This is a case report of a sliding hiatal hernia with severe gastroesophageal reflux (GER) after repair of congenital diaphragmatic hernia(CDH). It was not possible to determine whether the hiatal hernia is a de novo lesion which was missed at the original operation or a consequence of overzealous repair of the Bochdalek defect at the expense of weakening of the diaphragmatic crura. This case demonstrates that a sliding hiatal hernia can be a cause of severe gastroesophageal reflux that should be managed surgically.
Gastroesophageal Reflux ; Hernia ; Hernia, Hiatal ; Hernias, Diaphragmatic, Congenital ; Herniorrhaphy

Adult ; Female ; Fetal Organ Maturity ; Hernia, Diaphragmatic ; complications ; Hernias, Diaphragmatic, Congenital ; Humans ; Infant, Newborn ; Lung ; pathology ; Myocardium ; pathology ; Pregnancy

Child, Preschool ; Cleft Lip ; complications ; surgery ; Cleft Palate ; complications ; surgery ; Hernia, Diaphragmatic ; complications ; surgery ; Hernias, Diaphragmatic, Congenital ; Humans ; Male

PURPOSE: Since 1971, Nitrofen (2,4-dichloro-4`-nitrodiphenyl ether) herbicide has been known to induce variable congenital abnormalities in rats. However, until now there has been no animal model of congenital diaphragmatic hernia (CDH) using this herbicide in Korea. Therefore, CDH in rats using Nitrofen was tried for searching on pathogenesis or using therapeutic modalities of CDH. METHODS: 20 Sprague-Dawley rats ingested Nitrofen as indicated on the 9.5th day of gestation, and after sacrificing on the 21th day of gestation. 243 offspring were harvested for the examination of diaphragmatic herniations. Dissections were made along sites of herniations to confirm the diaphragmatic herniation's site and size, lung/body weight ratio comparison as well as to accomplish microscopic radial saccular counting and evaluation of alveolar septal wall maturations and a comparision was made between the groups. RESULTS: CDH appeared in 149 of 243 offspring. CDH only appeared in the posterior portion of the diaphragm regardless of size or the side of appearance. Left sided CDH was the most common single diaphragmatic anomaly, represented in 112 offspring. Right sided CDH was next, found in 31 offspring, and bilateral CDH was seen in 6 offspring. Left sided CDH was almost always associated with visceral herniations and with pulmonary hypoplasia on the affected side, which was proved in the decreased Lung/Body weight ratio and in decreased radial saccular counts and in increased alveolar septal wall thickness in the affected lungs of CDH. In94 offspring without CDH following exposure to Nitrofen, moderate pulmonary hypo plasia was microscopically observed. CONCLUSION: Initial Rat modeling of congenital diaphragmatic hernia using Nitrofen showed results of herniation and pulmonary hypoplasia of the affected lungs acceptable for further experimental studies on CDH and accompaning pulmonary abnormalities.
Animals ; Animals* ; Congenital Abnormalities ; Diaphragm ; Hernia, Diaphragmatic* ; Korea ; Lung ; Models, Animal* ; Pregnancy ; Rats* ; Rats, Sprague-Dawley

Congenital diaphragmatic hernia (CDH) is often associated with major anomalies and chromosomal abnormalities. Chromosomal abnormalities are usually detected in 9.5% to 34% of fetuses with CDH prenatally diagnosed and the defect has also been reported in association with multiple syndromes such as Pallister-Killian syndrome, Fryns syndrome, Di George syndrome and Apert syndrome. Among the chromosomal abnormalities associated with CDH, trisomy 21, 18, and 13 are most common. Association with complex chromosomal aberrations such as mosaicism has also been reported. However, CDH presented in a fetus with Y-autosome translocation is extremely rare. Herein, we reported a case of fetus with 46,XY/46,X,-Y, +der(Y)t(Y;1)(q12;q12) mosaicism who presented with CDH diagnosed by ultrasonography at 19 weeks' gestation.
*Chromosome Aberrations ; Hernia, Diaphragmatic/*congenital/*diagnosis/genetics ; Humans ; Mosaicism/*embryology ; Prenatal Diagnosis/*methods

