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1.
Pediatr Surg Int ; 39(1): 135, 2023 Feb 20.
Artículo en Inglés | MEDLINE | ID: mdl-36805329

RESUMEN

OBJECTIVE: Minimal Access Surgery (MAS) for Congenital Diaphragmatic Hernia (CDH) repair is well described, yet only a minority of surgeons report this as their preferred operative approach. Some surgeons find it particularly difficult to repair the defect using MAS and convert to laparotomy when a patch is required. We present in this study our institutional experience in using an easy and relatively cheap methodology to anchor the patch around the ribs using Endo Close™. This device has an application in MAS for tissue approximation using percutaneous suturing. METHODS AND TECHNIQUE: We retrospectively reviewed our database for patients undergoing MAS repair of CDH between 2009 and 2021. Outcome measures included length of surgery and recurrence rates after patch repair. Endo Close™ was used in all patients who required patch repair. We declare no conflict of interest and to not having received any funding from Medtronic (UK). The technique is as follows: (1) The edges of the diaphragm are delineated by dissection. When primary suture repair of the diaphragmatic hernia was unfeasible without tension, a patch was used. (2) The patch is anchored in place by two corner stitches at the medial and lateral borders. (3) The posterior border of the patch is fixed to the diaphragmatic edge by running or interrupted stitches. (4) For securing the anterior border, a non-absorbable suture is passed through the anterior chest wall and the patch border is taken with intracorporeal instruments. (5) Without making another stab incision, the Endo Close™ is tunnelled subcutaneously through the anterior chest wall. (6) The suture end is pulled through the Endo Close™ and the knot is tied around the rib. This procedure can be performed as many times as required to secure the patch. RESULTS: 58 patients underwent MAS surgery for repair of CDH between 2009 and 2021. 48 (82%) presented with a left defect. 34 (58%) had a patch repair. The length of patch repair surgery for CDH ranged from 100-343 min (median 197). There was only one patient (3%) in the patch repair cohort that had a recurrent hernia, diagnosed 12 months after the initial surgery. CONCLUSIONS: In our experience, MAS repair of CDH is feasible. We adopted a low threshold in using a patch to achieve a tension-free repair. We believe that the Endo Close™ is a cheap and safe method to help securing the patch around the ribs.


Asunto(s)
Hernias Diafragmáticas Congénitas , Humanos , Hernias Diafragmáticas Congénitas/cirugía , Estudios Retrospectivos , Procedimientos Quirúrgicos Mínimamente Invasivos , Costillas/cirugía , Procedimientos Neuroquirúrgicos
2.
Pediatr Surg Int ; 39(1): 82, 2023 Jan 16.
Artículo en Inglés | MEDLINE | ID: mdl-36645513

RESUMEN

PURPOSE: ECMO is an escalation treatment for hypoxic respiratory failure in patients with CDH. Open repair has been advocated after ECMO indicating that physiological changes associated to thoracoscopic repair were not well tolerated. METHODS: We have performed a retrospective review of all patients who underwent ECMO prior CDH repair over a 7 year period (2015-2021). Outcome measures were intra-operative Ph, PCO2, PO2 and FiO2 at 30 min, 1 h 30 min, and 2 h 30 min of surgery, operative time and recurrence rate. Data are shown in median (range). RESULTS: Eleven patients required ECMO prior CDH repair. Six of eleven (55%) were done thoracoscopically (Group A) and five of eleven (45%) via laparotomy (Group B). Two of six (33%) patients (Group A) were converted to a laparotomy, one of six (16%) patient developed a recurrence, and there was no recurrence in Group B. Two of five (40%) patients died within the first 60 days of life, whilst there was no death in Group A. Intra-operative values are shown below. CONCLUSION: Whilst this is a preliminary report of a limited number of patients, there is no obvious difference of intra-operative blood gas parameters during surgical repair in patients after ECMO. Thoracoscopic CDH repair may be considered in patients after ECMO.


Asunto(s)
Oxigenación por Membrana Extracorpórea , Hernias Diafragmáticas Congénitas , Humanos , Hernias Diafragmáticas Congénitas/cirugía , Resultado del Tratamiento , Toracoscopía , Estudios Retrospectivos
3.
Pediatr Surg Int ; 39(1): 185, 2023 Apr 24.
Artículo en Inglés | MEDLINE | ID: mdl-37095416

