Patterns of fetal perinasal fluid flow in cases of congenital diaphragmatic hernia

OBJECTIVE(S): Our purpose was to expand the previous reported series of observations of fetal perinasal fluid flow in cases of antenatally diagnosed congenital diaphragmatic hernia, characterize the timing parameters of the fetal breath cycle, and define the relationship of fetal perinasal fluid flow and the diaphragmatic component of fetal breathing movements. Our hypothesis was that characteristics of diaphragm-related and nondiaphragm-related perinasal fluid flow and other breath cycle characteristics differ in cases of congenital diaphragmatic hernia compared with controls. STUDY DESIGN: Fetal perinasal fluid flow velocity and fetal chest wall movements were studied in 24 cases of uncomplicated pregnancy, and flow was studied in 24 cases of antenatally diagnosed congenital diaphragmatic hernia at gestational ages ranging from 30 to 41 weeks. The examination of fetal perinasal fluid flow velocity was performed with use of an ultrasonography system applying color flow and spectral Doppler analysis. Breath-to-breath interval, time of inspiration, time of expiration, and peak inspiratory and expiratory velocities were determined for each type of perinasal flow. RESULTS: The study revealed that the time of expiration in cases of congenital diaphragmatic hernia at 30 to 36 and 37 to 41 weeks of gestation was significantly shorter than in cases of uncomplicated pregnancy. The ratio of time of inspiration and breath-to-breath interval in cases of diaphragmatic hernia was approximately 30% higher (p = 0.001) at 30 to 36 weeks of gestation than in cases of uncomplicated pregnancy. The study also showed that in cases of congenital diaphragmatic hernia the expiratory peak velocity ratio at 30 to 36 weeks of gestation was significantly lower than in cases of uncomplicated pregnancy. CONCLUSIONS: We conclude that by Doppler ultrasonography measurements of fetal perinasal fluid flow, in cases of congenital diaphragmatic hernia, we can evaluate the timing parameters of fetal diaphragm-related breath cycles, the relationship of intraalveolar and intraamniotic pressures, and fetal upper respiratory tract resistance. Fetuses with diaphragmatic hernia spent significantly more time with diaphragm-nonrelated perinasal flow than did fetuses in cases of uncomplicated pregnancy, which can cause the increased loss of lung liquid and consequently be associated with pulmonary insufficiency in the early neonatal period.     

Ultrasound features of congenital listeriosis--a case report

A cohort of 78 infants of gestational age less than 34 weeks was examined for Ureaplasma urealyticum colonization and neonatal morbidity. Ureaplasma urealyticum was cultured from nasopharyngeal, endotracheal and blood-culture samples. A child was considered as being colonized if any sample was positive. The children with perinatal U. urealyticum colonization (n = 11; 14%) differed from those with no colonization (n = 67) in two important aspects: (i) they had higher leucocyte counts on the first (18.6 vs 12.4 10(9)) and the second (29.0 vs 15.4 10(9)) days of life (p = 0.01, both days); and (ii) they more often needed high-frequency oscillatory ventilation (45% vs 13%, p = 0.02). This study showed that U. urealyticum colonization is associated with signs of the host defence response together with symptoms of respiratory tract involvement suggesting the pathogenicity of U. urealyticum in premature infants.     

Prediction of outcome in congenital diaphragmatic hernia

The case records of 59 patients with congenital diaphragmatic hernia (CDH) who presented between 1984 and 1997 were studied retrospectively. Included in the study were infants born with CDH who required respiratory support within the first 6 h of life. Twenty-three were excluded from the study for various reasons; 36 were enrolled in the study; the male-to-female ratio was 18:18. Twenty-nine hernias were left-sided and 7 were right-sided. All patients were ventilated using conventional techniques. Arterial blood gases were measured on average 1.76 h following admission and the initial period of resuscitation (range 1-4 h). Three formulae were applied in an attempt to predict outcome: ventilation index against PCO2, alveolar-arterial oxygen gradient, and a newly derived formula from this institution (Red Cross formula) that comprises respiratory rate x PCO2 x FiO2 x mean airway pressure/PaO2 x 6000. Seventeen patients (47.2%) survived and 19 died (52.8%); 21 became stable enough to undergo surgery. The average time from presentation until surgery was 1.98 days (range 6 h to 4 days). The Red Cross formula, with a 100% predictive value for mortality, 85% predictive value for survival, and an overall predictive value of 91.6%, appeared to be superior to the other formulae studied. The availability of a highly accurate predictive formula may facilitate management of this frequently lethal disease.     