Pulmonary hypoplasia(PH) commonly occurs in association with oligohydramnios and other congenital anomalies, especially congenital diaphragmatic hernia (CDH). Pulmonary hypoplasia is an important factor, as persistent pulmonary hypertension, in the prognosis of CDH. In some reports, there is a decrement of pulmonary surfactant in PH accompanying CDH. Recently, there are some reports that exogenous pulmonary surfactant therapy is effective in experimental animal model and neonatal respiratory distress with PH. We report a case of a 5 day-old male neonate, who had shown dyspnea and diagnosed as left pulmonary hypoplasia accompanying CDH. The CDH was surgically treated and the ipsilateral PH, with intratracheal administration of exogenous pulmonary surfactant postoperatively. After exogenous pulmonary surfactant application, the left lung volume was increased on chest roentgenogram and lung perfusion scan findings, and there was an improvement in oxygenation and clinical manifestations. We suggest that postoperative exogenous pulmonary surfactant replacement therapy is effective in the case of PH and further trials are needed to clarify the optimal dose and timing of supplementation of surfactant for treatment of infants with PH accompanying CDH.
Case Report ; Hernia, Diaphragmatic/*congenital ; Human ; Infant, Newborn ; Lung/*abnormalities ; Male ; Pulmonary Surfactants/*therapeutic use

Fryns syndrome is a lethal syndrome of multiple congenital anomalies first described by Fryns et al in 1979. A recently developed major diagnostic criteria includes abnormal face, small thorax with widely spaced hypoplastic nipples, distal limb and nail hypoplasia, lung hypoplasia with diaphragmatic hernia, central nervous system anomalies and congenital heart disease. The pathogenesis of Fryns syndrome is not clear. Of the major immediate life-threatening abnormalities of this syndrome, lung hypoplasia associated with diaphragmatic hemia has usually proven to be fatal. We report a case of Fryns syndrome, which has the prenatal ultrasonographic findings of Dandy-Walker malformation and renal hypoplasia.
Central Nervous System ; Dandy-Walker Syndrome ; Extremities ; Heart Defects, Congenital ; Hernia, Diaphragmatic ; Lung ; Nipples ; Thorax

The authors have experienced two cases of general anesthesia for repair of congenital diaphragmatic hernia. Both patients were female (1 year 5 months and 8 months of age) and had left sided Bochdalek hernia. Anesthesia was maintained with halothane-oxygen using nonrebreathing system with Ruben valve, and the patients tolerated the whole procedure very well. The importance of maintaining adequate ventilation with high concentrations of oxygen and avoiding abdominal distension has been stressed.
Anesthesia ; Anesthesia, General ; Female ; Hernia ; Hernias, Diaphragmatic, Congenital* ; Humans ; Oxygen ; Ventilation

Giant Meckel's diverticulum is a very rare lesion and its association with a congenital diaphragmatic hernia has not been reported previously. We report a case of newborn with a giant Meckel's diverticulum and congenital diaphragmatic hernia. A large round atypical air-filled bowel segment was found by chest radiography preoperatively, and a giant Meckel's diverticulum was located within the left hemithorax during surgery.
Hernia, Diaphragmatic/*complications/congenital/*pathology ; Human ; Infant, Newborn ; Male ; Meckel Diverticulum/*complications/*pathology/surgery

Epidermal Growth Factor ; physiology ; Fetal Diseases ; therapy ; Hernia, Diaphragmatic ; complications ; therapy ; Hernias, Diaphragmatic, Congenital ; Humans ; Lung ; abnormalities ; pathology ; Prenatal Diagnosis ; Pulmonary Surfactants ; pharmacology ; Tumor Necrosis Factor-alpha ; physiology