Chylothorax after congenital diaphragmatic hernia repair

Purpose

Chylothorax after congenital diaphragmatic hernia (CDH) repair contributes significantly to morbidity. Our aim was to identify factors contributing to chylothorax and effective treatment strategies.

Methods

We reviewed 171 patients with CDH from 1997 to 2008 and analyzed hernia characteristics, extracorporeal membrane oxygenation (ECMO) use, operative details, and treatment approaches for chylothorax.

Results

Ten (7%) patients developed chylothorax; all were left sided. Using univariate analysis, prenatal diagnosis, ECMO use, and patch repair were associated with development of chylothorax. Logistic regression analysis showed that patch repair was the only variable predictive of chylothorax (P = .028; confidence interval, 0.032-0.823). Although survival was not affected, patients with chylothorax had a significant increase in ventilator days and length of stay (t = 3.57; P = .000; t = 2.74; P = .007). All received thoracostomy and total parenteral nutrition. Six patients received octreotide, 5 of whom required pleurectomy because of failed medical management; the remaining patient died of overwhelming sepsis.

Conclusions

The incidence of chylothorax at our institution was relatively low. Patch repair was associated with the formation of chylothorax. Morbidity was substantial, but survival was not significantly affected. Total parenteral nutrition and thoracostomy were appropriate initial treatments. Octreotide was not an effective adjunct. Refractory cases were successfully treated with pleurectomy.     

Abdominal complications related to type of repair for congenital diaphragmatic hernia

BACKGROUND: Literature on congenital diaphragmatic hernia (CDH) over the past few decades has focused on prognostic factors and management of pulmonary hypertension/hypoplasia. Larger diaphragmatic defects may require patch closure, reported by some authors to be associated with poorer outcomes. In this study, we evaluate the impact synthetic material has on the need for subsequent abdominal operations, particularly recurrence and small bowel obstruction (SBO). METHODS: After obtaining IRB approval, all patients undergoing repair of congenital diaphragmatic from January, 1994 to December, 2004 were investigated. Records from primary and subsequent admissions were reviewed to identify those patients who underwent major procedures after repair of the diaphragmatic defect. Subsequent abdominal operations in these series were recurrent CDH repair, exploration for SBO and fundoplication. Patients who died prior to hospital discharge were excluded. Statistical comparisons were made using Fisher's exact test: significance was defined as P<0.05. RESULTS: During the study period, there were 81 survivors from CDH repair, 24 with a synthetic patch, and 57 without. Those with a patch repair had a significantly increased risk of recurrence, small bowel obstruction, and subsequent operation (Table 1). Eleven patients had nonabsorbable mesh patches, and 13 were repaired with absorbable (Surgisis-Gold; Cook Technology Inc., West Lafayette, IN). While there were no differences in recurrence between these two groups, four patients (31%) with Surgisis developed SBO compared with one patient (9%) repaired with a nonabsorbable synthetic. CONCLUSIONS: Incidence of SBO and recurrent CDH in patients with a patch was higher than those who underwent primary repair. There may also be a difference in the rate of subsequent SBO depending on the type of mesh used. A prospective trial is under way at our institution to help define this issue.     

Intrapericardial diaphragmatic hernia: a rare type of congenital diaphragmatic hernia

An extremely rare case of congenital intrapericardial diaphragmatic hernia is presented. Since 1981, only 14 cases have been reported in the literature. A 5-year-old girl presented with dyspnea on exertion and easy fatigability. Computed tomography was suggestive of an anterior diaphragmatic hernia. Laparoscopy followed by successful open repair of hernia was performed.     

腹腔镜小儿先天性膈疝修补术

目的探讨腹腔镜小儿先天性膈疝修补术的疗效。方法2002年6月-2005年12月，我们对11例小儿先天性膈疝（3例新生儿年龄3—24h，余8例2—24个月）施行三孔或四孔腹腔镜手术。还纳疝内容物，丝线缝合膈肌修补缺损。结果10例手术修补成功。1例新生儿腹腔镜手术后3d膈疝复发，再次腹腔镜手术修补成功。手术时间55—180min，平均100min，术中出血量1—2ml。术后1—2d开始进食。11例随访9—24个月，平均16个月，X线检查膈肌位置正常。结论腹腔镜小儿先天性膈疝修补术安全可靠，创伤小，可清晰显露术野，术中还可探查有无腹部其他先天性畸形。     

“Acquired” congenital diaphragmatic hernia

“Acquired” congenital diaphragmatic hernia (ACDH), has been defined as delayed or late appearance of a congenital diaphragmatic hernia after a documented time period of postnatal life with no evidence of herniation. Three new cases are presented. This diagnosis has been characterized on the basis of a review of these cases and 14 additional patients from the literature. A classification based upon timing of herniation and state of pulmonary development is presented.     

