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Pediatric Reports Jul 2023The term congenital diaphragmatic eventration (CDE) refers to an anatomical abnormality of the diaphragm. It is a very rare condition; however, early and prompt...
BACKGROUND
The term congenital diaphragmatic eventration (CDE) refers to an anatomical abnormality of the diaphragm. It is a very rare condition; however, early and prompt diagnosis is of very great importance due to possible life-threatening complications. Most severely affected patients are neonates, usually presented with respiratory distress symptoms. The aim of this study was to systematically review the existing literature and to consolidate data on CDE in neonates as well as to report a case of a neonate with congenital diaphragmatic eventration of the left hemidiaphragm and clinical signs and symptoms of the gastrointestinal tract.
METHODS
An electronic search of the PubMed and Scopus databases was performed regarding studies evaluating the clinical presentation, diagnosis methods, treatments, and outcomes of CDE in the neonatal population.
RESULTS
Data from 93 studies were integrated into our review, reporting 204 CDE cases, and according to them, the male/female ratio was 1/1 with a predominance of right-sided eventration. The diagnosis was primarily established by chest X-ray; surgical intervention was the most frequent treatment. The recurrence rate was 8.3% (9/109 cases).
CONCLUSIONS
Early and accurate diagnosis of CDE and repair of the diaphragm can prevent complications, reduce morbidity, and improve the quality of patient's life.
PubMed: 37606445
DOI: 10.3390/pediatric15030041 -
Journal of Clinical Medicine May 2023A congenital intrathoracic kidney (ITK) is a rare anomaly that is recognized to have four causes: renal ectopia with an intact diaphragm, diaphragmatic eventration,... (Review)
Review
INTRODUCTION
A congenital intrathoracic kidney (ITK) is a rare anomaly that is recognized to have four causes: renal ectopia with an intact diaphragm, diaphragmatic eventration, diaphragmatic hernia, and traumatic diaphragmatic rupture. We report a case of a prenatal-diagnosed ITK related to a congenital diaphragmatic hernia (CDH) and conducted a systematic review of all cases of the prenatal diagnosis of this association.
CASE PRESENTATION
A fetal ultrasound scan at 22 gestational weeks showed left CDH and ITK, hyperechoic left lung parenchyma, and mediastinal shift. The fetal echocardiography and karyotype were normal. Magnetic resonance imaging at 30 gestational weeks confirmed the ultrasound suspicion of left CDH in association with bowel and left kidney herniation. The fetal growth, amniotic fluid, and Doppler indices remained within the normal range over time. The woman delivered the newborn via an at-term spontaneous vaginal delivery. The newborn was stabilized and underwent non-urgent surgical correction; the postoperative course was uneventful.
CONCLUSIONS
CDH is the rarest cause of ITK; we found only eleven cases describing this association. The mean gestational age at diagnosis was 29 ± 4 weeks and 4 days. There were seven cases of right and four cases of left CDH. There were associated anomalies in only three fetuses. All women delivered live babies, the herniated kidneys showed no functional damage after their surgical correction, and the prognosis was favorable after surgical repair. The prenatal diagnosis and counseling of this condition are important in planning adequate prenatal and postnatal management in order to improve neonatal outcomes.
PubMed: 37297803
DOI: 10.3390/jcm12113608 -
Ultrasound in Obstetrics & Gynecology :... May 2010Intrathoracic liver herniation (ILH) is being used to estimate prognosis and hence guide antenatal interventions in fetal congenital diaphragmatic hernia (CDH). However,... (Meta-Analysis)
Meta-Analysis Review
OBJECTIVES
Intrathoracic liver herniation (ILH) is being used to estimate prognosis and hence guide antenatal interventions in fetal congenital diaphragmatic hernia (CDH). However, the literature regarding its utility in this role is conflicting. This review systematically examines the currently available evidence of ILH use in fetal CDH.
METHODS
MEDLINE and EMBASE databases were searched for the terms ((congenital diaphragmatic hernia) OR CDH) AND liver. Inclusion criteria were human case series of fetuses diagnosed with CDH using either ultrasound or magnetic resonance imaging. Included studies were required to have reported the antenatal liver position and the outcome (survival or not). Case reports, reviews and eventration series were excluded. Studies reporting similar cases from the same center over an overlapping time period were considered duplicates; only the larger of the studies were therefore included. Absolute totals were extracted and sums calculated. Fisher's exact test (FET) was used to compare survival rates in different groups.
RESULTS
The original search retrieved 338 studies. Applying inclusion/exclusion criteria and removing duplicates left 21 case series in 20 studies. Retrieved studies differed in the definitions of liver herniation, survival and treatment modality. In total, there were 407 fetuses in the liver-up (herniated) and 303 in the liver-down (not herniated) groups. Survival rates were 45.4% and 73.9%, respectively. The difference was statistically significant (FET = 56.4, P < 0.005). Sensitivity analysis for cases that had only conventional postnatal treatment was still significant (FET = 52.8, P < 0.005).
CONCLUSIONS
Liver herniation is associated with poorer prognosis in fetal CDH. Grading liver herniation or using it as part of a panel of markers may enhance the value of liver herniation as a prognostic test in fetal CDH.
Topics: Female; Gestational Age; Hernia, Diaphragmatic; Hernias, Diaphragmatic, Congenital; Humans; Liver Diseases; Pregnancy; Prenatal Diagnosis; Prognosis
PubMed: 20178116
DOI: 10.1002/uog.7586