Abstract：
PURPOSE:The congenital central alveolar hypoventilation syndrome (CCAHS), also known as Ondine curse, is characterized by the absence of adequate autonomic control of respiration. The purpose of our study is to review our 20-year experience with diaphragmatic pacing as a treatment modality for CCAHS. METHODS:After obtaining institutional review board approval, the medical records of all 6 patients who currently are in the diaphragmatic pacing program at the Montreal Children's Hospital, Montreal, QC, Canada were reviewed. In addition to demographic details, associated anomalies were noted. Data regarding age at surgery, technical approach, and short- and long-term complications, including equipment failure and replacement, were noted. We were interested in the long-term outcome for this group of patients, particularly their quality of life. RESULTS:Our group of patients is composed of 4 females and 2 males. Their age ranges between 4 and 23 years. They were operated on at an average age of 47.8 months. The surgical approach used was mainly bilateral axillary thoracotomy. Internal component failure is the most common complication. All patients are ventilator free during the day. They all are active and productive, either attending school or working full time. CONCLUSION:Diaphragmatic pacing is an effective treatment of Ondine disease. As equipment improved, there is much less need for replacement of components. Patients can lead a much more normal existence by being ventilator free at least during the day, enabling them to participate in normal daily activities.
journal_name
J Pediatr Surgjournal_title
Journal of pediatric surgeryauthors
Ali A,Flageole Hdoi
10.1016/j.jpedsurg.2007.12.013subject
Has Abstractpub_date
2008-05-01 00:00:00pages
792-6issue
5eissn
0022-3468issn
1531-5037pii
S0022-3468(07)00947-5journal_volume
43pub_type
杂志文章abstract：BACKGROUND:Perceptions toward surgical innovations are critical to the social processes that drive technology adoption. This study aims to capture attitudes of early adopter pediatric surgeons toward robotic technologies in order to clarify 1) specific features that are driving appeal, 2) limiting factors that are acti...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2014.05.017
更新日期：2014-10-01 00:00:00
abstract：:Partial drainage of a hepatic hydatid cyst into a pericystic blood vessel caused anaphylactic shock following minimal blunt abdominal trauma in a child. A case report including diagnostic procedures and treatment is presented. ...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/0022-3468(91)90916-h
更新日期：1991-02-01 00:00:00
abstract：:The records of 207 patients evaluated and treated for breast abnormalities during a 10-yr period were reviewed. Patients ranged in age from 1 wk through 16 yr. Seventy-eight per cent were female. Operative procedures were performed in 134 patients (64%). Most common diagnoses were fibroadenoma, premature thelarche, an...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(76)80187-x
更新日期：1976-06-01 00:00:00
abstract：:A full-term baby boy with a segmental defect of the ileal musculature associated with terminal ileal and biliary atresia is presented. The newborn had a dilated loop of the ileum 30 cm proximal to the ileal atretic site. Pathological study results showed absence of the intestinal musculature with relatively intact muc...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(98)90101-4
更新日期：1998-03-01 00:00:00
abstract：PURPOSE:Surgical correction of torticollis is occasionally necessary to curtail the facial deformity that can result from this condition. The resultant neck scar can be of suboptimal cosmesis, with consequent psychological distress for the child. We have previously described an endoscopic approach to forehead and brow ...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2007.10.008
更新日期：2008-03-01 00:00:00
abstract：PURPOSE:To determine whether hemodynamically significant bleeding occurs after pediatric femur fractures. METHOD:A retrospective chart review was performed; demographic and injury data were collected for all patients with the diagnosis of femur fracture over a 30-month period at a level/ pediatric trauma center. Inclu...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(96)90827-1
更新日期：1996-10-01 00:00:00
abstract：:Cystic duplication of the duodenum is a rare anomaly of the gastrointestinal tract. Patients may present in early childhood with symptoms of upper intestinal obstruction. Successful, staged management of a cystic duodenal duplication in an 880-g infant is described. ...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/0022-3468(93)90106-u
