Hernia diafragmatica en pediatria / Diaphragmatic hernia in pediatrics

Resumen La hernia diafragmática (HD) consiste en un trastorno en el desarrollo del músculo diafragmático, produciéndose el paso de contenido abdominal a la cavidad torácica. Las principales son hernia de Bochdalek, Morgagni y Hiatal.1 Malformación congénita que afecta a 1 de cada 2 200 recién nacidos. Está asociada a elevada morbimortalidad, principalmente por hipoplasia pulmonar e hipertensión.2 La ecografía prenatal determina el diagnóstico, pronóstico y terapia. La cirugía consiste en cerrar el defecto, siendo el objetivo del tratamiento mantener un adecuado intercambio gaseoso, evitar o minimizar la hipoplasia y la hipertensión pulmonar.1 Las hernias diafragmáticas presentes en el momento del nacimiento se diagnostican fácilmente. En contraste, el diagnóstico de las hernias después del periodo neonatal es muy variable, puede conducir a evaluaciones clínicas y radiológicas erróneas.3

Abstract The diaphragmatic hernia is a disorder in the development of the diaphragm, causing the passage of the abdominal contents into the thoracic cavity. The main ones are Bochdalek, Hiatal and Morgagni hernias1. Congenital malformation that affects 1 in 2 200 newborns. It is associated to high morbidity and mortality mainly due to lung hypoplasia and hypertension.2 Prenatal ultrasound determines the diagnosis, prognosis and therapy. The surgical procedure consists ofthe closure of the defect. The goal of the treatment is to maintain adequate gas exchange, to prevent or minimize hypoplasia and pulmonary hypertension.1 Congenital diaphragmatic hernias present at birth are readily recognized. In contrast, the diagnosis of diaphragmatic hernia after the neonatal period varies considerably owing to misleading clinical and radiologic features.3

Laparoscopic repair of a diaphragmatic hernia associated with radiofrequency ablation for hepatocellular carcinoma: lessons from a case and the review of the literature.

We describe the case of a patient with a diaphragmatic hernia associated with radiofrequency ablation for hepatocellular carcinoma who was successfully treated by laparoscopic surgery. A 62-year-old man with a long history of hepatitis C-induced liver cirrhosis was admitted to our institution because of recurrent postprandial periumbilical pain. Eight years earlier, he had undergone radiofrequency ablation for hepatocellular carcinoma at hepatic segment VIII. Computed tomography, gastrografin enema examination revealed transverse colon obstruction because of a diaphragmatic hernia. We diagnosed diaphragmatic hernia associated with the prior radiofrequency ablation treatment. The patient underwent laparoscopic repair of the diaphragmatic hernia. Though the patient experienced the recurrence once, relaparoscopic treatment has improved the patient's conditions. Thus, diaphragmatic hernia can develop as a complication of radiofrequency ablation treatment. A laparoscopic approach is safe, feasible, and minimally invasive, even in patients with cirrhosis who develop iatrogenic diaphragmatic hernia as a complication of radiofrequency ablation treatment.

Antenatal maternally-administered phosphodiesterase type 5 inhibitors normalize eNOS expression in the fetal lamb model of congenital diaphragmatic hernia.

