Salvaging the severe congenital diaphragmatic hernia patient: is a silo the solution?

Background

Infants with severe congenital diaphragmatic hernia (CDH) requiring extracorporeal membrane oxygenation (ECMO) have a high morbidity and mortality. We hypothesized that placement of an abdominal wall silo and staged abdominal wall closure may reduce problems associated with decreased abdominal domain in CDH.

Methods

We performed a retrospective review and identified 7 CDH patients requiring ECMO who had a silastic abdominal wall silo between 2003 and 2006. Variables analyzed included survival, ECMO duration, duration of silo, time to discharge, and long-term outcome.

Results

Predicted mean survival for the entire cohort using the published CDH Study Group equation was 47% (range, 9%-86%). All 7 patients (100%) survived. Extracorporeal membrane oxygenation duration averaged 15 days (range, 5-19 days). Four of the patients (58%) were repaired with a silo on ECMO, and 3 (42%) had their repair after ECMO. The abdominal wall defect was closed at a mean of 21 days (range, 4-41 days). Hospital stay after silo placement averaged 54 days (range, 20-170 days) with no infections or wound complications.

Conclusions

Abdominal wall silo placement in infants with CDH requiring ECMO appears to be an effective strategy for decreased abdominal domain. Further studies are warranted to determine the efficacy of such a strategy for these high-risk CDH patients.     

Polyurethane versus silicone port a cath: What's going on at removal?

Background

Port-a-cath (PAC) is an essential device in the management of the patients of chronic illness, but despite theirs benefits there are many complications either at the time of insertion or at time of removal. Our aim of this study is to evaluate the fracture rate of the catheter at removal time in comparison with catheter type either polyurethane or silicone.

Does V-A ECMO increase the likelihood of chylothorax after congenital diaphragmatic hernia repair?

Background: The authors noticed a relatively large number of patients with congenital diaphragmatic hernia (CDH) repair after extracorporeal membrane oxygenation (ECMO) who had a chylothorax (CT). The data are reviewed.Methods: The charts of patients from 1990 until 2000 with CDH, treated with or without ECMO, together with the charts of patients treated with ECMO for other reasons and patients with esophageal atresia (EA) repair were reviewed. The diagnosis of CT was made if aspirated fluid appeared chylous and contained more than 90% lymphocytes or if the triglyceride level was more than 1.50 mmol/L.Results: Eighty-nine patients with CDH were analyzed. Postoperatively, 10% had a CT—21% in CDH patients with ECMO treatment and 6% in CDH patients without ECMO treatment. This difference appeared to be significant (P < .05). The presence of a patch as independent variable for the development of CT also showed significance (P < .05).Conclusions: Chylothorax presented in almost all cases as a left-sided fluid accumulation, and a patch was present in the majority of patients with CDH. Therefor, CT should be considered the result of the severity of the defect rather than the consequence of ECMO as a therapeutic modality.     

Prenatal diagnosis of congenital diaphragmatic hernia: how should the babies be delivered?

Congenital diaphragmatic hernia (CDH) in many patients is diagnosed in utero. In these patients, the delivery can be planned as an elective cesarean, induced vaginal, or spontaneous vaginal delivery. The optimal method has yet to be determined.The aim of this study was to compare the outcome of patients with CDH delivered by different methods.

Methods

The Congenital Diaphragmatic Hernia Study Group was formed in 1995 to compile data on liveborn babies with CDH. Beginning in 2001, data concerning delivery were collected. By October 2005, delivery data were available on 1039 term and near-term infants without cardiac malformations. Five hundred forty-eight had a prenatal diagnosis and complete data on delivery (194 delivered by elective cesarean delivery, 121 by induced vaginal delivery, and 233 by spontaneous vaginal delivery). Patients delivered by a nonelective cesarean delivery were assigned to the delivery group for which they were originally planned.

