Minimally invasive surgery for pilonidal disease: Outcomes of the Gips technique-A systematic review and meta-analysis.

BACKGROUND: Pilonidal disease is a common inflammatory condition that significantly impacts quality of life. Currently, there is a tendency to favor minimally invasive procedures. The present review aims to summarize the evidence and assess the outcomes of the Gips procedure. METHODS: A systematic review was conducted on MEDLINE/Pubmed, Scopus, Web of Science, and Cochrane Library databases until December 2022. Eligible studies included patients with pilonidal disease submitted to the Gips procedure, reporting at least 1 of the following outcomes: wound complications, wound healing time, time to resume daily activities, and recurrence (International Prospective Register of Systematic Reviews protocol: CRD42023389269). The National Institutes of Health assessment tool was used for risk of bias evaluation. Meta-analysis was performed using OpenMeta[Analyst] and R software, and a subgroup analysis was performed when applicable. RESULTS: Thirteen observational studies with a total of 4,286 patients submitted to Gips were included. The pooled wound complications rate was 7.8% (95% confidence interval: 5.1-10.6), the median time to resume daily activities was 1 day (95% confidence interval: 1-2), and the mean wound healing time was 4.7 weeks (95% confidence interval: 3.0-6.4). Subgroup analysis showed that pooled recurrence rate was 6.5% (95% confidence interval: 5.2-7.8) up to 2 years and 38.9% (95% confidence interval: 27.1-50.7) after more than 2 years of surgery. Most results showed substantial heterogeneity across studies. CONCLUSION: Despite apparent favorable outcomes of the Gips procedure, there is a high recurrence rate over time. Because included studies had an observational nature and unstandardized methodologies, comparative randomized controlled trials with longer follow-ups are needed for high-level evidence regarding these outcomes.

Short and Medium-term Outcomes of Omphalocele and Gastroschisis: A Survey from a Tertiary Center.

OBJECTIVE: To characterize and compare the outcomes of omphalocele and gastroschisis from birth to 2 years of follow-up in a recent cohort at a tertiary center. METHODS: This is a retrospective clinical record review of all patients with gastroschisis and omphalocele admitted to the Neonatal Intensive Care Unit between January 2009 and December 2019. RESULTS: There were 38 patients, 13 of whom had omphalocele, and 25 of whom had gastroschisis. Associated anomalies were present in 6 patients (46.2%) with omphalocele and in 10 (41.7%) patients with gastroschisis. Compared with patients with omphalocele, those with gastroschisis had younger mothers (24.7 versus 29.6 years; p = 0.033), were born earlier (36 versus 37 weeks, p = 0.006), had lower birth weight (2365 ± 430.4 versus 2944.2 ± 571.9 g; p = 0.001), and had a longer hospital stay (24 versus 9 days, p = 0.001). The neonatal survival rate was 92.3% for omphalocele and 91.7% for gastroschisis. Thirty-four patients were followed-up over a median of 24 months; 13 patients with gastroschisis (59.1%) and 8 patients with omphalocele (66.7%) had at least one adverse event, mainly umbilical hernia (27.3% vs 41.7%), intestinal obstruction (31.8% vs 8.3%), or additional surgical interventions (27.3% vs 33.3%). CONCLUSION: Despite the high proportion of prematurity, low birth weight, and protracted recovery, gastroschisis and omphalocele (without chromosomal abnormalities) may achieve very high survival rates; on the other hand, complications may develop in the first years of life. Thus, a very positive perspective in terms of survival should be transmitted to future parents, but they should also be informed that substantial morbidity may occur in the medium term.

