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Postgraduate Medical Journal May 1986
Topics: Esophagitis, Peptic; Hernia, Diaphragmatic; Hernia, Diaphragmatic, Traumatic; Humans; Stomach Volvulus
PubMed: 3763538
DOI: 10.1136/pgmj.62.727.325 -
Ultrasound in Obstetrics & Gynecology :... Aug 1998The purpose of this study was to assess perinatal outcome in cases of prenatally diagnosed congenital diaphragmatic hernia and identify the prognostic value of various...
OBJECTIVE
The purpose of this study was to assess perinatal outcome in cases of prenatally diagnosed congenital diaphragmatic hernia and identify the prognostic value of various prenatal factors.
DESIGN
Retrospective review of fetal medicine, obstetric and histopathology records in all 34 cases of congenital diaphragmatic hernia identified prenatally between 1992 and 1996 at a tertiary referral fetal medicine unit.
RESULTS
Overall survival was 18%. None of those with bilateral (0/1) or right-sided (0/5) congenital diaphragmatic hernia survived, whereas 21% (6/28) of those with left-sided hernias survived. Prenatal karyotyping was performed in 71% (n = 24) and five chromosomal abnormalities were identified. The pregnancy was terminated in 15 cases (44%). In the 19 continuing pregnancies, the survival rate was 32% (6/19). In those with an isolated congenital diaphragmatic hernia, the survival rate was 38% (5/13), and in those in which the hernia was isolated and left-sided, the survival rate was 56% (5/9). In ongoing pregnancies diagnosed after 24 weeks, the survival rate was 33% (2/6) compared with 31% (4/13) in pregnancies diagnosed at < or = 24 weeks. Of those infants who had surgical repair, six out of seven (86%) survived.
CONCLUSIONS
These data clearly demonstrate an improved prognosis in fetuses with an isolated left-sided congenital diaphragmatic hernia.
Topics: Adolescent; Adult; Female; Hernia, Diaphragmatic; Hernias, Diaphragmatic, Congenital; Humans; Infant, Newborn; Pregnancy; Pregnancy Outcome; Prognosis; Retrospective Studies; Survival Rate; Ultrasonography, Prenatal
PubMed: 9744054
DOI: 10.1046/j.1469-0705.1998.12020107.x -
Asian Journal of Surgery Oct 2003Congenital diaphragmatic hernia (CDH) has a mortality rate of up to 77% despite optimal pre- and postnatal care. Fetuses with liver herniation, a low lung-to-head ratio,... (Review)
Review
Congenital diaphragmatic hernia (CDH) has a mortality rate of up to 77% despite optimal pre- and postnatal care. Fetuses with liver herniation, a low lung-to-head ratio, and an early diagnosis before 24 weeks have a particularly poor prognosis. In utero open repair of these fetuses does not improve patient survival. The PLUG (Plug the Lung Until it Grows) technique was reported to be able to reverse pulmonary hypoplasia in CDH. A foam plug or a titanium clip is used and the trachea can be unplugged using Ex Utero Intrapartum Tracheoplasty (EXIT) at birth. Since hysterotomy causes premature labour, a video-fetoscopic intrauterine technique of tracheal occlusion called Fetendo-PLUG was developed. Compared to those who receive standard postnatal care or fetal tracheal occlusion via open hysterotomy, patients who undergo Fetendo-PLUG are reported to have a higher survival rate of 75% and fewer fetal and maternal complications. A recent refinement is to use a detachable balloon for intratracheal occlusion through a single 5 mm port under real-time ultrasound guidance. Without the need for neck dissection, injury to the recurrent laryngeal nerves and trachea and vocal cord paresis can be minimized. The result of this form of treatment for CDH is promising, but further refinement of fetal instrumentation and development of effective tocolytic drugs are still required.