Iatrogenic diaphragmatic hernia following abdominal esophagogastrofundoplication: report of a case

The authors describe diagnosis and surgical treatment of a patient with iatrogenic diaphragmatic hernia following esophagogastrofundoduplication by Nissen's operation. The patient had presented a hiatal hernia with esophagitis chronic regurgitation and was submitted to esophagogastrofundoduplication. On the third postoperative day, the patient showed signs of dysphagia and intense dyspnea. The computerized tomography showed the presence of the gastric fundus and it's contents inside the leftpleural cavity. The patient was submitted to a left posterolateral thoractomy and an ischemic peptic ulcer in the gastric fundus, blocked by lung parenchyma was sutured. Then, the stomach was reduced into the abdominal cavity with diaphragmatic suture associated with esophageal and gastric fundus fixation to the right diaphragmatic pilar. The patient presented satisfactory immediate and late postoperative follow-up (1 year). The authors discuss and document aspects of diagnosis as well as surgical indication.     

Prenatal diagnosis of congenital heart disease

Owing to the widely different levels of experience of examiners, there is a large discrepancy in study results of second trimester ultrasound screening for fetal malformations, which is a result of varying levels of obstetric scanning expertise prevalent at the reporting centre. This holds particularly true for the prenatal diagnosis of congenital heart disease where detection rates ranging from 0 per cent to 60 per cent are being reported. On the other hand, congenital heart disease affects about 4-8 per 1000 live births and is a leading cause of infant mortality, whereas prenatal diagnosis could possibly prevent death and long-term morbidity in some of these neonates. Various screening concepts for more effective detection of congenital heart diseases are analysed in this article, including the more recent technique of early echocardiography between 13 and 15 weeks of gestation. High-risk groups are defined and the group of fetuses with increased thickness of nuchal translucency seems to be of particular interest.     

Contractile properties of intralobar pulmonary arteries and veins in the fetal lamb model of congenital diaphragmatic hernia

BACKGROUND/PURPOSE: Pulmonary hypertension plays a significant role in the pathophysiology of congenital diaphragmatic hernia (CDH). Although there has been an intensive research effort directed at mediators that may cause pulmonary vasoconstriction, no single agent has been identified. The authors hypothesize that there may be an alteration in the cGMP-nitric oxide (NO) pathway of vasodilatation contributing to the pulmonary hypertension observed in CDH. The purpose of these studies is to begin to elucidate vasoactive properties of pulmonary vessels with particular attention to the cGMP-NO pathway of vasodilatation in fetal lambs with CDH. METHODS: Fourth-generation pulmonary arteries and pulmonary veins were dissected from both right and left lungs of eight, 139-day gestational fetuses with surgically created CDH. Vessels were studied with standard isolated tissue bath techniques. Experiments examined basal release of NO in endothelium-intact PVs and PAs of both right and left lungs by measuring the contractile force of vessels constricted with norepinephrine (NE) in the presence and absence of the nitric oxide synthase (NOS) inhibitor N(omega)-nitro-L-arginine (L-NA). Concentration-response curves to the vasodilating agents zaprinast and A23187 were also obtained in vessels contracted by NE. RESULTS: Left and right pulmonary artery responses to NE are enhanced over those of historic controls. Pretreatment of left pulmonary arteries with L-NA enhances the vasoconstrictor response to NE, whereas right PAs show no increased response. Relaxation responses to A23187 and zaprinast, in both left and right pulmonary arteries were not different from control lambs. Relaxation responses of both left and right pulmonary veins to A23187 and zaprinast are blunted compared with controls. This blunting is significantly more in left pulmonary veins than right. Further, right but not left pulmonary veins display enhanced vasoconstrictive response to NE after L-NA pretreatment. CONCLUSIONS: The NO-cGMP pathway of vasodilatation is abnormal in the near term, fetal lamb with CDH. These abnormalities were most apparent in pulmonary veins and may reflect abnormal NOS activity or content between left and right lungs of the fetal lamb with CDH. Pulmonary arteries from CDH lambs have basal and stimulated NO release equal to that of historic controls but appear to be hypersensitive to exogenous vasoconstrictors.     

