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1.
J Neuropsychiatry Clin Neurosci ; 33(4): 295-306, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34280321

RESUMO

OBJECTIVE: The relationship between idiopathic and inherited (monogenic) forms of isolated and combined dystonia and psychiatric disorders remains unclear. In the present review, the authors aimed to provide increased clarity on this association through a systematic review of all controlled quantitative studies using a structured or semi-structured psychiatric interview to diagnose psychiatric disorders in individuals with these conditions. METHODS: Three databases were searched to identify 20 eligible studies, with a total of 1,275 participants fulfilling inclusion criteria. Eligible articles were quality appraised and divided into four sections (idiopathic forms of dystonia [N=11], early-onset torsion dystonia [N=2], gene mutation positive myoclonus dystonia; DYT-SGCE [N=6], and rapid-onset dystonia-parkinsonism [N=1]). RESULTS: For each study, results were grouped into subcategories (overall psychiatric comorbidity, anxiety disorders, mood disorders, substance misuse, and other [personality disorder and cognitive impairment]). For idiopathic dystonia, higher rates of psychiatric comorbidity, including mood and anxiety disorders, were noted when cases were compared with both healthy control subjects and control groups with a medical comorbidity. However, for major depressive disorder and obsessive-compulsive disorder (OCD) specifically, no differences were seen between groups. Study subjects with DYT-SGCE appeared to be at higher risk of psychiatric comorbidity, major depressive disorder, OCD, and alcohol dependence than control populations. CONCLUSIONS: Overall, the prevalence of psychiatric comorbidity appears to be increased in individuals with idiopathic and inherited (monogenic) forms of isolated and combined dystonia compared with control subjects. This finding is not consistent for all comparisons, and further research is required to understand the nature of these associations and the underlying causative etiologies.


Assuntos
Transtornos de Ansiedade/epidemiologia , Distonia , Transtornos do Humor/epidemiologia , Comorbidade , Distonia/epidemiologia , Distonia/genética , Humanos , Mutação
2.
J Pediatr Gastroenterol Nutr ; 69(2): e34-e38, 2019 08.
Artigo em Inglês | MEDLINE | ID: mdl-30921256

RESUMO

AIMS AND OBJECTIVES: The aim of this review is develop a reliable and valid questionnaire that assesses patient-reported experience measures (PREMs) and patient-reported outcome measures (PROMs) of caregivers, families, and patients with severe constipation and fecal incontinence who failed conservative treatments and require a formal bowel management program (BMP). METHODS: A 5-step iterative process was utilized to ensure the reliability and validity of the final instrument. Parents or guardians of patients undergoing our week-long BMP were first asked 20 open-ended questions aimed at understanding the challenges in managing their child's condition. Responses were transformed into an initial 41-item survey constituted of 2 constructs relating to PREMs (24 items) and PROMs (17 items). Survey items were then administered to 359 parents and guardians undergoing BMP. Cognitive interviews were performed with 20 families to assess face validity and to further refine the survey. Instrument readability and reliability was assessed by Flesch-Kincaid and Crohnbach α analyses. Items that weakly correlated were deleted to yield a final instrument that was both valid and reliable. RESULTS: A 33-statement survey was developed that encompasses important physical and emotional health concerns, quality of life, treatment success, financial considerations, schooling, parental employment, and social concerns. The final instrument, the Patient-Reported Experience and Outcome Measure in a Bowel Management Program (PREOM-BMP), is divided into 2 separate constructs that assess important patient-reported experience (22 items) and outcome (11 items) measures. Reliability analyses on the final instrument yielded a Crohnbach α of 0.925. CONCLUSIONS: The PREOM-BMP offers physicians and nursing professionals a brief and valid tool to measure the impact of a bowel management program on both the child and the family unit.