Results

The overall survival among the 548 patients was 69%. It was highest in patients delivered by cesarean delivery (71%) followed by those delivered through induced vaginal delivery (70%) and spontaneous vaginal delivery (67%). The difference was not statistically significant.Fifty-three percent of all patients survived without extracorporeal membrane oxygenation (ECMO). This was significantly higher after cesarean delivery (60%) than after induced vaginal delivery (49%) or spontaneous vaginal delivery (49%) (P < .05).At 30 days of age, 45% of the patients delivered by cesarean delivery had survived and were on room air. This was slightly lower after induced vaginal delivery (37%) or after spontaneous vaginal delivery (37%), although not statistically significant.

Conclusion

Cesarean delivery was associated with a slightly better outcome in terms of a significantly higher survival without the use of extracorporeal membrane oxygenation, although there was no significant difference in total survival. Because this study was not randomized, it is not possible to determine if the elective cesarean delivery was the cause for the better outcome or if centers favoring elective cesarean delivery by protocol are more skillful in the management of patients with CDH. Mode of delivery for term and near-term infants with CDH deserves further prospective study.     

Nitrofen-induced congenital diaphragmatic hernia in rat embryo: what model?

Purpose

The aim of this study was to investigate the natural history of congenital diaphragmatic hernia (CDH) in rat embryos with special attention to the pathomorphologic changes of diaphragm, liver, intestines, and lungs at various stages of embryonic development.

Methods

Pregnant Sprague-Dawley rats were given, via a gavage tube, 100 mg nitrofen (Wako Chemicals, Neuss, Germany) on day 10.5 of gestation. Fetuses were harvested by laparotomy on day 15.5, 16.5, 18, and 21. Anatomic study of the diaphragm, herniated viscera, and lungs was performed under stereoscope with special attention to the diaphragmatic defect.

Results

CDH occurred in 44 embryos on right, in 10 on the left, and in 34 bilateral. In the youngest embryos, the small defect was located in the dorsomedial portion of the diaphragm very close to the aortic hiatus. During pregnancy, the defect enlarged progressively to occupy more than half of the affected hemidiaphragm on day 21. In all animals, regardless of their age and side of the defect, the liver was found inside the chest. The intrathoracic mass was formed by ingrown hepatic tissue originating from the dorsal surface of the intraabdominal liver. It appeared as an accessory liver lobe. The amount of intrathoracic liver increased rapidly. From around day 18, the thoracic portion of liver, when examined in sagittal plane, was bent forward and assumed an uncinate shape. The stomach and small bowel loops were displaced into the chest in the oldest examined fetuses. The first sign of reduction of size of the lung was seen in 16, 5-day old embryos, and at further stages of embryonic development the lung growth impairment strictly paralleled the ingrowth of the liver. The natural history of the right and left CDH were very similar. In fetuses with bilateral CDH, asymmetry regarding size of the defect and volume of intrathoracic hepatic mass was noted with larger defects on the left side in more than 60% of animals.

Conclusions

Pathogenesis of nitrofen-induced diaphragmatic defect in the rat embryo and CDH in the human fetus seem to differ significantly. Nitrofen induces a complex malformation of the embryonic diaphragm and the liver. CDH in rat embryo bears more resemblance to a model of space-occupying lesion than to human CDH. The induction of localized hepatic proliferation may be a result of disturbance of as yet unknown regulatory interaction between hepatic and mesenchymal diaphragmatic cells.     

Esophageal adenocarcinoma: a long-term complication of congenital diaphragmatic hernia?

A 22-year-old man presented with a lower esophageal adenocarcinoma having been treated for a left-sided congenital diaphragmatic hernia (CDH) as a neonate. There is no known association or similar case reported in the literature. We review the known long-term sequelae of CDH, possible theories for the occurrence of adenocarcinoma, and its implications for follow-up of patients with CDH.     

Corticosteroids for fetuses with congenital diaphragmatic hernia: can we show benefit?