Experimental fetal tracheal ligation reverses the structural and physiological effects of pulmonary hypoplasia in congenital diaphragmatic hernia

Infants with congenital diaphragmatic hernia (DH) and profound pulmonary hypoplasia are currently unsalvageable. The authors previously demonstrated that tracheal ligation (TL) accelerates fetal lung growth and reverses the pulmonary hypoplasia of fetal nephrectomy. The purpose of this study was to determine if the pulmonary hypoplasia of experimental DH could be similarly reversed and, if so, whether the resulting lungs would show better function than those of their DH counterparts. Eighteen fetal lambs were divided into three experimental groups of six animals each. In group 1, DH was created at 90 days' gestation. In group 2, DH was created at 90 days' gestation and TL performed during the same operation. Group 3 consisted of sham-operated controls. These animals were delivered near full-term, and their lungs analyzed by standard morphometric techniques. Ten additional fetal lambs were divided into two experimental groups of five animals each. In group 4, DH was created at 90 days' gestation. In group 5, DH was created at 90 days' gestation and TL performed 20 days later, at 110 days' gestation. These animals were pressure-ventilated via tracheostomy over a 2-hour period in which PaO2, PaCO2, and compliance were measured. Intratracheal pressure (ITP) was measured at the time of delivery in all groups. Upon retrieval, DH animals had abdominal viscera in the chest and small lungs; in contrast, DH/TL animals had the herniated viscera reduced from the chest by enlarged lungs. DH/TL lungs showed markedly increased growth, with significant increases in lung volume:body weight ratio (LV:BW; P = .0001), alveolar surface area (ALV.SA; P = .0001), and alveolar number (ALV#) (P = .0001) when compared with those of the DH or control group. This growth was associated with a normal maturation pattern based on histological appearance, normal airspace fraction, and normal alveolar numerical density. ITP in the DH/TL group was increased when compared with that of DH and control animals (P = .0001). Total lung DNA and protein were both elevated in the DH/TL animals (P = .0001). However, the DNA:protein ratio remained normal, suggesting lung growth had occurred through cell proliferation, not by hypertrophy. When ventilated over a range of settings, DH/TL lungs were more compliant (P = .0001) and achieved higher PaO2s (P < .003) and lower PaCO2s (P = .0001) than their DH counterparts. From these data, the authors conclude: (1) Experimental fetal DH produces hypoplastic lungs that are not capable of adequate gas exchange with conventional ventilation.(ABSTRACT TRUNCATED AT 400 WORDS)     

Combined heart and single-lung transplantation in complex congenital heart disease

We present a patient with a history of tricuspid and pulmonary atresia who underwent a classic Glenn shunt and a Potts shunt during childhood, resulting in different right and left pulmonary physiology. Because of progression of cardiopulmonary disease and the fact that the right lung was "protected," the patient underwent combined heart-left single-lung transplantation. The postoperative course was uneventful. Potential early and late advantages of this approach include simplifying of the operative procedure and mitigating the potential effects of obliterative bronchiolitis.     

Laparoscopic repair of chronic traumatic diaphragmatic hernia: case report and review of the literature

This study compared the efficacy and tolerability of nisoldipine coat core (CC) 10-40 mg o.d. and hydrochlorothiazide (HCTZ) 25-50 mg o.d. Patients with mild-to-moderate essential hypertension received either nisoldipine CC 10 mg o.d. or HCTZ 25 mg o.d. Treatment was titrated at two-weekly intervals as necessary. The primary efficacy endpoint was a defined reduction in diastolic blood pressure (DBP). Response rates were similar for both the nisoldipine CC- and HCTZ-treated groups (74% and 70%, respectively). Secondary efficacy endpoints were reductions in both diastolic and systolic blood pressures (SBP). At treatment endpoint, the change from baseline in SBP was 16.2 mmHg for the nisoldipine CC group and 14.9 mmHg for the HCTZ group. Both drugs were well tolerated, and adverse events were generally minor and typical of these antihypertensive agents. Drug-related adverse events were greater in the nisoldipine CC- than the HCTZ-treated patients (50% and 37%, respectively). Nisoldipine CC was shown to demonstrate antihypertensive efficacy similar to HCTZ in the treatment of mild-to-moderate hypertension.     

Surfactant decreases pulmonary vascular resistance and increases pulmonary blood flow in the fetal lamb model of congenital diaphragmatic hernia

INTRODUCTION: Experiments using animal models of neonatal respiratory distress syndrome have shown a decrease in pulmonary vascular resistance (PVR) with surfactant replacement, whereas studies with the lamb model of congenital diaphragmatic hernia (CDH) have demonstrated improvement in oxygenation and lung mechanics with this therapy. The aim of the present study was to measure the effects of surfactant replacement therapy on the pulmonary hemodynamics of the lamb model of CDH. METHODS: Ten lambs with surgically created CDH and five control lambs were instrumented at term, with the placental circulation intact. Ultrasonic flow probes were positioned around the main pulmonary artery and the common origin of the left and right pulmonary arteries to record total lung and main pulmonary artery blood flow. Catheters were inserted to record systemic, pulmonary, and left atrial pressure. Five CDH animals received 50 mg/kg of surfactant by tracheal instillation just before delivery. All 15 animals were then ventilated for 4 hours. RESULTS: Correcting the surfactant deficiency in the CDH lamb resulted in a significant increase in pulmonary blood flow, a decrease in PVR, and a reduction in right-to-left shunting. These improvements in hemodynamics were associated with a significant improvement in gas exchange over 4 hours. CONCLUSION: The fetal lamb model of CDH has elevated PVR in comparison to controls. Prophylactic surfactant therapy reduces this resistance and dramatically increases pulmonary blood flow while reducing extrapulmonary shunt. A surfactant deficiency may be partially responsible for the persistent pulmonary hypertension in neonates with CDH.     