OBJETIVO: Caracterizar e comparar os desfechos do onfalocelo e gastrosquisis desde o nascimento até aos 2 anos de seguimento numa coorte recente de um centro terciário. MéTODOS: Este é um estudo retrospectivo em que foi feita uma revisão dos registos clínicos de todos os pacientes com gastrosquisis e onfalocelo que foram internados na unidade de cuidados intensivos neonatais, entre janeiro de 2009 e dezembro de 2019. RESULTADOS: Identificamos 38 pacientes, 13 dos quais tinham onfalocelo e 25 dos quais tinham gastrosquisis. Anomalias associadas estavam presentes em 6 pacientes (46.2%) com onfalocelo e 10 (41.7%) com gastrosquisis. Comparativamente com os pacientes com onfalocelo, os pacientes com gastrosquisis tinham mães mais jovens (24.7 versus 29.6 anos; p = 0.033), nasceram mais precocemente (36 versus 37 semanas, p = 0.006), com menor peso ao nascimento (2,365 ± 430.4 versus 2,944.2 ± 571.9 g; p = 0.001), e o internamento teve uma duração mais longa (24 versus 9 dias, p = 0.001). A taxa de sobrevivência neonatal foi de 92.3% para o onfalocelo e 91.7% para a gastrosquisis. Trinta e quatro pacientes foram seguidos durante um tempo mediano de seguimento de 24 meses: 13 com gastrosquisis (59.1%) e 8 com onfalocelo (66.7%) apresentaram pelo menos um evento adverso, sobretudo hérnia umbilical (27.3% vs 41.7%), obstrução intestinal (31.8% vs 8.3%) ou intervenções cirúrgicas adicionais (27.3% vs 33.3%). CONCLUSãO: Apesar da alta proporção de prematuridade, de baixo peso e de recuperação lenta, os gastrosquisis, assim como os onfalocelos (sem anomalias cromossómicas), podem ter uma taxa de sobrevivência muito alta; por outro lado, nos primeiros anos de vida, podem surgir complicações não desprezíveis. Assim, aos futuros pais pode ser transmitida uma perspectiva muito positiva em termos de sobrevivência, embora eles também devam ser informados de que pode ocorrer morbidade substancial no médio prazo.

Short and Medium-term Outcomes of Omphalocele and Gastroschisis: A Survey from a Tertiary Center / Resultados a curto e médio prazo do onfalocelo e gastrosquisis: uma investigação de um centro terciário

Abstract Objective To characterize and compare the outcomes of omphalocele and gastroschisis from birth to 2 years of follow-up in a recent cohort at a tertiary center. Methods This is a retrospective clinical record review of all patients with gastroschisis and omphalocele admitted to the Neonatal Intensive Care Unit between January 2009 and December 2019. Results There were 38 patients, 13 of whom had omphalocele, and 25 of whom had gastroschisis. Associated anomalies were present in 6 patients (46.2%) with omphalocele and in 10 (41.7%) patients with gastroschisis. Compared with patients with omphalocele, those with gastroschisis had younger mothers (24.7 versus 29.6 years; p=0.033), were born earlier (36 versus 37 weeks, p=0.006), had lower birth weight (2365±430.4 versus 2944.2±571.9 g; p=0.001), and had a longer hospital stay (24 versus 9 days, p=0.001). The neonatal survival rate was 92.3% for omphalocele and 91.7% for gastroschisis. Thirty-four patients were followed-up over a median of 24 months; 13 patients with gastroschisis (59.1%) and 8 patients with omphalocele (66.7%) had at least one adverse event, mainly umbilical hernia (27.3% vs 41.7%), intestinal obstruction (31.8% vs 8.3%), or additional surgical interventions (27.3% vs 33.3%). Conclusion Despite the high proportion of prematurity, low birth weight, and protracted recovery, gastroschisis and omphalocele (without chromosomal abnormalities) may achieve very high survival rates; on the other hand, complications may develop in the first years of life. Thus, a very positive perspective in terms of survival should be transmitted to future parents, but they should also be informed that substantial morbidity may occur in the medium term.