Endobronchial migration of prosthetic patch after congenital diaphragmatic hernia repair

Surgical repair of congenital diaphragmatic hernia (CDH) can be performed by means of either direct suturing of the diaphragm or positioning of a prosthetic patch. However, half of all prosthetic patches show evidence of reherniation. We describe the case of an 8-year-old girl who presented with prosthesis dislocation and fistulization in the right lower bronchus as a complication of a CDH repair that she underwent when she was 1 year old. Abdominal ultrasound and magnetic resonance imaging suggested a hernia relapse, whereas chest computed tomographic scan failed to identify the diaphragmatic defect. Only fibrobronchoscopy allowed fistulization of the prosthesis into the bronchi to be correctly diagnosed.     

Laparoscopic repair of congenital diaphragmatic hernia complicated with sliding hiatal hernia with reflux in adult

INTRODUCTIONCongenital diaphragmatic hernia (CDH) in adults is a relatively rare condition being asymptomatic in the majority of cases. Symptomatic CDH should prompt surgical management because they may lead to intestinal obstruction or severe pulmonary disease. This is the first reported case of a symptomatic CDH complicated with sliding hiatal hernia (SHH).PRESENTATION OF CASEA 65 years old women with reflux and dysphagia was complaining of postprandial paroxysmal dyspnea and epigastric pain radiating to her back. Upper endoscopy diagnosed sliding and para-esophageal diaphragmatic hernia with severe esophagitis. Computed tomography-scan revealed a large Bochdalek hernia at the left diaphragm.DISCUSSIONDiagnostic laparoscopy was decided, which confirmed the SHH, but also revealed a CDH defect at the tendonous part of the left diaphragm. The left bundle of the right crus was intact, separating the two hernia components (sliding and congenital). Extensive adhesiolysis was performed, dissecting and separating the stomach away from the diaphragm. Posterior cruroplasty at the esophageal hiatus was performed for the SHH with Nissen fundoplication as antireflux procedure. Primary continuous suture repair was performed for the CDH, reinforced with prosthetic mesh on top. Operative time was 150 min with no morbidity. The patient was discharged home uneventfully the third postoperative day. On 12-months follow-up, she reported no symptoms and improvement in quality of life.CONCLUSIONLaparoscopy is a unique method for a precise diagnosis of symptomatic congenital diaphragmatic hernia in adults being also a safe and viable technique for a successful repair at the same time. Experience of advanced laparoscopic surgery is required.     

Minimally invasive repair of congenital diaphragmatic hernia

Purpose

Operative approach, including minimally invasive surgery (MIS) in the repair of congenital diaphragmatic hernia (CDH), is variable among institutions. The short-term recurrent hernia rate is not well described. We evaluated the in-hospital recurrence rate of MIS repairs of infants with CDH from the Congenital Diaphragmatic Hernia Registry.

Methods

Prospectively collected data from infants with CDH were analyzed from the Congenital Diaphragmatic Hernia Registry from January 1995 to January 2010. Recurrent hernia was defined as reoperations during initial hospitalization. Operative approaches included abdominal, thoracic, laparoscopic, and thoracoscopic techniques.

Results

Five thousand four hundred eighty infants with CDH were identified, of which 4516 (82.4%) were repaired. Operative data were available in 4390 infants. One hundred fifty-one infants (3.4%) underwent MIS repairs with 12 reported recurrences (7.9%) compared with 114 for open techniques (2.7%, P < .05). Minimally invasive surgery demonstrated a significant increased odds for recurrence (odds ratio, 3.59; 95% confidence interval, 1.92-6.71) after adjusting for gestational age, birth weight, patch repair, and extracorporeal membrane oxygenation.

Conclusion

Minimally invasive techniques appear to have a significant higher recurrent hernia rate, with thoracoscopy being the highest. Although adjusted for patch repair, other factors with regard to disease severity may contribute to differences in outcomes among centers. This study is limited to short-term recurrence during initial hospitalization.     

