Right congenital diaphragmatic hernia a well-known pathology?

Abstract

In right congenital diaphragmatic hernia (RCDH), several clinical diagnostic pitfalls are possible and should be known to those caring for infants and children with this disorder. The records of the 18 patients at Hotel Dieu de France Hospital with a history of CDH between 1990 and 1999 were collected; those of the ten who had a RCDH were reviewed retrospectively. The mean age at diagnosis was 6 months; the male-to-female ratio was 2:3. The delay between the first symptom and the diagnosis ranged between 0 and 10.5 months (mean 4.5 months). An acute presentation was observed in four cases, consisting of respiratory distress in three; the 4th presented with gastric volvulus and intestinal obstruction. The presenting symptoms were mild in four cases; recurrent respiratory infections in three and failure to thrive in one. The diagnosis was incidental in two cases during the evaluation of respiratory symptoms attributed to an atrial septal defect. The radiologic findings provided by a chest radiograph (CxR) were sufficient to make an accurate diagnosis in eight cases and peritoneography was useful in one. In six cases, the presenting CxR had been misinterpreted as normal or acute lobar pneumonia. Pathologic findings at surgery consisted of lateral and posterior right diaphragmatic defects in nine cases; the defect was lateral and anterior in one. A hernia sac was found in seven cases; malrotation was present in three. Surgical correction was done by an abdominal approach in nine cases and a thoracic approach in one. The diaphragmatic defect was repaired by transverse closure in six cases, diaphragm plication in three and prosthetic closure in one. The postoperative outcome was uneventful in eight cases. Two patients died. Thus, RCDH seems to cause less severe symptoms than left-sided LCDH. It usually manifests beyond the neonatal period as respiratory or gastrointestinal symptoms. The diagnosis should be made easily by a CxR. The presence of a hernia sac correlated with a mild presentation. An abdominal surgical approach is preferred.

Redefining outcomes in right congenital diaphragmatic hernia

“…Predictors of disease severity and survival have been proposed from these data ( Table 1), but it remains unclear whether they are applicable to infants with rightsided defects. Available R-CDH series are underpowered to draw conclusions about this less prevalent subpopulation [21,23,25]. We report a 16-year, single-institution experience with 40 R-CDH and 227 L-CDH patients to better define R-CDH outcome and determine whether predictors of disease severity in R-CDH mimic those in the more prevalent L-CDH population.…”

“…Although reports with a specific focus on R-CDH [24,25] have recently become available and have provided new insights into R-CDH behavior, they lack an L-CDH control group and cannot identify factors that may be responsible for disparate right-left CDH outcomes. Lastly, some R-CDH studies are not applicable to the management of neonates, as their data include analyses of older children with R-CDH [23]. To address these limitations and clarify inconsistencies in the literature regarding R-CDH outcome, we have reviewed our institutional CDH database with specific focus on R-CDH to determine whether factors traditionally linked to poor prognosis in L-CDH also apply to R-CDH.…”

“…Such correlations are biased to reflect the behavior of more prevalent left-sided CDH (L-CDH) and are less applicable to the underrepresented subpopulation of right-sided CDH (R-CDH). Published R-CDH outcome data are therefore largely inconsistent [3,15,17,[21][22][23][24].…”

Right-sided congenital diaphragmatic hernia: high utilization of extracorporeal membrane oxygenation and high survival

“…These series give varying rates of survival, with some reporting a higher mortality for R-CDH than leftsided CDH (L-CDH), whereas others report a lower mortality for R-CDH [3,4,[8][9][10]. Similarly, some congenital anomalies have been reported as more common in R-CDH than L-CDH, whereas other anomalies have similar frequencies in each form [11,12].…”

Treatment of a case with right-sided diaphragmatic hernia associated with an abnormal vessel communication between a herniated liver and the right lung

“…The male to female ratio is about 1:1.25 [3]. The characteristics of right-sided CDH have been described less thoroughly than left-sided CDH, and rightsided CDH appears to have some pathologic aspects that differ from left-sided CDH [5,6].…”