RESUMEN

PURPOSE: To identify markers of previous ovarian torsion and outline the outcomes according to US appearance and operative management. METHODS: A retrospective single-centre review of neonatal ovarian cysts from January 2000 to January 2020. Data on postnatal cyst size and sonographic features and operative treatment were co-related with outcomes of ovarian loss and histology. RESULTS: 77 females were included with 22 simple and 56 complex cysts, one patient had bilateral cysts. 9/22 (41%) simple cysts regressed spontaneously in a median of 13 weeks (8-17). Complex cysts regressed spontaneously less frequently, 7/56(12%, P = 0.01), in 13 weeks (7-39). 38/56 (68%) complex and 12/22 (55%) simple cysts were treated operatively. 21/22 (95%) ovaries with initially simple cyst were salvaged compared to 20/56(36%) with initially complex cyst (P < 0.001). A fluid-debris level in 23/26 complex cysts was most associated with ovarian loss (P = 0.0006). Presence of viable ovarian stromal tissue was seen in 8/20 (40%) excised specimens during ovarian sparing procedures and in 5/30 (17%) oophorectomies for necrotic appearing ovaries. CONCLUSIONS: Fluid-debris level on US is significantly associated with ovarian loss likely due to previous torsion. Simple cysts are viable and often regress spontaneously. The finding of viable ovarian stromal tissue in resected specimens supports attempting ovarian preservation wherever possible.


Asunto(s)
Quistes Ováricos , Ultrasonografía Prenatal , Embarazo , Recién Nacido , Femenino , Humanos , Estudios Retrospectivos , Quistes Ováricos/cirugía
4.
Pediatr Surg Int ; 40(1): 17, 2023 Dec 08.
Artículo en Inglés | MEDLINE | ID: mdl-38063992

RESUMEN

AIM: To review our experience of laparoscopic inguinal hernia repair (LIHR) regarding complication rates, the practice of closing the asymptomatic patent processes vaginalis (PPV), and comparison of complication rates between pre-term (< 37 week gestation) and term infants. METHODS: Retrospective review of LIHR performed between 2009 and 2021. Repair was performed by intracorporal single or double purse string/purse string + Z-stitch using a non-absorbable suture. Data were analyzed using Chi-squared/Mann-Whitney and are quoted as median (range). RESULTS: 1855 inguinal rings were closed in 1195 patients (943 (79%) male). 1378 rings (74%) were symptomatic. 492 (41%) patients were pre-term. Corrected gestational age at surgery was 55 weeks (31 weeks-14.6 years) and weight 5.9 kg (1-65.5). Closure of contralateral PPV was higher in the premature group (210/397 [53%] vs. 265/613 [43%] p = 0.003). There were 23 recurrences in 20 patients, of whom 10 had been born prematurely. The only factor significantly associated with a lower recurrence was use of a second stitch (p = 0.011). CONCLUSION: This is the largest single-center reported series of LIHR. LIHR is safe at any age, the risk of recurrence is low, and can be corrected by re-laparoscopy. Use of a Z-stitch or second purse string is associated with a significantly lower rate of recurrence.


Asunto(s)
Hernia Inguinal , Laparoscopía , Hidrocele Testicular , Lactante , Femenino , Humanos , Masculino , Hernia Inguinal/cirugía , Resultado del Tratamiento , Herniorrafia , Recurrencia , Hidrocele Testicular/cirugía , Estudios Retrospectivos
5.
Pediatr Surg Int ; 38(5): 769-775, 2022 May.
Artículo en Inglés | MEDLINE | ID: mdl-35338381

RESUMEN

PURPOSE: The safety of minimally invasive surgery (MIS) was questioned in the COVID-19 pandemic due to concern regarding disease spread. We continued MIS during the pandemic with appropriate protective measures. This study aims to assess the safety of MIS compared to Open Surgery (OS) in this setting. METHODS: Operations performed during 2020 lockdown were compared with operations from the same time-period in 2019 and 2021. Outcomes reviewed included all complications, respiratory complications, length of stay (LOS) and operating surgeon COVID-19 infections (OSI). RESULTS: In 2020, MIS comprised 52% of procedures. 29% of MIS 2020 had complications (2019: 24%, 2021: 15%; p = 0.08) vs 47% in OS 2020 (p = 0.04 vs MIS). 8.5% of MIS 2020 had respiratory complications (2019: 7.7%, 2021: 6.9%; p = 0.9) vs 10.5% in OS 2020 (p = 0.8 vs MIS). Median LOS[IQR] for MIS 2020 was 2.5[6] days vs 5[23] days in OS 2020 (p = 0.06). In 2020, 2 patients (1.2%) were COVID-19 positive (MIS: 1, OS: 1) and there were no OSI. CONCLUSION: Despite extensive use of MIS during the pandemic, there was no associated increase in respiratory or other complications, and no OSI. Our study suggests that, with appropriate protective measures, MIS can be performed safely despite high levels of COVID-19 in the population.