Background and Purpose

Prenatal corticosteroids have been used in fetuses with congenital diaphragmatic hernia (CDH). We tested the utility of steroids by 2 methods.

Methods

Mothers carrying fetuses with CDH were randomized to 3 weekly doses of betamethasone or placebo starting at 34 weeks. Patients were followed until death or discharge. In a separate cohort study, the CDH Registry was used to compare infants who received prenatal steroids to those who had not.

Results

Thirty-four patients were enrolled at 7 centers, with 32 completing the trial. There were 15 placebo and 17 steroid patients. There was no difference in survival, length of stay, duration of ventilation, or oxygen use at 30 days. For the cohort study, we looked at infants older than 34 weeks who were born after October 2000 when data on prenatal steroids were collected. There were 1093 patients; 390 were evaluable, with 56 receiving steroids. There was no difference in survival, length of stay, ventilator days, or oxygen use at 30 days.

Conclusion

Neither the trial nor the CDH Registry suggest that late prenatal corticosteroids benefit fetuses with CDH. More than 1700 mothers and fetuses would need to be enrolled in a trial to show a 10% improvement in survival. It is unlikely that late steroids offer benefit to most fetuses with CDH.     

Patch repair for congenital diaphragmatic hernia: is it really a problem?

BackgroundLarge congenital diaphragmatic hernia (CDH) defects often require the use of synthetic patches for tension-free repair. Although high rates of recurrence and other morbidities have been previously reported, our favorable perception of patch repair prompted this review.MethodsA single-center retrospective chart review of CDH cases repaired between January 1, 1999, and October 1, 2010. Patch repairs were performed by multiple surgeons with an effort to construct a tension-free dome-shaped patch.ResultsOne hundred eighty-four children underwent CDH repair of whom 99 (53.8%) required a patch. Seventy-four (74.7%) of the 99 patients who underwent patch repair survived to discharge and were compared with 75 primary repair survivors. Of those undergoing patch repair, 88% were prenatally diagnosed, 55% had liver herniation, and 22 (29.9%) were repaired on extracorporeal membrane oxygenation. Two patients experienced a recurrence after a patch repair and 3 after a primary repair for a rate of 5.4% and 4.0%, respectively (P = 1.0).ConclusionsThese results demonstrate that synthetic patch repair for CDH can be performed with a very low rate of recurrence challenging the need for alternative approaches to diaphragmatic replacement. High rates of recurrence reported for patch repair may be technical rather than intrinsic to the patch.     

Extracorporeal membrane oxygenation and congenital diaphragmatic hernia: should any infant be excluded?

Mortality in infants with congenital diaphragmatic hernia (CDH) remains high despite improvements in neonatal and surgical care because many infants develop persistent pulmonary hypertension of the newborn (PPHN) following repair. Since 1984, extracorporeal membrane oxygenation (ECMO) has been used as rescue therapy in all infants (n = 25) with PPHN following CDH repair when conventional management failed, with an overall survival of 60%. Repair was performed in this hospital on 12 infants and in other hospitals in 13 infants transferred for consideration of ECMO after repair. Mortality was the same in the group repaired here and those transferred for ECMO. Although complications were frequent in the surviving group, they were successfully managed with nonoperative or operative therapy. Selective use of ECMO has been advocated in CDH patients based on various predictors of high mortality such as "best" PO2 postrepair less than 100 mm Hg, oxygenation index greater than 40, and ventilation index greater than 1,000 with PCO2 greater than 40. Seven surviving infants following ECMO would have been classified as unsalvageable by at least one parameter if selection criteria based on these parameters had been used. We conclude from this series that current predictors of high mortality in CDH patients are unreliable when ECMO is used. Surgeons caring for infants with CDH should consider the use of ECMO in all infants.     

Does creating a dome reduce recurrence in congenital diaphragmatic hernia following patch repair?

