patients. Incidentally diagnosed lesions constituted 15% of English patients, whereas none of the Egyptian cases were discovered incidentally. There were variations in diagnostic imaging; England: Ultrasound (USS) (54), magnetic resonance imaging (MRI) (37), and computed tomography (CT) (only one)-Egypt: USS (35), CT (17), and MRI (only one). Minimally invasive surgery (MIS) was performed in 15% of English and 23% of Egyptian patients (P = 0.334). Ovarian-sparing surgery (OSS) was performed in: England 35%, Egypt 37%; P = 0.851. OSS was performed using MIS in 87.5% (7/8) of English patients and 100% (8/8) of Egyptians. Patients presented as emergencies generally had open oophorectomies: England; 86.7% open and 80% oophorectomy-Egypt; 100% open and 90% oophorectomy. Recurrences or metachronous disease occurred in 5.6% of English and 5.7% of Egyptian patients. CONCLUSIONS: There were no significant differences regarding surgical management, tumor pathology, and recurrence or metachronous disease. However, age, incidental diagnosis, and imaging modalities showed notable differences. MIS was correlated with ovarian preservation, whereas emergency surgery generally resulted in open oophorectomy.
Assuntos
Gerenciamento Clínico , Procedimentos Cirúrgicos Minimamente Invasivos/métodos , Neoplasias Ovarianas/cirurgia , Ovariectomia/métodos , Adolescente , Criança , Pré-Escolar , Egito/epidemiologia , Inglaterra/epidemiologia , Feminino , Humanos , Incidência , Lactente , Imageamento por Ressonância Magnética , Neoplasias Ovarianas/diagnóstico , Estudos Retrospectivos , UltrassonografiaRESUMO
AIM: No consensus exists about the optimal surgical technique for esophageal replacement. This study reports the surgical outcomes for the gastric pull-up and the colonic interposition procedures. MATERIALS AND METHODS: A retrospective review of children undergoing esophageal replacement surgery between January 2001 and June 2015 across four different pediatric surgery centers was conducted. Data collected included indications, epidemiology, surgical technique, complications, and outcomes. Patients were divided into group A, those that had a gastric pull-up procedure and group B, those that had a colonic interposition procedure. RESULTS: In total, 50 patients were included; 29 in group A and 21 in group B. Indications included esophageal atresia, caustic ingestion, and infective esophageal stricture. The median age at the time of surgery was 13 months. The mean length of follow-up was 5.2 years. Three patients died giving a mortality rate of 6%; 2 in group A and 1 in group B.In both groups, early postoperative complications included infective complications, such as wound infections, sepsis, and pneumonia (11), anastomotic leak (7), and respiratory complications (7). Late complications included adhesive bowel obstruction (2), anastomotic strictures (4), redundancy (1), and jejunostomy problems (1). Septic complications and anastomotic strictures occurred more frequently in group B. Further surgery was needed in eight patients; this was significantly higher in group B. Full oral feeding was achieved within 6 months in 91.5%. CONCLUSION: The gastric pull-up and colonic interposition have comparable mortality and outcomes. The colonic interposition was associated with a higher rate of early septic complications, anastomotic strictures, and need for further surgery.
Assuntos
Colo/transplante , Atresia Esofágica/cirurgia , Estenose Esofágica/cirurgia , Esofagoplastia/métodos , Estômago/cirurgia , Criança , Pré-Escolar , Atresia Esofágica/mortalidade , Estenose Esofágica/mortalidade , Feminino , Seguimentos , Humanos , Lactente , Masculino , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Estudos Retrospectivos , Resultado do TratamentoRESUMO
Abdominal wall defects in foetuses include gastroschisis, exomphalos, bladder exstrophy complex, cloacal exstrophy and body stalk syndrome. The defects that occur more commonly are gastroschisis and exomphalos. In this review we assess the current evidence regarding the incidence, perinatal risk factors, antenatal and postnatal management and outcome for both these conditions. A review of the current surgical practices for management of gastroschisis and exomphalos is discussed.