Asunto(s)
COVID-19 , Pandemias , COVID-19/epidemiología , Control de Enfermedades Transmisibles , Humanos , Tiempo de Internación , Procedimientos Quirúrgicos Mínimamente Invasivos/métodos , Estudios Retrospectivos
6.
Pediatr Surg Int ; 37(9): 1161-1165, 2021 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-34081161

RESUMEN

Gastrointestinal stromal tumor (GIST) is a rare cancer of mesenchymal origin mostly seen in adult and elderly populations. Therefore, the prognostic and therapeutic features of pediatric GIST are not clearly defined. Clinical knowledge has been largely extrapolated from case series and adult studies. In this systematic review, we aimed to analyze the health outcome metrics of pediatric GIST. Medline and Embase databases were searched using relevant key terms. The original search retrieved 1,892 titles; 27 studies with 184 patients (68% female) were included for final review. The primary tumors were located in the stomach (165/184, 90%), small bowel (12/184, 7%), and elsewhere (7/184, 4%). Individual patient data were available in 125 cases with a median follow-up of 6.7 years. All patients underwent surgical resection, which varied from wide local excision to total gastrectomy. There were 12 deaths (10%), 65 (52%) patients were alive with no evidence of disease, and 31 cases (25%) were alive with disease. Tumor size > 5 cm, high mitotic index, and spindle morphology were predictive of mortality. Pediatric GIST has a more favorable prognosis and different characteristics versus adult tumors. There is a crucial need for international consensus and specific pediatric guidelines for the treatment of this rare tumor.


Asunto(s)
Tumores del Estroma Gastrointestinal , Adulto , Anciano , Niño , Femenino , Tumores del Estroma Gastrointestinal/epidemiología , Tumores del Estroma Gastrointestinal/cirugía , Humanos , Intestino Delgado , Masculino , Pronóstico , Estudios Retrospectivos , Resultado del Tratamiento
7.
Pediatr Surg Int ; 36(2): 165-169, 2020 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-31646379

RESUMEN

PURPOSE: Congenital intrathoracic stomach (CIS) is an uncommon pediatric surgical diagnosis where > 2/3rd of the stomach lies within the chest through a hiatus defect. We reviewed our recent experience with this condition. METHODS: A retrospective single-center review of children with a diagnosis of CIS (2007-2018) was performed. Patient demographics, presentation, imaging and management were assessed. Results are expressed as median (range). RESULTS: Eleven patients (6 girls) were identified with onset of symptoms at 2 (0-26) months of age. Presenting symptoms were vomiting (8/11), respiratory symptoms (4/11) and failure to thrive (2/11). Two patients had Marfan's syndrome. An upper gastrointestinal contrast study demonstrated gastric herniation in all. All were corrected laparoscopically with hiatus repair and fundoplication [age at surgery 10.5 (1.5-34.5) months]. A concurrent gastrostomy was done in children ≤ 6 months (n = 5). Enteral feeds were commenced on post-operative day one in 9 and second post-operative day in 2. At 7 (0-95) months follow-up, all were on full enteral feeds. One patient had a recurrence 6 months post-operatively, which was re-operated laparoscopically without any further recurrence. CONCLUSION: This is the largest reported series of children with CIS. All could be managed laparoscopically with no conversions and a low recurrence.


Asunto(s)
Fundoplicación/métodos , Gastrostomía/métodos , Hernia Hiatal/cirugía , Herniorrafia/métodos , Laparoscopía/métodos , Estómago/cirugía , Preescolar , Femenino , Hernia Hiatal/congénito , Hernia Hiatal/diagnóstico , Humanos , Lactante , Recién Nacido , Masculino , Recurrencia , Estudios Retrospectivos
8.
Pediatr Hematol Oncol ; 31(8): 703-16, 2014 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-25247398

RESUMEN

The role of surgery in the management of advanced staged neuroblastoma (NBL) is controversial. A systematic review and meta-analysis is reported to address robust evidence for curative "gross total tumor resection" (GTR) in Stage 3 and Stage 4 neuroblastoma. Studies were identified using Medline, Embase, and Cochrane databases using pre-specified search terms. Primary outcomes were 5-year overall (OS) and disease-free survival (DFS) after GTR and subtotal resection (STR) in Stage 3 or 4 NBL. Data were analyzed using Review Manager. The Mantel-Haenszel method and a random effects model was utilized to calculate odds ratios (95% CI). Fifteen studies (five Stage 3 and 13 Stage 4) met full inclusion criteria. The pooled odds ratio for 5 year OS in Stage 3 following GTR compared to STR was 2.4 (95% CI 1.19-4.85). In Stage 4 disease, the pooled odds ratio for 5 year overall survival (OS) following GTR compared to STR was 1.65 (95% CI 0.96-1.91); a pooled odds ratio for 5 year DFS following GTR compared to STR was 1.55 (95% CI 1.12-2.14). A clear survival benefit is shown for GTR over STR in Stage 3 NBL only. Though some advantage can be demonstrated for GTR as defined by DFS in Stage 4 NBL GTR did not significantly improve OS in Stage 4 disease.


