Standardized reporting for congenital diaphragmatic hernia--an international consensus.

BACKGROUND/PURPOSE: Congenital diaphragmatic hernia (CDH) remains a significant cause of neonatal death. A wide spectrum of disease severity and treatment strategies makes comparisons challenging. The objective of this study was to create a standardized reporting system for CDH. METHODS: Data were prospectively collected on all live born infants with CDH from 51 centers in 9 countries. Patients who underwent surgical correction had the diaphragmatic defect size graded (A-D) using a standardized system. Other data known to affect outcome were combined to create a usable staging system. The primary outcome was death or hospital discharge. RESULTS: A total of 1,975 infants were evaluated. A total of 326 infants were not repaired, and all died. Of the remaining 1,649, the defect was scored in 1,638 patients. A small defect (A) had a high survival, while a large defect was much worse. Cardiac defects significantly worsened outcome. We grouped patients into 6 categories based on defect size with an isolated A defect as stage I. A major cardiac anomaly (+) placed the patient in the next higher stage. Applying this, patient survival is 99% for stage I, 96% stage II, 78% stage III, 58% stage IV, 39% stage V, and 0% for non-repair. CONCLUSIONS: The size of the diaphragmatic defect and a severe cardiac anomaly are strongly associated with outcome. Standardizing reporting is imperative in determining optimal outcomes and effective therapies for CDH and could serve as a benchmark for prospective trials.

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

UNLABELLED: 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.

Cranial CT for diagnosis of intracranial complications in adult and pediatric patients during ECMO: Clinical benefits in diagnosis and treatment.

RATIONALE AND OBJECTIVES: To evaluate the clinical utility of cranial computed tomography (CT) in pediatric and adult patients during ongoing extracorporeal membrane oxygenation (ECMO) treatment from acute respiratory failure and to assess the frequency of intracranial hemorrhage (ICH) and infarction during the treatment. MATERIALS AND METHODS: The medical records of 123 consecutive patients, 54 children (ages 3 months-17 years) and 69 adults (ages 18-62 years), treated with ECMO over a 10-year period were searched for cranial CT performed during ECMO. Indications for CT, CT findings, impact on clinical management, and patient outcome were noted. In addition, all CT scans were reviewed for the frequency of ICH or infarction. RESULTS: Seventy-eight patients had cranial CT while on ECMO. ICH or cerebral infarction were detected in 45 (37%) of the 123 patients. Eighteen patients (15%) had focal hemorrhage, 11 (9%) focal infarction, and 16 (13%) general brain edema. In 16 of the 45 patients, the CT findings were decisive to withdraw the ECMO treatment. Five patients were weaned from ECMO, and in four patients the findings motivated cranial surgery during ECMO. In the remaining 20 patients with less extended intracranial pathology, the ECMO treatment was continued with high survival. CONCLUSION: Cranial CT has an important role during ECMO treatment to reveal or exclude severe intracranial complications where ECMO treatment should be discontinued. Less severe complications have a favorable prognosis with continued treatment. Our study suggests an underreporting of intracranial complications in adults and pediatric patients on ECMO because of low utilization of neuroimaging.

MRI of the brain and thorax during extracorporeal membrane oxygenation: preliminary report from a pig model.

Early diagnosis of cerebral hypoxic ischemic complications during extracorporeal membrane oxygenation (ECMO) is important to guide further treatment. However, diagnostic methods available during ECMO are limited, especially in adults and older children. Magnetic resonance imaging (MRI) is a sensitive and noninvasive method for assessment of vessel patency and brain parenchymal changes, and for measurement of brain perfusion. The use of MRI during ECMO has, to our knowledge, never been reported. We report the first animal experiment with MRI examination during ECMO. After a preliminary test with the mobile ECMO system in the MRI environment, a healthy pig was put on venoarterial ECMO, transported to the MRI department, and examined with sequences for anatomy and function of the brain and thorax. The results showed that the ECMO system was not adversely affected by the magnetic field at a distance from the camera where positioning and examination of the animal was possible. High-quality anatomical and functional images of the brain, heart, and thoracic vessels were acquired. The results suggest that MRI may be used for early diagnosis of cranial complications in patients on ECMO. MRI may also provide a useful tool for further research on flow dynamics and brain perfusion during ECMO.

Chest and abdominal CT during extracorporeal membrane oxygenation: Clinical benefits in diagnosis and treatment.

RATIONALE AND OBJECTIVE: This study aims to evaluate the clinical usefulness of thoracic and abdominal computed tomography (CT) as an adjunct to bedside diagnostic imaging in patients on extracorporeal membrane oxygenation (ECMO) therapy because of severe acute respiratory failure. MATERIALS AND METHODS: Imaging records for 118 consecutive thoracic and abdominal CT examinations performed in 63 patients (22 neonates, 15 children, and 26 adults) on ECMO therapy during an 8-year period were retrospectively reviewed. Reported CT findings were compared with concurrent bedside radiographs and ultrasounds. The clinical importance and effect on treatment of each CT finding was determined by reviewing the medical records. RESULTS: CT showed 30 clinically important complications in 20 different patients that directly impacted on the treatment, but were not diagnosed with bedside imaging. Of the 30 complications, 15 (50%) were surgically treated, 11 (37%) required percutaneous invasive procedures, and 4 (13%) were managed conservatively. Despite the serious complications, 13 of 20 patients (65%) survived. CONCLUSION: Both chest and abdominal CT have an important clinical role in patients on ECMO therapy because of acute respiratory failure, as a complement to bedside imaging, to exclude or show complications and expedite early invasive treatment, when needed.