Resumo Objetivo Caracterizar e comparar os desfechos do onfalocelo e gastrosquisis desde o nascimento até aos 2 anos de seguimento numa coorte recente de um centro terciário. Métodos Este é um estudo retrospectivo em que foi feita uma revisão dos registos clínicos de todos os pacientes com gastrosquisis e onfalocelo que foram internados na unidade de cuidados intensivos neonatais, entre janeiro de 2009 e dezembro de 2019. Resultados Identificamos 38 pacientes, 13 dos quais tinham onfalocelo e 25 dos quais tinham gastrosquisis. Anomalias associadas estavam presentes em 6 pacientes (46.2%) com onfalocelo e 10 (41.7%) com gastrosquisis. Comparativamente com os pacientes com onfalocelo, os pacientes com gastrosquisis tinham mães mais jovens (24.7 versus 29.6 anos; p=0.033), nasceram mais precocemente (36 versus 37 semanas, p=0.006), com menor peso ao nascimento (2,365±430.4 versus 2,944.2±571.9 g; p=0.001), e o internamento teve uma duração mais longa (24 versus 9 dias, p=0.001). A taxa de sobrevivência neonatal foi de 92.3% para o onfalocelo e 91.7% para a gastrosquisis. Trinta e quatro pacientes foram seguidos durante umtempo mediano de seguimento de 24meses: 13 com gastrosquisis (59.1%) e 8 com onfalocelo (66.7%) apresentaram pelo menos um evento adverso, sobretudo hérnia umbilical (27.3% vs 41.7%), obstrução intestinal (31.8% vs 8.3%) ou intervenções cirúrgicas adicionais (27.3% vs 33.3%). Conclusão Apesar da alta proporção de prematuridade, de baixo peso e de recuperação lenta, os gastrosquisis, assim como os onfalocelos (sem anomalias cromossómicas), podem ter uma taxa de sobrevivência muito alta; por outro lado, nos primeiros anos de vida, podem surgir complicações não desprezíveis. Assim, aos futuros pais pode ser transmitida uma perspectiva muito positiva em termos de sobrevivência, embora eles também devam ser informados de que pode ocorrermorbidade substancial no médio prazo.

Simultaneous Testicular and Appendix Testis Torsion: A Novel Clinically Relevant Association.

ABSTRACT: Torsion of the appendix testis (TAT) and testicular torsion (TT) are the most common causes of acute scrotum in the pediatric population. They usually present as separate conditions and have distinct managements. We report a unique case of a 16-year-old boy with concomitant TAT and TT. The role of TAT as a trigger factor to TT is addressed, and its clinical repercussion on the management of acute scrotum is discussed.

Herlyn-Werner-Wunderlich Syndrome: Report of a Prenatally Recognised Case and Review of the Literature.

Herlyn-Werner-Wunderlich syndrome, defined by the triad of uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis, is a rare Mullerian malformation, usually diagnosed after menarche, when symptoms related to hematocolpos arise. Rarely, this malformation is diagnosed in the neonatal period, normally following prenatal diagnosis of renal agenesis. Herein, a case recognized on prenatal imagiology that underwent surgery on the fourth day of life is reported. The records of prepubertal cases were also collected, addressing the clinical and imagiological features. In the presence of a solitary kidney and/or a pelvic mass on prenatal ultrasound, Herlyn-Werner-Wunderlich syndrome should be considered, enabling neonatal treatment.

Measuring otoplasty outcome: Expanding the validity to caregivers' perspective and to Portuguese-speaking children.

