Respiratory function after repair of congenital diaphragmatic hernia.

Respiratory function studies were carried out in 22 infants who had successful repair of diaphragmatic herniae of the Bochdalek type. Thoracic gas volume was initially reduced in only 3 of these, but subsequent studies showed that improvement occurred. There were no consistent abnormalities in either dynamic compliance or mean pulmonary conductance. This is evidence that there is rapid adaptation which compensates for any alteration in the parenchymatous tissue in the lungs or abnormalities in the bronchial tree in infants soon after the repair of congenital diaphragmetic herniae. Further studies are necessary to determine the changes in these lungs with growth.     

Lung function after repair of congenital diaphragmatic hernia

To study lung development after repair of congenital diaphragmatic hernia 20 patients operated on between 1960 and 1976 were followed-up at 6-22 years of age. All had had an uneventful postoperative course, had no concomitant disease and were subjectively well with an ordinary physiological performance. The investigation showed a mean increase of residual volume by one third of predicted normal values. Fractional perfusion and ventilation were reduced by 20% on the hernia side compared with the contralateral side. Functional impairment owing to pulmonary hypoplasia was ruled out by the existence of a normal working capacity and maintenance of a normal alveolar gas exchange during exercise on a high work load.     

Lung function in children after repair of congenital diaphragmatic hernia.

Sixteen children who had had congenital diaphragmatic herniae repaired either in the neonatal period (11), or in early childhood (5), were reviewed at between 7 and 19 years of age. Only minor abnormalities were shown in their chest x-rays and in a variety of lung function tests. This suggests that the long-term prognosis in this condition is good.     

Pulmonary function after repair of congenital diaphragmatic hernia — a short review

In congenital diaphragmatic hernia the patients are generally born with pulmonary hypoplasia. The degree of this hypoplasia determines the patients chance of survival. The clinical impression is that the babies who survive the initial critical phase do well later on and are free of respiratory symptoms. A few detailed followup examinations on pulmonary function have been published and are briefly reviewed in this paper. There is some disagreement regarding lung volumes, but perfusion and ventilation relative to volume is lower in the hernia side lung. This, however, seems to be without greater functional importance as the physical performance and oxygen uptake during exercise is normal.     

Gastroesophageal reflux and diaphragmatic motility after repair of congenital diaphragmatic hernia.

At the Department of Pediatric Surgery in Graz, 31 boys and 23 girls were operated on for congenital diaphragmatic hernia (CDH) from 1978 to 1994. In 49 patients the defect was on the left, in five on the right side. In 46 cases, the hernia was diagnosed within the first week of life; in eight children at a later date. 19 children (35%) died. 25 of the 35 survivors (71%) came to a follow-up examination on average 9.4 (1-17) years after the operation. 24 h pH-monitoring or manometry and Upper G.I. series revealed pathological gastroesophageal reflux (GER) in 16 patients. Nine children were treated conservatively; in seven patients an antireflux procedure was performed. A thoracic position of the stomach or left liver lobe, presence of a hernial sac, gestational age, prenatal diagnosis, use of a patch or severity of lung hypoplasia did not significantly influence the incidence of GER. In three patients, a hiatal hernia was found. The motility of the diaphragm was documented with M-mode sonography (n = 18); a restricted motility could be demonstrated in five patients. GER is very common in patients after repair of CDH. We recommend long-term follow-up with special interest in respect of GER.     

先天性膈疝术后食管功能及胃食管反流的临床观察

目的 了解先天性膈疝修补术后预后及术后食管功能和胃食管反流情况。方法 对46例先天性膈疝修补术后进行随访，13例进行了24h食管双极pH及24h动态压力测定，了解胃食管功能。结果 13例进行了24h食管pH及压力测定的膈疝修补术后患儿中，7例有胃食管反流，其中3例术中应用补片，4例术前显示胃疝入胸腔。结论 先天性膈疝修补术后可出现胃食管反流，24h测压及pH测定表现为食管蠕动功能明显减弱，这种食管下端蠕动功能减弱与膈疝修补术后出现胃食管连接解剖异常及膈肌发育不良可能相关。     

Laparoscopic repair of a congenital diaphragmatic hernia

The standard treatment of congenital diaphragmatic hernia is by open operation. We have successfully performed a laparoscopic repair in an asymptomatic 2-year-old child. We recommend this as a safe alternative treatment modality in diaphragmatic hernias presenting past infancy. The role of laparoscopic correction in the newborn period, however, remains to be determined.     

