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.     

Decreased cerebral oxygen saturation during thoracoscopic repair of congenital diaphragmatic hernia and esophageal atresia in infants

Background/Purpose

Congenital diaphragmatic hernia (CDH) and esophageal atresia with tracheoesophageal fistula (EA/TOF) can be repaired thoracoscopically, but this may cause hypercapnia, acidosis, and reduced cerebral oxygenation. We evaluated the effect of thoracoscopy in infants on cerebral oxygen saturation (cSO₂), arterial blood gases, and carbon dioxide (CO₂) absorption.

Methods

Eight infants underwent thoracoscopy (6 CDH and 2 EA/TOF). Serial arterial blood gases were taken. Regional cSO₂ was measured using near-infrared spectroscopy. Absorption of insufflated CO₂ was calculated from exhaled ¹³CO₂/¹²CO₂ ratio measured by mass spectrometry.

Results

CO₂ absorption increased during thoracoscopy with a maximum 29% ± 6% of exhaled CO₂ originating from the pneumothorax. Paco₂ increased from 9.4 ± 1.3 kPa at the start to 12.4 ± 1.0 intraoperatively and then decreased to 7.6 ± 1.2 kPa at end of operation. Arterial pH decreased from 7.19 ± 0.04 at the start to 7.05 ± 0.04 intraoperatively and then recovered to 7.28 ± 0.06 at end of operation. Cerebral hemoglobin oxygen saturation decreased from 87% ± 4% at the start to 75% ± 5% at end of operation. This had not recovered by 12 (74% ± 4%) or 24 hours (73% ± 3%) postoperatively.

Conclusions

This preliminary study suggests that thoracoscopic repair of CDH and EA/TOF may be associated with acidosis and decreased cSO₂. The effects of these phenomena on future brain development are unknown.     

Survival of severe congenital diaphragmatic hernia has morbid consequences

Background/Purpose

Fetal tracheal occlusion (TO) was developed in an attempt to enhance prenatal lung growth and improve survival in fetuses with severe congenital diaphragmatic hernia (CDH). We conducted a randomized, controlled clinical trial in 24 fetuses with severe left CDH (liver herniated into the thorax and low lung-to-head ratio) to compare survival after endoscopic fetal TO vs standard perinatal care (control) and prospectively followed up the 16 survivors (9 control, 7 TO) to compare neurodevelopmental, respiratory, surgical, growth, and nutritional outcomes.

Methods

At 1 and 2 years old, subjects underwent evaluation consisting of medical and neurological history and physical, developmental testing, nutritional assessment, oxygen saturation and pulmonary function testing, chest radiograph, and echocardiogram. Growth and developmental measures were corrected for prematurity. Data were analyzed by Mann-Whitney rank sum test, Fisher's Exact test, and logistic and linear regression.

Results

Infants with TO were significantly more premature at birth (control vs TO, 37.4 ± 1.0 vs 31.1 ± 1.7 weeks; P < .01). Growth failure (z score for weight <2 SDs below mean) was severe in both groups at 1 year of age (control vs TO, 56% vs 86%; P = .31). There was considerable catch-up growth by age 2 years (growth failure: control vs TO, 22% vs 33%; P = .19). There were no differences in other growth parameters. There were also no differences in neurodevelopmental outcome at 1 and 2 years. Supplemental oxygen at hospital discharge was a significant predictor of worse neurodevelopmental outcome at 1 and 2 years old (P = .05 and P = .02, respectively). Hearing loss requiring amplification has been diagnosed in 44% of the group (control vs TO, 44% vs 43%; P = 1.0).

Conclusions

In this group of infants with severe CDH, there were no differences in outcome at 2 years old despite significant prematurity in the TO group. Oxygen supplementation at hospital discharge identified the most vulnerable group with respect to neurodevelopmental outcome, but all infants had significant growth failure, and hearing impairment is a substantial problem in this population. Severe CDH carries significant risk of chronic morbidity.     