OBJECTIVE: The GBI (Glasgow Benefit Inventory) and the GCBI (Glasgow Children's Benefit Inventory) are used to evaluate patient-reported Health-Related Quality of Life (HRQoL) following otoplasty for prominent ears. The aim of this study was to validate them through patients' and caregivers' perspectives and in Portuguese language versions. STUDY DESIGN: After translation to Portuguese, the GBI and the GCBI were applied to patients (n = 255) who underwent otoplasty throughout a five-year span and to their caregivers, respectively. A second set of questionnaires (retest) was applied to assess reliability. Caregivers were subsequently contacted to evaluate aesthetic satisfaction, recommendation to others, postoperative complications and main motivation for surgery. SETTING: Paediatric Surgery Department of a tertiary hospital in Porto, Portugal. PARTICIPANTS: 134 patients (aged 10.9 ± 3.6 years, 61.2% males) and their caregivers (52.5% responders). MAIN OUTCOME MEASURES: Impact on HRQoL using the Portuguese GBI and GCBI (global score, -100 to 100). Validity assessed by principal component factor analysis, Cronbach's α and Intraclass Correlation Coefficient (ICC). RESULTS: The GBI was 30.1 ± 25.7 and the GCBI was 22.6 ± 23.0. For the GBI, three subscales were identified, explaining 63.3% of variance. For the GCBI, two subscales were identified, accounting for 66.8% of variance. Both the GBI and the GCBI showed a high internal consistency (α = 0.92 and 0.94, respectively). 79% were satisfied and 92% recommended the surgery. Statistically significant higher scores were found in satisfied patients, in those who underwent surgery due to teasing, and in females. CONCLUSIONS: The Portuguese GBI and GCBI proved to be valid and reliable to evaluate HRQoL following otoplasty in Portuguese-speaking children. The outcome was very favourable; almost all patients would recommend the surgery.

Biliary Atresia - Clinical Series.

INTRODUCTION: Biliary atresia is the main cause of death by hepatic failure and the main indication for liver transplant in children. This study aims to analyze the population with this diagnosis, treated between 2000 and 2015 at Hospital de São João. MATERIAL AND METHODS: Descriptive, observational, and retrospective study, including the patients with biliary atresia, diagnosed and treated between January 1, 2000 and December 31, 2015. We analyzed epidemiologic, clinical, biochemical, and image data, as well as registered complications and present status. RESULTS: Eighteen patients were evaluated. The median age at time of Kasai portoenterostomy was 63 days of life, with better prognosis for those patients who had surgery before 72 days. The procedure was successful in 2/3 of cases. There was a significant association between recurrent cholangitis and survival. Five cases of transplant and 2 deaths, one of them after transplant, were registered. Survival with native liver was 77.8%, 72.2%, and 64.2% at 1, 5, and 10 years of follow-up, respectively. DISCUSSION: The presentation and evolution of patients was similar to other studies. However, there was a higher surgical success and survival rates at 5 and 10 years of follow-up than most series. Age at surgery and recurrence of cholangitis were the only factors significantly related to prognosis. CONCLUSION: In spite of the low number of patients (1,125/year), our results were similar to those of other reference centers.

INTRODUÇÃO: A atresia das vias biliares representa a principal causa de morte por insuficiência hepática e a principal indicação para transplante hepático em idade pediátrica. O objetivo deste estudo é analisar a população com este diagnóstico, tratada entre 2000 e 2015 no Hospital de São João. MATERIAL E MÉTODOS: Estudo descritivo, observacional e retrospetivo, incluindo-se doentes com atresia das vias biliares diagnosticados e tratados entre 1 de Janeiro de 2000 e 31 de Dezembro de 2015. Foram analisados dados epidemiológicos, clínicos, bioquímicos, imagiológicos, idade à data da cirurgia, complicações ocorridas e o estado atual. RESULTADOS: Foram avaliados 18 doentes. A idade mediana à data da portoenterostomia de Kasai foi de 63 dias de vida, verificando-se um melhor prognóstico nos pacientes operados antes dos 72 dias. Foi constatada drenagem biliar adequada em 2/3 dos casos aos 6 meses. Verificou-se uma associação significativa entre a ocorrência de episódios recorrentes de colangite e a sobrevida. Registaram-se 5 transplantes e 2 óbitos, um dos quais pós-transplante. A sobrevida com fígado nativo foi de 77.8%, 72.2% e 64.2% ao 1º, 5º e 10º anos de seguimento, respetivamente. DISCUSSÃO: A apresentação e evolução dos pacientes coincidiu, de um modo geral, com o descrito na literatura. No entanto, constatou-se uma taxa de sucesso operatório e de sobrevida com fígado nativo aos 5 e 10 anos superiores à maioria das séries. A idade à data de cirurgia e a recorrência dos episódios de colangite foram os únicos fatores que se mostraram significativamente relacionados com o prognóstico. CONCLUSÃO: Apesar do número reduzido de doentes (1,125/ano), foram registados resultados comparáveis a outros centros de referência.

