Abstract：
PURPOSE:There is a known association between Cornelia de Lange syndrome (CdLS) and congenital diaphragmatic hernia (CDH), with CDH being the cause of death in 5%-20% of CdLS cases. We aimed to identify and describe patients with CDLS and CDH. We hypothesized that CdLS would be associated with high-risk CDH and poor outcomes. METHODS:CDH Study Group patients from 1995 to 2019 were included. Those with CdLS were reviewed retrospectively. Rates of repair and outcomes were compared between patients with and without CdLS. RESULTS:We identified 9,251 CDH patients. Of those, 21 had confirmed CdLS. CdLS patients had a lower birth weight (2.2±0.57 kg) than non-CdLS patients (2.9±0.64 kg) (p<0.001). 5-min Apgar scores were lower in CdLS patients (6, 4-7) than non-CdLS patients (7, 5-8) (p=0.014). Only 33% of CdLS patients underwent diaphragmatic repair compared to 84.2% of non-CdLS patients (p<0.001). Mortality was 76% for CdLS patients compared with 29% for non-CdLS patients (p<0.001). Of the 7 CdLS patients who underwent repair, 5 survived to hospital discharge. CONCLUSIONS:Infants with CdLS and CDH have a poor prognosis. However, CdLS patients who undergo repair can survive to discharge; therefore, the concomitant diagnosis of CdLS and CDH is not necessarily a contraindication to repair. Early recognition of these anomalies can assist with counseling and prognostication. TYPE OF STUDY:Retrospective comparative study LEVEL OF EVIDENCE: III.
journal_name
J Pediatr Surgjournal_title
Journal of pediatric surgeryauthors
Gupta VS,Khan AM,Ebanks AH,Lally PA,Lally KP,Harting MT,Congenital Diaphragmatic Hernia Study Group.doi
10.1016/j.jpedsurg.2020.06.003subject
Has Abstractpub_date
2020-06-11 00:00:00eissn
0022-3468issn
1531-5037pii
S0022-3468(20)30412-7pub_type
杂志文章abstract：:Necrotizing fasciitis is a rare but serious condition with a poor prognosis both in adults and in children. Retroperitoneal localization is mostly associated with fatal outcome. Early diagnosis, extensive and repeated surgical debridement, and use of antibiotics are necessary. Herein the authors report on a 4-year-old...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(98)90219-6
更新日期：1998-05-01 00:00:00
abstract：PURPOSE:The serial transverse enteroplasty (STEP) tapers and lengthens the gut to improve adaptation. Mortality has decreased with multidisciplinary intestinal rehabilitation programs (IRP) allowing more time to reach adaptive potential. We reviewed our STEP experience to compare surgical outcomes between early and lat...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2019.01.051
更新日期：2019-05-01 00:00:00
abstract：BACKGROUND:Extrahepatic portal vein thrombosis (EHPVT) is an important cause of portal hypertension in children. Rex shunt has been used successfully to treat these patients. METHODS:We report our experience in 19 infants and children (5 months to 14 years) with HPVT eligible for a mesenteric-portal surgical shunt wit...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2010.09.091
更新日期：2011-01-01 00:00:00
abstract：BACKGROUND:Rhomboid excision with Limberg flap (RELF) repair has been shown to be effective in the management of pilonidal disease (PD) in adults. Wide excision allows complete removal of diseased tissue, and the rotational flap allows tensionless coverage as well as helps flatten the natal crease, which is believed to...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2008.11.033
更新日期：2009-08-01 00:00:00
abstract：BACKGROUND/PURPOSE:Long-term sequelae caused by associated anomalies or respiratory and gastrointestinal disorders are common after the repair of esophageal atresia (EA). The aim of this study was to assess the effect of these sequelae on the health-related quality of life (HRQoL) of patients with EA. METHODS:A questi...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2004.10.014
更新日期：2005-02-01 00:00:00
abstract：PURPOSE:Neuroblastoma is the most common extracranial solid tumor in children. Twenty percent of all neuroblastomas arise in the thorax. This study evaluates the open vs thoracoscopic resection of thoracic neuroblastoma. METHODS:A retrospective chart review was conducted from the medical records of all children underg...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2010.03.018
更新日期：2010-08-01 00:00:00
abstract：:Mesenchymal hamartoma of the chest wall (MHCW) is a rare condition. Previously, surgical resection has been advocated with considerable post-operative morbidity. Evidence for conservative management is lacking because the natural history of MHCW is unknown. We present serial measurements of an antenatally detected MHC...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2010.08.057
更新日期：2010-12-01 00:00:00
abstract：BACKGROUND:Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) is the most severe form of functional intestinal obstruction in the newborn. To date, multivisceral transplantation has been the only accepted treatment modality for these patients, and the results have met with marginal success. We report the...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2007.09.073
更新日期：2008-02-01 00:00:00
abstract：:A classification of vaginal anomalies has been derived, which permits logical operative decisions. This tool allows the assignment of increasingly involved reconstructive operations to progressively more complex vaginal anatomies. The outcome of this approach in 49 vaginal reconstructions performed in 36 patients over...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/0022-3468(95)90573-1