Assuntos
Constipação Intestinal/prevenção & controle , Incontinência Fecal/prevenção & controle , Medidas de Resultados Relatados pelo Paciente , Inquéritos e Questionários , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Reprodutibilidade dos Testes , Adulto Jovem
3.
J Digit Imaging ; 32(5): 761-765, 2019 10.
Artigo em Inglês | MEDLINE | ID: mdl-30350007

RESUMO

Surgeons have a steep learning capacity to understand 2-D images provided by conventional cloacagrams. Imaging advances now allow for 3-D reconstruction and 3-D models; but no evaluation of the value of these techniques exists in the literature. Therefore, we sought to determine if advances in 3-D imaging would benefit surgeons, lead to accelerated learning, and improve understanding for operative planning of a cloaca reconstruction. Questionnaires were used to assess the understanding of 2-D and 3-D images by pediatric surgical faculty and trainees. For the same case of a cloacal malformation, a 2D contrast study cloacagram, a 3D model rotatable CT scan reconstruction, a software enhanced 3D video animation (which allowed the observer to manipulate the structure in any orientation), and a printed physical 3D cloaca model that could be held in the observer's hand were employed. Logistic mixed effect models assessed whether the proportion of questions about the case that were answered correctly differed by imaging modality, and whether the proportion answered correctly differed between trainee and attending surgeons for any particular modality. Twenty-nine pediatric surgery trainees (27 pediatric general surgery and 2 pediatric urology surgery trainees) and 30 pediatric surgery and urology faculty participated. For trainees, the percentage of questions answered correctly was: 2-D 10.5%, 3-D PACS 46.7%, 3-D Enhanced 67.1%, and 3-D Printed 73.8%. For faculty, the total percentage of questions answered correctly was: 2-D 22.2%, 3-D PACS 54.8%, 3D Enhanced 66.2%, and 3-D printed 74.0%. The differences in rates of correctness across all four modalities were significant in both fellows and attendings (p < 0.001), with performance being lowest for the 2-D modality, and with increasing percentage of correct answers with each subsequent modality. The difference between trainees and attendings in correctness rate was significant only for the 2-D modality, with attendings answering correctly more often. The 2-D cloacagram, as the least complex model, was the most difficult to interpret. The more complex the modality, the more correct were the responses obtained from both groups. Trainees and attendings had similar levels of correct answers and understanding of the cloacagram for the more advanced modalities. Mental visualization skills of anatomy and complex 3-D spatial arrangements traditionally have taken years of experience to master. Now with novel surgical education resources of a 3-D cloacagram, a more quickly advancing skill is possible.


Assuntos
Cloaca/anatomia & histologia , Imageamento Tridimensional/métodos , Modelos Anatômicos , Pediatria/métodos , Cirurgiões , Cloaca/diagnóstico por imagem , Fluoroscopia , Humanos
4.
J Surg Res ; 203(2): 398-406, 2016 06 15.
Artigo em Inglês | MEDLINE | ID: mdl-27363649

RESUMO

BACKGROUND: The aim of this study was to establish the rate of screening for associated cardiac, vertebral, spinal cord, urologic, and limb anomalies vertebral ano-rectal cardiac tracheo-esophageal renal limb (VACTERL) in children with anorectal malformation (ARM). METHODS: We performed a retrospective cohort study using the Medicaid Analytic eXtract database which contains enrollment and utilization claims and demographic information from all Medicaid enrollees. Patients born between January 2005 and December 2008 with International Classification of Diseases, Ninth Revision codes for a diagnosis of ARM within 30 days of life, an ARM procedure code during the first year of life, and a minimum of 12 months of continuous enrollment were included. VACTERL screening was determined using International Classification of Diseases, Ninth Revision and Current Procedural Terminology codes for diagnostic tests used to detect these anomalies. RESULTS: A total of 406 patients were identified (231 males). Evaluation of the spinal vertebrae was performed in 94% of patients (381 of 406). Spinal cord evaluation was performed in 57% (231 of 406): 52% (121 of 231) received spinal ultrasound (US), 24% (56 of 231) received spinal magnetic resonance imaging, and 23% (54 of 231) received both. Sacral radiographs were performed in 8% (32 of 406) and 77% (313 of 406) underwent an echocardiogram. Genitourinary evaluation was performed in 84% (341 of 406): 67% (229 of 341) received renal US, 8% (27 of 341) received abdominal US, and 25% (85 of 341) received both. Limb evaluation was recorded in 19% (76 of 406). Multiple screening including an echocardiogram, spinal radiograph, spinal cord evaluation, and renal evaluation was performed in 45% (181 of 406); 2% (7 of 406) did not receive any screening tests. CONCLUSIONS: Low VACTERL screening in children with ARM suggests that associated anomalies may be undiagnosed which may lead to increased long-term morbidity.