除草醚诱导的小鼠先天性膈疝肺发育不良模型的建立

目的 建立小鼠先天性膈疝(CDH)模型.方法 实验组10只妊娠第8天的BABL/C小鼠通过灌胃给于除草醚25 mg/只,正常对照组给予橄榄油,于妊娠第20天剖宫产取出子鼠,解剖显微镜下观察子鼠有无膈疝形成,测定子鼠体重及双肺重量,HE染色观测肺组织发育情况,逆转录-聚合酶链反应(RT-PCR)检测肺组织SP-B、SP-C和VEGF表达水平.结果 实验组69只子鼠中有膈疝形成者39只,成功率为56.5%,子鼠双肺重量较对照组显著降低(P＜0.01);实验组有膈疝形成和无膈疝形成者的肺组织均发育不良,处于假腺体期和原始肺小管期;与对照组比较,实验组胎肺组织中SP-B、SP-C和VEGF表达水平均显著下调(P＜0.01),且与膈疝形成与否无关.结论 采用除草醚能在小鼠成功建立CDH模型,具有简便、成功率高的优点,为深入研究CDH发病机制及其治疗提供了新的手段.     

Early repair of congenital diaphragmatic hernia on extracorporeal membrane oxygenation

Background

Timing of repair of congenital diaphragmatic hernia (CDH) in babies that require stabilization on extracorporeal membrane oxygenation (ECMO) remains controversial. Although many centers delay operation until physiologic stabilization has occurred or ECMO is no longer needed, we repair soon after ECMO has been initiated. The purpose of this study is to determine if our approach has achieved acceptable morbidity and mortality.

Methods

Charts of live-born babies with CDH treated at our institution between 1993 and 2007 were retrospectively reviewed. Data were then compared with The Congenital Diaphragmatic Hernia Study Group and Extracorporeal Life Support Organization registries.

Results

Forty-eight (39%) patients required ECMO Thirty-four of these 48 neonates were cannulated before operative repair. Venoarterial ECMO was used exclusively. The mean (SD) time of repair from cannulation was 55 (21) hours. Survival for this subset of patients was 71%. Three patients (8.8%) who underwent repair on ECMO experienced surgical site hemorrhage that required intervention.

Conclusion

Early repair of CDH in neonates on ECMO can be accomplished with acceptable rates of morbidity and mortality.     

Laparoscopic repair of congenital diaphragmatic hernia in a 6-month-old child

This report describes the laparoscopic approach of closure of a congenital left posterolateral diaphragmatic hernia in a 6-month-old boy. The pros and cons of such an approach are discussed.     

Robotic repair of Morgagni congenital diaphragmatic hernia in an infant

We describe a robotic repair of a large Morgagni congenital diaphragmatic hernia in a 12-month-old infant using the da Vinci surgical robot.     

Free fascia lata repair for a second recurrent congenital diaphragmatic hernia

Repair of recurrent congenital diaphragmatic hernia (CDH) continues to be a difficult problem. Although several materials have been used to repair recurrent CDH, the ideal material has yet to be established. We report the successful use of an autologous free fascia lata graft to repair the diaphragm following a second recurrence of CHD. The fascia lata is one of the strongest fascia in the body and is easy to obtain without extremity functional loss even in children. This procedure is regarded as effective for the repair of recurrent CDH.     

Long-term functional evaluation of diaphragmatic motility after repair of congenital diaphragmatic hernia

Background/Purpose

A few studies have taken into account the diaphragmatic function in patients successfully treated for congenital diaphragmatic hernia (CDH). Monodimensional sonography has been reported to be useful in assessing the diaphragmatic motility. Aim of the present study was to investigate, in a long-term follow-up, the diaphragmatic function after CDH repair.

Methods

Ten patients, with a mean age of 16 (5-26) years, were enrolled. All had had a left diaphragmatic hernia repaired, but no one received a patch. Ten subjects of matched age were used as controls. The diaphragmatic excursions appear, at M-mode sonography, as a sinusoid; the amplitude of the curve on the vertical axis measured the movement in centimeters. Chest x-ray and spirometry were also performed in CDH patients.

Results

A reduced diaphragmatic motility on the left (treated) side was recorded. The amplitude of the contraction was significantly reduced when compared with the contralateral side (1.19 ± 0.2 vs 2.33 ± 0.9 cm; P = .017) and was also significantly reduced in comparison with the motion of the left side of controls (1.19 ± 0.2 vs 1.83 ± 0.4 cm; P = .01). There was no difference in the amplitude of contraction between the left and right sides of control patients and between the right side of CDH patients and the controls. Spirometry was normal in all patients but one, who had a slight reduction of ventilation on the left side.