Trans-umbilical laparoscopic-assisted appendectomy in the pediatric population: comparing single-incision and 2-trocar techniques.

INTRODUCTION: Trans-umbilical laparoscopic-assisted appendectomy (TULAA) combines advantages of open surgery with benefits of laparoscopy and can be performed either by single-incision or with two trocars; the present study aims to compare outcomes and cost of these techniques in a pediatric serie. MATERIAL AND METHODS: Patients undergoing TULAA between July 2013 and June 2014 were retrospectively reviewed. There were two groups: A (single-incision) - one umbilical trocar for a telescope with inbuilt working channel; and B (2-trocar) - one umbilical trocar for a 'standard' telescope plus a supra-pubic trocar for instruments. RESULTS: One-hundred and eighty-nine patients (112 males, age: 12[2-17] years) underwent TULAA; 32(17%) presented with perforated appendicitis. Group A encompassed 157 and group B 32 patients; age, gender, proportion of perforated appendicitis, and follow-up were similar. Operative time was 46 [21-145] min in group A, and 54.5[30-111] in group B (A vs B, p = 0.087). Length of hospital stay was 2 [1;22] days in group B, and 2 [0;24] in group A (A vs B, p = 0.136). The prevalence of post-operative complications was similar: 11 (7%) cases in group A and 2 (6.3%) intra-abdominal abscesses in group B. Using disposable trocars, the more expensive starting point of single-incision technique may be reversed after 27 procedures. CONCLUSION: Single-incision and 2-trocar TULAA are effective techniques with low complication rates and similar outcomes. Despite being technically more demanding, single incision may be advantageous because there are no visible scars, and potentially cheaper in hospitals where disposable trocars are used.

BOTOX-A injection of salivary glands for drooling.

BACKGROUND AND PURPOSE: Drooling is a challenging entity to manage. Botulinum toxin A (BOTOX-A) infiltration of salivary glands is a promising alternative to surgical treatment. This study aims to assess the outcome of BOTOX-A salivary glands infiltration in children with drooling. METHODS: Patients treated between January 2012 and March 2015 were enrolled. BOTOX-A was injected in the parotid and submandibular glands under ultrasound control and general inhalational anesthesia. The outcome was evaluated through the DSFS: Drooling Severity (1-best to 5-worst) and Frequency (1 to 4) Scale, that was applied before treatment, and 1-, 3-, and 6-month after injection. The inclusion criteria were a DSS ≥4 and/or DFS ≥3. Statistical significance was set at 5%. RESULTS: There were 17 patients aged 12.1±5.1 [4-19]years, all of them with neurologic impairment. After the first injection, 13 (76.5%) patients had reduction of the severity (S) and 12 (70.6%) of the frequency (F) scale; in 6 (35.5%) patients drooling resolved completely. Pre-treatment S+F score was 8.59±0.71 [7-9]; it decreased significantly to 4.65±2.32 (p=0.001) at 1-month post-injection evaluation. At 3-month and 6-month the scores were also significantly lower than the pre-treatment one (4.00±1.96, p=0.002; 5.36±2.20, p=0.005; respectively), but there was a significant increase between the 3-month and 6-month evaluations (p=0.01). With a follow-up of 20.1±9.2 [4-38] months, 4 out of the 13 successful injections needed a second one after 7.5±3.1 [3-10] months. The patient with the longest time not requiring re-injection had 28months of follow-up. One (6%) patient presented mild dysphagia that regressed spontaneously. All but two (88%) parents/caregivers would repeat the treatment. CONCLUSIONS: BOTOX-A seems to be an effective minimal invasive treatment for drooling with few complications. After 6months the need for re-injection becomes substantial but it may not be necessary for several months. Further studies are needed to establish the most effective dosage and frequency of injections. LEVEL OF EVIDENCE: IV.