Assuntos
Anormalidades Múltiplas/diagnóstico , Canal Anal/anormalidades , Malformações Anorretais/diagnóstico , Esôfago/anormalidades , Cardiopatias Congênitas/diagnóstico , Rim/anormalidades , Deformidades Congênitas dos Membros/diagnóstico , Triagem Neonatal/estatística & dados numéricos , Padrões de Prática Médica/estatística & dados numéricos , Coluna Vertebral/anormalidades , Traqueia/anormalidades , Feminino , Humanos , Recém-Nascido , Masculino , Medicaid , Estudos Retrospectivos , Estados Unidos
5.
Pediatr Surg Int ; 30(7): 733-6, 2014 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-24879556

RESUMO

BACKGROUND AND AIMS: At routine groin surgery in male paediatric patients occasionally the vas deferens may be absent. This finding usually leads to investigations to establish the status of the contralateral vas deferens and the status of the kidneys. It is not uncommon to find either an ipsilateral renal agenesis or congenital bilateral absence of the vas deferens. The latter finding prompts a test for cystic fibrosis. We report three patients who upon investigation were found to have the rare combination of congenital bilateral absence of the vas deferens and unilateral renal agenesis, and discuss the possible embryological basis, the clinical management and the long-term implications of these findings. PATIENTS AND METHODS: We present three patients who were incidentally found to have absence of the vas deferens whilst undergoing elective groin surgery and following further tests were diagnosed with congenital bilateral absence of the vas deferens and unilateral renal agenesis. The case notes were reviewed, together with the results of radiological investigations, cystic fibrosis screening and the status of the contralateral vas deferens. RESULTS: All three patients were found to have congenital bilateral absence of the vas deferens, unilateral renal agenesis and were not found to have cystic fibrosis. CONCLUSIONS: The combination of congenital bilateral absence of the vas deferens and unilateral renal agenesis, without cystic fibrosis, is rare and not reported previously in the paediatric literature. These findings require appropriate counselling of the parents and child, with regards to the long-term implications of infertility and renal function.


Assuntos
Anormalidades Congênitas/cirurgia , Nefropatias/congênito , Rim/anormalidades , Procedimentos Cirúrgicos Urológicos/métodos , Ducto Deferente/anormalidades , Anormalidades Congênitas/genética , Família , Humanos , Lactente , Rim/cirurgia , Nefropatias/genética , Nefropatias/cirurgia , Laparoscopia , Masculino
6.
J Pediatr Surg ; 58(2): 270-274, 2023 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-36384941

RESUMO

INTRODUCTION: The aim of this study is to report on the outcomes of patients born with cloacal malformation, managed at a single institution more than the last 28 years. The focus of this study is the long term renal and colorectal outcomes. METHODS: Patients were identified from the departmental database from 1994 to 2021. The medical records and operative notes were retrospectively reviewed. RESULTS: Twenty-one patients fulfilled the inclusion criteria. Eleven long common channel (LCC) and ten short common channel (SCC) cloacae patients were identified. Median age at the time of primary reconstruction was 11 months in both groups. In the LCC group, seven (63.6%) patients underwent a Total Urogenital Mobilisation (TUM), and 4 (36.4%) required a vaginal replacement. 6/11 (54.5%) of patients required drainage of a hydrocolpos. In the SCC group, four patients required a TUM, two patients underwent mobilisation of the rectum and vagina alone, and three underwent rectal mobilisation alone. Two patients have required renal transplant for congenital renal dysplasia, and two have developed chronic renal failure associated with the sequalae of vesicoureteric reflux. Eleven (52.3%) of the patients manage their bowels with an antegrade continent enema (ACE), and two of the LCC cloaca are defunctioned with a colostomy. Clean intermittent catheterisation is performed by 12 (57%) of the patients, either per urethra or via a Mitrofanoff channel. CONCLUSION: The urinary and faecal continence are the main challenges in the management of cloaca patients. Many require surgical intervention to achieve social continence. LEVEL OF EVIDENCE: Level IV.