Asunto(s)
Neuroblastoma/mortalidad , Neuroblastoma/cirugía , Niño , Preescolar , Terapia Combinada , Inglaterra , Humanos , Lactante , Neuroblastoma/epidemiología , Neuroblastoma/patología , Procedimientos Neuroquirúrgicos , Análisis de Supervivencia , Resultado del Tratamiento
9.
J Pediatr Surg ; 59(2): 192-196, 2024 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-38016850

RESUMEN

AIM OF THE STUDY: We describe the short- and medium-term outcomes following open and laparoscopic assisted oesophageal replacement surgery in a single tertiary paediatric surgical centre. METHODS: A retrospective review (institutional audit approval no. 3213) on patients who underwent open or laparoscopic-assisted oesophageal replacement (OAR vs. LAR) at our centre between 2002 and 2021 was completed. Data collected (demographics, early complications, stricture formation, need for oesophageal dilatations, and mortality) were analysed using GraphPad Prism v 9.50 and are presented as median (IQR). RESULTS: 71 children (37 male) had oesophageal replacement surgery at a median age of 2.3 years (IQR 4.7 years). 51 were LAR (6 conversions). Replacement conduit was stomach (n = 67), colon (n = 3), or jejunum (n = 1). Most gastric transpositions had a pyloroplasty (46/67) or pyloromyotomy (14/67). Most common pathology was oesophageal atresia (n = 50 including 2 failed transpositions), caustic injury (n = 19 including 3 due to button battery), stricture of unknown cause (n = 1), and megaoesophagus (n = 1). There were 2 (2.8 %) early postoperative deaths at 2 days (major vessel thrombosis), 1 month (systemic sepsis), and one death at 5 years in the community. The rate of postoperative complications were comparable across LAR and OAR including anastomotic leak, pleural effusions, or early strictures. More patients with caustic pathology needed dilatations (60 % vs 30 % in OA, p = 0.05). CONCLUSIONS: Outcomes of open and laparoscopic-assisted oesophageal replacement procedures are comparable in the short and medium term. Anastomotic stricture is higher in those with caustic injury. LEVEL OF EVIDENCE: IV.


Asunto(s)
Cáusticos , Atresia Esofágica , Estenosis Esofágica , Laparoscopía , Niño , Humanos , Masculino , Preescolar , Estenosis Esofágica/epidemiología , Estenosis Esofágica/etiología , Estenosis Esofágica/cirugía , Constricción Patológica/cirugía , Atresia Esofágica/cirugía , Atresia Esofágica/complicaciones , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/etiología , Complicaciones Posoperatorias/cirugía , Laparoscopía/métodos , Estudios Retrospectivos
10.
Children (Basel) ; 9(8)2022 Jul 30.
Artículo en Inglés | MEDLINE | ID: mdl-36010044

RESUMEN

Consensus on the optimal management of asymptomatic congenital pulmonary airway malformation (CPAM) is lacking, and comparison between studies remains difficult due to a large variety in outcome measures. We aimed to define a core outcome set (COS) for pediatric patients with an asymptomatic CPAM. An online, three-round Delphi survey was conducted in two stakeholder groups of specialized caregivers (surgeons and non-surgeons) in various European centers. Proposed outcome parameters were scored according to level of importance, and the final COS was established through consensus. A total of 55 participants (33 surgeons, 22 non-surgeons) from 28 centers in 13 European countries completed the three rounds and rated 43 outcome parameters. The final COS comprises seven outcome parameters: respiratory insufficiency, surgical complications, mass effect/mediastinal shift (at three time-points) and multifocal disease (at two time-points). The seven outcome parameters included in the final COS reflect the diversity in priorities among this large group of European participants. However, we recommend the incorporation of these outcome parameters in the design of future studies, as they describe measurable and validated outcomes as well as the accepted age at measurement.

11.
Pediatr Blood Cancer ; 57(7): 1254-60, 2011 Dec 15.
Artículo en Inglés | MEDLINE | ID: mdl-21523901

RESUMEN

PURPOSE: The role of surgery in the management of neuroblastoma yields conflicting reports. We report a 20-year experience from a UK centre in the context of evolving cancer therapies for neuroblastoma. METHODS: Hospital records of 91 neuroblastoma patients from 1985 to 2005 were studied. Patient demographics, data from operating notes and tumour biology (MYCN status) where available were analysed. RESULTS: Surgery consisted of primary resection or delayed operation following tumour biopsy/chemotherapy. Overall survival was 100% for stage 1(n = 3), 90% for stage 2 (n = 10), 46% for stage 3 (n = 13), 13% for stage 4 (n = 55) and 56% for stage 4S disease (n = 9). During the eras 1985-1994 versus 1995-2005, survival for stage 3 lesions was 25% and 80% (P = 0.04) with marginal increase in survival observed in stage 4 disease (12% vs. 22%, P = 0.083). Delayed tumour resection was not performed in 20 (36%) stage 4 patients due to progressive disease. Complete tumour resection was achieved in 62% of stage 3-4 patients during 1995-2005 compared to 38% in 1985-1994. The extent of surgical resection (complete vs. partial) showed no significant differences in overall survival or relapse rates. Postoperative morbidity occurred in 15.7% of cases emphasising technical challenges in resection of neuroblastoma. No child with MYCN amplification survived versus 59% survival in non-amplified cases (P = 0.012). CONCLUSIONS: While complete tumour resection may be desirable in advanced neuroblastoma (stage 3-4) these findings suggest that the radicality of operation is not significantly associated with better overall survival/relapse. Improving outcomes in the 1995-2005 era for patients with stage 3-4 tumours complements the introduction of new high dose-intensive chemotherapy regimens and other adjuvant therapies for this enigmatic disease.


