Surgical Complications in Children with CDH: A Multivariate Analysis.

INTRODUCTION: Several medical and surgical improvements in the treatment of congenital diaphragmatic hernia (CDH) patients have led to a higher survival rate. However, some of these improvements also lead to an increased morbidity rate. This study aims to determine the contribution different medical and surgical treatments have had on the development of surgical complications. METHOD: All CDH patients treated in a single centre between 2000 and 2015 were retrospectively evaluated. Multivariate logistic regression was used to estimate the independent effects of several treatment options that could influence the surgical outcome by adjustment for multiple risk factors. RESULTS: Sixty of the 197 surgically repaired CDH patients had surgical complications. There were more haemorrhagic complications in the ECMO compared to non-ECMO group (27% vs. 2%, p < 0.001). The use of inhaled nitric oxide was also significantly related to haemorrhage (OR = 13.0 (95% CI 1.1-159)). After adjustment for other risk factors, chylothorax was neither significantly associated with ECMO treatment (OR = 1.6 (95% CI 0.5-5.2) nor with patch repair (OR = 2.1: 95% CI 0.7-6.1). A recurrence occurred more often in patients with pulmonary hypertension (OR = 10.0 (95% CI 1.5-65.8) and after treatment with an abdominal patch (OR = 11.3: 95% CI 1.5-84.4). CONCLUSION: ECMO treatment and the inhalation of nitric oxide are used in the most severe CDH patients but are associated with a higher risk on surgical haemorrhage. The recurrence rate is associated with both the use of an abdominal patch and the presence of pulmonary hypertension, regardless of medical treatment.

Congenital diaphragmatic eventration and hernia sac compared to CDH with true defects: a retrospective cohort study.

Congenital diaphragmatic eventration (CDE) and congenital diaphragmatic hernia (CDH) with or without hernia sac are three different types of congenital diaphragmatic malformations, which this study evaluates. All surgically treated patients with CDE or Bochdalek type CDH between 2000 and 2016 were included in this retrospective analysis. Demographics, CDH-characteristics, treatment, and clinical outcome were evaluated. In total, 200 patients were included. Patients with an eventration or hernia sac had no significant differences and were compared as patients without a true defect to patients with a true defect. The 1-year survival of patients with a true defect was significantly lower than patients with no true defect (76% versus 97%, p = 0.001). CDH with no true defect had significantly better short-term outcomes than CDH with true defect requiring patch repair. However, at 30 days, they more often required oxygen supplementation (46% versus 26%, p = 0.03) and had a higher recurrence rate (8% versus 0%, p = 0.006) (three eventration and two hernia sac patients). Conclusion: Patients without a true defect seem to have a more similar clinical outcome than CDH patients with a true defect, with a better survival. However, the recurrence rate and duration of oxygen supplementation at 30 days are higher than CDH patients with a true defect.What is Known:• Congenital diaphragmatic hernia with or without hernia sac and congenital diaphragmatic eventration (incomplete muscularization) are often treated similarly.• Patients with hernia sac and eventration are thought to have a relatively good outcome, but exact numbers are not described.What is New:• Congenital diaphragmatic eventration and patients with hernia sac seem to have a more similar clinical outcome than Bochdalek type CDH with a true defect.• Patients without a true defect (eventration or hernia sac) have a high recurrence rate.

A systematic review and meta-analysis of surgical morbidity of primary versus patch repaired congenital diaphragmatic hernia patients.

Large studies comparing the surgical outcome of primary versus patch repair in congenital diaphragmatic hernia (CDH) patients are rare. This study aims to evaluate the incidence of surgical complications in both types of CDH repair. PubMed, EMBASE, Cochrane and Web of Science were searched for peer-reviewed articles. Studies on CDH between 1991 and August 2020 were systematically screened and meta-analyses were performed. Primary outcomes of this review were: haemorrhage, chylothorax, recurrences and small bowel obstruction (SBO). A total of 6436 abstracts were screened, after which 25 publications were included (2910 patients). Patch repaired patients have a 2.8 times higher risk on developing a recurrence (20 studies) and a 2.5 times higher risk on developing a chylothorax (five studies). Moreover, they have a two times higher risk on developing a SBO. No studies could be included that evaluated the incidence of surgical haemorrhage between these patients. Although the quality of the studies was relatively low, patch repaired patients have a higher risk on developing a recurrence, chylothorax and small bowel obstruction. Large prospective studies are required to adjust for severity of disease, to reveal the true causative factors in order to minimize the risk on these surgical complications in both types of patients.

Malrotation in Congenital Diaphragmatic Hernia: Is It Really a Problem?

INTRODUCTION: Congenital diaphragmatic hernia (CDH) has been associated with other congenital malformations, such as intestinal rotation abnormalities. However, there is no standard evaluation and treatment of intestinal rotation during initial CDH repair. This study evaluates intestinal malrotation in CDH patients. MATERIALS AND METHODS: All patients with a CDH treated in a high-volume center between 2000 and 2015 were retrospectively evaluated. Demographics, gastrointestinal characteristics, surgical treatment, and abdominal outcomes (acute surgery, small bowel obstruction, and volvulus) were described. RESULTS: A total of 197 CDH patients were surgically repaired. In 76 (39%) patients, a malrotation was described at initial CDH repair, in 39 (20%) patients, a normal rotation, but 82 patients (42%) had no report on intestinal rotation. During follow-up (range: 2-16 years), 12 additional malrotations were diagnosed, leading to a prevalence of at least 45% (n = 88). These were mostly diagnosed due to acute small bowel obstruction, of which three had a volvulus. Of the 12 later diagnosed malrotations, 58% required acute surgery, compared to 3% of the 76 initially diagnosed patients (p < 0.001). CONCLUSION: Malrotation is associated with CDH, with a prevalence of at least 45% and in 21% a normal intestinal rotation. "Missed" malrotations have a higher risk on need for acute surgery later in life.