Assuntos
Cloaca , Vagina , Feminino , Animais , Humanos , Lactente , Cloaca/cirurgia , Estudos Retrospectivos , Vagina/cirurgia , Vagina/anormalidades , Reto/cirurgia , Uretra/cirurgia
7.
J Pediatr Surg ; 57(2): 250-256, 2022 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-34848077

RESUMO

BACKGROUND: We conducted a questionnaire-based study of pouch function, quality of life, sexual function, and fertility among patients who had undergone ileal pouch anal anastomosis (IPAA) in childhood and who are now more than 18 years old. METHODS: A consecutive series of patients were asked to complete the following questionnaires: Pouch function score (PFS), short form 36 (SF-36), International index of erectile function (IIEF) (males), and Female sexual function index (FSFI) (females). Fertility in females was also assessed. Data are quoted as a median (SD). RESULTS: Of 144 patients who had IPAA, 101 were eligible, and 70 responded. Mean age at surgery was 14 years, and mean current age is 26 years. Most patients had either ulcerative colitis or familial adenomatous polyposis. The questionnaire was completed by 38/70 (54%). Median PFS score was 6 (5). SF-36 were lower than previous reports. Median FSFI was 30 (7.6), 84% of possible maximum. Median IIEF was 69, 92% of possible maximum. Successful conception was reported in 5/7 women who had tried. However, there were several miscarriages and two surgical emergencies during pregnancies. CONCLUSIONS: IPAA can be performed in childhood with similar pouch function to reported adult series. Quality of life appears poorer, but sexual function is maintained. There may be an association with adverse events in pregnancy. LEVEL OF EVIDENCE: IV.


Assuntos
Polipose Adenomatosa do Colo , Colite Ulcerativa , Bolsas Cólicas , Proctocolectomia Restauradora , Polipose Adenomatosa do Colo/cirurgia , Adolescente , Adulto , Anastomose Cirúrgica , Colite Ulcerativa/cirurgia , Feminino , Fertilidade , Humanos , Masculino , Gravidez , Qualidade de Vida , Resultado do Tratamento
8.
J Immigr Minor Health ; 24(3): 570-579, 2022 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-34595614

RESUMO

Prior investigations have examined risk factors associated to postpartum depression in immigrant women, but depression during pregnancy has received less attention. This study describes the prevalence and early determinants of antenatal depression among recent (≤ 5 years) and long-term immigrants (> 5 years), compared to Canadian-born women. 503 women completed standardized self-report questionnaires measuring sociodemographics and psychosocial factors. Multivariate logistic regressions identified first trimester risk factors for depression in each immigrant group. The prevalence of depressive symptoms was highest for recent immigrant (25.3-30.8%) compared to long-term immigrant (16.9-19.2%) and Canadian-born women (11.7-13.8%). Among recent immigrants, multiparity, higher stress and pregnancy-specific anxiety in early pregnancy increased the risk of antenatal depression. Among long-term immigrants, stress in the first trimester was significantly associated with antenatal depressive symptoms. Knowledge of modifiable risk factors (pregnancy-specific anxiety and stress) may help improve antenatal screening and inform the development of tailored interventions to meet the mental health needs of immigrant women during the perinatal period.


Assuntos
Depressão Pós-Parto , Emigrantes e Imigrantes , Canadá/epidemiologia , Depressão/psicologia , Depressão Pós-Parto/diagnóstico , Feminino , Humanos , Masculino , Paridade , Gravidez , Fatores de Risco
9.
BJPsych Bull ; 46(6): 336-341, 2022 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-34231454

RESUMO

MRCPsych courses play a key role in helping trainees prepare for Membership examinations and specialist training. Historically, their social aspect, although arguably as important, has perhaps not been sufficiently prioritised. During a pandemic, when teaching is largely delivered virtually, the social benefit of meeting peers is highlighted by its absence. Given the future likelihood of increased virtual teaching, it is of paramount importance to explore ways of enhancing the sociability of teaching courses. In addition to the social needs of trainees, there is a recognised need to increase and integrate the neuroscience component of the curriculum to better meet the needs of modern day mental health research, treatment and practice. This article describes how the Birmingham MRCPsych course successfully addressed both these issues with a 2-day 'residential' in October 2019, and considers whether future residentials could be delivered virtually.