PURPOSE: Pulmonary hypertension (pHTN), a main determinant of survival in congenital diaphragmatic hernia (CDH), results from in utero vascular remodeling. Phosphodiesterase type 5 (PDE5) inhibitors have never been used antenatally to treat pHTN. The purpose of this study is to determine if antenatal PDE5 inhibitors can prevent pHTN in the fetal lamb model of CDH. METHODS: CDH was created in pregnant ewes. Postoperatively, pregnant ewes received oral placebo or tadalafil, a PDE5 inhibitor, until delivery. Near term gestation, lambs underwent resuscitations, and lung tissue was snap frozen for protein analysis. RESULTS: Mean cGMP levels were 0.53±0.11 in placebo-treated fetal lambs and 1.73±0.21 in tadalafil-treated fetal lambs (p=0.002). Normalized expression of eNOS was 82%±12% in Normal-Placebo, 61%±5% in CDH-Placebo, 116%±6% in Normal-Tadalafil, and 86%±8% in CDH-Tadalafil lambs. Normalized expression of ß-sGC was 105%±15% in Normal-Placebo, 82%±3% in CDH-Placebo, 158%±16% in Normal-Tadalafil, and 86%±8% in CDH-Tadalafil lambs. Endothelial NOS and ß-sGC were significantly decreased in CDH (p=0.0007 and 0.01 for eNOS and ß-sGC, respectively), and tadalafil significantly increased eNOS expression (p=0.0002). CONCLUSIONS: PDE5 inhibitors can cross the placental barrier. ß-sGC and eNOS are downregulated in fetal lambs with CDH. Antenatal PDE5 inhibitors normalize eNOS and may prevent in utero vascular remodeling in CDH.

Rare diaphragmatic complications following cytoreductive surgery and HIPEC: report of two cases.

Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) have been proven to be an effective treatment modality for selected patients with peritoneal surface malignancies. The most common surgical complications after CRS and HIPEC are anastomotic leakage, intestinal obstruction and peripancreatitis. This case report presents two patients with early and late postoperative diaphragmatic hernia after CRS and HIPEC. One patient that initially had pseudomyxoma peritonei complained of persistent chest pain and dyspeptic disorder after peritonectomy and HIPEC and underwent reoperation due to a left diaphragmatic hernia containing the splenocolic flexure. The other patient had gastric cancer and peritoneal carcinomatosis and developed anastomotic leakage of the transverse colon anastomosis and pleural empyema. Stripping of the diaphragm is often performed during CRS, sometimes combined with diaphragmatic resection. A herniation through a diaphragmatic defect is rarely diagnosed during either the early or late postoperative period. Direct suture of the defect or closure with synthetic or biological tissue are possible surgical techniques for repair with a good prognosis.

Dietary vitamin A intake below the recommended daily intake during pregnancy and the risk of congenital diaphragmatic hernia in the offspring.

BACKGROUND: Vitamin A has been related to the etiology of congenital diaphragmatic hernia (CDH). We performed a case-control study to investigate whether maternal dietary vitamin A intake is related to CDH in the offspring. METHODS: Thirty-one pregnancies diagnosed with CDH and 46 control pregnancies were included during the study. After CDH diagnosis and inclusion of controls by risk set sampling, maternal vitamin A intake was investigated with a food frequency questionnaire. Serum retinol and retinol-binding protein were determined. Univariable and multivariable logistic regression models were used to calculate risk estimates with adjustment for potential confounders. RESULTS: We found no significant differences in the overall nutrient and vitamin A intake between case and control mothers. After stratification in body mass index (BMI) categories, case mothers with normal weight showed a lower energy adjusted vitamin A intake (685 vs. 843 µg retinol activity equivalents [RAEs] / day; p = 0.04) and a slightly lower serum retinol (1.58 vs. 1.67 µmol/L; p = 0.08) than control mothers. Vitamin A intake <800 µg retinol activity equivalents (recommended daily intake) in normal weight mothers was associated with a significantly increased CDH risk (odds ratio [OR], 7.2; 95% confidence interval [CI], 1.5-34.4; p = 0.01). Associations were not significantly different in underweight and overweight mothers. CONCLUSIONS: In normal-weight mothers, dietary vitamin A intake during pregnancy below the recommended daily intake is significantly associated with an increased risk of a child with CDH. This finding supports the retinoid hypothesis in human CDH, but warrants further investigation in larger study populations. Birth Defects Research (Part A), 2013. © 2013 Wiley Periodicals, Inc.