Congenital diaphragmatic hernia is associated with nonscrotal testes.

BACKGROUND: Congenital diaphragmatic hernia (CDH) is a rare birth defect with a high mortality and morbidity. Nonscrotal testes (NST) are the most reported genital anomaly in boys. Both defects have known associated anomalies, but little is known about the association between CDH and NST. This study evaluates this association and the location of the NST in a large cohort of male CDH survivors. Moreover, we analyzed possible associative factors for NST in CDH patients. METHODS: A cohort of CDH patients, born between January 2000 and March 2014 and treated in a high volume expertise center, was evaluated retrospectively. Boys with a minimum follow-up of 18months were included. The patients were evaluated for testes location, performed orchidopexy, and possible associative factors such as birth weight, gestational age, other congenital anomalies and CDH characteristics (surgical treatment, approach and ECMO). RESULTS: Seventy-five CDH patients were included. Twenty-seven (36%) were diagnosed with NST, of which 22 (29%) received orchidopexy. In 54 patients (72%) there were reports on testes location at birth and location was known for all patients at the age of 18months, although side of NST was unknown in four. The location of the NST was mostly ipsilateral to the CDH (n=20, 87%), of which eight (35%) had a bilateral NST with a unilateral CDH. There were no significant differences in birth weight, gestational age, and CDH specific characteristics in patients with or without NST. CONCLUSION: This study shows a strong association between CDH and NST, with a prevalence of 36%. However, no specific characteristics of the CDH were related to the NST. The testes of all male CDH patients should be thoroughly evaluated in the first year of their life, to ensure a proper and timely treatment. LEVEL OF EVIDENCE: Level IV; case series.

Factors related to long-term surgical morbidity in congenital diaphragmatic hernia survivors.

BACKGROUND: Patients born with a congenital diaphragmatic hernia (CDH) have a high mortality and morbidity. After discharge, complications and long-term morbidity are still encountered. This study describes the factors related to the surgical long-term outcomes in CDH survivors. METHODS: A cohort of CDH patients born between 2000 and 2014, with a minimum of two years follow up, were included in this retrospective study. Demographics, CDH specific characteristics, treatment, and long-term surgical outcome were evaluated using multivariate logistic regression analyses. RESULTS: 112 patients were included, with a mean follow up of 7.3 years (SD 3.8). The majority had primary repair, but 31% received patch repair. Recurrence was reported in 7% of all patients. However, recurrence risk increased for patients with extracorporeal membrane oxygenation (ECMO) treatment (ORadjusted: 6.3, 95% CI: 1.2-33.9). This risk was highest for patients needing both ECMO and patch repair (OR: 11.2, 95% CI: 2.3-54.1). Small bowel obstructions (SBO) were observed in 20% and was associated with patch repair (ORadjusted: 3.5, 95% CI: 1.2-10.0), but ECMO treatment seemed to reduce this risk (ORadjusted: 0.2, 95% CI: 0.0-1.0). Thoracic deformations (36%) was diagnosed most often after patch repair, especially when ECMO was needed (60%) as well. CONCLUSIONS: This retrospective study shows that the incidence of surgical long-term morbidity of CDH is relatively high, with different factors accounting for this. Diaphragmatic hernia recurrence was strongest associated with ECMO treatment in combination with patch repair, while SBO's were associated with patch repair, with an unexpected protective effect of ECMO treatment. TYPE OF STUDY: Retrospective comparative study - Level III evidence.

Bilateral congenital diaphragmatic hernia: prognostic evaluation of a large international cohort.

BACKGROUND: Congenital diaphragmatic hernia (CDH) is a lethal birth defect, which occurs in 1:2000-3000 live births. Bilateral CDH is a rare form (1%), with a high mortality. This study presents the outcomes of the largest cohort of bilateral CDH patients. METHODS: The records of patients with bilateral CDH from the Congenital Diaphragmatic Hernia Registry born between 1995 and 2015 were retrospectively analyzed to identify parameters associated with mortality. RESULTS: Eighty patients with a bilateral CDH were identified. Overall mortality was 74% (n=59). Apgar scores at 1 and 5min were statistically lower in the non-survivors compared to the survivors (median 3.0 and 5.0, versus 6.5 and 8.0, respectively, p<0.001). All survivors were repaired (n=21), compared to 22% of the non-survivors (n=17). The type of repair was equally divided in the survivors (52% primary versus 48% patch), while non-survivors were mainly patch repaired (82% versus 12%). Nineteen were treated with extracorporeal membrane oxygenation (ECMO) (24%), only three of them survived. When calculating the risk on mortality for the patients who lived until repair, ECMO had an adjusted odds ratio for mortality of 10.8 (95% CI: 2.0-57.7) and patch repair 5.2 (95% CI: 0.8-34.9). CONCLUSIONS: The treatment of bilateral CDH patients remains challenging with a high mortality rate. Lower Apgar-scores, ECMO (probably as a surrogate for the severity of disease), and patch repair were negatively associated with outcome. LEVEL OF EVIDENCE: Level IV study.