Lung liquid,fetal lung growth,and congenital diaphragmatic hernia

There is now a large body of evidence supporting the role of lung liquid in normal and experimental fetal lung growth. It is clear that tracheal ligation causes retention of lung liquid increased intratracheal pressure, and lung hyperplasia and that this hyperplastic effect can reverse the experimental pulmonary hypoplasia of clinically relevant entities such as congenital diaphragmatic hernia. Most importantly, this reversal is both structural and physiologic, as these lungs are more compliant and capable of normal gas exchange. In clinical cases of pulmonary hypoplasia, focusing initial therapy on active promotion of lung growth by exploiting a mechanism that may be a part of normal pulmonary development offers some hope of improved outcome in a group of patients that are currently unsalvageable.     

Surgical complications,especially gastroesophageal reflux disease,intestinal adhesion obstruction,and diaphragmatic hernia recurrence,are major sequelae in survivors of congenital diaphragmatic hernia

Purpose

This study aimed to characterize the surgical complications, especially gastroesophageal reflux disease (GERD), intestinal adhesion obstruction (IAO), and diaphragmatic hernia recurrence, in patients with congenital diaphragmatic hernia (CDH).

Methods

Between January 1995 and December 2013, we determined the incidence of surgical complications and their predictors in CDH patients. We also examined whether the CDH repair and patch closure were associated with the incidence of IAO and the severity of adhesion.

Results

Seventy-four CDH survivors were evaluated. GERD occurred in 28 patients (37.8 %) and recurred in 8 patients (10.8 %). Stomach herniation was a risk factor for GERD, and occurred in 25 patients. IAO occurred in 13 patients (17.6 %). In 240 neonatal laparotomies in the same period, the incidence of IAO was significantly higher in patients who underwent CDH repair than in patients who underwent other neonatal laparotomy (p = 0.023). Surgical time and intraoperative bleeding were significantly greater following CDH repair with an artificial patch compared with CDH repair with direct closure.

Conclusion

Surgical complications are major sequelae in survivors of CDH repair. CDH repair and artificial patch closure were significantly associated with the incidence of IAO and the severity of adhesion.     

Association of hiatus hernia with postero-lateral diaphragmatic hernia (Bochdalek's hernia)

A boy with hiatus hernia following the repair of the left postero-lateral diaphragmatic hernia (Bochdalek's hernia) was reported. At the age of one month, the repair of Bochdalek hernia was performed with transabdominal approach. At that time the stomach was located in the normal position. Eight days after the repair he developed vomiting and hiatus hernia was revealed by barium esophagram. Antireflux surgery was required because there was no response to the conservative management for two months. Esophageal pH study and manometric study were very useful for the diagnosis of hiatus hernia or GER and the evaluation of antireflux surgery.     

Intra-abdominal pressure monitoring in newborns with gastroschisis,omphalocele, and diaphragmatic hernia

Surgeons generally attempt to close even large abdominal defects primarily and without an implant if possible, so that the risk on an increase in intra-abdominal pressuresis always present. We recorded intra-abdominal pressures using a paracaval transducer in 14 children with gastroschisis (n = 6), omphalocele (n = 2), or diaphragmatic hernia (n = 6). Only 1 child had increased intra-abdominal pressure and postoperative problems including anuria. This method makes it possible to record intra-abdominal pressure immediately after closure of the abdominal wall, to detect too-small interponates, and to monitor the clinical course. We confirmed our results by postoperative flow measurements in the inferior vena cava using pulsed Doppler sonography. Offprint requests to: R. J. Schück     

Diaphragmatic hernia and eventration

Summary Five cases of diaphragmatic abnormalities are presented, one with congenital absence of the diaphragm and herniation of the gut into the thorax, and four wtth eventration. One case of eventration was associated with malrotation of the gut with a low placed liver and gallbladder and one with a high placed right kidney. Two patients with eventration presented with tuberculosis. Clinical features which might suggest a diagnosis have been presented. From the Department of Pediatrics, Medical College, Jabalpur.     

Fetal surgery for lung lesions,congenital diaphragmatic hernia,and sacrococcygeal teratoma

After more than 2 decades of experimental and clinical work, fetal surgery is an accepted treatment option for highly selected fetuses with life-threatening anomalies. Fetal lung masses associated with hydrops are nearly 100% fatal. These lesions can be resected in utero if they are predominantly solid or multicystic. Thoracoamniotic shunt placement may be effective in the setting of a single large cyst. Fetuses diagnosed with left congenital diaphragmatic hernia before 26 weeks' gestation with associated liver herniation and a low right lung to head circumference ratio have a relatively poor prognosis with conventional therapy after birth, but in utero therapeutic approaches have yet to show a comparative survival benefit. A prospective randomized trial is required to critically evaluate the efficacy of fetal tracheal occlusion for severe diaphragmatic hernia. Fetal sacrococcygeal teratoma complicated with progressive high output cardiac failure may benefit from in utero resection of the tumor.     