Asunto(s)
Neuroblastoma/mortalidad , Neuroblastoma/terapia , Antineoplásicos/uso terapéutico , Instituciones Oncológicas/estadística & datos numéricos , Quimioterapia Adyuvante , Preescolar , Terapia Combinada , Supervivencia sin Enfermedad , Femenino , Humanos , Lactante , Recién Nacido , Estimación de Kaplan-Meier , Masculino , Estadificación de Neoplasias , Neuroblastoma/patología , Procedimientos Neuroquirúrgicos/métodos , Estudios Retrospectivos , Resultado del Tratamiento , Reino Unido
12.
ANZ J Surg ; 91(5): 1000-1004, 2021 05.
Artículo en Inglés | MEDLINE | ID: mdl-33506998

RESUMEN

BACKGROUND: Numerous factors have been identified as carrying prognostic value in neuroblastoma (NB) and therefore incorporated in risk stratification of disease. Here, we investigate the association of anatomical site of NB with molecular biology and clinical outcomes. METHODS: A total of 117 patients with NB were studied over a 30-year period. Tumour location was confirmed with computed tomography/magnetic resonance imaging. Data on molecular biology were obtained as testing became available. Chi-squared, Fisher's exact test and Kaplan-Meier log-rank tests were used for statistical analysis. RESULTS: Tumour originated in the thoracic region (thoracic NB, TNB) in 15 patients (13%), adrenal gland (adrenal NB, ANB) in 88 patients (75%) and abdominal/paravertebral chain (paravertebral NB, PVNB) in 14 patients (12%). Overall survival (OS) for ANB was significantly lower (38%; P = 0.015). ANB cases were more frequently diagnosed at stage IV (69%; P = 0.001). MYCN amplification was noted in 33% of ANB cases and associated with lower OS (17% versus 62% MYCN non-amplified ANB; P = 0.01). The vast majority of TNB and PVNB were non-MYCN amplified (100% and 86%, respectively) and carried better prognosis (OS 86% and 83%, respectively). Forty-two percent of ANB cases were diploid and had lower OS (20% versus 71% hyperdiploid ANB; P = 0.079). TNB and PVNB were found to be mostly hyperdiploid (86% and 100%, respectively) with better OS (83% and 33%, respectively). Segmental chromosomal alterations had prognostic significance in those with PVNB (P = 0.03). CONCLUSION: TNB tumours have better outcomes than adrenal tumours. This may be due to varied factors reported here including non-metastatic disease at presentation, non-amplification of the MYCN oncogene and overall favourable molecular biology characteristics.


Asunto(s)
Neuroblastoma , Humanos , Lactante , Estimación de Kaplan-Meier , Biología Molecular , Proteína Proto-Oncogénica N-Myc/genética , Neuroblastoma/diagnóstico por imagen , Neuroblastoma/genética , Pronóstico
13.
Eur J Pediatr Surg ; 31(5): 385-389, 2021 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-32932540

RESUMEN

INTRODUCTION: The prognosis of stage 4S/MS neuroblastoma has traditionally been reported as excellent, yet conflicting treatment protocols exist for this enigmatic disease. To critically address this question, we have undertaken a systematic review of published studies to accurately determine outcomes for infants with stage 4S/MS neuroblastoma. MATERIALS AND METHODS: Studies were identified using MEDLINE, Embase, and Cochrane databases using the relevant search terms. Literature reviews, case reports, and adult studies were excluded. Data were extracted independently following article selection by three authors and reviewed by the senior author. RESULTS: The original search retrieved 2,325 articles. Following application of exclusion criteria and removing duplicate data, 37 studies (1,105 patients) were included for final review. Overall patient survival was 84%. Twelve studies (544 patients) recorded MYCN status. Mortality in MYCN amplified tumors was 56%. Chromosome 1p/11q status was reported in four studies and 1p/11q deletion carried a 40% fatality rate. Management included observation only (201 patients, 8.5% mortality), surgical resection of primary tumor only (153 patients, 6.5% mortality), chemotherapy only (186 patients, 21% mortality), radiotherapy (5 deaths, 33% mortality), chemotherapy with surgery (160 patients, 10% mortality), surgery with radiotherapy (21 patients, 19% mortality), radiotherapy with chemotherapy (42 patients, 29% mortality), and surgery with chemotherapy and radiotherapy (27 patients, 33% mortality). CONCLUSION: There is a significant mortality observed in stage 4S/MS neuroblastoma infants with a dismal outcome observed in those patients with MYCN amplification and 1p/11q deletion. Those patients suitably amenable for conservative management or surgery to excise the primary tumor carry the best prognosis.