10.
European J Pediatr Surg Rep ; 9(1): e41-e45, 2021 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-34026419

RESUMO

Cloacal exstrophy is a rare malformation that presents as a lower midline abdominal wall defect which affects the gastrointestinal and genitourinary systems. The components of cloacal exstrophy characteristically include omphalocele, exstrophy of perineal structures, and imperforate anus. Most of these patients also have renal anomalies such as pelvic kidney, fused kidneys, or solitary kidneys. This congenital condition can also be associated with spinal issues, such as spinal dysraphism. When combined with spinal defects, it is referred to as the omphalocele, exstrophy, imperforate anus, and spinal defects (OEIS) complex, and is one of the most challenging surgical conditions to manage. Here, we present a unique case of a low-birth-weight patient with OEIS and a liver containing giant omphalocele and the novel surgical technique used to manage her cloacal exstrophy whereby the cecal plate was not separated from the bladder halves, but rather left for an autoaugment, and the ileum was connected to the hindgut.

11.
Pediatr Surg Int ; 26(2): 141-4, 2010 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-19707772

RESUMO

Circumcision can be undertaken using a variety of techniques. For the technique to be successful, it should be easy to perform, avoid excessive haemorrhage and achieve a good cosmetic and functional result with minimal postoperative care. Naturally, there are differences between circumcision in the paediatric and adult populations and here we review the literature on recent advances that have been made in paediatric circumcision and the use of a sutureless technique with tissue glue for wound approximation.


Assuntos
Circuncisão Masculina/métodos , Técnicas de Sutura , Adesivos Teciduais , Humanos , Masculino , Resultado do Tratamento
12.
European J Pediatr Surg Rep ; 8(1): e45-e47, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-32793406

RESUMO

Herein we present a case of 5-month-old female born with a cloaca. She underwent a colostomy at birth and then underwent endoscopy and cloacagram to plan for the definitive reconstruction. The case is presented with a focus on the reconstructive strategies, and questions for the readers are posed in a quiz format.

13.
Semin Pediatr Surg ; 29(6): 150995, 2020 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-33288142

RESUMO

In this review, the care of children with a previously repaired anorectal malformation is explored. We know that the surgical care of children with anorectal malformations is complex; however, despite an increased understanding of the congenital anomaly and significant technical advances in the operative repair, many of these children continue to have poor functional outcomes. In this article we focus on the common surgical complications, discuss typical presentations, consider appropriate investigations, and review the risks and benefits of revisional surgery in those patients that are 'not doing well' following their primary reconstruction.


Assuntos
Assistência ao Convalescente/métodos , Malformações Anorretais/cirurgia , Procedimentos Cirúrgicos do Sistema Digestório/métodos , Procedimentos de Cirurgia Plástica/métodos , Cuidados Pós-Operatórios/métodos , Complicações Pós-Operatórias/diagnóstico , Complicações Pós-Operatórias/terapia , Anormalidades Múltiplas/diagnóstico , Anormalidades Múltiplas/cirurgia , Malformações Anorretais/diagnóstico , Humanos , Complicações Pós-Operatórias/etiologia , Reoperação/métodos , Resultado do Tratamento
14.
Pilot Feasibility Stud ; 5: 121, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31720000

RESUMO

BACKGROUND: There is no definitive method of accurately diagnosing appendicitis before surgery. We evaluated the feasibility of collecting breath samples in children with abdominal pain and gathered preliminary data on the accuracy of breath tests. METHODS: We conducted a prospective pilot study at a large tertiary referral paediatric hospital in the UK. We recruited 50 participants with suspected appendicitis, aged between 5 and 15 years. Five had primary diagnosis of appendicitis. The primary outcome was the number of breath samples collected. We also measured the number of samples processed within 2 h and had CO2 ≥ 3.5%. Usability was assessed by patient-reported pain pre- and post-sampling and user-reported sampling difficulty. Logistic regression analysis was used to predict appendicitis and evaluated using the area under the receiver operator characteristic curve (AUROC). RESULTS: Samples were collected from all participants. Of the 45 samples, 36 were processed within 2 h. Of the 49 samples, 19 had %CO2 ≥ 3.5%. No difference in patient-reported pain was observed (p = 0.24). Sampling difficulty was associated with patient age (p = 0.004). The logistic regression model had AUROC = 0.86. CONCLUSIONS: Breath tests are feasible and acceptable to patients presenting with abdominal pain in clinical settings. We demonstrated adequate data collection with no evidence of harm to patients. The AUROC was better than a random classifier; more specific sensors are likely to improve diagnostic performance. TRIAL REGISTRATION: ClinicalTrials.gov, NCT03248102. Registered 14 Aug 2017.

