Abstract:
:Sirenomelia, or the mermaid syndrome, is the most extreme example of the caudal regression syndrome. It invariably presents with lower limb fusion, sacral and pelvic bony anomalies, absent external genitalia, anal imperforation, and renal agenesis or dysgenesis. Because of the resultant oligohydramnios, these infants most often have Potter's facies and pulmonary hypoplasia. There are approximately 300 cases reported in the literature, 15% of which are associated with twinning, most often monozygotic. The syndrome of caudal regression is thought to be the result of injury to the caudal mesoderm early in gestation. It has been suggested that the association of the most extreme form of caudal regression, sirenomelia, with monozygotic twinning may represent developmental arrest of the primitive streak, with creation of a second primitive streak that gives rise to the usually normal twin. The embryology of the various presentations of the caudal regression syndrome may be further delineated by studying infants with this dramatic and fatal syndrome.
journal_name
J Pediatr Surgjournal_title
Journal of pediatric surgeryauthors
Di Lorenzo M,Brandt ML,Veilleux Adoi
10.1016/0022-3468(91)90614-ysubject
Has Abstractpub_date
1991-11-01 00:00:00pages
1334-6issue
11eissn
0022-3468issn
1531-5037pii
S0022346891000726journal_volume
26pub_type
杂志文章abstract:BACKGROUND:Though patients with progressive familial intrahepatic cholestasis (PFIC) typically require liver transplantation, initial surgical treatment includes partial biliary diversion (PBD) to relieve jaundice-associated pruritus. This study was undertaken to describe long-term PFIC outcome data, which are currentl...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2010.02.010
更新日期:2010-05-01 00:00:00
abstract:BACKGROUND/PURPOSE:Closure of abdominal wall defects in children poses a challenge for pediatric surgeons. We describe a technique using tissue expanders placed either intraperitoneally or in the abdominal wall to aid in the reconstruction of a variety of complex abdominal wall defects. METHODS:The tissue expanders ar...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2010.11.020
更新日期:2011-02-01 00:00:00
abstract::Solid pseudopapillary tumors of the pancreas (SPTP) are very rare, and an SPTP arising in a pancreatic rest has been reported only 4 times previously and never in association with the jejunum. We report this unusual case of a 16 year old girl who presented with 4 days of intermittent, crampy abdominal pain and was fou...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2009.01.074
更新日期:2009-04-01 00:00:00
abstract::An infant girl with the megacystis microcolon intestinal hypoperistalsis syndrome is reported. The presence of polyhydramnios and the absence of upper urinary tract abnormalities, differentiate this patient from similar cases previously reported. Although the etiology of this disease is unknown, partial success with t...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(81)80571-4
更新日期:1981-10-01 00:00:00
abstract:BACKGROUND/PURPOSE:Intestinal resection is the most frequent surgical procedure for bowel stenoses in Crohn's disease (CD). Recurrence of strictures, particularly with ileocolonic disease, often requires resection of lengthy segments of bowel, potentially resulting in short bowel syndrome. Different techniques of stric...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/jpsu.2003.50173
更新日期:2003-05-01 00:00:00
abstract::A personal experience with 105 cloacal malformations is described, showing the wide range of anatomy that can occur with the urogenital sinus, the vagina(s), and the rectum, as well as in the external features of the perineum. Follow-up is presented on 98 patients, excluding 4 who died preoperatively, 2 who are preope...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/0022-3468(92)90393-l
更新日期:1992-07-01 00:00:00
abstract::Over 200 cases of colonic hemangioma have been reported, with more than 50% occurring in the rectosigmoid. These benign vascular neoplasms usually present in childhood, often with life-threatening hemorrhage. Lack of awareness of the condition continues to lead to errors in diagnosis and management, and may contribute...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(88)80018-6
更新日期:1988-11-01 00:00:00
abstract::In 2015, the Truth and Reconciliation Commission called for health care institutions to address structural barriers that affect Indigenous families seeking health care in Canada. Pediatric care centers in Canada have failed to adequately address these, resulting in continued negative and life-threatening impacts on In...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章,评审