Minimal access surgery in the management of pediatric urolithiasis.

BACKGROUND: In contrast to adult patients, a relatively large number of open surgical procedures are still needed in the treatment of urolithiasis in children. Since almost all open surgical techniques may be reproduced by minimal access surgery (MAS), there is a rationale to apply the latter in the management of pediatric urolithiasis. Our study aimed to assess the feasibility and outcome of MAS in the treatment of pediatric urinary calculi. METHODS: The charts of patients with urolithiasis submitted to MAS between 1994 and 2007 were retrospectively reviewed. The inclusion criteria were contraindication for and failure of lithotripsy or endourology techniques. Demographic data, lithiasis characterization (location, dimension, composition), predisposing factors (anatomic or metabolic) and surgical approach (technique and outcome) were evaluated. RESULTS: Fifteen consecutive patients (eight girls, seven boys) with a median age of 108 months (range: 10-297 months) were elected for MAS. Eleven (73%) children had associated urogenital malformations and three (20%) presented metabolic abnormalities. A total of 17 procedures were performed laparoscopically: three nephrolithotomy (one transperitoneal, two by retroperitoneoscopy), four pyelolithotomies (retro), three ureterolithotomy (trans) and seven cystolithotomies (suprapubic approach). Five patients underwent concomitant correction of urological anomalies (three calyceal diverticula, one obstructive megaureter, one ureteropelvic junction obstruction). Complete removal of calculi was accomplished in 14 (82%) procedures. There were two perioperative complications (one intraperitoneal vesical perforation and one perivesical urinoma). At a median follow up of 4 years (range, 1 month to 11 years), four patients have developed recurrence. CONCLUSIONS: MAS is an effective and safe approach for urolithiasis in children who are not candidates for minimally invasive modalities.

[Secondary Alveolar Bone Grafting in Orofacial Cleft: A Survey of a Portuguese Tertiary Hospital]. / Enxerto Ãsseo na Fenda Lábio-Palatina: Experiência de um Hospital de Referência Português.

INTRODUCTION: Orofacial clefts are the most frequent craniofacial birth defects. In the presence of bone defect, the secondary alveolar bone grafting is the correction method most commonly accepted by clinicians. This study evaluates the result of this technique in a tertiary hospital. MATERIAL AND METHODS: Analysis of the secondary alveolar bone grafting performed between 2007 and 2014, with inclusion of cases in which the iliac crest was the donor site for the graft and the clinical and imaging information was complete. The success of the intervention was assessed radiographically using the Bergland scale (type I-IV) and correlated to variables associated with pathology and/or surgical correction. RESULTS: Of the 32 secondary alveolar bone grafting performed, 29 met the inclusion criteria: 13 cases (44.8%) corresponded to complete unilateral pre-foramen clefts; four (13.8%) to complete bilateral pre-foramen clefts; eight (27.6%) to transforamen unilateral clefts and four (13.8%) to bilateral transforamen clefts. According to the scale of Bergland (applied with a mean follow-up of 8 ± 5 months) six were classified as type I, 15 as type II, five as type III and three as type IV. No association was found between the effectiveness of the surgery and the type of orofacial clefts, the presence of the incisor and the canine eruption phase. Five patients were re-operated (three of type II and two of type III at baseline). DISCUSSION: In this series, the secondary alveolar bone grafting was effective in most patients (72% type I and II) regardless of the type of orofacial clefts. The relatively high proportion of failures (10.3%) and subsequent need for re-intervention (17%) justify long-term follow up and the continuation of this study. CONCLUSION: It is important to highlight the multidisciplinary involvement in order to identify previously the ideal timing for each intervention and to optimize results.