Asunto(s)
Neuroblastoma/mortalidad , Humanos , Lactante , Recién Nacido , Proteína Proto-Oncogénica N-Myc , Estadificación de Neoplasias , Neuroblastoma/genética , Neuroblastoma/terapia
14.
BMJ Open ; 11(4): e044544, 2021 04 12.
Artículo en Inglés | MEDLINE | ID: mdl-33846152

RESUMEN

INTRODUCTION: A worldwide lack of consensus exists on the optimal management of asymptomatic congenital pulmonary airway malformation (CPAM) even though the incidence is increasing. Either a surgical resection is performed or a wait-and-see policy is employed, depending on the treating physician. Management is largely based on expert opinion and scientific evidence is scarce. Wide variations in outcome measures are seen between studies making comparison difficult thus highlighting the lack of universal consensus in outcome measures as well. We aim to define a core outcome set which will include the most important core outcome parameters for paediatric patients with an asymptomatic CPAM. METHODS AND ANALYSIS: This study will include a critical appraisal of the current literature followed by a three-stage Delphi process with two stakeholder groups. One surgical group including paediatric as well as thoracic surgeons, and a non-surgeon group including paediatric pulmonologists, intensive care and neonatal specialists. All participants will score outcome parameters according to their level of importance and the most important parameters will be determined by consensus. ETHICS AND DISSEMINATION: Electronic informed consent will be obtained from all participants. Ethical approval is not required. After the core outcome set has been defined, we intend to design an international randomised controlled trial: the COllaborative Neonatal NEtwork for the first CPAM Trial, which will be aimed at determining the optimal management of patients with asymptomatic CPAM.


Asunto(s)
Evaluación de Resultado en la Atención de Salud , Investigadores , Niño , Consenso , Técnica Delphi , Humanos , Recién Nacido , Proyectos de Investigación , Encuestas y Cuestionarios
15.
Pediatr Blood Cancer ; 55(1): 206-7, 2010 Jul 15.
Artículo en Inglés | MEDLINE | ID: mdl-20310004

RESUMEN

We report a case of Peutz-Jeghers syndrome (PJS) in a 2-year old with precocious puberty secondary to a Sertoli-Leydig cell tumour. Family history of PJS and other neoplasms were discovered. The tumour was excised and the STK11 gene deletion identified in both patient and father. Screening revealed hamartomatous gastric polyps, which were removed. Current recommendations for screening of children with PJS begin at age 8 years, based on reported occurrence of complications 1. This report illustrates the importance of considering early screening, along with close clinical review and patient/parent education, for detection of life threatening neoplasms and complications.


Asunto(s)
Mucosa Gástrica/patología , Hamartoma/diagnóstico , Neoplasias Ováricas/diagnóstico , Síndrome de Peutz-Jeghers/diagnóstico , Pólipos/diagnóstico , Tumor de Células de Sertoli-Leydig/diagnóstico , Neoplasias Gástricas/diagnóstico , Quinasas de la Proteína-Quinasa Activada por el AMP , Preescolar , Femenino , Mucosa Gástrica/cirugía , Eliminación de Gen , Hamartoma/complicaciones , Hamartoma/genética , Hamartoma/cirugía , Humanos , Neoplasias Ováricas/complicaciones , Neoplasias Ováricas/genética , Síndrome de Peutz-Jeghers/complicaciones , Síndrome de Peutz-Jeghers/genética , Pólipos/genética , Pólipos/cirugía , Proteínas Serina-Treonina Quinasas/genética , Tumor de Células de Sertoli-Leydig/complicaciones , Tumor de Células de Sertoli-Leydig/genética , Neoplasias Gástricas/complicaciones , Neoplasias Gástricas/genética , Neoplasias Gástricas/cirugía
16.
Eur J Pediatr Surg ; 30(1): 33-38, 2020 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-31707729