doi:10.1016/j.jpedsurg.2020.01.029
更新日期:2020-05-01 00:00:00
abstract:OBJECTIVE:Malrotation with midgut volvulus is a time-sensitive pediatric surgical disease that requires emergent operative exploration to avoid bowel loss; however, it also requires specialized pediatric care. The purpose of this study was to identify disparities in bowel resection in children who underwent emergency s...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2019.01.061
更新日期:2019-09-01 00:00:00
abstract::A 12 yr old black male presented with a 3 day history of left inguinal and testicular pain associated with nausea, vomiting, and fever. Left groin exploration demonstrated a markedly inflammed patent process vaginalis containing purulent exudate. Concomitant abdominal exploration demonstrated a perforated gangrenous a...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/0022-3468(77)90417-1
更新日期:1977-10-01 00:00:00
abstract:BACKGROUND:Open Ladd's procedure is the gold standard for the correction of intestinal malrotation and laparoscopic approach remains controversial. This study aimed to evaluate our experience in laparoscopic management of malrotation. METHODS:Single center retrospective study including patients who underwent a laparos...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2018.09.023
更新日期:2019-09-01 00:00:00
abstract:BACKGROUND:Meconium plug syndrome (MPS) is associated with Hirschsprung disease (HD) in 13-38% of cases. This study sought to assess institutional variation in utilization of rectal biopsy in children with MPS and the likelihood of diagnosing HD in this population. METHODS:Patients with MPS on contrast enema in the fi...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2019.02.036
更新日期:2019-06-01 00:00:00
abstract::Although the survival for infants with abdominal wall defects (AWD) has dramatically improved, agreement on the optimum surgical approach has not been reached. From October 1970 through March 1983, 31 neonates with gastroschisis and 14 neonates with omphalocele were treated. Reduction of the herniated viscera with pri...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(83)80010-4
更新日期:1983-12-01 00:00:00
abstract:PURPOSE:The aim of this study was to identify the most accurate prenatal predictors of outcomes and need for fetal surgery for fetuses with high-risk lung masses and vascular tumors. METHODS:The records of all fetuses with high-risk lung mass (congenital cystic adenomatoid malformation-volume ratio > 1.6 or findings o...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2011.10.019
更新日期:2012-01-01 00:00:00
abstract::Small intestinal myoelectric activity has been studied extensively in adult humans and in many animal models. However, little is known about gut myoelectrical activity in newborns, a population susceptible to primary and secondary motility disorders. We report the development of a chronic neonatal piglet model for ass...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/0022-3468(90)90075-k
更新日期:1990-03-01 00:00:00
abstract:BACKGROUND/PURPOSE:Vascular anomalies are a diverse set of lesions with distinct clinical behaviors, whose biomolecular characteristics are largely undefined. Common hemangiomas proliferate during the first year of life, then involute at a variable pace over several years. Other vascular tumors may involute much more q...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2003.11.043
更新日期:2004-03-01 00:00:00
abstract:BACKGROUND/PURPOSE:Previous reports of laparoscopic repair of Morgagni hernias in children have involved relatively complex laparoscopic techniques. This report describes a simpler method of repair that we have applied to 4 children. METHODS:Four children with retrosternal (Morgagni) hernias underwent primary laparosc...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/jpsu.2003.50163
更新日期:2003-05-01 00:00:00
abstract:PURPOSE:The aim of this study is to determine the incidence of paratubal cysts (PTCs) in a pediatric and adolescent population. METHODS:An institutional review board-approved retrospective chart review was performed between the years of 1998 and 2008 at a single children's hospital. Inclusion criteria were met if path...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2011.04.054
更新日期:2011-11-01 00:00:00