Topics: Animals; Female; Follow-Up Studies; Gestational Age; Hernia, Diaphragmatic; Hernias, Diaphragmatic, Congenital; Humans; Pregnancy; Pregnancy Outcome; Pregnancy, Animal; Risk Assessment; Sheep; Treatment Outcome; Ultrasonography, Prenatal
PubMed: 14530115
DOI: 10.1016/S1015-9584(09)60314-6 -
BMJ Case Reports Nov 2012Acute gastric volvulus associated with congenital diaphragmatic hernia is an unusual surgical emergency and its presentation in adulthood is extremely rare. Gastric... (Review)
Review
Acute gastric volvulus associated with congenital diaphragmatic hernia is an unusual surgical emergency and its presentation in adulthood is extremely rare. Gastric volvulus is an uncommon condition which is difficult to diagnose and treat. This case describes a middle aged man presenting with post-traumatic acute abdomen and finally being diagnosed as a case of gastric volvulus due to congenital diaphragmatic hernia which was till now asymptomatic and undiagnosed. Clinical examination and chest x-ray was suggestive of hiatus hernia. Contrast-enhanced CT of thorax and abdomen revealed hiatus hernia and gastric volvulus. A provisional diagnosis of post-traumatic hiatus hernia with associated gastric volvulus was made and was operated on an emergency basis. Surprisingly, the intraoperative findings revealed significant adhesions near the diaphragm suggestive of congenital diaphragmatic hernia. Hence, a diagnosis of gastric volvulus due to congenital diaphragmatic hernia was made which remained asymptomatic and undiagnosed till adulthood. He responded well to operative management and remained asymptomatic afterwards.
Topics: Diagnosis, Differential; Hernia, Diaphragmatic; Hernia, Hiatal; Hernias, Diaphragmatic, Congenital; Humans; Male; Middle Aged; Radiography; Stomach Volvulus
PubMed: 23195824
DOI: 10.1136/bcr-2012-007332 -
Orphanet Journal of Rare Diseases Jun 2013The development of new therapeutics has led to progress in the early management of congenital diaphragmatic hernia (CDH) in pediatric intensive care units (PICU). Little...
BACKGROUND
The development of new therapeutics has led to progress in the early management of congenital diaphragmatic hernia (CDH) in pediatric intensive care units (PICU). Little is known about the impact on the quality of life (QoL) of children and their family. The aim of this study was to assess the impact of CDH treated according to the most recent concepts and methods outlined above on child survivors' QoL and their parents' QoL.
PATIENTS AND METHODS
This study incorporated a cross-sectional design performed in two PICU (Marseille, France). Families of CDH survivors born between 1999 and 2008 were eligible. The following data were recorded: socio-demographics, antenatal history and delivery, initial hospitalization history. Self-reported data were collected by mail, including current clinical problems of the children (13-symptom list), children's QoL (Kidscreen-27 questionnaire), and parents' QoL (Short-Form 36 questionnaire). Children's QoL score was compared with controls and QoL of survivors of childhood leukemia. Parent's QoL was compared with controls. Non-parametric statistics were employed.
RESULTS
Forty-two families agreed to participate and questionnaires were completed by 32 of them. Twenty-one children had a current clinical problems related to CDH. All the QoL scores of CHD survivors were significantly lower compared with controls. The physical well-being dimension was significantly higher for CHD survivors compared with survivors of childhood leukemia. Gastro-esophageal reflux at discharge, antenatal diagnosis, length of stay in the PICU, and neuropsychological and respiratory issues significantly impacted QoL scores of children. The parents of CHD survivors had significantly poorer score in emotional role dimension compared with controls.
CONCLUSION
The impact of CDH on QoL seems to be important and must be understood by clinicians who treat these children and their parents.
Topics: Adult; Child; Child, Preschool; Female; France; Health Status; Hernia, Diaphragmatic; Hernias, Diaphragmatic, Congenital; Humans; Intensive Care Units, Pediatric; Male; Parents; Quality of Life; Surveys and Questionnaires; Survivors
PubMed: 23786966
DOI: 10.1186/1750-1172-8-89 -
Ultrasound in Obstetrics & Gynecology :... Mar 2013To investigate the prognostic value of a hernia sac in isolated congenital diaphragmatic hernia (CDH).
OBJECTIVE
To investigate the prognostic value of a hernia sac in isolated congenital diaphragmatic hernia (CDH).