Transumbilical scarless surgery with thoracic trocar: easy and low-cost / 대한외과학회지

PURPOSE: Single-site laparoscopic surgery has become increasingly common. We herein report an easy and low-cost thoracic trocar technique (TTT) for these types of procedures and recommend the simpler name "transumbilical scarless surgery" (TUSS) to minimize confusion in nomenclature. METHODS: We retrospectively reviewed patients who underwent TUSS by TTT using a thoracic trocar and surgical glove in our hospital between November 2011 and November 2012. Operating time, postoperative stay, and complications were detailed. RESULTS: A total of 101 TUSS by TTT were successfully performed, comprising appendectomy (n = 63), ovarian cyst excision (n = 7), splenectomy (n = 5), nephroureterectomy (n = 5), orchidopexy (n = 4), pyeloplasty (n = 3), nephrolithotomy (n = 2), orchiectomy (n = 2), varicocelectomy (n = 2), lymphangioma excision (n = 2), ureterectomy (n = 1), Morgagni diaphragmatic hernia repair (n = 1), ovarian detorsion (n = 1), antegrade continence enema (n = 1), intestinal resection anastomosis (n = 1), and intestinal duplication excision (n = 1). Kirschner wires were used for some organ traction. Nine patients required an additional port, but no major complications occurred. The postoperative stay (mean +/- standard deviation) was 3.2 +/- 1.4 days, and operating time was 58.9 +/- 38.3 minutes. CONCLUSION: We recommend the simpler name of TUSS to minimize confusion in nomenclature for all transumbilical single-incision laparoendoscopic surgeries. TTT is an easy and low-cost TUSS technique.

Randomized double-blind placebo-controlled study of the efficacy of continuous infusion of local anesthetic to the diaphragm closure following laparoscopic hiatal hernia repair.

BACKGROUND: Laparoscopic repair of hiatal hernia can result in significant postoperative pain requiring use of narcotics and patient dissatisfaction. A catheter-based delivery method that has demonstrated effectiveness and safety in other laparoscopic and open procedures (ON-Q, I Flow Corporation) was used to deliver pain medicine. This randomized double-blind, placebo-controlled study evaluated the efficacy of continuous infusion of local anesthetic to the diaphragm closure post laparoscopic hiatal hernia repair. METHODS: After obtaining Institutional Review Board approval, qualifying patients undergoing laparoscopic repair of hiatal hernia voluntarily consented to the study protocol. Standard techniques for routine closure of hiatal hernia repair were used. The ON-Q pain pump catheter was placed adjacent to the sutures used to repair the hiatal hernia, so that it rested between the diaphragm and the collagen patch used to reinforce the hernia repair. The pump infused either bupivacaine 0.5% or NaCl 0.9% at 2 cc/h for 5 days postoperatively. Patients kept a daily diary for pain scores, number of narcotic pain pills taken, and number of nausea pills taken. RESULTS: Of the 46 patients enrolled in the study, seven were dropped for adverse events or noncompliance; 20 were given placebo (0.9% NaCl) and 19 were given 0.5% bupivacaine. CONCLUSION: This randomized double-blind, placebo-controlled trial showed no advantage in using the ON-Q pain pump in terms of providing measurable reduction of pain or concomitant narcotic or nausea medication use. Further studies are indicated to determine alternatives for reducing postoperative pain after laparoscopic hiatal hernia repair.

Prenatal retinoic acid upregulates connexin 43 (Cx43) gene expression in pulmonary hypoplasia in the nitrofen-induced congenital diaphragmatic hernia rat model.