Assuntos
Parede Abdominal/anormalidades , Gastrosquise/cirurgia , Hérnia Umbilical/cirurgia , Parede Abdominal/patologia , Parede Abdominal/cirurgia , Gastrosquise/epidemiologia , Gastrosquise/patologia , Guias como Assunto , Hérnia Umbilical/epidemiologia , Hérnia Umbilical/patologia , Humanos , Incidência , Recém-Nascido , Fatores de Risco , Resultado do TratamentoRESUMO
Adhesions following intra-abdominal surgery are a major cause of small bowel obstruction. The nature of surgical interventions in children (especially neonates) increases the risk of adhesion-related complications. Following laparotomy in neonates, the collective literature reveals an aggregate mean incidence of adhesive small bowel obstruction (ASBO) of 6.2%; malrotation, 14.2%; gastroschisis, 12.6%; necrotising enterocolitis, 10.4%; exomphalos, 8.6%; Hirschsprung's disease, 8.1%; congenital diaphragmatic hernia, 6.3% and intestinal atresia, 5.7%. In children beyond the neonatal period, the aggregate mean incidence was 4.7%; colorectal surgery, 14%; open fundoplication, 8.2%; small bowel surgery, 5.7%; cancer surgery, 5.5%; choledochal cyst, 3.1%; appendicectomy, 1.4% and pyloromyotomy, 0.1%.
Assuntos
Procedimentos Cirúrgicos do Sistema Digestório/efeitos adversos , Obstrução Intestinal/epidemiologia , Aderências Teciduais/epidemiologia , Criança , Humanos , Lactente , Recém-Nascido , Obstrução Intestinal/etiologia , Aderências Teciduais/etiologiaRESUMO
Recurrence rate after a congenital diaphragmatic hernia repair is high especially after a patch repair. Recurrence can be asymptomatic, followed by respiratory or gastrointestinal symptoms and the diagnosis is usually confirmed radiologically. We present an unusual case of radiologically diagnosed recurrent left diaphragmatic hernia but at surgery was found to be a gastro-pleural fistula that occurred as a complication following fundoplication surgery.
Assuntos
Fundoplicatura/efeitos adversos , Fístula Gástrica/diagnóstico , Hérnias Diafragmáticas Congênitas , Doenças Pleurais/diagnóstico , Fístula do Sistema Respiratório/diagnóstico , Pré-Escolar , Diagnóstico Diferencial , Feminino , Fístula Gástrica/etiologia , Fístula Gástrica/cirurgia , Hérnia Diafragmática/diagnóstico , Hérnia Diafragmática/cirurgia , Humanos , Doenças Pleurais/etiologia , Doenças Pleurais/cirurgia , Recidiva , Fístula do Sistema Respiratório/etiologia , Fístula do Sistema Respiratório/cirurgia , Resultado do TratamentoRESUMO
PURPOSE: Pelvic ultrasound (US) has been used in various different ways to assess severity of constipation. We evaluated the use of a new US scoring system to determine if there is any correlation between symptom severity and US findings. METHODS: Data were collected prospectively on all children attending the constipation clinic from January 2007 to July 2008. All patients completed a standardised symptom severity score sheet (SSS) for constipation. US was done with the probe placed 2 cm above the pubic symphysis. A new US scoring system designed to assess the faecal loading and its effect on the bladder was used. Patients with severe symptoms were followed up with few patients requiring as many as four follow up visits. During each follow-up visit SSS and US scoring was completed. RESULTS: A total of 500 patients were studied (male 317, female 183) with a median age of 8 years (8 months, 18 years). Statistical analysis was done using SPSS software (version 15.0). Pearson's correlation was used to assess the correlation between SSS and US score. P value <0.05 was considered to represent a statistically significant correlation between symptoms and ultrasound findings. CONCLUSION: US is an easy and quick tool that can be used in the out-patient setting to assess severity of constipation. The new US scoring system is a reliable method that can be used in conjunction with clinical examination for assessing the long-term improvement of children who are undergoing treatment for chronic constipation.