METHODS
Our database was searched to identify all consecutive cases of CDH referred to our fetal medicine unit between January 2004 and August 2011. Presence or absence of a hernia sac was assessed in liveborn cases using surgery or postnatal autopsy reports. We studied the correlation between the presence of a hernia sac and prenatal findings and perinatal morbidity and mortality.
RESULTS
Over the study period, there were 70 cases with isolated CDH born alive in which either a surgery or autopsy report was available. Neonatal death, either preoperative or postoperative, occurred in 1/18 (5.6%) infants with a hernia sac and in 17/52 (32.7%) cases without a hernia sac (P = 0.03). Patients with a hernia sac had a significantly higher observed to expected pulmonary volume on prenatal magnetic resonance imaging (51.9 vs 39.3%, P = 0.01). Neonatal morbidity in surviving infants was lower in the group with a hernia sac, although not significantly.
CONCLUSION
The presence of a hernia sac is associated with a higher pulmonary volume and a better overall prognosis for CDH.
Topics: Female; Hernia, Diaphragmatic; Hernias, Diaphragmatic, Congenital; Humans; Infant, Newborn; Infant, Newborn, Diseases; Magnetic Resonance Imaging; Pregnancy; Prenatal Diagnosis; Prognosis; Retrospective Studies; Survival Rate
PubMed: 22605546
DOI: 10.1002/uog.11189 -
Annals of Surgery Oct 1992One hundred ten infants with congenital diaphragmatic hernia (CDH) developed life-threatening respiratory distress in the first 6 hours of life. Associated anomalies...
One hundred ten infants with congenital diaphragmatic hernia (CDH) developed life-threatening respiratory distress in the first 6 hours of life. Associated anomalies were present in 33%. Twenty-eight of 65 infants (43%) treated before 1987 (pre-extracorporeal membrane oxygenation [ECMO] era) survived after immediate CDH repair, and mechanical ventilation with or without pharmacologic support. Only two of 16 (12.5%) infants requiring a prosthetic diaphragmatic patch survived. Since 1987, 31 of 46 (67.4%) infants with birth weight, gestational age, and severity of illness similar to the pre-1987 group survived. All patients were immediately intubated and ventilated. Seven (four with lethal chromosomal anomalies) infants died before treatment, and 30 stabilized (partial pressure of carbon dioxide [PCO2] < 50; partial pressure of oxygen [PO2] > 100; pH > 7.3) and underwent delayed CDH repair at 5 to 72 hours. Fifteen did well on conventional support and survived. Fifteen infants deteriorated after operation: 11 were placed on ECMO with eight survivors, and four infants were not considered ECMO candidates. Nine babies failed to stabilize initially and were placed on ECMO before CDH repair (alveolar-arterial gradient > 600 and oxygenation index > 40), and seven survived. The overall survival rate was 80% at 3 months in this ECMO-treated group. Early mortality was due to inability to wean from ECMO (one), intracranial hemorrhage (one), liver injury (one), and pulmonary hypoplasia (one). Nine of 11 babies requiring a prosthetic patch in the post-1987 ECMO group survived (81.8%). There were three late post-ECMO deaths (3 to 18 months) of right heart failure (two) and sepsis (one). Symptomatic gastroesophageal reflux occurred in nine cases, six requiring a fundoplication in the bypass babies. Recurrent diaphragmatic hernia occurred in nine cases (five ECMO). The overall survival rate was significantly improved in the delayed repair/ECMO group (67% versus 43%; p < 0.05) and was most noticeable in infants requiring a prosthetic diaphragm (81.2% versus 12.5%; p < 0.005). These data indicate that early stabilization, delayed repair, and ECMO improve survival in high-risk CDH. Early deaths are related to pulmonary hypertension and can be reversed by ECMO.
Topics: Extracorporeal Membrane Oxygenation; Female; Hernia, Diaphragmatic; Humans; Infant, Newborn; Male; Respiratory Insufficiency; Survival Rate; Time Factors
PubMed: 1417195
DOI: 10.1097/00000658-199210000-00009 -
BMJ Case Reports Jan 2013Osteogenesis imperfecta and congenital diaphragmatic hernia are both conditions that can occur due to genetic mutation. We present the first case to be reported of a...