更新日期：1993-12-01 00:00:00
abstract：:Heterotopic gastric mucosa (HGM) is a rare, but acknowledged source of gastrointestinal pathology in pediatric patients. Sometimes clinically confused with a Meckel's diverticulum, HGM diagnosis is often made postoperatively by pathology. We present a case of jejunal HGM with a positive technetium pertechnetate scan i...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2015.01.016
更新日期：2015-05-01 00:00:00
abstract：BACKGROUND:Previous studies of recombinant human thrombin (rThrombin) enrolled adult and adolescent patients. This phase 4, open-label, single-group study was conducted in pediatric patients undergoing synchronous burn wound excision and skin grafting to provide information regarding the safety and immunogenicity of rT...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2011.05.022
更新日期：2011-10-01 00:00:00
abstract：INTRODUCTION:Bacterial intestinal translocation plays an important role in neonatal sepsis. We aimed to elucidate the importance of such translocation in causing central line associated blood stream infection (CLABSI) in patients undergoing gastrointestinal surgery (GIS). METHODS:Using a database of pediatric patients...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2020.02.012
更新日期：2020-08-01 00:00:00
abstract：:We report three additional cases of primary extrarenal Wilms' tumor and review those cases previously documented. Analysis of the location, histopathology, treatment, and survival of these cases supports the following conclusions: Wilms' tumor may occur in an extrarenal location without primary renal involvement and m...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(89)80568-8
更新日期：1989-12-01 00:00:00
abstract：:This report describes a case of thoracoscopic repair of esophageal atresia with a rare type D tracheoesophageal fistula in a child with complex congenital heart disease. We demonstrate the feasibility of thoracoscopic repair and anesthetic management in a child with complex congenital heart disease. ...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2007.05.013
更新日期：2007-09-01 00:00:00
abstract：PURPOSE:The management of patients with short bowel syndrome is complicated by the paucity of methods to assess in vivo the absorptive capacity of the remaining bowel. The purpose of this experiment was to assess the feasibility of using urinary recovery of 3-0 methylglucose (3-0 MG) as a quantitative measure of carboh...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1053/jpsu.2001.22951
更新日期：2001-05-01 00:00:00
abstract：BACKGROUND/PURPOSE:The role of serum gamma-glutamyl transpeptidase (GGT) levels in predicting clinical outcomes after Kasai portoenterostomy (KPE) is unknown. This study analyzed whether postoperative GGT along with the aspartate aminotransferase-to-platelet ratio index (APRi) predicted prognosis of biliary atresia (BA...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2017.10.001
更新日期：2018-03-01 00:00:00
abstract：:Three newborn boys presented with features suggestive of classic low anorectal abnormality. However, during surgery they were found to have an intermediate anorectal abnormality and a rectoperineal fistula rather than an anocutaneous fistula. The surgical treatment of these infants is discussed. ...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/0022-3468(94)90085-x
更新日期：1994-04-01 00:00:00
abstract：BACKGROUND:Extralobar pulmonary sequestrations (EPS) are a rare benign congenital bronchopulmonary foregut malformation. Complete resection is necessary to confirm the diagnosis with histopathologic examination. The aim of this study was to describe the laparoscopic minimally invasive surgery (MIS) for a small series o...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2010.06.033
更新日期：2010-11-01 00:00:00
abstract：:Tracheal agenesis is a rare and usually lethal congenital malformation of the forgut. Although some infants can be resuscitated with an intra-esophageal intubation temporarily, long-term airway management is difficult because of the collapsing airway. We report a long-term survivor with tracheal agenesis in whom a Gor...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2008.06.013
更新日期：2008-10-01 00:00:00
abstract：:In this case of bronchopulmonary foregut malformation (BPFM), the bronchus of the anomalous pulmonary tissue was reconstructed and tracheoplasty for associated congenital tracheal stenosis was performed at 5 days of age. After reconstruction the right lung was ventilated, which was followed by improved blood gas data....