Thoracoscopic repair of congenital diaphragmatic hernia in children

Minimal invasive surgery (MIS) has been first proposed in case of delayed congenital diaphragmatic hernia (CDH). Since then, about 32 cases of thoracoscopic CDH approach in newborns have been published. Conditions of thoracoscopy are reviewed and enlightened with our preliminary series. The advantages of thoracoscopy versus a laparoscopic approach are detailed. Since 1999, all children presenting with CDH after the immediate neonatal period were offered a MIS approach. We started treating stable newborns suffering a CDH by thoracoscopic procedures in 2003. In cases of late presentations, 10 thoracoscopies and 1 laparoscopy were performed. Among them, 4 patients suffered from an incarcerated hernia. One conversion to a thoracotomy, 1 video-assisted thoracic surgery (VATS), and 2 conversions to laparoscopies were required for the reduction of hernia contents. In those last cases, the hernia defects were sutured, coming back to the thoracoscopic approach. Six newborns with neonatal diagnosis of CDH were primarily treated by thoracoscopy. Four procedures were completed without difficulty. The diaphragm was approximated with interrupted 2/0 nonabsorbable sutures. On the lateral part of the defect, in which there is a lack of diaphragm against the ribs, plegetted rib-anchoring stitches were used. A wide defect requiring a patch needed for conversion to a thoracotomy; in this case, we encountered a very rare pericardial defect and had difficulties in reducing the liver. In another case, we went to a VATS with a less than 2-cm opening to insert a Gore Tex patch, which was required. Thoracoscopy for delayed CDH repair seems to be easy and feasible with good results. A combined procedure with both thoracoscopy and laparoscopy has proven its usefulness in case of incarcerated hernia. Thoracoscopic CDH repair in newborns is not feasible in every case due to the patient's conditions. Criteria for eligibility need more cases to be evaluated. The advantages and disadvantages of thoracoscopy versus laparoscopy are reviewed. Nevertheless, the thoracoscopic approach seems easier.     

腹腔镜治疗新生儿膈疝的探讨

目的探讨腹腔镜手术治疗新生儿膈疝的可行性。方法新生儿膈疝5例（生后1h、2d、7d、12d和24d）行腹腔镜手术治疗，其中，产前B超确诊2例，产后X线确诊3例；女4例，男1例；左侧膈疝4例，右侧膈疝1例。手术采用3孔或4孔腹腔镜法，还纳疝内容物，丝线缝合膈肌修补缺损。结果5例患儿在腹腔镜下顺利完成手术，手术时间85～160min，平均115min，手术出血量〈5ml，均无术中并发症，其中1例行腹腔镜手术后2周复发，再经腹腔镜手术治愈；另外1例行腹腔镜手术后1d复发行开腹补片修补术成功。5例患儿已随访6个月～4年，术前症状完全消失，无术后并发症发生。结论腹腔镜技术治疗新生儿膈疝具有暴露清楚、损伤小、打击小、恢复快、切口美观等优点，是一种可行方法。但由于新生儿腹腔镜手术普遍存在腹腔空间狭小的问题，特别是膈疝手术，将疝内容物还纳入腹腔后空间将进一步缩小，修补缝合手术操作较困难，需要熟练的腹腔镜技术。     

Extracorporeal membrane oxygenation — succesful treatment after repair of congenital diaphragmatic hernia