Introdução: A fenda lábio-palatina é a malformação congénita craniofacial mais frequente. Na presença de defeito ósseo, a técnica de enxerto ósseo alveolar secundário é o método de correção mais consensual entre os autores. Neste estudo avalia-se o resultado da aplicação desta técnica num hospital terciário. Material e Métodos: Análise dos enxertos ósseos alveolares secundários realizados entre 2007 e 2014, sendo incluídos os casos em que a crista ilíaca foi a região dadora e em que a informação clínica e imagiológica estava completa. A eficácia da intervenção foi avaliada radiologicamente com recurso à escala de Bergland (tipo I-IV), e correlacionada com variáveis associadas à patologia e/ou correção cirúrgica. Resultados: Dos 32 enxertos ósseos alveolares secundários realizados, 29 cumpriam os critérios de inclusão: 13 casos (44,8%) correspondiam a fendas pré-forâmen unilaterais completas; quatro (13,8%) a fendas pré-forâmen bilaterais completas; oito (27,6%) a fendas transforâmen unilaterais e quatro (13,8%) a fendas transforâmen bilaterais. Pela escala de Bergland (aplicada com um seguimento médio de 8 ± 5 meses), seis eram do tipo I, 15 do tipo II, cinco de tipo III e três do tipo IV. Não foi encontrada associação entre a eficácia da intervenção cirúrgica e o tipo de fenda lábio-palatina, presença do incisivo e fase de erupção do canino. Cinco doentes foram submetidos a novo enxerto ósseo alveolar (três tipo II e dois tipo III na avaliação inicial). Discussão: Na presente série, o enxerto ósseo alveolar foi eficaz na maioria dos doentes (72%, tipo I e II), independentemente do tipo de fenda lábio-palatina. A proporção de falências (10,3%) e a necessidade ulterior de reintervenção (17%) foram relativamente altas justificando o seguimento a longo-prazo e a continuação deste estudo. Conclusão: Importa realçar o envolvimento multidisciplinar para identificação atempada do momento ideal para intervenção e otimi-zação dos resultados.

Complete Ureteral Duplication: Outcome of Different Surgical Approaches.

INTRODUCTION: The surgical management of complete ureteral duplication anomalies is not consensual. OBJECTIVE: To characterize the pediatric population who underwent surgery for complete ureteral duplication and assess the outcomes of different approaches. MATERIAL AND METHODS: Clinical records from patients treated between January 2008 and June 2014 were retrospectively reviewed. Epidemiology, diagnosis, clinical manifestations and surgical procedures were collected and analysed. Ureteral units were divided into two groups: A, with ureterocele; and B, without it. RESULTS: Forty-one ureteral units from 32 patients with complete duplication underwent surgery. In group A (n = 18), the selected primary procedure was: ureterocele punction (12); ureter reimplantation (3); pyelopyelostomy (2); heminephrectomy (1). A reintervention was required in 3 of the 12 units submitted to punction: heminephrectomy (1), ureteroureterostomy (1), and ureteric reimplantation (1). In group B (n = 23), STING was performed in 10 units, ureteric reimplantation in 3, pyelopyelostomy in 3, ureteroureterostomy in 1, and heminephrectomy in 6; two cases required reintervention. DISCUSSION: A conservative primary approach was favoured in cases with ureterocele and/or reflux in hemisystems worth preserving (53.7%); it was effective per se in 75% (n = 9/12) units in group A and 80% (n = 8/10) in group B. An ablative primary procedure was adopted in 17% (n = 7/41) cases, 5.6% of group A (n = 1/18) and 26.1% of group B (n = 6/23). CONCLUSIONS: A conservative approach is effective as a primary and isolated procedure in the majority of cases with ureterocele or vesicoureteral reflux. Further studies are needed to establish the advantages over primary invasive or ablative approaches.