PURPOSE: Connexin 43 (Cx43), a major gap junction protein, is necessary for alveologenesis and plays an important role in the differentiation of type II to type I alveolar epithelial cells. Knockout mice of Cx43 display severe pulmonary hypoplasia (PH). Prenatal administration of retinoic acid (RA) is known to stimulate alveologenesis in nitrofen-induced PH. Recent studies revealed that retinoids upregulate Cx43 expression. We hypothesized that gene expression of Cx43 is downregulated during alveologenesis and that administration of RA upregulates Cx43 expression in the nitrofen-induced PH. METHODS: Pregnant rats were exposed to olive oil or nitrofen on day 9 (D9) of gestation. Retinoic acid was given intraperitoneally on D18, D19, and D20. Fetal lungs were harvested on D18 and D21 and divided into control, nitrofen, control+RA (D21), and nitrofen+RA (D21). The Cx43 expression levels were determined using reverse transcription polymerase chain reaction and immunohistochemistry. RESULTS: On D18 and D21, Cx43 relative messenger RNA expression levels were significantly downregulated in nitrofen compared with those in the control group. On D21, expression levels of Cx43 were significantly upregulated in nitrofen+RA and control+RA compared with those in nitrofen group. Immunohistochemical studies confirmed these results. CONCLUSION: Downregulation of Cx43 expression may interfere with normal alveologenesis. Upregulation of Cx43 pulmonary gene expression after RA treatment may promote lung growth by stimulating alveologenesis in nitrofen-induced PH.

[Protective effects of tetrandrine against congenital diaphragmatic hernia in rats].

OBJECTIVE: To study the protective effect of the prenatal use of tetrandrine (TET) against congenital diaphragmatic hernia (CDH) in rats and possible mechanisms. METHODS: Pregnant female Sprague-Dawley rats were randomly divided into 3 groups: control, nitrofen and TET treatment. The later two groups were administered with nitrofen by gavage on day 9.5 of gestation. On day 18.5 of gestation, TET (30 mg/kg) was given by gavage (once a day, for three days) in the TET treatment group. On day 21 of gestation, parts of pregnant rats were delivered by cesarean section and amniotic fluid was collected. The fetal rats were examined for a diaphragmatic hernia. Lung histologic evaluations with microscope and immunohistochemistry staining of TNF-α were performed. TNF-α in amniotic fluid was detected using ELISA. The remaining pregnant rats were allowed to deliver spontaneously at term. The survival of pup rats was observed until 24 hrs of age. RESULTS: In the nitrofen group, significant lung hypoplasia was presented not only in fetuses with CDH but also in those without CDH. Stronger expression of TNF-α was observed in fetal lungs and amniotic fluid in the nitrofen group, even when CDH was absent. The TET treatment group showed improved lung development compared with the nitrofen group. The incidence of large diaphragmatic hernia in the TET treatment group was lower than that in the nitrofen group (P<0.05), and the expression of TNF-α in fetal lungs and amniotic fluid in the TET treatment group was also lower than in the nitrofen group (P<0.01). The 24-hr survival rate of pup rats in the TET group was higher than that in the nitrofen group (P<0.01). CONCLUSIONS: Prenatal use of TET can improve nitrofen-induced pulmonary hypoplasia, decrease the incidence of large diaphragmatic hernia and increase the survival rate of pup rats, possibly through a reduction in the production of TNF-α in fetal lungs and amniotic fluid in rats with CDH.

Transabdominal subcostal approach in surgical management of Morgagni hernia.

OBJECTIVE: Morgagni hernia is an uncommon type of diaphragmatic hernias whose pathophysiology is not clear. We aimed to retrospectively evaluate morbidity, mortality, and treatment outcomes in 13 cases of Morgagni hernia treated with the subcostal approach. MATERIAL AND METHODS: Between 1993 and 2009, 13 patients with Morgagni hernia were operated in our department. Of the 13 patients with a median age of 65 years (range: 13-78), 12 were female. Chest roentgenograms, thorax computed tomography (CT), and barium enema roentgenographic studies were used as diagnostic utilities. The contents of the hernia, as diagnosed with CT and confirmed at surgery, were omentum and colon in all patients. RESULTS: All the patients were operated electively except one patient on ventilation treatment. Transabdominal repair with the subcostal approach was performed in all patients. There was no operative morbidity and mortality. The median hospital stay was 8 days (range: 6-14 days). There was no recurrence during the follow-up of 48.8 months (1-132 months). CONCLUSION: We recommend the transabdominal subcostal approach in patients with Morgagni hernia for surgical exposure, easy repair of the hernia sac, and low morbidity.