Long-term follow-up of patients with high-risk congenital diaphragmatic hernia

Background/Purpose

Recent advances including prenatal diagnosis, high-frequency oscillatory ventilation, and nitric oxide inhalation therapy have gradually improved the survival of high-risk congenital diaphragmatic hernia. However, the factors affecting the long-term outcome of these patients have not been well established.

Methods

Thirty-three children with ages 4.1 ± 2.5 years underwent clinical examination including growth measurements, echocardiography, ventilation, and perfusion scintigraphy.

Results

No late death was observed. Common complications were frequent respiratory tract infection (13 patients) and bowel obstruction (5 patients underwent surgery). Although frequent respiratory tract infection decreased with increasing age, patients with frequent respiratory tract infection had a decreased uptake of lung ventilation and perfusion scintigraphy on the affected side and had a decreased height for age and weight for height. No significant difference in lung ventilation and perfusion scintigraphy was observed between patients treated with and without extracorporeal membrane oxygenation, those requiring oxygen more than 1 month, and between those with and without prenatal diagnosis. Patients with a patch repair had decreased uptake on lung perfusion scintigraphy. Although frequent respiratory tract infection may be owing to hypoplasia of the ipsilateral lung, it may impair recovery of the hypoplastic lung.

Conclusion

These results indicate that monitoring for respiratory tract infection in addition to nutritional assessment should be required in the follow-up of patients with congenital diaphragmatic hernia at high risk.     

Management of oesophageal atresia in association with congenital diaphragmatic hernia

Oesophageal atresia (OA) with or without a tracheo-oesophageal fistula (TOF) is rarely associated with congenital diaphragmatic hernia (CDH). The combination poses significant management problems and the mortality rate is high. Two patients with CDH and associated OA are presented and their management outlined. Early recognition of the TOF is essential to prevent increasing respiratory compromise by gaseous distension of herniated bowel within the thorax. A staged surgical approach involving initial repair of the CDH allowing left lung function to improve prior to right thoracotomy and ligation of the TOF with oesophageal anastomosis may improve outcome. The presence of other life-threatening anomalies and/or chromosomal abnormalities must be evaluated prior to embarking upon definitive treatment.     

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。     

Radiographic changes in the diaphragm after repair of congenital diaphragmatic hernia

Background/Purpose

The growth and function of the repaired diaphragm have not been well elucidated, which may contribute to pulmonary function and chest wall deformity. We measured the lower lung diameter at the top of the diaphragm (LLD), diaphragmatic diameter (DD), and diaphragmatic height (DH) on the posteroanterior plain chest radiograph using a picture archive and communication system.

Methods

Thirty-six children aged 10.4 ± 4.8 years with congenital diaphragmatic hernia underwent clinical evaluation, including plain chest radiograph and a lung ventilation/perfusion scan. As a control, chest radiographs of 89 children aged 9.0 ± 5.5 years with minor surgery were analyzed.

Results

The LLD, DD, and DH in controls were significantly correlated with age; each value was then expressed as a percentage of age-based estimated values. Ipsilateral LLD and DD were significantly decreased. The perfusion of the ipsilateral lung was best correlated with ipsilateral DD. Five patients had chest wall deformity, and 7 had scoliosis (Cobb angle >10°). Patients with scoliosis had decreased ipsilateral LLD, DD, and DH. The Cobb angle was correlated with LLD and DD.

Conclusion

The growth of the repaired diaphragm may be impaired, which contributes to decreased perfusion of the ipsilateral lung and scoliosis. The LLD and DD are simple but useful parameters in the follow-up of patients with CDH.     

Cardiopulmonary anatomy and function in long-term survivors of mild to moderate congenital diaphragmatic hernia

Background/purpose

In the last decades, several studies regarding cardiopulmonary sequelae in survivors of congenital diaphragmatic hernia (CDH) have been published, but results often are conflicting, and controversies still exist. The aim of this study was to assess cardiopulmonary anatomic and functional outcome in a group of long-term survivors of CDH of mild to moderate degree.