Introdução: O tratamento cirúrgico das duplicações ureterais completas não é consensual. Objetivos: Caraterizar a população pediátrica submetida a cirurgia para tratamento de duplicações ureterais completas e avaliar resultados de diferentes abordagens. Material e Métodos: Processos clínicos de doentes tratados entre janeiro de 2008 e junho de 2014 foram retrospetivamente revistos. Dados acerca de epidemiologia, diagnóstico, manifestações clínicas e procedimentos cirúrgicos foram recolhidos. As unidades ureterais foram divididas em dois grupos: A, com ureterocelo; e B, sem ureterocelo. Resultados: Quarenta e uma unidades ureterais de 32 doentes com duplicação completa foram intervencionados. No grupo A (n = 18), o procedimento primário selecionado foi: punção de ureterocelo (12); reimplantação de ureter (3); pielopielostomia (2) e heminefrectomia (1). Foi necessário reintervir em três dos 12 casos submetidos a punção: heminefrectomia (1), ureteroureterostomia (1) e reimplantação (1). No grupo B (n = 23), foi efetuado STING em 10 unidades, reimplantação ureteral em três, pielopielostomia em três, ureteroureterostomia em um, e heminefrectomia em seis; dois casos necessitaram de reintervenção. Discussão: Foi favorecida uma abordagem primária conservadora para tratamento de ureterocelo ou refluxo em hemissistemas a preservar (53,7%; n = 22/41), tendo sido eficaz per se em 75% (n = 9/12) unidades do grupo A e 80% (n = 8/10) do grupo B. Uma abordagem ablativa primária foi adotada em 17% (n = 7/14) casos, 5,6% do grupo A (n = 1/18) e 26,1% do grupo B (n = 6/23). Conclusão: Uma abordagem conservadora é eficaz como procedimento primário isolado na maioria dos casos com ureterocelo ou refluxo. Mais estudos são necessários para estabelecer as suas vantagens sobre abordagens primárias invasivas ou ablativas.

Pectus Carinatum Evaluation Questionnaire (PCEQ): a novel tool to improve the follow-up in patients treated with brace compression.

OBJECTIVES: A questionnaire (Pectus Carinatum Evaluation Questionnaire, PCEQ) was developed to be applied in follow-up of patients with Pectus Carinatum (PC). After validation of the PCEQ, we aimed to quantify the compliance to brace compression and to assess factors that could influence this treatment in patients with PC. METHODS: From July 2008 to July 2014, 56 patients with PC were treated with the Calgary Protocol of compressive bracing at Paediatric Surgery Department of Hospital São João. Forty patients (71%) completed the questionnaire. The PCEQ was divided into four sections: (i) compliance; (ii) symptoms; (iii) social influence; (iv) activities. For the validation process of the PCEQ, principal components analysis (PCA), orthogonal varimax or oblimin rotation and Cronbach's α coefficient were used. To evaluate the association between compliance and other sections of the questionnaire, we estimated the Pearson's correlation between compliance factor scores ('Compliance Days' and 'Compliance Hours') and the final score of each new questionnaire component identified by PCA ('Chest Pain', 'Dyspnoea', 'Back Pain', 'Parents' Influence', 'Friends' Influence', 'Activities', 'Time To Compliance'). For the sections 'Symptoms', 'Social Influence' and 'Activities', we estimated final scores as the sum of the questions that constitute each component. For the section 'Compliance', the factor scores were estimated by the regression method. RESULTS: After PCA analysis, the PCEQ found nine different components with high reliability. When analysing the compliance of our study group, the final score for 'Activities' revealed a significant correlation with the factor score for 'Compliance Hours' (r = 0.382, P = 0.015). The final score for 'Time To Compliance' showed a significant correlation with both factor scores for 'Compliance Hours' (r = -0.765, P < 0.001) and 'Compliance Days' (r = -0.345, P < 0.029). CONCLUSIONS: The PCEQ seems to be an important tool to follow up patients with PC treated by brace compression. Practical steps, such as developing a tight schedule in the early follow-up period or applying the PCEQ in first visits after initiating brace therapy, can be taken in order to increase compliance with brace therapy and improve the quality of life.