Testicular descent, cryptorchidism and inguinal hernia: the Melbourne perspective

Cryptorchidism is the commonest congenital genitourinary anomaly in males and results when the testis does not descend into its normal intrascrotal position during development. In full-term infants, the incidence is approximately 3% at birth. Cryptorchidism results in several abnormalities, including attenuated spermatogenesis, infertility and a greater risk of malignancy. The normal mechanism of testicular descent appears to be multi-staged, with various anatomical factors and hormonal influences, but the exact process is still unclear. In this article we review the current theories of normal testicular descent, with a focus on the hormones and anatomical factors, and current treatments for undescended testis.     

Acute gastric volvulus and congenital diaphragmatic hernia,case report and review

Congenital diaphragmatic hernia (CDH) is the result of the incomplete fusion and closure of the pleuroperitoneal canal during the fetal development. CDH is usually diagnosed prenatally but, if undiagnosed, the clinical presentation ranges from asymptomatic children to serious respiratory or gastrointestinal symptoms. Acute gastric volvulus associated with CDH is a rare surgical emergency in children. We report two cases of acute gastric volvulus associated with CDH and review the literature.Key words: Acute gastric volvulus, children, congenital diaphragmatic hernia, gastropexy     

Cardiac dysfunction in congenital diaphragmatic hernia: Pathophysiology,clinical assessment,and management

Cardiac dysfunction is an important consequence of pulmonary hypertension in congenital diaphragmatic hernia and a determinant of disease severity. Increased afterload leads to right ventricular dilatation and diastolic dysfunction. Septal displacement and dysfunction impair left ventricular function, which may also be compromised by fetal hypoplasia. Biventricular failure contributes to systemic hypotension and hypoperfusion. Early and regular echocardiographic assessment of cardiac function and pulmonary artery pressure can guide therapeutic decision-making, including choice and timing of pulmonary vasodilators, cardiotropes, ECMO, and surgery.     

Diaphragmatic hernia

We describe a case of acute dyspnea in a patient with Marfan syndrome secondary to bowel herniation into the thoracic cavity.     

Congenital diaphragmatic hernia: new models,new ideas

An animal model for congenital diaphragmatic hernia following interference with the development of the primary lung bud by 2,4-dinitro-p-diphenylether (nitrofen) is described. It has been used for pathogenetic studies to evaluate the presence of pulmonary hypoplasia and a closing defect of the diaphragm. Functional studies revealed abnormal surfactant levels and differences in pressure/volume curves following birth and during artificial ventilation for 6 h together with a disturbed antioxidant enzyme response. This animal model opens up new ways of studying the effects of prenatal hormonal modulation (corticosteroids, thyrotrophin-releasing hormone) on lung development as a novel therapeutic modality.     

Sex and congenital diaphragmatic hernia

Background and purpose: Human studies note sex reversal syndromes and sex difference(s) in the incidence of congenital diaphragmatic hernia (CDH). Epidemiology surveys record a higher incidence of CDH in females, whilst other reports cite a higher frequency in males. Nitrofen, a teratogen, produces experimental CDH. This agent is speculated to interfere with retinoid acid–steroid signalling pathways and may also be linked with sexual differentiation. This study was designed therefore to test the hypothesis that nitrofen may influence sexual phenotype and frequency of CDH. Methods: Time mated Sprague Dawley rats were dosed with nitrofen at day 9.5 to generate predominantly left sided CDH. Fetuses were delivered by caesarean section on days 20 or 21 of gestation (term=day 22). External genitalia were examined to define external genital phenotype. The abdominal cavity was opened and the genito-urinary system carefully examined. The internal genital organs were assigned a phenotype and findings correlated with external appearances. The diaphragm of each fetus was studied for the absence or presence of CDH and the laterality of defect recorded. Controls (non nitrofen fed) were used for all comparative analysis. Results: Control (n=600) and nitrofen exposed offspring (n=504) had equal frequencies of males and females. CDH occurred with similar incidence in male and female nitrofen treated pups. In all nitrofen exposed fetuses and normal controls, internal and external genitalia concorded without evidence of significant genital tract malformations or intersex states. Conclusions: Prenatal nitrofen exposure is not associated with significant gender differences (or prenatal loss) in the risk of CDH. Genital tract malformations do not appear to accompany CDH in the nitrofen model.     

Recurrent bronchopneumonia and Morgagni hernia

The Morgagni hernia occurs behind the sternum and is a rare diaphragmatic defect although the late diagnosis may result in important clinic symptoms and poor prognosis. The authors present a case report of a child with 11 months and respiratory symptoms with chronic pulmonary infections and several hospitalizations without etiologic diagnosis. The conclusion was for the early Morgagni hernia diagnosis and the prevention of chronic complications.