1.A Case of Sliding Hiatal Hernia associated with Bochdalek Hernia Repair
Seok Jin NAM ; Hyun Hahk KIM ; Suk Koo LEE
Journal of the Korean Association of Pediatric Surgeons 1996;2(2):129-132
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
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Hernia
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Hernia, Hiatal
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Hernias, Diaphragmatic, Congenital
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Herniorrhaphy
4.Congenital Diaphragmatic Hernia in Rats Using Nitrofen: An Animal Model.
Whan Bong LEE ; Jai Kyoo LEE ; Pyong Chan LEE ; Byung Jun SO ; Kwon Mook CHAE
Journal of the Korean Surgical Society 2001;60(4):355-360
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
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Animals*
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Congenital Abnormalities
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Diaphragm
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Hernia, Diaphragmatic*
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Korea
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Lung
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Models, Animal*
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Pregnancy
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Rats*
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Rats, Sprague-Dawley
5.Prenatal Diagnosis of Congenital Diaphragmatic Hernia in a Fetus with 46,XY/46,X,-Y,+der(Y)t(Y;1)(q12;q12) Mosaicism: A Case Report.
Hyun Young AHN ; Jong Chul SHIN ; Yeon Hee KIM ; Hyun Sun KO ; In Yang PARK ; Sa Jin KIM ; Jong Gu RHA ; Soo Pyung KIM
Journal of Korean Medical Science 2005;20(5):895-898
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
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Hernia, Diaphragmatic/*congenital/*diagnosis/genetics
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Humans
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Mosaicism/*embryology
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Prenatal Diagnosis/*methods
6.Anesthetic Experience with Two Cases of Congenital Diaphragmatic Hernia .
II Soo KYUN ; Moo Kil PARK ; Jung Ung LEE
Korean Journal of Anesthesiology 1973;6(2):91-96
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
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Anesthesia, General
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Female
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Hernia
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Hernias, Diaphragmatic, Congenital*
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Humans
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Oxygen
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Ventilation
7.Giant Meckel's Diverticulum Associated with a Congenital Diaphragmatic Hernia.
In Suk KANG ; Soo Min AHN ; Airi HAN ; Jung Tak OH ; Seok Joo HAN ; Seung Hoon CHOI
Yonsei Medical Journal 2004;45(1):177-179
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
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Human
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Infant, Newborn
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Male
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Meckel Diverticulum/*complications/*pathology/surgery
8.Exogenous pulmonary surfactant replacement therapy in a neonate with pulmonary hypoplasia accompanying congenital diaphragmatic hernia: a case report.
Chong Woo BAE ; Chang Kyun JANG ; Sa Jun CHUNG ; Yong Mook CHOI ; Soo Myoung OH ; Tae Suk LEE ; Ok Young SHIN
Journal of Korean Medical Science 1996;11(3):265-270
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
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Hernia, Diaphragmatic/*congenital
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Human
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Infant, Newborn
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Lung/*abnormalities
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Male
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Pulmonary Surfactants/*therapeutic use
9.A Case of Fryns Syndrome.
Jun Ho KIM ; Jin Hwa JEONG ; Sung Min CHO
Journal of the Korean Pediatric Society 2000;43(9):1269-1273
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
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Dandy-Walker Syndrome
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Extremities
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Heart Defects, Congenital
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Hernia, Diaphragmatic
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Lung
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Nipples
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Thorax
10.A series of observation on the expression of TGF-beta1 in the lung of nitrofen-induced congenital diaphragmatic hernia rat model.
Chang XU ; Zhong-Xian CHEN ; Wen-Ying LIU ; Yuan-Xiang WANG ; Zhong-Xun XIONG
Chinese Journal of Surgery 2009;47(4):301-304
OBJECTIVETo access the expression of transforming growth factor beta1 (TGF-beta1) in the lung of Nitrofen-induced congenital diaphragmatic hernia rat model.
METHODSTwelve timed-pregnant Sprague-Dawley rats were randomly divided into two groups, namely control group and CDH group on day 9.5 of gestation. Each rat in the CDH group was given 125 mg of Nitrofen (dissolved in seed fat) by gavage. Each rat in the control group was given the same dose of single oil. On day 16 of gestation, the two groups mentioned above were divided into three subgroups, and fetuses were delivered by cesarean section respectively on day 16, 18 and 21 of gestation. After the fetuses were checked for diaphragmatic hernia, lung tissue weight (LW) and body weight (BW) of each fetus on gestational day 21 were recorded. Lung histologic evaluations were made with microscope and TGF-beta1 immunohistochemistry staining were performed with image analyzing.
RESULTSAt day 16 of gestation, no visible diaphragm closure was observed in all fetuses. Diaphragmatic hernia was observed in 32 of the 44 rat fetuses of the CDH groups on day 18 and day 21 of gestation (72.7%). Lw/Bw of the 21d subgroups of CDH group were lower than those of corresponding control group (P < 0.01). Observed under the microscope, the lungs of fetuses in CDH groups showed marked hypoplasia. The expression of TGF-beta1 was detected in cytoplasmic, without definite expression in nuclear. It was significantly stronger that the expression of TGF-beta1 was in the lungs of the CDH group than that of the control group (P < 0.01).
CONCLUSIONSNitrofen interferes with lung development in early stage of the fetal before the diaphragm developed. TGF-beta1 would be one of the important factors which lead to pulmonary hypoplasia.
Animals ; Disease Models, Animal ; Female ; Hernia, Diaphragmatic ; metabolism ; Hernias, Diaphragmatic, Congenital ; Lung ; metabolism ; Random Allocation ; Rats ; Rats, Sprague-Dawley ; Transforming Growth Factor beta1 ; metabolism