Methods

Twenty-four children aged 8.15 ± 2.80 years underwent clinical examination with growth assessment, chest radiographs, echocardiography, pulmonary perfusion scintigraphy, static lung volumes measurement, and spirometry.

Results

Mean Z scores of weight for age and height for age were within normal values. Echocardiography showed normal anatomy and function in all but 3 patients with isolated CDH, in whom minor alterations were detected. Mean perfusion to the affected side was significantly lower (45.16 ± 5.30%; P < .0001) but still within normal range. Four children showed a substantial impairment of perfusion to the hernia side. The mean spirometric values and pulmonary volumes were normal. However, a mild restrictive pattern was evident in 6 children (27.3%), an obstructive pattern in 3 (13.6%), and a mixed obstructive and restrictive impairment in 1.

Conclusions

Hypoplastic lungs of mild to moderate CDH survivors continue to cause pulmonary morbidity in some children many years after the correction of the defect. In particular, lung perfusion appears to be impaired in 20% of the patients and pulmonary function in 45%, without any significant cardiac or developmental sequelae. The negative correlation between FEV₁ and duration of ventilation at presentation (r = −0.49; P = .026) may be caused by the consequences of lung hypoplasia, but initial ventilatory management may contribute to increased pulmonary morbidity. Relationship between perfusion and FEF_25-75 (r = 0.61; p = 0.004) could reflect an equivalent degree of reduction in the caliber of distal airways and pulmonary vascular tree.     

先天性膈疝的诊治进展

先天性膈疝是新生儿外科危重症之一,目前病因及发病机制尚未明确。多数患儿可以产前发现,并能依据影像学检查结果对患儿的预后进行评估。外科手术是从根本上治疗先天性膈疝的主要手段。目前外科手术方式包括开放手术和腔镜手术两种。随着微创外科的日益发展,更多中心开始对新生JLN疝行腔镜下膈疝修补术治疗。     

The decreasing incidence of pneumothorax and improving survival of infants with congenital diaphragmatic hernia

In the 6-year period between 1977 and 1982 inclusive, 75 newborn infants with congenital diaphragmatic hernia of Bochdelek underwent corrective surgery during the first 24 hours of life. A total of 40 infants (53%) survived. Beginning in January 1980, a standardized approach to care including early use of mechanical ventilation and paralysis with pancuronium as well as dopamine use prior to any Priscoline infusion, was instituted. To determine whether these approaches improved outcome, term infants without malformations from the years 1977 to 1979 were compared with a similar group treated after institution of standardized care between 1980 and 1982 inclusive. The infants were comparable in all respects, but survival improved from 45% to 82% between the two periods (P less than 0.03). There was an associated decrease in the incidence of pneumothorax (45% in first period; 14% in second period) paralleled by a concomitant increase in pancuronium use (18% and 85%, respectively). Although factors responsible for the improved survival are multifactorial these data indicate the detrimental effect of pneumothorax on outcome and the beneficial effect of a standardized approach to care using conventional intensive care techniques.     

In utero incarceration of congenital diaphragmatic hernia

In utero diagnosis of incarcerated congenital diaphragmatic hernia has never been reported. In our case, congenital diaphragmatic hernia presented at 34 weeks of gestation with dilated bowel loops, pleural effusion, and ascites on fetal ultrasound. Preterm delivery and emergency exploration revealed a tight posterolateral diaphragmatic defect with extensive bowel infarction.     

Associated malformations in delayed presentation of congenital diaphragmatic hernia

Background/Purpose

Delayed presentation of congenital diaphragmatic hernia (CDH) has been considered rare, and clinical manifestations differ from the more common newborn entity. Associated malformations in late-appearing CDH have been reported in a few patients. The authors reviewed their clinical experience to catalogue the frequency and clinical importance of associated malformations in patients with late-presenting CDH.

Methods

The records of patients greater than 1 month of age with Bochdalek type CDH treated in the authors’ clinic, from 1991 to 2001, were retrospectively reviewed. Twenty patients (age range, 1 month to 10 years) were included in the study.

