Bochdalek hernia (BH) is an uncommon form of diaphragmatic hernia. The rarity of this hernia and its nonspecific presentation leads to delay in the diagnosis, with the potential risk of complications. This review summarizes the relevant aspects of its presentation and management, based on the present evidence in the literature. A literature search was performed on PubMed, Google Scholar, and EMBASE for articles in English on BH in adults. All case reports and series from the period after 1955 till January 2015 were included. A total of 180 articles comprising 368 cases were studied. The mean age of these patients was 51 years (range 15-90 years) with a male preponderance of 57% (211/368). Significantly, 6.5% of patients were above 70 years, with 3.5% of these being above 80 years. The majority of the hernias were on the left side (63%), with right-sided hernias and bilateral occurring in 27% and 10%, respectively. Precipitating factors were noted in 24%, with 5.3% of them being pregnant. Congenital anomalies were seen in 11%. The presenting symptoms included abdominal (62%), respiratory (40%), obstructive (vomiting/abdominal distension; 36%), strangulation (26%); 14% of them were asymptomatic (detected incidentally). In the 184 patients who underwent surgical intervention, the surgical approach involved laparotomy in 74 (40.27%), thoracotomy in 50 (27.7%), combined thoracoabdominal approach in 27 (14.6%), laparoscopy in 23 (12.5%), and thoracoscopic repair in 9 (4.89%). An overall recurrence rate of 1.6% was noted. Among these patients who underwent laparoscopic repair, 82% underwent elective procedure; 66% underwent primary repair, with 61% requiring interposition of mesh or reenforcement with or without primary repair. The overall mortality was 2.7%. Therefore, BH should form one of the differential diagnoses in patients who present with simultaneous abdominal and chest symptoms. Minimal access surgery offers a good alternative with short hospital stay and is associated with minimum morbidity and mortality.

OBJECTIVE: This systematic review aims to evaluate current choice in practice and outcomes of biomaterials used in patch repair of congenital diaphragmatic hernia (CDH). BACKGROUND: Multiple biomaterials, both novel and combinations of pre-existing materials are employed in patch repair of large size CDH. METHODS: Literature search was performed across Embase, Medline, Scopus, and Web of Science. Publications that explicitly reported patch repair, material used and recurrences following CDH repair were selected. RESULTS: Sixty-three papers were included, presenting data on 4598 patients of which 1811 (39.4%) were managed using 19 types of patches. Goretex® (GTX) (n=1259) was the most frequently employed patch followed by Surgisis® (n=164), Dualmesh® (n=114), Marlex®/GTX® (n=56), Tutoplast dura® (n=40), Dacron® (n=34), Dacron®/GTX® (n=32), Permacol® (n=24), Teflon® (n=24), Surgisis®/GTX® (n=15), Sauvage® Filamentous Fabric (n=13), Marlex® (n=9), Alloderm® (n=8), Silastic® (n=4), Collagen coated Vicryl® mesh (CCVM) (n=1), Mersilene® (n=1), and MatriStem® (n=1) Biomaterials were further subgrouped as: Synthetic non-resorbable (SNOR) (n=1458), Natural-resorbable (NR) (n=249), Combined natural and synthetic non-resorbable (NSNOR) (n=103), and Combined natural and synthetic resorbable (NSR) (n=1). Overall recurrence rate for patch repair was 16.8% (n=305). For patch types with n>20, recurrence rate was lowest in GTX/Marlex (3.6%), followed by Teflon (4.2%), Dacron (5.6%), GTX (13.8%), Permacol (16.0%), Tutoplast Dura (17.5%), Dualmesh (20.2%), SIS/GTX (26.7%), SIS (36.6%), and Dacron/GTX (37.5%).When analysed by biomaterial groups, recurrence was highest in NSR (100%), followed by NR (32.9%), NSNOR (17.5) and SNOR the least (14.0%). CONCLUSION: In this cohort, over one-third of CDH were closed using patches. To date, 19 patch types/variations have been employed for CDH closure. GTX is the most popular, employed in 70% of patients; however excluding smaller cohorts (n<20) GTX/Marlex is associated with the lowest recurrence rate (3.6%). SNOR was the material type least associated with recurrence while NSR experienced recurrence in every instance.