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/0022-3468(95)90418-2
更新日期：1995-10-01 00:00:00
abstract：:According to Gitlin, alpha-fetoprotein (AFP), albumin, prealbumin, alpha-1-antitrypsin and transferrin are normal products of the human yolk sac. They are expected to reappear in human endodermal sinus tumor (yolk sac tumor). The synthesis of alpha-fetoprotein and other serum proteins by human endodermal sinus tumor w...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(78)80207-3
更新日期：1978-02-01 00:00:00
abstract：:Unilateral cryptorchidism was induced in newborn Wistar rat pups. Using DNA flow cytometry to compare scrotal testes of control and cryptorchid animals, there was a significant difference decrease in the haploid cell population in cryptorchid animals from 40 days of age. Orchidopexy was performed at 30 days (at the ti...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/0022-3468(91)90994-5
更新日期：1991-04-01 00:00:00
abstract：:The authors report the rare case of a dipygus monster. The infant had an omphalocele, a secondary pelvis with two limbs, an accessory pelvic kidney, and two bladders. At operation, the secondary pelvis and extremities were removed, and the omphalocele was repaired. The postoperative course was complicated by wound inf...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(79)80140-2
更新日期：1979-10-01 00:00:00
abstract：:Cystic lesions of the spleen are uncommon, about 600 cases being reported in the world literature. This report concerns three pediatric patients treated by partial splenectomy for benign cyst. In the world literature most of the cystic lesions of the spleen are treated by splenectomy and more recently by partial splen...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(86)80357-8
更新日期：1986-09-01 00:00:00
abstract：BACKGROUND/PURPOSE:Inguinal hernia repair and orchidopexy are among the most common operations in boys. The impact on future fertility has not been conclusively defined. This study evaluates sperm quality after previous inguinal surgery. METHODS:Spermiograms of men with a desire to conceive children were analyzed. His...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2018.10.012
更新日期：2019-01-01 00:00:00
abstract：:We report our initial experience and describe the operative technique used in three patients who underwent laparoscopic splenectomy for hematologic disorders between September 1991 and February 1992. No early or late complications have been found at an average follow-up of 9 months. All have remained asymptomatic and ...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/0022-3468(93)90033-h
更新日期：1993-05-01 00:00:00
abstract：:Vaginal reflux during voiding cystourethrography (VCUG) is a common finding in young females. Reflux of urine into the uterus, fallopian tubes, and peritoneal cavity documented by contrast studies is rare and considered abnormal. Chronic retrograde flow of urine and its contaminants through an incompetent cervical os ...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(83)80200-0
更新日期：1983-08-01 00:00:00
abstract：BACKGROUND:Abdominal compartment syndrome (ACS) is a pathological increase of the intra-abdominal pressure (IAP) with dysfunction of one or more organs. There is lack of clarity in neonates regarding what intravesical pressure (IVP) value, a surrogate marker for IAP, indicates the need for intervention for ACS. METHOD...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2019.08.003
更新日期：2020-07-01 00:00:00
abstract：:During the years 1982 through 1991, stitch granulomata complicated 15 of 2,447 inguinal herniotomies. The lesions were diagnosed 3 to 120 months (mean +/- SE, 2.46 +/- 0.65 years) following surgery. Granulomata were associated with male sex (P = .02), emergency herniotomy (P = 0.016), surgery during the neonatal perio...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/0022-3468(93)90442-n
更新日期：1993-11-01 00:00:00
abstract：:Four adolescent girls, aged 12 to 14 years, were seen for evaluation of spontaneous nipple discharge, two of whom had associated breast lumps in the ipsilateral breast. Clinical examination showed the discharge to be arising from one or several of Montgomery's areolar tubercles, with the breast lumps localized to the ...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(88)80409-3
更新日期：1988-08-01 00:00:00
abstract：BACKGROUND:Mucosal atrophy and bowel shortening are the hallmark of proximal intestinal diversion for extensive necrotizing enterocolitis (NEC) or Thiry-Vella fistulas (TVF), in which the ends of a defunctionalized loop of intestine are exteriorized as stomas. Recombinant human interleukin-11 (rhIL-11) is a pleiotropic...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1053/jpsu.2000.7826
更新日期：2000-07-01 00:00:00
abstract：:Of 33 infants with pelviureteric obstruction, most presented in the first month of life with an abdominal mass and most were males. Half of these patients had bilateral renal pathology. The association between cystic dysplasia in the hydronephrotic kidney and contralateral cystic renal abnormality in patients with uni...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/0022-3468(76)90170-6
更新日期：1976-02-01 00:00:00