RESUMEN

INTRODUCTION: Congenital duodenal obstruction (CDO) repair can be performed open or laparoscopically. We aimed to determine the potential benefit of laparoscopic repair regarding tolerance of enteral feeding, postoperative pain, hospital stay, and complication rate. MATERIALS AND METHODS: In a single-center retrospective cohort study, we compared neonates with isolated CDO operated open versus laparoscopically from 2010 to 2019. No transanastomotic tubes were used, and anastomoses were created in a side-to-side fashion in all cases. An early feeding policy is applied for all cases operated at our institution. Statistical comparison was performed using the Mann-Whitney's test or Fisher's exact test where appropriate. RESULTS: Forty-one patients analyzed were similar regarding body weight, gestational age, and proportion of patients with trisomy 21. Median follow-up was 21 months. Four (20%) out of 20 laparoscopic procedures started laparoscopically were converted to open. Comparing the 21 open with the 16 laparoscopically completed patients, median anesthetic duration was shorter by 18% in the open versus laparoscopic completed group (218 vs. 179 minutes, respectively; p = 0.025). Median postoperative time to full enteral feeds was shorter by 4 days in the first group (7 vs. 11 days, respectively; p = 0.028). In accordance, the median duration of parenteral nutrition (PN) was less than half in the laparoscopic completed compared with the open group (5 vs. 11.5 days, respectively; p = 0.031). Postoperative opioids were required for only half the duration in the laparoscopically completed group compared with open (2 vs. 4 days, respectively; p = 0.026). Outcomes such as length of stay, the occurrence of strictures or adhesions requiring reintervention, or line sepsis were similar in both groups. CONCLUSION: Patients undergoing laparoscopic CDO repair at our institution benefited from shorter time to full enteral feeds, and reduced the need for PN as well as postoperative pain medication.


Asunto(s)
Obstrucción Duodenal/congénito , Obstrucción Duodenal/cirugía , Laparoscopía , Anomalías Múltiples , Peso Corporal , Conversión a Cirugía Abierta , Obstrucción Duodenal/complicaciones , Nutrición Enteral , Femenino , Estudios de Seguimiento , Humanos , Recién Nacido , Recien Nacido Prematuro , Laparoscopía/efectos adversos , Tiempo de Internación , Masculino , Tempo Operativo , Dolor Postoperatorio , Nutrición Parenteral , Estudios Retrospectivos , Factores de Tiempo
17.
Essays Biochem ; 45: 121-33, 2008.
Artículo en Inglés | MEDLINE | ID: mdl-18793128

RESUMEN

Understanding how mammalian cells function requires a dynamic perspective. However, owing to the complexity of signalling networks, these non-linear systems can easily elude human intuition. The central aim of systems biology is to improve our understanding of the temporal complexity of cell signalling pathways, using a combination of experimental and computational approaches. Live-cell imaging and computational modelling are compatible techniques which allow quantitative analysis of cell signalling pathway dynamics. Non-invasive imaging techniques, based on the use of various luciferases and fluorescent proteins, trace cellular events such as gene expression, protein-protein interactions and protein localization in cells. By employing a number of markers in a single assay, multiple parameters can be measured simultaneously in the same cell. Following acquisition using specialized microscopy, analysis of multi-parameter time-lapse images facilitates the identification of important qualitative and quantitative relationships-linking intracellular signalling, gene expression and cell fate.


Asunto(s)
Imagenología Tridimensional , Biología de Sistemas , Animales , Supervivencia Celular , Colorantes Fluorescentes/metabolismo , Genes Reporteros , Humanos , Proteínas Luminiscentes/metabolismo
18.
J Pediatr Surg ; 54(2): 251-254, 2019 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-30503023

RESUMEN

OBJECTIVE: The objective of this study was to report our multidisciplinary diagnostic approach for patients with anterior mediastinal masses (AMM). METHODS: A retrospective review of patients with AMM at a tertiary pediatric surgical oncology centre (January 2011-December 2016) was performed. We analyzed data on clinical presentation, mode of tissue diagnosis, anesthetic techniques, and complications. RESULTS: Of the 44 patients admitted with AMM (median age 11 years, 27 males and 17 females), 22 had respiratory symptoms. Imaging revealed tracheobronchial compression in 26 children. Twenty patients had a lymph node biopsy. Ten patients had image-guided core biopsy of the mediastinal mass, and 2 had mediastinoscopic biopsy of a paratracheal lymph node. One patient with likely recurrence of a relapsed metastatic ethmoid carcinoma did not have a biopsy. The diagnosis was made from alternative tissues, such as pleural fluid in 4 and peripheral blood in 7 patients. Twenty-five anesthetics were assessed, as 14 patients required no or only local anesthesia, and 5 had unavailable anesthetic notes. Eighteen of 25 patients were anesthetized maintaining spontaneous breathing, mostly by means of ketamine sedation. There were no major anesthetic complications. CONCLUSION: Safe tissue diagnosis of anterior mediastinal masses can be obtained by a personalized multidisciplinary approach. Use of alternative tissues, local anesthesia, and ketamine sedation help minimize the need for general anesthesia, muscle paralysis, and controlled ventilation. LEVEL OF EVIDENCE: IV (Case Series with no Comparison Group).