Results

Associated malformations were documented in 16 of the patients (80%) and included malrotation in 12 patients, umbilical hernia in 2, pulmonary hypoplasia in 4, pulmonary sequestration in 1, gastroesophageal duplication cyst coexisting with polysplenia in 1, atrial septal defect and ventricular septal defect in 1, hydronephrosis in 1, wandering spleen in 1, talipes equinovarus in 1, and type I diabetes mellitus in 1.

Conclusions

The results of this study show a significant incidence and a wide spectrum of associated malformations. These findings suggest that patients with late CDH should be evaluated carefully for additional anomalies that may help to establish correct diagnosis and treatment.     

Octreotide for chylous effusions in congenital diaphragmatic hernia

Background/Purpose

Chylothorax is a frequent complication in congenital diaphragmatic hernia (CDH) infants and is associated with significant morbidity. The optimal treatment strategy remains unclear. We hypothesize that octreotide decreases chylous effusions in infants with CDH.

Methods

This is a retrospective study of all infants with CDH admitted to our institution from October 2006 to October 2011.

Results

Eleven (12%) infants developed a chylothorax. Five infants were managed conservatively with thoracostomy and total parenteral nutrition. Six infants were started on octreotide therapy. None of the infants required surgical intervention to stop the effusion. There was no significant difference in survival to discharge, length of stay, or average daily chest tube output between groups. There appeared to be a temporally associated drop in chest tube output upon initiation of octreotide in two infants; however, the overall rate of decline in chest tube drainage was unchanged. In addition, there were infants in the conservative group who demonstrated a similar drop in daily chest tube output despite the absence of octreotide.

Conclusions

Our data suggest that the majority of chylous effusions in CDH infants resolve with conservative therapy alone.     

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.     

先天性膈疝的围手术期处理

目的探讨新生儿先天性膈疝(NCDH)的围手术期处理。方法对我院自1996—2003年救治20例NCDH进行回顾性分析。患儿是由本院新生儿重症监护(NICU)中心转运,转运前予气管插管保持呼吸通畅。然后进行急诊手术,术后持续用呼吸机支持。结果20例NCDH中有2例死亡,1例术后放弃治疗,17例存活出院。经6个月以上随访,临床和X线胸部检查均提示肺发育良好,心脏、纵隔正常。结论早期诊断和院前处理,适时行膈疝修补术及术后呼吸机支持,是提高NCDH治愈率的有效措施。     

Mortality prediction in congenital diaphragmatic hernia

Background

A validated risk stratification tool for congenital diaphragmatic hernia (CDH) is required for accurate outcomes analyses. Existing mortality-predictive models include those of the CDH Study Group (CDHSG) based on birth weight and 5-minute Apgar score, the Canadian Neonatal Network (CNN) based on gestational age and admission score in Score for Neonatal Acute Physiology version II, and the Wilford Hall/Santa Rosa clinical prediction formula (WHSR_PF) derived from blood gas measurements. The purpose of this study was to evaluate the calibration and discrimination of these predictive models using the Canadian Pediatric Surgical Network dataset.

Methods

Neonatal risk variables and birth hospital survivorship were collected prospectively in 11 perinatal centers, between May 2005 and October 2006. Actual vs predicted outcomes were analyzed for each equation to measure the calibration and discrimination of each model.

Results

Twenty (21.2%) of 94 infants with CDH died during birth hospitalization. The CDHSG model demonstrated superior discrimination (area under the receiver operator characteristic curve = 0.85; CNN = 0.79; WHSR_PF = 0.63). Model calibration reflected by the Hosmer-Lemeshow P value was poorest with the WHSR_PF = 0.37 and comparable between CDHSG and CNN (0.48 and 0.46, respectively).

Conclusion

Predictive outcome models are essential for risk-adjusted outcome analysis of CDH. The ideal predictive equation should prove robust across CDH datasets.