Current surgical management of congenital diaphragmatic hernia: a report from the Congenital Diaphragmatic Hernia Study Group

BACKGROUND: Repair of congenital diaphragmatic hernia (CDH) has changed from an emergent procedure to a delayed procedure in the last decade. Many other aspects of management have also evolved since the first successful repair. However, most reports are from single institutions. The lack of a large multicenter database has hampered progress in the management of congenital diaphragmatic hernia (CDH) and makes determination of the current standard difficult. METHODS: The CDH study group was formed in 1995 to collect data from multiple institutions in North America, Europe, and Australia. Participating centers completed a registry form on all live-born infants with CDH during 1995 and 1996. Demographic information, data about surgical management, and outcome were collected for all patients. RESULTS: Sixty-two centers participated, with 461 patients entered. Overall survival was 280 of 442 patients (63%) where survival was recorded. The defect was left-sided in 78%, right-sided in 21%, and bilateral in 1%. A subcostal approach was used in 91% of patients, with pleural drainage used in 76%. A patch of some kind was used in just over half (51%) of the patients, with polytetrafluoroethylene being the most commonly used material (81%) in those patients with a patch. The mean surgical time was 102 minutes, with an average blood loss of 14 mL (range, 0 to 500 mL). The overwhelming majority of patients underwent repair between 6:00 AM and 6:00 PM (289 of 329, 88%). Nineteen percent of patients had surgical repair on extracorporeal membrane oxygenation (ECMO) at a mean time of 170 hours into the ECMO course (range, 10 to 593 hours). The mean age at surgery in patients not treated with ECMO was 73 hours (range, 1 to 445 hours). CONCLUSIONS: The multicenter nature of this report makes it a snapshot of current management. The data would indicate that prosthetic patching of the defect has become common, that after-hours repair is infrequent, and that delayed surgical repair has become the preferred approach in many centers. Furthermore, the mean survival rate of 63% indicates that despite decades of individual effort, the CDH problem is far from solved. This highlights the need for a centralized database and cooperative multicenter studies in the future.     

Prediction of morbidity during infancy after repair of congenital diaphragmatic hernia

Recent studies have suggested there may be a high incidence of pulmonary and extrapulmonary sequelae among infants who have undergone repair of congenital diaphragmatic hernia (CDH). The aim of this study was to identify factors that predict high-risk cases to facilitate counseling of parents. Morbidity, defined as conditions requiring treatment and/or hospitalization during follow-up, was documented. Only five of 15 cases that had been diagnosed antenatally (at 16 to 26 weeks' gestation) and underwent surgical repair after preoperative stabilization were without morbidity at the time of follow-up. Respiratory problems were identified in seven (chylothorax in 3, recurrent infections in 4) and failure to thrive in four; three infants required further gastrointestinal surgery. A comparison of infants with and without morbidity showed that the only significant difference between the groups was in the duration of respiratory support. A requirement for respiratory support for more than 10 days had 90% sensitivity, 100% specificity, and a positive predictive value for morbidity at follow-up of 100%.     

Massive gastric enlargement with delayed presentation of congenital diaphragmatic hernia: report of three cases and review of the literature

In three cases of congenital diaphragmatic hernia presenting long after the newborn period, there was striking enlargement of the intraabdominal stomach. In each case there was herniation of the entire small bowel into the thorax and absence of a hernial sac. Despite the enormous capacity of the stomach, which was suggestive of obstruction preoperatively, no site of obstruction could be demonstrated at operation. This enlargement disappeared following surgical repair of the hernia.     

Wandering spleen presenting as duodenal obstruction after repair of congenital diaphragmatic hernia

The most common presentation of the wandering spleen in children is torsion with infarction. Duodenal obstruction by the spleen has not been reported previously. Wandering spleen can accompany congenital diaphragmatic hernia (CDH) because of its loss of retroperitoneal fixation. If absence of normal splenic fixation is found during repair of CDH, splenopexy should be performed to eliminate the risk of torsion, infarction, or, as described here, duodenal obstruction.