Biological materials for diaphragmatic repair: initial experiences with the PeriGuard Repair Patch®.

BACKGROUND: We sought to analyze the efficacy of a bovine pericardial patch (PeriGuard®) for diaphragmatic repair. METHODS: Seven consecutive patients (6 males, median age 56 years) scheduled for diaphragmatic resection and/or repair were enrolled in this study. In all cases diaphragmatic repair was performed with a PeriGuard Repair Patch® (Synovis, St. Paul, MN, USA). At follow-up (median: 12 months; range: 6-18 months), quality of life, signs of reherniation and incorporation of mesh were assessed through clinical examination, blood samples and CT or MRT scan. RESULTS: Diagnosis on admission included sarcoma (n = 2), mesothelioma (n = 1), squamous cell carcinoma (n = 1), parachordoma (n = 1) and large congenital or posttraumatic herniation (n = 2). At follow-up successful diaphragmatic repair with no signs of reherniation, graft dehiscence or seroma formation was confirmed for all patients. Recorded inflammatory markers [C-reactive protein (CRP), white blood cell count (WBC) and procalcitonin (PCT)] reached their peak values between postoperative day (POD) 4 and POD 7. Values ranged from 122-282 mg/L for CRP, 0.4-4.6 µg/L for PCT and 6.2-15.6 Tsd/µL for WBC. Overall oncological results were good and 5 out of 6 survivors reported a fully reestablished quality of life. CONCLUSION: We consider the PeriGuard Repair Patch® a viable alternative to synthetic materials for diaphragm replacement. Moreover, we advise carrying out cautious follow-up in patients undergoing extensive oncological resection to learn more about the biological behavior of the bovine PeriGuard Repair Patch® after diaphragmatic repair.

Protective effects of tetrandrine against congenital diaphragmatic hernia in rats / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To study the protective effect of the prenatal use of tetrandrine (TET) against congenital diaphragmatic hernia (CDH) in rats and possible mechanisms.</p><p><b>METHODS</b>Pregnant female Sprague-Dawley rats were randomly divided into 3 groups: control, nitrofen and TET treatment. The later two groups were administered with nitrofen by gavage on day 9.5 of gestation. On day 18.5 of gestation, TET (30 mg/kg) was given by gavage (once a day, for three days) in the TET treatment group. On day 21 of gestation, parts of pregnant rats were delivered by cesarean section and amniotic fluid was collected. The fetal rats were examined for a diaphragmatic hernia. Lung histologic evaluations with microscope and immunohistochemistry staining of TNF-α were performed. TNF-α in amniotic fluid was detected using ELISA. The remaining pregnant rats were allowed to deliver spontaneously at term. The survival of pup rats was observed until 24 hrs of age.</p><p><b>RESULTS</b>In the nitrofen group, significant lung hypoplasia was presented not only in fetuses with CDH but also in those without CDH. Stronger expression of TNF-α was observed in fetal lungs and amniotic fluid in the nitrofen group, even when CDH was absent. The TET treatment group showed improved lung development compared with the nitrofen group. The incidence of large diaphragmatic hernia in the TET treatment group was lower than that in the nitrofen group (P<0.05), and the expression of TNF-α in fetal lungs and amniotic fluid in the TET treatment group was also lower than in the nitrofen group (P<0.01). The 24-hr survival rate of pup rats in the TET group was higher than that in the nitrofen group (P<0.01).</p><p><b>CONCLUSIONS</b>Prenatal use of TET can improve nitrofen-induced pulmonary hypoplasia, decrease the incidence of large diaphragmatic hernia and increase the survival rate of pup rats, possibly through a reduction in the production of TNF-α in fetal lungs and amniotic fluid in rats with CDH.</p>

Effects of estrogen on lung development in a rat model of diaphragmatic hernia.