Conclusion

M-mode sonography appears as a very useful tool in quantitative evaluation of diaphragmatic movements and should be extensively used during follow-up of patients after CDH repair. Motility of the repaired diaphragmatic is reduced, even after a long period, but this does not affect the respiratory function in patients who survived CDH repair.     

Thoracoscopic repair for congenital diaphragmatic hernia: lessons from 45 cases

Purpose

To describe the surgical technique, initial results, and overview indications of thoracoscopic repair of congenital diaphragmatic hernia (CDH).

Materials and Methods

A retrospective review was undertaken of patients with CDH who underwent thoracoscopic repair by the same surgeon from January 2001 to January 2005. Patients underwent surgery under general anesthesia. Reduction of the hernia contents was carried out using 1 optical trocar and 2 operating trocars. Pleural insufflation with carbon dioxide was maintained at a pressure of 2 to 4 mm Hg. The hernia defect was repaired using nonabsorbable interrupted sutures with extracorporeal knots.

Results

There were 45 patients, including 29 boys and 16 girls. Among 19 newborn patients, there were 13 patients younger than 7 days. The other 26 patients were infants and elders. The hernia was located in the left side in 37 patients and in the right side in 8 patients. The mean operative time was 54 minutes. Conversion was required in 4 patients. There were no complications. However, there were 2 postoperative deaths.

Conclusions

Thoracoscopic repair is feasible and safe for children with CDH, including selective newborn. The technique causes minimal trauma, results in good respiratory function, and promotes early recovery.     

Immunohistochemical distribution of bombesin-positive pulmonary neuroendocrine cells in a congenital diaphragmatic hernia

Morphometrical analyses of the immunohistochemical expression of bombesin, which is one of the peptides produced by pulmonary neuroendocrine (PNE) cells, were carried out on the bronchioles of human congenital diaphragmatic hernia (CDH) neonates, and the findings were then compared with those in a gestational and postnatal age-matched control group. As a result, no difference was found in the number of bombesin-positive cells between the lungs of the control group and the unaffected side lungs in the CDH group except for the ratio of the bombesin-positive cells per unit of the bronchiolar surface area (P<0.05). However, compared with the lungs in the control group, the affected side of the lungs in the CDH group showed a significant increase in the expression of bombesin, namely, the ratio of the bombesin-positive cells per bronchiole (P<0.05), the ratio of the bombesin-positive cells per unit perimeter of the bronchioles (P<0.05), and the ratio of the bombesin-positive cells per unit of the bronchiolar surface area (P<0.01). These results thus suggest that hyperplasia of the PNE-cell system in the lungs of the CDH cases, especially on the affected side, exists in human fetuses. We also further speculate that PNE cells may thus play a role in the problems associated with CDH during intrauterine life in human beings.     

Late-presenting congenital diaphragmatic hernia

Background

Late-presenting congenital diaphragmatic hernia (CDH) is a rare subset of CDH, most of the information derived from small series or case reports. The aim of this study was to document the clinical manifestations of late-presenting CDH using a large multicenter database.

Methods

Information about late-presenting CDH (diagnosed at later than 30 days of age) was identified from the database of the CDH Study Group (3098 cases collected during 1995-2004) and reviewed retrospectively.

Results

Seventy-nine cases (2.6%) from 30 centers met the inclusion criteria. Seven cases had a Morgagni hernia. There were 50 males (65%) and 27 females (35%). The mean age at diagnosis was 372 days (32 days to 15 years). Major associated anomalies (10 cardiac and 7 chromosomal abnormalities) were identified in 12 cases (15%). Presenting symptoms were respiratory in 20 (43%), gastrointestinal in 15 (33%), both in 6 (13%), and none (asymptomatic) in 5 (11%). The hernia was left-sided in 53 (69%), right-sided in 21 (27%), and central or bilateral in 3 (4%). Patients with gastrointestinal symptoms invariably had left-sided hernias (n = 19), whereas patients with respiratory symptoms (n = 24) seemed equally likely to have right- or left-sided lesions. A primary repair without patch was done in all cases with 100% survival.