PURPOSE: The aim of this study was to review all available studies reported in the English-language literature from 1975 through 1998, and by meta-analysis assess the importance of prenatal diagnosis, associated malformations, side of hernia, timing of surgery, and study population on mortality rates in patients with congenital diaphragmatic hernia (CDH). METHODS: One-hundred-two studies were identified, and 51 studies (2,980 patients) fulfilled the prespecified inclusion criteria. Studies were grouped according to study population into: (I) fetuses diagnosed prenatally; (II) neonates admitted to a treatment center; and (III) population-based studies. RESULTS: Pooled total mortality rate was significantly higher in category I than in category III (75.6% v 58.2%, P < .001). Pooled hidden postnatal mortality rate (deaths before admittance to a treatment center) in population-based studies was 34.9%. Prenatally diagnosed patients in both category II and III had significantly higher mortality rates than those diagnosed postnatally. Mortality rates were significantly higher among CDH infants with associated major malformations compared with isolated CDH in all 3 categories. An increased mortality rate in right-sided CDH was found in category II and III. CONCLUSIONS: Prenatal diagnosis of CDH, presence of associated major malformations, and the study population have a major influence on mortality rate. The very high mortality rate in studies of fetuses with a prenatal diagnosis of CDH should be taken into account in prenatal counselling.

BACKGROUND: Congenital diaphragmatic hernia (CDH), is an uncommon but severe condition in which there is a developmental defect in the fetal diaphragm, resulting in liver and bowel migrating to the chest cavity and impairing lung development and function for the neonate. This condition can be diagnosed during pregnancy and as such, is potentially amenable to in-utero prenatal intervention. Neonatal surgical repair is possible, but even with early surgical repair and improving neonatal management, neonatal morbidity and mortality is high. Prenatal interventions described to date have included maternal antenatal corticosteroid administration and fetal tracheal occlusion, with both methods aiming to improve lung growth and maturity. However surgical procedures have potential maternal complications, as the uterus and amniotic sac are breached in order to gain access to the fetus. OBJECTIVES: To compare the effects of prenatal versus postnatal interventions for CDH on perinatal mortality and morbidity, longer-term infant outcomes and maternal morbidity, and to compare the effects of different prenatal interventions with each other. SEARCH METHODS: We searched the Cochrane Pregnancy and Childbirth Group's Trials Register (31 August 2015) and reference lists of retrieved studies. SELECTION CRITERIA: All published (including those published in abstract form), unpublished, and ongoing randomised controlled trials comparing prenatal and postnatal interventions for fetuses with CDH. Quasi-RCTs were eligible for inclusion but none were identified. Trials using a cross-over design are not eligible for inclusion. DATA COLLECTION AND ANALYSIS: Two review authors evaluated trials for inclusion and methodological quality without consideration of their results according to the stated eligibility criteria and extracted data independently. Data were checked for accuracy. MAIN RESULTS: We identified 11 studies for potential inclusion. Of those, we included three studies involving 97 women. Two additional studies are ongoing.Two trials examined in-utero fetal tracheal occlusion with standard (postnatal) care in fetuses with severe diaphragmatic hernia. Whilst the trials utilised fetal interventions that were similar, there were important differences in how access was gained to the fetus and in the timing and mode of delivery. Therefore, we did not combine these trials in meta-analysis and the results are examined in separate comparisons. One trial examined the effect of antenatal corticosteroids versus placebo. Overall, the methodological quality of the trials was variable and no data were available for a number of this review's secondary outcomes. In-utero fetal occlusion by maternal laparotomy versus standard postnatal management (one trial, 24 women)For the primary infant outcome (perinatal mortality), there were no data suitable for inclusion in the analysis. There was no difference between groups in terms of long-term infant survival (risk ratio (RR) 1.06, 95% confidence interval (CI) 0.66 to 1.69). In-utero fetal occlusion by minimally invasive fetoscopy versus standard postnatal management (one trial, 41 women)The primary infant outcome (perinatal mortality) was not reported. Minimally invasive fetoscopy was associated with a small reduction in the mean gestational age at birth (mean difference (MD) -1.80 weeks, 95% CI -3.13 to -0.47), but there was no clear difference in the risk of preterm birth before 37 weeks (RR 1.75, 95% CI 0.78 to 3.92). Long-term infant survival (three to six months) (RR 10.50, 95% CI 1.48 to 74.71) was increased with the intervention when compared with standard management, and there was a corresponding reduction in pulmonary hypertension (RR 0.58, 95% CI 0.36 to 0.93) associated with the intervention. There was no difference between groups in terms of preterm ruptured membranes (< 37 weeks) (RR 1.47, 95% CI 0.56 to 3.88) or maternal infectious morbidity (RR 3.14, 95% CI 0.14 to 72.92), and there were no maternal blood transfusions. Antenatal corticosteroids versus placebo (one trial, 32 women)We also included one trial (involving 32 women) examining the effect of antenatal corticosteroids versus placebo. There was no clear difference in the incidence of perinatal mortality (our primary infant outcome) between the group of women who received antenatal corticosteroids and the placebo control (RR 1.24, 95% CI 0.50 to 3.08). Data (mean only) were reported for two of our secondary outcomes (mechanical ventilation and days of hospital admission) but standard deviations (SDs) were not provided. For the purposes of this review and to permit further analysis we have estimated the SDs based on the reported P values reported in the trial report, although our estimation does assume that the SD is the same in both the intervention and control groups. There were no differences between the antenatal corticosteroid group and the placebo control in terms of days of mechanical ventilation (MD 18.00 days, 95% CI -14.77 to 50.77) or days of hospital admission (MD 17.00 days, 95% CI -13.93 to 47.93) . AUTHORS' CONCLUSIONS: There is currently insufficient evidence to recommend in-utero intervention for fetuses with CDH as a part of routine clinical practice. We identified three small studies, with only one study adequately reporting on the primary outcome of this review - perinatal mortality, and there were few data pertaining to many of this review's secondary outcomes.WIth regard to the administration of antenatal corticosteroids, there remains a gap in current research, and a large multicentre trial with adequate statistical power should be undertaken to answer this unresolved question. More studies are needed to further examine the effect of in-utero fetal tracheal occlusion on important neonatal outcomes and long-term infant survival and health. Long-term follow-up is of particular importance, and should include morbidity and mortality measures. Further studies should examine the benefits of an in-utero intervention on subgroups with moderate and severe congenital diaphragmatic hernia. Indeed, there are three ongoing studies, being conducted by European, North and South American fetal medicine centres, which will contribute to this gap. Ongoing research and any implementation into clinical practice should include standardisation of the procedure, inclusion criteria and long-term childhood follow-up.