PURPOSE: The study aimed to observe the influence of estradiol on rat models with congenital diaphragmatic hernia (CDH) and understand the potential mechanism. METHODS: Eleven pregnant female Sprague-Dawley rats were randomly divided into 3 groups on day 9.5 of gestation: group C (n = 2) was administered 2 mL of olive oil, whereas group N (n = 3) and group E (n = 6) were administered 200 mg of nitrofen. Antenatal estradiol was given subcutaneously to group E on days 18.5, 19.5, and 20.5 of gestation. Histologic evaluations, incidence of CDH, and the immunoreactivity of transforming growth factor (TGF)-ß1 in lung were observed. In addition, the mRNA levels of TGF-ß1, type I TGF-ß receptor (TßRI), and type II TGF-ß receptor (TßRII) were determined. RESULTS: Histologically, the lungs of group N fetuses were hypoplastic compared with those of group C and had thick-walled septa with poorly developed saccules. Group E showed improved mesenchymal differentiation with well-developed saccules. There was no significant difference between the incidence of CDH in group N and that in group E. The expression of TGF-ß1 in lung tissue and arterioles in group N were significantly higher than those in group C and E. Moreover, relative mRNA expression levels of TGF-ß1 and TßRI in group N were markedly higher than those in group C, whereas those in group E were significantly decreased compared with group N. CONCLUSIONS: Estradiol can promote lung development in rats with CDH. The down-regulation of TGF-ß1 and its signaling pathway may play a role in this effect.

Physiological and clinical characteristics of gastroesophageal reflux after congenital diaphragmatic hernia repair.

BACKGROUND/PURPOSE: Gastroesophageal reflux (GER) is an important sequela of congenital diaphragmatic hernia (CDH) repair. This study investigated the physiological and clinical characteristics of GER in CDH survivors. METHODS: A total of 52 CDH survivors were investigated retrospectively. Esophageal acid exposure was evaluated with 24-h esophageal pH monitoring in all patients, and esophageal anatomical and motor functional abnormalities were examined with videomanometry in 16 patients. RESULTS: Fundoplication was necessary in 1 patient. Medical treatment with acid suppression or rikkunshito, a traditional Japanese medicine, was successful in nine patients, and the reflux symptoms were ameliorated at the age of 3 years. The percentage of total time the esophageal pH was below 4.0 (reflux index: RI) ranged from 0.1 to 44.3%. No patient with an RI < 10% had reflux symptoms requiring treatment. The basal lower esophageal sphincter (LES) tone ranged from 15 to 35 mmHg (median 25 mmHg). Esophageal peristalsis was preserved in all of the patients examined, except one who had failed peristalsis and poor clearance in the dilated esophagus. CONCLUSION: The motor function of the esophageal body and LES is usually preserved in CDH survivors despite the wide range of esophageal acid exposure in early infancy. Those with symptomatic GER outgrow it, unless associated with advanced respiratory distress or neurological impairment.

Laparoscopic dual mesh repair of a diaphragmatic hernia of Bochdalek in a symptomatic elderly patient.

Bochdalek hernia is a congenital posterolateral diaphragmatic defect. It is usually diagnosed in newborns and children; the mortality rate is high due to further congenital anomalies such as pulmonary hypoplasia and pulmonary hypertension. In adulthood, the diagnosis is often incidental, while sometimes it is related to gastrointestinal or respiratory symptoms, or to herniated viscera complications. Thus, surgical treatment is advocated. We herein report a case of an 86-year-old woman with dyspnea and dysphagia. After the diagnosis by barium enema and CT scan of the herniated stomach and the greater omentum through a left side foramen of Bochdalek, she underwent a successful laparoscopic dual mesh repair and was discharged on the 6th postoperative day.

Understanding abnormal retinoid signaling as a causative mechanism in congenital diaphragmatic hernia.