PurposeTo develop a quantifiable approach to identify a dome shape of the repaired diaphragm using post-operative chest radiograph and to determine if a dome-shaped prosthetic patch repair is associated with a decreased rate of CDH recurrence.MethodsWe conducted a retrospective review of all neonates evaluated at our institution from January 2004 to August 2017 with left- and right-sided CDH with at least 6 months of follow-up after CDH repair. Patch use, post-operative imaging and postnatal outcomes were analyzed. Neonates with patch repair were divided into two groups based on the presence of a dome. Using postoperative chest radiographs, the presence of a dome was classified as having a vertical-horizontal diaphragm ratio (VHDR) greater than 20%. Primary outcome was CDH recurrence after repair.ResultsWe identified 192 neonates who met our inclusion criteria. Cohort survival was 96%, recurrence rate was 15%, 78% had a left-sided CDH; 54% received a patch repair, of which 58% had a type C defect. Of the 104 infants with patch repairs, the CDH recurrence rate was 22% (n = 23) at a median age of 8.5 months (3.8, 20.1). Although neonates with a dome repair had more ECMO use and longer hospital stay, their recurrence rate was about half of those with a non-dome repair (14% vs 28%, p = 0.07).ConclusionsA dome-shaped repair may reduce recurrence following prosthetic patch repair of congenital diaphragmatic hernia. A larger, multi-institutional study is needed to statistically validate this clinically significant observation.Type of StudyRetrospective review.Level of EvidenceIII.     

Platelet-derived growth factor inhibition--a new treatment of pulmonary hypertension in congenital diaphragmatic hernia?

Increased pulmonary vascular resistance causing pulmonary artery hypertension is a major problem in the treatment of congenital diaphragmatic hernia with a strong association to mortality. We here report a patient with intractable pulmonary hypertension at 4 weeks of age unresponsive to conventional treatment. After administration of the platelet-derived growth factor (PDGF) receptor antagonist imatinib, pulmonary artery pressure gradually decreased to acceptable levels and the patient's clinical condition gradually improved.     

Editorial Commentary: What's Hamada With Partial Rotator Cuff Repair?

Early repair of acute traumatic rotator cuff tears has been shown to restore functional range of motion, whether or not there is complete healing. The ability to predict those cuff tears that can achieve only a partial repair can help with preoperative patient counseling. The Hamada classification can be predictive in determining outcomes in the treatment of massive rotator cuff tears.     

Minimally invasive congenital diaphragmatic hernia repair: a 7-year review of one institution’s experience

Background  Minimally invasive surgery (MIS) has been described for the repair of congenital diaphragmatic hernias (CDH) in neonates, infants, and children. This report evaluates patient selection, operative technique, and clinical outcomes for MIS repair of CDHs from a single center’s experience. Methods  All cases of CDH at a tertiary care pediatric hospital with an initial attempt at MIS repair from January 2001 to December 2007 were reviewed. Results  A total of 22 children underwent an initial attempt at MIS repair of their CDH (5 Morgagni and 17 Bochdalek hernias). The children ranged in age from 1 day to 6 years (mean, 13.9 ± 23 months) and weighed 2.2 to 21 kg (mean, 7.4 ± 5.50 kg) at the time of the operation. All five Morgagni hernias were managed successfully with laparoscopic primary repair. Six of the Bochdalek hernias were found in infants and children (age range, 6–71 months). All these were managed successfully with primary repair by an MIS approach (2 by laparoscopy and 4 by thoracoscopy). The remaining 11 Bochdalek hernias were found in neonates (age range, 1 day to 8 weeks). Four of the Bochdalek hernias were right-sided. Nine of the Bochdalek hernias in neonates were repaired thoracoscopically. One neonate required conversion to laparotomy, and another underwent conversion to thoracotomy. Four of the neonates with Bochdalek hernias required a prosthetic patch. Two of the neonates also had significant associated congenital cardiac defects. Overall, there were two recurrences involving one 3-day-old who underwent a primary thoracoscopic repair and another 3-day-old who underwent a thoracoscopic patch repair. The follow-up period ranged from 5 months to 5 years. Conclusions  Morgagni hernias can be managed successfully by laparoscopy, whereas thoracoscopy is preferred for neonatal Bochdalek hernias. Either approach can be successful for infants and children with Bochdalek hernias. Additionally, patients with congenital cardiac defects and those requiring prosthetic patches can undergo a MIS CDH repair with a successful outcome.     