BACKGROUND: Emanuel Syndrome (ES), a rare chromosomal disorder caused by a supernumerary chromosome 22 derivative (der(22)t(11;22)), was identified in a fetus with congenital diaphragmatic hernia (CDH) at our fetal center. We aimed to identify a precedent for clinical care and patient outcomes to guide family decision-making. METHODS: This non-funded and non-registered study queried the entire CDH Registry (CDHR) including >10,000 patients since 1995 and conducted a systematic literature review for patients with concomitant ES and CDH. RESULTS: Literature review captured 12 citations and identified 9 patients with CDH+ES from over 400 known ES cases. Given the rarity of the disease and to reduce bias, there were no exclusion criteria aside from non-English language. Of these 9, two underwent surgical CDH repair with neither surviving. The CDHR identified 6 patients with ES, all reported after 2013 and prenatally diagnosed. Median estimated gestational age was 39 weeks (range 37-40) and median birth weight was 2.72 kg (range 2.4-3.4 kg). 3 patients died within the first few postnatal days; surgical repair was not offered due to "anomalies" and "pulmonary hypertension" in two and one family chose comfort measures. The other 3 patients underwent surgical repair, and 2 were supported with ECMO. Two patients survived to discharge, incurring surgical comorbidities associated with severe CDH including gastrostomy dependence, tracheostomy, and CDH recurrence. CONCLUSIONS: ES patients with CDH have potential to tolerate repair and survive to discharge, however with significant additional morbidity combined with severe challenges inherent to ES. This represents the largest series of patients with CDH and ES to date. LEVEL OF EVIDENCE: IV (Case series with no comparison group).

BACKGROUND: Traumatic diaphragmatic hernia (TDH) is rare in children, most often occurring following blunt thoracoabdominal trauma from high energy mechanisms, such as motor vehicle collisions (MVC). We performed a systematic review to describe injury details and management. METHODS: Following PRISMA guidelines, a systematic literature search was performed to identify publications of blunt TDH in patients < 18 y old. Conflicts were resolved by consensus. Data were collected on demographics, TDH location, mechanism of injury, associated intraabdominal injuries (IAI), management, and outcomes. Denominators vary depending on number of patients with such information reported. RESULTS: Fifty-eight articles were reviewed with 142 patients with TDH. The median age was seven y (range 0.25-16). Most were left-sided (85 of 126, 67.5%). MVC was the most common mechanism (66 of 142, 46.5%). IAI was present in 50.0% (57 of 114), most commonly liver injuries (25 of 57, 43.9%). Delayed diagnoses occurred in 49.6% (57 of 115, range 8 h-10 y), and were more common with right-sided TDH (76.0% versus 48.5%, P = 0.02). Chest radiography was 59.0% sensitive for TDH, while computed tomography sensitivity was 65.8%. Operative repair was performed on all surviving patients, and all underwent primary diaphragm repair. The overall mortality was 11.3% (n = 16), with four attributable to the TDH. There were no reported recurrences over a median follow-up of 12 mo. CONCLUSIONS: Pediatric TDH is a rare diagnosis with a high rate of associated IAI and delayed diagnosis. Primary diaphragm repair was performed in all cases. Surgeons should maintain a high suspicion for diaphragm injury in blunt thoracoabdominal trauma.