15.
Eur J Pediatr Surg ; 29(2): 150-152, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-29232722

RESUMO

INTRODUCTION: Repair of anorectal malformations (ARMs), primarily or with a reoperation, may be performed in certain circumstances without a diverting stoma. Postoperatively, the passage of bulky stool can cause wound dehiscence and anastomotic disruption. To avoid this, some surgeons keep patients NPO (nothing by mouth) for a prolonged period. Here, we report the results of a change to our routine from NPO for 7 days to clear fluids or breast milk. MATERIALS AND METHODS: After primary or redo ARM surgery, patients given clear liquids were compared to those who were kept strictly NPO. Age, indication for surgery, incision type, use of a peripherally inserted central catheter (PICC) line, and wound complications were recorded. RESULTS: There were 52 patients, including 15 primary and 37 redo cases. Group 1 comprised 11 female and 15 male patients. The mean age at surgery was 4.9 years (standard deviation [SD]: 2.3). There were 8 primary cases and 18 redo cases. Twelve (46.6%) received a PICC line. The average start of clear liquids was on day 5.3 (SD: 2.2) after examination of the wound, and the diet advanced as tolerated. The first stool passage was recorded on average on day 2.3 (SD: 1.3). Four minor wound complications and no major wound complications occurred.Group 2 comprised 14 females and 12 male patients. The mean age at surgery was 3.5 (SD: 2.4) years. There were 7 primary and 19 redo cases. One (3.8%) patient required a PICC line. A clear liquid diet was started within 24 hours after surgery. A regular diet was started on average on day 5.8 (SD: 1.3). The first stool passage was recorded on an average of day 1.6 (SD: 0.9). Three minor wound complications occurred; however, there was no significant difference between the two groups (SD: 0.71). One major wound complication occurred. However, there was no significant difference in major wound complications between the groups (SD: 0.33). CONCLUSION: No increase in wound problems was noted in children receiving clear liquids or breast milk compared with the strict NPO group, and PICC line use was reduced. We believe this change in practice simplifies postoperative care without increasing the risk of wound complications.


Assuntos
Malformações Anorretais/cirurgia , Nutrição Enteral/métodos , Cuidados Pós-Operatórios/métodos , Complicações Pós-Operatórias/prevenção & controle , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Masculino , Complicações Pós-Operatórias/epidemiologia , Reoperação , Estudos Retrospectivos , Estomas Cirúrgicos , Resultado do Tratamento
16.
Eur J Pediatr Surg ; 29(4): 378-383, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-29909603

RESUMO

INTRODUCTION: Previous research in children with Hirschsprung's disease (HD) and Down's syndrome (DS) has focused on colorectal outcomes. We set out to review urinary outcomes in this patient group. MATERIALS AND METHODS: The medical records of all patients aged five years and older with HD were reviewed, and patients and caregivers filled out the Vancouver Symptom Score at intake, which is designed and validated to diagnose dysfunctional elimination syndrome. RESULTS: A total of 104 patients with HD were included in this study. Of these, 16 (15%) patients had DS. There were no significant differences in the prevalence of enterocolitis or colorectal symptoms between patients with or without DS. Five of 88 (6%) patients without DS and 7 of 16 (44%) (p = 0.00001) with DS reported having urinary accidents. Patients with HD and DS scored higher on the Vancouver score (9 vs. 17.5; p = 0.007), indicating more severe urinary symptoms. Patients who also reported fecal accidents scored significantly higher on the Vancouver (12 vs. 9; n = 61; p = 0.016), indicating more problems. CONCLUSION: Patients with DS appear to be a unique subset of HD patients who have a higher prevalence of urinary symptoms after surgery. In the postoperative care of patients with HD and DS, a strong focus should be placed on postoperative urinary care in addition to their bowel care. This could significantly ease care and contribute to the quality of life of the parents and the patient.