更新日期：1995-02-01 00:00:00
abstract：BACKGROUND:Laparoscopic cholecystectomy is the standard surgical management of biliary disease in children, but there has been a paucity of studies addressing outcomes after pediatric cholecystectomies, particularly on a national level. We conducted the first study to address the effect of surgeon specialty and volume ...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2011.10.054
更新日期：2012-04-01 00:00:00
abstract：PURPOSE:The aim of this study is to identify the diagnostic values of serum exosomal miRNA-34s of patients with HB in a large Asian group and explore the prognostic value of the exosomal miRNA-34s panel compared with other risk factors. METHODS:We retrospectively reviewed 89 children with HB. Among these patients, 63 ...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2016.09.070
更新日期：2017-04-01 00:00:00
abstract：BACKGROUND/PURPOSE:The need for transitional care has gained increased focus in the treatment of patients with congenital colorectal disorders. We aimed to acquire in-depth knowledge about the experiences of adult patients with Hirschsprung's disease (HD) and their suggestions for transitional care. METHODS:Binational...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2020.05.015
更新日期：2020-05-16 00:00:00
abstract：BACKGROUND:Chronic infection of ingrown toenails may lead to exogenous osteomyelitis. Therefore, plain x-rays are commonly taken in children with significant inflammation. We evaluated the preoperative radiologic findings and their clinical significance, especially with regard to exogenic osteomyelitis. PATIENTS AND M...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2009.05.032
更新日期：2009-11-01 00:00:00
abstract：:The authors report on two adults who had jejunal dilatation after having had resection for jejunal atresia in the neonatal period. Both patients presented 20 years after the initial procedure, with severe iron deficiency anemia, marked jejunal dilatation proximal to the old anastomotic site (which was not narrowed), a...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/0022-3468(94)90238-0
更新日期：1994-12-01 00:00:00
abstract：PURPOSE:Infants born with congenital diaphragmatic hernia (CDH) demonstrate a wide variability in postnatal catch-up lung growth. The goals of this study were to assess the pulmonary development of children born with CDH using sequential ventilation-perfusion (V/Q) scintigraphy and to identify the perinatal factors tha...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2006.09.011
更新日期：2007-01-01 00:00:00
abstract：:In Gaucher disease, partial splenectomy has been suggested for alleviating the complications of splenomegaly as well as for avoiding the immunologic compromise and potential acceleration of bony and hepatic involvement that may follow total splenic resection. However, the fate of the splenic remnant has been reported ...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(89)80520-2
更新日期：1989-06-01 00:00:00
abstract：BACKGROUND:Ultrasound (US) evaluation of some fetal anomalies provides limited information. Anatomic details that affect prognosis and selection for fetal therapy, such as liver herniation and pulmonary hypoplasia in congenital diaphragmatic hernia (CDH) and airway patency in giant neck masses, may be difficult to deli...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(98)90315-3
更新日期：1998-04-01 00:00:00
abstract：RATIONALE:Recurrent tracheoesophageal fistulas (RTEFs) remain a therapeutic challenge because open surgical approaches have been associated with substantial rates of morbidity, mortality, and repeat recurrences. Recently, endoscopic techniques for the repair of RTEF have provided an alternative approach with the potent...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2007.08.062
更新日期：2008-01-01 00:00:00
abstract：:Of 156 children undergoing elective inguinal herniotomy under general anesthesia, 81 also had a regional nerve block using Bupivacaine. All were treated as outpatients and recovery was assessed by postanesthetic room nurses and by interrogating the parents as to the child's behavior at home. It was concluded that the ...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(80)80757-3
更新日期：1980-08-01 00:00:00
abstract：:The authors report the case of bilateral gonadoblastomas in a phenotypic female, with a 46,XY karyotype, with campomelic dysplasia. Although campomelic dysplasia with gonadal dysgenesis should be expected to contribute to an increased risk of gonadoblastoma, this is the first documented case report of campomelic dyspl...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/0022-3468(95)90468-9
更新日期：1995-12-01 00:00:00
abstract：UNLABELLED:The absorption of carbon dioxide (CO2) used for positive pressure pneumoperitoneum may lead to an increased CO2 load. CO2 elimination during laparoscopy has not been investigated previously in paediatrics. The aim of this study was to characterise the pattern of CO2 elimination during laparoscopic surgery in...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1053/jpsu.2003.50021
更新日期：2003-01-01 00:00:00
abstract：BACKGROUND/PURPOSE:Transanal endorectal pull-through (TEPT) is the latest development in treatment of Hirschsprung's disease (HD). This prospective study was designed to evaluate the safety and efficacy of 1 stage TEPT technique in the management of patients with HD. METHODS:One hundred forty-nine children (116 boys a...