Congenital diaphragmatic hernia (CDH) is a frequently occurring source of severe neonatal respiratory distress. It has been hypothesized that abnormal retinoid signaling contributes to the etiology of this developmental anomaly. Here, we use rodent models toward specifically understanding the role of retinoid signaling in the developing diaphragm and how its perturbation is a common mechanism in drug-induced CDH. This includes monitoring of retinoic acid (RA) response element (RARE) activation with RARE-lacZ mice, RA supplementation studies, systematic analyses of the expression profile of key elements in the RA signaling pathway within the developing diaphragm, and the in utero delivery of a RA receptor (RAR) antagonist. These data demonstrate the timing of RARE perturbation by CDH-inducing teratogens and the efficacy of RA supplementation. Furthermore, a detailed profile of retinoid binding proteins, synthetic enzymes, and retinoid receptors within primordial diaphragm cells was obtained. The expression profile of RAR-alpha was particularly striking in regard to its overlap with the regions of primordial diaphragm affected in multiple CDH models. Blocking of RAR signaling with the pan-RAR antagonist BMS493 induced a very high degree of CDH, with a marked left-right sidedness that depended on the timing of drug delivery. Collectively, these data demonstrate that retinoid signaling is essential for normal diaphragm development, providing further support to the hypothesis that abnormalities related to the retinoid signaling pathway cause diaphragmatic defects. This study also yielded a novel experimental model that should prove particularly useful for further studies of CDH.

Effect of prenatal tetrandrine administration on transforming growth factor-beta1 level in the lung of nitrofen-induced congenital diaphragmatic hernia rat model.

PURPOSE: Tetrandrine (Tet) is a bisbenzylisoquinoline alkaloid isolated from the root of Stephania tetrandra, which has been used in traditional Chinese medicine to treat patients with silicosis, asthma, and pulmonary hypertension, and others and can be used as a pulmonary therapeutic agent. We hypothesized that it can also improve the lung growth in congenital diaphragmatic hernia (CDH) for its multiple biological effects. There are increasing evidences that suggest transforming growth factor beta1(TGF-beta1) plays a crucial role in fetal lung growth and morphogenesis. The aim of this study was to evaluate the effect of prenatal administration of Tet and to investigate its possible mechanism on the expression of TGF-beta1 in the lung of nitrofen-induced CDH rat model. METHODS: A CDH model was induced in pregnant Sprague-Dawley rats by administration of nitrofen on day 9.5 of gestation (Ed9.5 term, day 22). Tetrandrine (30 mg/kg) was given through gavage (once a day, for 3 days) on Ed11.5. Accordingly, there were 3 groups as follows: control (n = 9), CDH (n = 9), and CDH + Tet (n = 9). All the fetuses were delivered by cesarean delivery on Ed16.5, 18.5, and 21.5, respectively, to check if diaphragmatic hernia existed on each fetus, then the lung tissue weight (LW) and body weight (BW) of each fetus were recorded. Histologic evaluations and TGF-beta1 immunohistochemistry staining in the lung sample were performed for image analysis. RESULTS: Diaphragmatic hernia was observed in 95 of the 112 rat fetuses in CDH and CDH + Tet groups on Ed18.5 and Ed21.5 (84.8%), the incidence between the 2 groups had no statistical significance (P = .642). Lung weight/body weight in the CDH group and the CDH + Tet group were lower than that in the control group (P < .01), and LW/BW in the CDH group was lower than that in the CDH + Tet group (P < .05). Observed under the light microscope and electron microscope, marked hypoplasia of the lungs in fetuses among the CDH groups was observed, in contrast to improvement of the lungs in CDH + Tet fetuses. Statistical differences in morphological parameters (percentage of alveoli area, counting bronchus) were found even on Ed16.5 when diaphragm had not closed (P < .01). The number of type II pneumocytes and lamellar bodies in each group had no significant difference (P > .05). The immunoreactivity of TGF-beta1 in CDH group and CDH + Tet group were markedly stronger than that in the control group (P < .01). In addition, TGF-beta1 expression in the CDH group was stronger than that in the CDH + Tet group (P < .01). CONCLUSION: Nitrofen can interfere with lung development early in the fetal rat development before and separate from diaphragm development, and increased expression of TGF-beta1 in the lung of CDH rat model may suppress lung growth and development. Prenatal treatment with Tet can improve the growth of the lung of the nitrofen-induced CDH fetuses and its mechanism seems to be involved in downregulating the expression of TGF-beta1. It is a likely new approach to treat CDH and its coexistent lung hypoplasia by maternal Tet administration.