Operative repair in congenital diaphragmatic hernia: How long do we really need to wait?

ObjectiveTo analyze preoperative cardiopulmonary support and define preoperative stability relative to timing of surgical repair for CDH neonates not on ECMO.Study designWe retrospectively analyzed repeated measures of oxygenation index (OI; Paw*FiO₂×100/PaO₂) among 158 neonates for temporal preoperative trends. We defined physiologic stability using OI and characterized ventilator days and discharge age relative to delay in repair beyond physiologic stability.ResultsThe OI in the first 24 h of life was temporally reliable and representative of the preoperative mean (ICC 0.70, 95% CI 0.61–0.77). A pre-operative OI of ≤ 9.4 (AUC 0.95) was predictive of survival. Surgical delay after an OI ≤ 9.4 resulted in increased ventilator days (1.4, 95% CI 1.1–1.9) and discharge age (1.5, 95% CI 1.2–2.0). When prospectively applied to a subsequent cohort, an OI ≤ 9.4 was again reflective of physiologic stability prior to repair.ConclusionOI values are temporally reliable and change minimally after 24 h age. Delay in surgical repair of CDH beyond initial stability increases ventilator days and discharge age without a survival benefit.Level of evidencePrognosis study, Level III.     

Multiple injections for low back pain: What’s the future?

To examine the strength of evidence available for multiple facet joint injections (FJIs) and medial branch blocks (MBBs), and to report on the variations in the NHS England framework using the getting it right first time (GIRFT) data. Systematic review using patient, intervention, comparison, outcome and study strategy. The literature search using Cochrane, MEDLINE and EMBASE databases using MeSH terms: lumbar spine, spinal injection and facet joint (“Appendix A”). Three studies were identified that investigated the efficacy of multiple FJIs or MBBs. None of these studies reported sustained positive outcomes at long-term follow-up. There is a paucity of levels I and II evidence available for the efficacy of multiple FJIs and MBBs in treating low back pain. GIRFT data show a high degree of variation in the use of multiple FJIs, which would not be supported by the literature. These slides can be retrieved under Electronic Supplementary Material.     

Does the ex utero intrapartum treatment to extracorporeal membrane oxygenation procedure change outcomes for high-risk patients with congenital diaphragmatic hernia?

PurposeIn the most severe cases of congenital diaphragmatic hernia (CDH), significant barotrauma or death can occur before advanced therapies such as extracorporeal membrane oxygenation (ECMO) can be initiated. We have previously examined the use of the ex utero intrapartum treatment (EXIT) to ECMO procedure (EXIT with placement on ECMO) in high-risk infants and reported a survival advantage. We report our experience with EXIT to ECMO in a more recent cohort of our patients with most severe CDH.MethodsEvery patient with less than 15% predicted lung volume during January 2005 to December 2010 was included. We obtained data on prenatal imaging, size and location of the defect, and survival.ResultsSeventeen high-risk infants were identified. All 17 (100%) received ECMO and required a patch. Six children were delivered by EXIT to ECMO, and only 2 (33%) survived. An additional patient was delivered by EXIT to intubation with ECMO on standby and died. Of the 10 children who did not receive EXIT, 5 (50%) survived.ConclusionsNo clear survival benefit with the use of the EXIT to ECMO procedure was demonstrated in this updated report of our high-risk CDH population. The general application of EXIT to ECMO for CDH is not supported by our results.