Assuntos
Síndrome de Down/complicações , Doença de Hirschsprung/cirurgia , Complicações Pós-Operatórias/etiologia , Incontinência Urinária/etiologia , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Doença de Hirschsprung/complicações , Humanos , Masculino , Complicações Pós-Operatórias/epidemiologia , Fatores de Risco , Resultado do Tratamento , Incontinência Urinária/epidemiologia , Adulto Jovem
17.
J Pediatr Surg ; 54(8): 1590-1594, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31027906

RESUMO

INTRODUCTION: Cloacal malformations, a confluence of the urinary tract, vagina and rectum into a single common channel, has a broad and complex anatomic spectrum requiring an imaging tool for visualization, measurement, and surgical planning for the reconstruction of these structures. We evaluated the role of 3-D fluoroscopy for this purpose, as it offers a combination of spatial correlation with precise anatomic measurements. METHODS: We examined our imaging protocol for patients with a cloacal malformation and report our experience with rotational fluoroscopy and 3-D reconstruction in 16 consecutive patients referred for cloacal reconstruction. The length of the common channel (CC), the length of the urethra from the bladder neck to the common channel, and the height (and existence or absence) of a vagina or vaginas were determinants of the surgical procedures used for the repair. RESULTS: We performed 16 consecutive 3-D cloacagrams (age range 4 months to 9 years) using a new protocol (Figure 1) that provided the following data which helped with surgical planning: Gynecologic: 3 cases with a single vagina, 5 cases with a duplicated Mullerian system (3 of which were asymmetric) and 2 cases with high vaginas requiring vaginal replacement. Colorectal: Four had a high rectum requiring an abdominal approach, and 6 had a rectum reachable via a posterior sagittal approach. Urologic: Two ectopic ureters requiring reimplantation, 3 patients had vesicoureteral reflux (1 bilateral, 2 unilateral), 1 patient had no bladder, and 7 had a normal sized bladder. Common channel length and urethral length were demonstrated in all cases and used to decide between a total urogenital mobilization or a separation of vagina(s) from the common channel, urogenital separation. CONCLUSION: The 3-D cloacagram can help predict the surgical plan for urologic, gynecologic, and colorectal components of the cloacal repair. It can predict the CC length as well as the length of the urethra. It helps with predicting the need for vaginal replacement and whether an abdominal approach is needed for the rectum. Its effectiveness is based on the ability to adequately distend structures and see their distal most extent, an advantage over other modalities such as MRI. Added benefits (particularly from the 3D view) include a better spatial understanding of the defect and the diagnosis of concomitant urological abnormalities such as vesicoureteral reflux and ectopic ureters. Disadvantages to this procedure include the need for general anesthesia and a higher exposure to radiation. LEVEL OF EVIDENCE: 3.


Assuntos
Anormalidades Congênitas , Fluoroscopia , Imageamento Tridimensional , Reto , Sistema Urinário , Vagina , Criança , Pré-Escolar , Anormalidades Congênitas/diagnóstico por imagem , Anormalidades Congênitas/cirurgia , Feminino , Humanos , Lactente , Reto/anormalidades , Reto/diagnóstico por imagem , Sistema Urinário/anormalidades , Sistema Urinário/diagnóstico por imagem , Vagina/anormalidades , Vagina/diagnóstico por imagem
18.
J Pediatr Surg ; 54(10): 1988-1992, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-30879755

RESUMO

PURPOSE: A complication of the surgical management of anorectal malformations (ARMs) is a retained remnant of the original fistula (ROOF) formerly called a posterior urethral diverticulum (PUD). A ROOF may have multiple presentations or may be incidentally discovered during the work-up of ARM after previous surgery. We sought to define the entity and the surgical indications for excision of a ROOF when found. METHODS: We performed a retrospective cohort study of all male patients who presented to our center following previous repair for ARM at another institution, who came for evaluation of problems with urinary and/or fecal continence, from 2014 to 2017. Charts were reviewed for symptoms, original type of malformation, preoperative imaging, treatment, and postoperative follow-up. RESULTS: Of 180 referred male patients, 16 had a ROOF. 14 underwent surgical repair to address this and for other redo indications, and 2 did not require intervention. 13 patients had an additional reason for a redo such as anal mislocation or rectal prolapse. Indications for ROOF excision were urinary symptoms (e.g. UTI, dribbling, passage of mucous via urethra, stone formation), to make a smoother posterior urethra for intermittent catheterization, or for prophylactic reasons. Patients were repaired at an average age of 4.2 years, using a PSARP only approach with excision of the ROOF for all except one patient who needed a laparotomy due to abdominal extension of the ROOF. No patient needed a colostomy. The original ARM repairs of the patients were PSARP (9), laparoscopic assisted (4) and abdominoperineal pullthrough (3). Preoperative evaluation included pelvic MRI, VCUG, and cystoscopy. The ROOF was visualized on 14 of 16 MRIs, 10 of 14 VCUGs, and 14 of 15 cystoscopies. Urinary symptoms associated with a ROOF and ease of catheterization were improved in all repaired cases. CONCLUSION: Patients not doing well from a urinary or bowel standpoint post ARM pull-through need a complete evaluation which should include a check for a ROOF. Both modalities MRI and cystoscopy are needed as a ROOF can be missed on either alone. A VCUG was not reliable in identifying a ROOF. Excision is needed in patients to improve urinary symptoms associated with these lesions and to minimize the small but theoretical oncologic risk present in a ROOF. LEVEL OF EVIDENCE: Level III.