BACKGROUND: Post-hepatectomy diaphragmatic hernia is the second most common cause of acquired diaphragmatic hernia. This study aims to review the literature on this complication's incidence, treatment and prognosis. METHODS: Following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, we systematically searched PubMed for all studies related to acquired diaphragmatic hernias after hepatectomy. RESULTS: We included 28 studies in our final analysis, comprising 11,368 hepatectomies. The incidence of post-hepatectomy diaphragmatic hernia was 0.75% (n = 86). The most frequent type of hepatectomy performed was right hepatectomy (79%, n = 68), and the indications for liver resection were a liver donation for living donor transplantation (n = 40), malignant liver tumors (n = 13), and benign tumors (n = 11). The mean onset between liver resection and the diagnosis of diaphragmatic hernia was 25.7 months (range, 1-72 months), and the hernia was located on the right diaphragm in 77 patients (89.5%). Pain was the most common presenting symptom (n = 52, 60.4%), while six patients were asymptomatic (6.9%). Primary repair by direct suture was the most frequently performed technique (88.3%, n = 76). Six patients experienced recurrence (6.9%), and three died before diaphragmatic hernia repair (3.5%). CONCLUSION: Diaphragmatic hernia is a rare complication occurring mainly after right liver resection. Repair should be performed once detected, given the not-negligible associated mortality in the emergency setting.

INTRODUCTION: Complicated diaphragmatic hernia (DH) can be congenital or acquired. Congenital diaphragmatic hernias (CDH) are rare and often can be asymptomatic until adulthood. Traumatic diaphragmatic hernia (TDH) is a complication that occurs in about 1-5% of victims of road accidents and in 10-15% of penetrating traumas of the lower chest. CDH and TDH are potentially life-threatening conditions, and the management in emergency setting still debated. This study aims to evaluate the surgical treatment options in emergency setting. METHODS: A bibliographic research reporting the item "emergency surgery" linked with "traumatic diaphragmatic rupture" and "congenital diaphragmatic hernia" was performed. Several parameters were recorded including sex, age, etiology, diagnosis, treatment, site and herniated organs. RESULTS: The research included 146 articles, and 1542 patients were analyzed. Most of the complicated diaphragmatic hernias occurred for a diaphragmatic defect due to trauma, only 7.2% occurred for a congenital diaphragmatic defect. The main diagnostic method used was chest X-ray and CT scan. Laparotomic approach still remains predominant compared to the minimally invasive approach. CONCLUSION: Surgery is the treatment of choice and is strongly influenced by the preoperative setting, performed mainly with X-ray and CT scan. Minimally invasive approach is safe and feasible but is highly dependent on the surgeon's expertise, especially in emergency setting.

The case of a 36-year-old man with an unsuspected delayed posttraumatic diaphragmatic hernia is described. This entity often presents with vague clinical symptoms and non-specific radiographic findings long after the initiating trauma has been forgotten. Therefore, the preoperative diagnosis of this entity is notoriously difficult, but failure to make this diagnosis carries a high mortality rate. A high index of suspicion must be maintained.

BACKGROUND: Diaphragmatic hernia (DH) is extremely rarely described during pregnancy. Due to the rarity, there is no diagnostic or treatment algorithm for DH in pregnancy. AIM: To summarize and define the most appropriate diagnostic methods and therapeutic options for DH in pregnancy based on scarce literature. METHODS: Literature search of English-, German-, Spanish-, and Italian-language articles were performed using PubMed (1946-2021), PubMed Central (1900-2021), and Google Scholar. The PRISMA protocol was followed. The search terms included: Maternal diaphragmatic hernia, congenital hernia, pregnancy, cardiovascular collapse, mediastinal shift, abdominal pain in pregnancy, hyperemesis, diaphragmatic rupture during labor, puerperium, hernie diaphragmatique maternelle, hernia diafragmática congenital. Additional studies were identified by reviewing reference lists of retrieved studies. Demographic, imaging, surgical, and obstetric data were obtained. RESULTS: One hundred and fifty-eight cases were collected. The average maternal age increased across observed periods. The proportion of congenital hernias increased, while the other types appeared stationary. Most DHs were left-sided (83.8%). The median number of herniated organs declined across observed periods. A working diagnosis was correct in 50%. DH type did not correlate to maternal or neonatal outcomes. Laparoscopic access increased while thoracotomy varied across periods. Presentation of less than 3 days carried a significant risk of strangulation in pregnancy. CONCLUSION: The clinical presentation of DH is easily confused with common chest conditions, delaying the diagnosis, and increasing maternal and fetal mortality. Symptomatic DH should be included in the differential diagnosis of pregnant women with abdominal pain associated with dyspnea and chest pain, especially when followed by collapse. Early diagnosis and immediate intervention lead to excellent maternal and fetal outcomes. A proposed algorithm helps manage pregnant women with maternal DH. Strangulated DH requires an emergent operation, while delivery should be based on obstetric indications.