Conclusions

Presenting symptoms of late-onset CDH can be respiratory or gastrointestinal, but presentation with gastrointestinal problems was more common in left-sided hernias, whereas respiratory symptoms predominated in right-sided lesions. The prognosis is excellent once the correct diagnosis is made.     

胸腔镜治疗新生儿先天性膈疝的围手术期监护

目的探究胸腔镜治疗新生儿先天性膈疝（CDH）的围手术期监护护理效果。 方法选取2010年1月至2018年10月,首都医科大学附属北京潞河医院就诊的64例先天性膈疝患儿,随机分为综合护理组（32例）和常规护理组（32例）,常规护理组给予围手术期常规护理,综合护理组则在此基础上给予综合护理。分别观测并比较2组患儿的围手术期指标、血气分析指标、舒适度、依从性以及护理质量评分和术后并发症发生情况。 结果综合护理组患儿的手术时间、术中出血量分别为（114.29±11.54）min、（2.58±1.01）ml与常规护理组（116.83±12.73）min、（2.81±1.03）ml比较,差异无统计学意义（t=0.862、0.900,P=0.392、0.356）;综合护理组住院时间、术后机械通气时间（12.18±4.11）d、（2.04±1.17）d均低于常规护理组（17.52±5.25）d、（2.89±2.03）d,差异均有统计学意义（t=4.670、2.115,P<0.001、=0.038）。2组患儿血气指标在术前、气腹后10 min、放气后30 min 3个时间点组间比较,差异均无统计学意义（P>0.05）。气腹后10 min与术前比较,2组患儿组内PaCO₂、气道峰压、呼气末CO₂分压均增加,差异均有统计学意义（P<0.05）;放气后30 min与术前比较,2组患儿的PaCO₂、气道峰压、呼气末CO₂分压均差异无统计学意义（P>0.05）。综合护理组患儿的舒适度、依从性和护理质量评分分别为（91.05±10.22）分、（91.48±7.64）分和（93.11±10.08）分均高于常规护理组（81.69±9.81）分、（80.27±7.05）分和（80.97±8.19）分,差异均有统计学意义（t=3.853、6.288、5.450,P均<0.001）。综合护理组术后并发症的发生率（3.125%）低于常规护理组（21.875%）,差异有统计学意义（χ²=5.143,P=0.023）。 结论综合护理干预在胸腔镜膈疝修补术治疗新生儿CDH中具有重要作用,可明显缩短患儿术后恢复时间,使血气指标维持正常平稳,有效降低术后并发症的发生率,显著提高患儿的舒适度、依从性和护理满意度,值得临床推广。     

Octreotide for treatment of chylothorax after repair of congenital diaphragmatic hernia

Chylothorax is a recognized cause of morbidity after repair of congenital diaphragmatic hernia (CDH). Management may include prolonged hospitalization with cessation of enteral feedings, repeated aspiration, chest tube drainage, total parenteral nutrition, and introduction of a medium chain triglyceride (MCT) diet as the effusion resolves. The authors report that the successful deployment of octreotide, a somatostatin analogue, hastened resolution of a postoperative chylothorax in a newborn infant with CDH.     

Influence of extracorporeal membrane oxygenation on subsequent surgeries after congenital diaphragmatic hernia repair

Purpose

Congenital diaphragmatic hernia (CDH) is a complex anomaly requiring intensive pulmonary and hemodynamic management. Survival has increased in this population placing them at risk for subsequent morbidities including surgery. The purpose of this study is to review the need for subsequent surgeries in the CDH population.

Methods

After receiving institutional review board approval, a retrospective chart review of all CDH patients between 1980 and 2007 was conducted noting subsequent surgeries and the impact of extracorporeal membrane oxygenation (ECMO) on the types of surgical procedures. Comparison of groups was done by Fisher's Exact test or nonparametric Wilcoxon rank-sum test where appropriate. A P value of less than .05 was considered significant.

Results

Data were analyzed for 227 of 294 patients during this period. Extracorporeal membrane oxygenation support was used in 45% of patients. Subsequent surgery was required in 117 patients. Seventy patients in the ECMO group (69%) required a subsequent operation. The most common operative procedures included inguinal hernia/orchiopexy, antireflux, and recurrent diaphragmatic hernias.

Conclusion

In this series, ECMO survivors are at a high risk for requiring subsequent surgeries compared to the total CDH group. This information can be used as an education tool for referring physicians and parents as they care for this group of children.