Assuntos
Malformações Anorretais/cirurgia , Divertículo/diagnóstico , Fístula/diagnóstico , Laparoscopia/efeitos adversos , Complicações Pós-Operatórias/diagnóstico , Doenças Uretrais/diagnóstico , Malformações Anorretais/fisiopatologia , Pré-Escolar , Cistoscopia , Divertículo/etiologia , Divertículo/cirurgia , Fístula/etiologia , Fístula/cirurgia , Humanos , Lactente , Imageamento por Ressonância Magnética , Masculino , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/cirurgia , Reoperação , Estudos Retrospectivos , Doenças Uretrais/etiologia , Doenças Uretrais/cirurgia
19.
Surv Ophthalmol ; 63(3): 406-436, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29100897

RESUMO

The association between socioeconomic position and morbidity and mortality has long been recognized. We evaluate the evidence for an association between multiple aspects of deprivation and ocular health in a global context. This is a systematic review of studies that evaluated deprivation in the adult population in the context of the major acquired causes of visual loss such as cataract, diabetic eye disease, glaucoma, age-related macular degeneration, and ocular trauma. The search strategy identified relevant studies reported between 1946 and August 2016, with randomized control trials, case-control, cohort, and cross-sectional study designs being selected for inclusion. The studies identified in this review from across the world demonstrate the extent to which the common themes such as low educational attainment and low income may be associated with increased incidence of various sight-threatening conditions and may adversely affect access to specialist assessment and delivery of treatment. Health inequality may always persist, but an increased recognition of the importance of the various impacts of deprivation may empower policy makers to target limited resources to the most vulnerable groups in order to deliver the greatest benefit.


Assuntos
Oftalmopatias/etiologia , Disparidades nos Níveis de Saúde , Transtornos da Visão/etiologia , Escolaridade , Humanos , Renda , Fatores Socioeconômicos
20.
Front Surg ; 5: 67, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30483511

RESUMO

Introduction: Operative surgical videos are a popular educational resource, not commonly a part of a peer-reviewed article. We wanted to evaluate the impact of either reading a peer-reviewed manuscript or watching an operative video on a surgeon's confidence in performing a complex case. Methods: Pediatric surgeons and fellows were asked to complete an initial questionnaire to assess their confidence (formulated as a score) in the diagnosis and operative repair of anal stenosis and rectal atresia. Results: Of 101 pediatric surgeons and fellows, 52 (51%) were randomized into a "manuscript" group and 49 (49%) into a "video" group. The mean confidence before the intervention was the same in the two groups (6.4 vs. 6.6). Attending surgeons started with more confidence than trainees (7.1 vs. 5.3, p < 0.001). In the manuscript group, the average confidence increased to 7.7 (p = 0.005), and in the video group the average confidence increased to 7.9 (p = 0.001) globally. Trainees in the video group significantly improved their confidence to a score of 6.6 (p = 0.035), as did attending surgeons to 8.5 (p = 0.01). In the manuscript group, only attendings significantly improved their confidence by 1.5-8.3 (p < 0.001), whereas trainees did not with a difference of 1.3 (p = 0.194). When considering experience level, physicians who reported never having performed this surgery improved only by reading the manuscript (3.9-6.2) (p = 0.004), not by watching the video (5.4-6.6) (p = 0.106). Surgeons with experience doing this operation (>5 times) did not improve their confidence by reading the manuscript (p = 0.10), nor by watching the video (p = 0.112). Conclusion: Reviewing either a detailed manuscript or operative video on the surgical management of rectal atresia and anal stenosis demonstrated a significant increase in self-reported confidence. Trainees benefitted the most from operative videos, whereas experienced surgeons did not improve their confidence by reading the manuscript nor watching the video.

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