abstract::The management of children with a mass in the head of the pancreas is not well defined. The medical records of 3 children with obstructive jaundice because of a mass in the head of the pancreas over a 4-year period were reviewed retrospectively. Abdominal ultrasonography and computed tomography showed intrahepatic and...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2006.02.029
更新日期:2006-06-01 00:00:00
abstract::The joining of widely separated proximal and distal esophageal segments in esophageal atresia remains a challenging problem. Livaditis introduced the operation of circular myotomy as an effective means of bridging such a wide gap. Three babies with esophageal atresia were successfully treated in this manner at the Hos...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(78)80453-9
更新日期:1978-08-01 00:00:00
abstract:PURPOSE:Ionizing radiation is used for the insertion of surgically implanted venous access devices (SIVADs) with children at the highest risk of cumulative radiation effects from these procedures. This study examines the radiation dose in a pediatric population during intraoperative radiological screening. METHODS:A r...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章,评审
doi:10.1016/j.jpedsurg.2015.04.017
更新日期:2015-07-01 00:00:00
abstract:BACKGROUND:The distribution of ganglion cells in the transition zone of Hirschsprung Disease (HD) colons is extremely variable. Determining the resection margin based on intraoperative biopsies may be imprecise. Multiphoton microscopy (MPM) is a novel imaging technology with the ability to visualize tissues in real tim...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2013.03.025
更新日期:2013-06-01 00:00:00
abstract::The authors report on an infant who had multifocal mesenchymal hamartoma of the right posterior chest wall. The tumors were found incidentally, on a chest radiograph, during routine evaluation for upper respiratory tract infection. Resection of both lesions with chest wall reconstruction was performed, with a good res...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(96)90684-3
更新日期:1996-05-01 00:00:00
abstract:BACKGROUND/PURPOSE:Percutaneous endoscopic gastrostomy (PEG) is of great benefit to a defined population of children, but gastrostomy has been implicated in causation or exacerbation of gastroesophageal reflux (GER). The aim of this study was to quantitatively and qualitatively analyze the effect of PEG on GER. METHOD...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1053/jpsu.2002.30267
更新日期:2002-02-01 00:00:00
abstract:BACKGROUND/PURPOSE:Acute Respiratory Distress Syndrome (ARDS) results in significant morbidity and mortality in pediatric trauma victims. The objective of this study was to determine risk factors and outcomes specifically related to pediatric trauma-associated ARDS (PT-ARDS). METHODS:A retrospective cohort (2007-2014)...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2018.07.005
更新日期:2019-07-01 00:00:00
abstract:PURPOSE:Advances in percutaneous endoscopic gastrostomy (PEG) and laparoscopic (LAP) techniques, including LAP-assisted PEG, offer alternatives to the standard open gastrostomy technique. This study compares the outcomes of the PEG and LAP techniques. METHODS:All gastrostomy tube placements were reviewed at our instit...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2010.02.079
更新日期:2010-06-01 00:00:00
abstract::Neuroblastomas located in the apex of the hemithorax or in the lower cervical region may make complete resection via a cervical or a thoracic approach difficult. The authors recently managed two patients with cervicothoracic neuroblastomas through an approach using the trap-door incision often applied in the setting o...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/0022-3468(95)90127-2
更新日期:1995-04-01 00:00:00
abstract:BACKGROUND:Hirschsprung disease (HSCR, Online Mendelian Inheritance in Man 142623) is a typical developmental disorder of the enteric nervous system in which ganglion cells fail to innervate the lower gastrointestinal tract during embryonic development. SOX10 gene is involved in the normal development of the enteric ne...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2011.05.017
更新日期:2011-10-01 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::Müllerian inhibiting substance (MIS), a gonadal hormone important in sexual differentiation, is high (10 to 70 ng/mL) in human male serum postnatally for several years before declining during the peripubertal period, but is undetectable in female serum until the onset of puberty. The sexually dimorphic secretion of MI...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/0022-3468(93)90245-g
更新日期:1993-03-01 00:00:00