Asunto(s)
Ganglios Linfáticos/patología , Neoplasias del Mediastino/diagnóstico , Neoplasias del Mediastino/patología , Adolescente , Anestesia General , Anestesia Local , Anestésicos Disociativos , Niño , Preescolar , Femenino , Humanos , Biopsia Guiada por Imagen , Ketamina , Masculino , Neoplasias del Mediastino/complicaciones , Mediastinoscopía , Grupo de Atención al Paciente , Derrame Pleural/etiología , Enfermedades Respiratorias/etiología , Estudios Retrospectivos
19.
Eur J Pediatr Surg ; 29(3): 243-246, 2019 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-29490378

RESUMEN

AIM: Regular anal dilatations are commonly recommended in the postoperative management following posterior sagittal anorectoplasty (PSARP) in anorectal malformations (ARM). We hypothesized that routine postoperative dilatations may not affect surgical outcomes following PSARP. We compare surgical outcomes of routine postoperative dilatations versus no routine postoperative dilatations from two United Kingdom tertiary pediatric surgical centers. MATERIALS AND METHODS: This is retrospective records review of patients undergoing definitive surgery for ARM in two tertiary surgical centers in the UK over 5 years. Center A used a protocol of routine postoperative dilatations, and center B used a protocol, which used dilatations only for clinical indications of stricture. Data collected included ARM type, operative procedures, and postoperative interventions. All post-operative interventions under general anesthesia (GA) were compared between groups. RESULTS: From 2011 to 2015, 49 procedures (46 PSARPs) were performed in center A and 54 (52 PSARPs) in center B. Median follow up period was 31 months (interquartile range [IQR] 18-48). The first postoperative anal calibration under GA was documented for 43 (86%) patients in center A and for 42 (78%) patients in center B. Following this, center A followed routine postoperative dilatation (RPD) at home, and center B reserved further dilatations for specific indications. RPD was performed for 100% of patients in center A versus 8% in center B. Further anal dilatations under GA were performed in 19 (38%) children in center A and in 17 (34%) children in center B (p = 0.68). In center A, 10 patients (22%) needed further surgery versus 14 (28%) in center B (p = 0.48). CONCLUSION: The use of routine postoperative dilatations does not significantly improve surgical outcomes following PSARP in ARM.


Asunto(s)
Malformaciones Anorrectales/cirugía , Obstrucción Intestinal/prevención & control , Cuidados Posoperatorios/métodos , Complicaciones Posoperatorias/prevención & control , Enfermedades del Recto/prevención & control , Dilatación , Femenino , Estudios de Seguimiento , Humanos , Lactante , Obstrucción Intestinal/epidemiología , Obstrucción Intestinal/etiología , Masculino , Complicaciones Posoperatorias/epidemiología , Enfermedades del Recto/epidemiología , Enfermedades del Recto/etiología , Estudios Retrospectivos , Centros de Atención Terciaria , Resultado del Tratamiento , Reino Unido
20.
BMC Pediatr ; 8: 37, 2008 Sep 24.
Artículo en Inglés | MEDLINE | ID: mdl-18816390

RESUMEN

BACKGROUND: The diagnosis of infantile hypertrophic pyloric stenosis (IHPS), although traditionally clinical, is now increasingly dependent on radiological corroboration. The rate of negative exploration in IHPS has been reported as 4%. The purpose of our study was to look at elements of supportive clinical evidence leading to positive diagnosis, and to review these with respect to misdiagnosed cases undergoing negative exploration. METHODS: All infants undergoing surgical exploration for IHPS between January 2000 and December 2004 were retrospectively analysed with regard to clinical symptoms, examination findings, investigations and operative findings. RESULTS: During the study period, 343 explorations were performed with a presumptive diagnosis of IHPS. Of these, 205 infants (60%) had a positive test feed, 269 (78%) had a positive ultrasound scan and 175 (55%) were alkalotic (pH >or=7.45 and/or base excess >or=2.5). The positive predictive value for an ultrasound (US) diagnosis was 99.1% for canal length >or=14 mm, and 98.7% for muscle thickness >or=4 mm. Four infants (1.1%) underwent a negative surgical exploration; Ultrasound was positive in 3, and negative in 1(who underwent surgery on the basis of a positive upper GI contrast). One US reported as positive had a muscle thickness <4 mm. Two false positive US were performed at peripheral hospitals. One infant had a false positive test feed following a positive ultrasound diagnosis. Two infants had negative test feeds. CONCLUSION: A 1% rate of negative exploration in IHPS compares favourably with other studies. However potential causes of error were identified in all 4 cases. Confident diagnosis comprises a combination of positive test feed and an 'in house US' in an alkalotic infant. UGI contrast study should not be used in isolation to diagnose IHPS. If the test feed is negative, strict diagnostic measurements should be observed on US and the pyloric 'tumour' palpated on table under anaesthetic before exploration.


Asunto(s)
Estenosis Pilórica/diagnóstico , Píloro/patología , Alcalosis/sangre , Reacciones Falso Positivas , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Examen Físico , Valor Predictivo de las Pruebas , Estenosis Pilórica/complicaciones , Estenosis Pilórica/cirugía , Píloro/diagnóstico por imagen , Píloro/cirugía , Reproducibilidad de los Resultados , Estudios Retrospectivos , Ultrasonografía/métodos , Vómitos/etiología , Vómitos/patología
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