Osteogenesis imperfecta and congenital diaphragmatic hernia are both conditions that can occur due to genetic mutation. We present the first case to be reported of a child with both osteogenesis imperfecta and congenital diaphragmatic hernias, showing that the incidence of this presentation may be more than chance.
Topics: Abnormalities, Multiple; Diagnosis, Differential; Digestive System Surgical Procedures; Follow-Up Studies; Hernia, Diaphragmatic; Hernias, Diaphragmatic, Congenital; Humans; Infant; Male; Osteogenesis Imperfecta; Tomography, X-Ray Computed
PubMed: 23345474
DOI: 10.1136/bcr-2012-007341 -
The Journal of Thoracic and... Nov 1996From 1985 to 1993, 49 patients (35 women and 14 men) with diaphragmatic hernia and associated anemia underwent surgical repair. The median age was 64.5 years (range 24...
From 1985 to 1993, 49 patients (35 women and 14 men) with diaphragmatic hernia and associated anemia underwent surgical repair. The median age was 64.5 years (range 24 to 84 years). Hematologic and gastroenterologic evaluations revealed no other potential cause of bleeding. Each patient had a diaphragmatic hernia. The median time between the diagnosis of anemia and surgical repair was 36 months (range 1 to 334 months). Forty-five patients (91.8%) had received replacement therapy, including iron for 43 and blood transfusions for 32 (median 6 units; range 2 to 70 units). Forty-six patients (93.9%) had symptoms: heartburn in 28, early satiety with bloating in 19, regurgitation in 11, dysphagia in 7, and aspiration in 4. Preoperative upper gastrointestinal endoscopic evaluation demonstrated gastric erosions at the level of the hiatus in 22 patients (44.9%), esophagitis in 7, stenosis in 1, and Barrett's disease in 1. An uncut Collis-Nissen fundoplication was performed in 44 patients, Belsey fundoplication in 2, a cut Collis-Nissen fundoplication, Nissen fundoplication, and Hill repair in 1 each. There was one operative death (2% mortality). Complications occurred in 18 patients (36.7%). Follow-up was complete and ranged from 4 to 103 months (median 63 months). Forty-five patients (91.8%) had resolution of their anemia. Functional results were excellent in 40 patients (81.6%), good in 2 (4.1%), fair in 4 (8.2%), and poor in 3 (6.1%). In most patients with diaphragmatic hernia and associated anemia refractory to medical treatment, surgical repair can result in successful resolution of the anemia.
Topics: Adult; Aged; Aged, 80 and over; Anemia, Refractory; Endoscopy, Gastrointestinal; Female; Fundoplication; Hernia, Diaphragmatic; Humans; Male; Middle Aged; Radiography; Retrospective Studies; Treatment Outcome
PubMed: 8911332
DOI: 10.1016/S0022-5223(96)70149-6 -
Medicine Apr 2018Diaphragmatic hernia (DH) in pediatrics following living donor liver transplantation (LDLT) has been seldom reported in the past. (Review)
Review
RATIONALE
Diaphragmatic hernia (DH) in pediatrics following living donor liver transplantation (LDLT) has been seldom reported in the past.
PATIENT CONCERNS
We report successful diagnosis and treatment of three pediatric cases with DH secondary to LDLT, discuss the possible etiology, and review the relevant literature.
DIAGNOSES
The primary disease was biliary atresia and DH was diagnosed by computed tomography scan or x-ray of chest.
INTERVENTIONS
Laparotomy was performed successfully to repair the DH.
OUTCOMES
The respiratory and digestive function was gradually recovered in 1 to 2 weeks after repair operation. In 2 to 8 months follow-up, patients were asymptomatic without any respiratory or digestive complications.
LESSONS
DH post-LDLT should be recognized as a possible complication when a left lateral segment graft is used. Careful clinical examination and prompt surgery could minimize complications.
Topics: Biliary Atresia; Child, Preschool; Female; Hernia, Diaphragmatic; Humans; Infant; Liver Transplantation; Living Donors; Male; Postoperative Complications; Prognosis; Reoperation
PubMed: 29642174
DOI: 10.1097/MD.0000000000010346