The effect of maternal betamethasone and fetal tracheal occlusion on pulmonary vascular morphometry in fetal rabbits with surgically induced diaphragmatic hernia: a placebo controlled morphologic study.

OBJECTIVES: We studied the vascular effects of betamethasone (BM) and/or tracheal occlusion (TO) in fetal rabbits with surgically induced congenital diaphragmatic hernia (CDH). METHODS: At day 23 (pseudoglandular phase; term = 31 d), 54 ovarian-end fetuses from 27 does underwent induction of CDH. Thirteen did receive either 0.05 mg/kg BM, on days 28 and 29 with a 24-h interval, or 14 saline [controls (CTR)]. At day 28, one ovarian-end fetus underwent TO and harvesting was at term. In total, we compared (ANOVA) lung-to-body weight ratio (LBWR) and vascular morphometric indices in survivors from the following groups (n - number alive at delivery): CDH (9); CDH + TO (10); unoperated controls (14); CDH + BM (10); CDH + TO (9); controls CTR + BM (13). RESULTS: Maternal BM had no effect on LBWR. LBWR was comparable to normal in CDH fetuses undergoing TO. Both TO and BM have an effect on medial thickening due to CDH which is larger when both interventions are combined. CONCLUSIONS: Both TO and BM lessen peripheric muscularization present in CDH lungs and their effect is cumulative.

[Diaphragmatic hernia presenting with colonic strangulation]. / Colonileus som komplikation til spontant diafragmahernie.

Diaphragmatic hernia may be asymptomatic and may have a delayed clinical manifestation. We describe a 78-year-old man who developed colonic obstruction as a complication to a diaphragmatic hernia. Unlike most diaphragmatic hernias, this case was not associated with a trauma. Normally, the diagnosis is made clinically by means of thoracic X-ray, computer tomography or magnetic resonance imagining of the abdomen. In this patient, barium enema showed a tumor-like stenosis of the left colonic flexure, but intraoperative findings demonstrated a defect in the left diaphragm with herniation of the left colonic flexure and the greater omentum.

[Congenital diaphragmatic hernia in an adult: case report of acute abdomen]. / Ernia diaframmatica congenita nell'adulto: un caso di addome acuto.

Bochdalek diaphragmatic hernia usually begins during childhood, but may be an occasional finding even in adults. The treatment of choice is surgical repair to avoid herniated bowel complications. The operation often requires a combined approach consisting in thoracotomy and laparotomy. This is a convenient solution to eliminate the vascular risks (if there are additional concomitant embryonic defects, such as intestinal malrotation). We report a case of a female, aged 45 years, with epigastric cramp-like pain for 4 days and tenderness in the right abdominal quadrants during physical examination; the standard laboratory data showed decreased blood levels of calcium and potassium. Chest and abdominal X-rays revealed significant, widespread colic distension and the presence of a colic loop in the chest. We confirmed these results by CT and barium enema and proceeded with urgent surgery consisting in a right hemicolectomy (extended as far as the left part of the tranverse colon) for volvulus and with stitching of the diaphragmatic gap. We also discovered incomplete intestinal malrotation. After surgery, complete remission of the clinical symptoms was achieved. This case report demonstrates that, despite the apparent clinical silence, congenital diaphragmatic hernia in an adult may often manifest itself with particular gravity calling for urgent surgery.