journal_title：Journal of pediatric surgery
pub_type： 临床试验,杂志文章,多中心研究
doi：10.1016/j.jpedsurg.2003.11.038
更新日期：2004-03-01 00:00:00
abstract：:Aortoesophageal fistula (AOF) in children with no history of cardiac disease or trauma is extremely rare, and survival is even rarer. We present a case of a 2-year-old boy who presented with massive hematemesis secondary to a primary aortoesophageal fistula and was found at endoscopy to have a submucosal hematoma. He ...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2011.09.054
更新日期：2011-12-01 00:00:00
abstract：PURPOSE:Ingrown toenails in children are a common problem with a high recurrence rate. The objective of this retrospective data review was to compare simple excision of the nail matrix with excision plus phenol (EPP) application in the treatment of ingrown toenails. METHODS:The charts of 69 children who underwent surg...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2004.09.051
更新日期：2005-01-01 00:00:00
abstract：:A 5-year-old girl presented with a painful abdominal mass. Abdominal magnetic resonance imaging (MRI) showed 3 separate masses. Tumor markers including lactate dehydrogenase (LDH), cancer antigen-125 (CA-125), beta-subunit of human chorionic gonadotropin (beta-hCG) and neuron-specific enolase (NSE) were elevated. At o...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2004.05.023
更新日期：2004-09-01 00:00:00
abstract：:Primary fetal hydrothorax presents a wide spectrum of severity ranging from small, harmless effusions, to life-threatening thoracic compression. To define natural history and management, we reviewed 32 cases seen at two large perinatal centers from 1980 to 1987. Spontaneous resolution of the effusions was seen in thre...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(89)80509-3
更新日期：1989-06-01 00:00:00
abstract：:This article reviews of the current evidence-based treatment standards for children with Wilms tumor. In this article, a summary of recently completed clinical trials by the Children's Oncology Group is provided, the current diagnostic evaluation and surgical standards are discussed, and the surgical impact on current...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章,评审
doi：10.1016/j.jpedsurg.2018.09.005
更新日期：2019-03-01 00:00:00
abstract：:Pneumothorax is a common complication of ventilator therapy and usually responds to tube thoracostomy. Persistent pneumothorax with a large air leak may indicate the presence of a bronchopleural fistula (mean weight 1360 g) with (BPF) required FIO2 of 1.0, a peak inspiratory pressure of 33 cm H2O and were hypoxemic (x...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(80)80746-9
更新日期：1980-08-01 00:00:00
abstract：:A mass spectrometer blood gas catheter system for the continuous in vivo analysis of Po2 and Pco2 has been presented. This system has been experimentally evaluated and demonstrated to be reliable and reproducible over a period of 30 hr following insertion of the catheter in the abdominal aorta. Although the present ca...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(76)80078-4
更新日期：1976-12-01 00:00:00
abstract：:Children requiring surgical intervention for pancreatic disease may be at risk long term for exocrine insufficiency and glucose intolerance. Pediatric surgeons must balance the need to perform adequate surgical resection while preserving as much normal pancreatic parenchyma as possible. Neoplasms of the middle pancrea...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2006.12.023
更新日期：2007-04-01 00:00:00