A case of congenital diaphragmatic hernia successfully treated by extracorporeal membrane oxygenation (ECMO) is reported. A female baby weighing 3.4 kg was admitted 3 h after birth because of respiratory distress. Her left diaphragmatic hernia was repaired 5 h after birth by laparotomy. The AaDO₂ levels were higher than 500 mmHg before and after the operation. She was managed by high-frequency ventilation and administration of tolazoine for the first 58 h. These effects were temporary, however, and she reverted into persistent fetal circulation three times. Therefore, ECMO was started at 59 h after birth. When the flow rate of ECMO reached 320 ml/min, the newborn entered a state of complete lung rest. Her general condition improved slowly, and ECMO was successfully terminated after 60 h of bypass. This was the first successful case in Japan. Offprint requests to: M. Nagaya     

先天性膈疝术后肺功能转归的临床研究

目的探索先天性膈疝患儿术后肺功能的转归情况。方法收集2016年11月至2019年3月在上海交通大学医学院附属新华医院小儿外科门诊随访并行肺功能检查的膈疝患儿肺功能数据,同时收集患儿治疗时的临床资料,包括患儿性别、膈疝类型、Apgar评分、出生体重、肝脏位置、血气分析、呼吸机维持时间和住院周期等进行统计分析。结果45例患儿纳入分析,其中男19例,女26例;左侧膈疝39例,右侧6例。共行89次肺功能检查,随访时间为(9.3±7.3)个月,20例患儿行1次检查,25例行2次及以上检查。以最近一次肺功能检查数据为准,45例患儿中呼吸功能正常者有7例(15.6%),呼吸功能存在异常组有38例(84.4%)。肺功能检查表现为潮气呼吸功能正常、阻塞性通气功能障碍、混合性通气功能障碍、限制性通气功能障碍的分别为10次、62次、11次和6次。肺功能检查时间、膈疝类型和肝脏疝入情况与肺功能检查结果存在相关性(P<0.05)。与限制性通气功能障碍组患儿的月龄相比,阻塞性通气功能障碍组患儿的月龄较大[(8.9±6.9)个月比(1.3±0.5)个月,P=0.001]。25例接受多次肺功能检查结果显示,随着患儿年龄的增长,其"达峰时间比"和"达容时间比"显著增加(P<0.05)。结论部分先天性膈疝患儿术后早期存在不同程度的肺功能异常,需给予高度重视;年龄、膈疝类型和肝脏疝入情况可能是影响肺功能异常的危险因素。     

Intraoperative ventilation during thoracoscopic repair of neonatal congenital diaphragmatic hernia

Purpose

To evaluate the optimal ventilation mode during thoracoscopic repair (TR) of neonatal congenital diaphragmatic hernia (CDH), we compared high-frequency oscillatory ventilation (HFOV) with conventional mechanical ventilation (CMV).

Methods

Twenty-three neonatal CDH cases who underwent TR without intraoperative inhalation of nitric oxide at our institution between 2007 and 2016 were reviewed. Patients were initially ventilated with HFOV, which was converted to CMV if the HFOV settings were decreased to FiO₂ <0.4, stroke volume <4 mL/kg and mean airway pressure <12 cmH₂O. Arterial blood gases in the perioperative period were compared between HFOV and CMV.

Results

Seventeen patients were ventilated with HFOV (group I), and six patients were ventilated with CMV (group II). Preoperative PaCO₂ was significantly higher and pH was significantly lower in group I compared with group II. In both groups I and II, intraoperative PaCO₂ increased significantly and pH decreased significantly compared with preoperation. Although intraoperative PaCO₂ and pH were not different between the groups, group II showed greater worsening of intraoperative PaCO₂ and pH as compared to their respective preoperative values.

Conclusions

HFOV seems to prevent deterioration of hypercapnia and acidosis to a greater extent than CMV during TR in neonatal cases of CDH, although patients can also be ventilated with CMV.

     

Outcomes of congenital diaphragmatic hernia repair on extracorporeal life support

Purpose

Extracorporeal life support (ECLS) is applied to refractory pulmonary hypertension in congenital diaphragmatic hernia (CDH). We evaluate the single-center outcomes of infants with CDH to determine the utility of late repair on ECLS versus repair post-decannulation.

Methods

Records of infants with CDH (2004–2014) were retrospectively reviewed.

Results

CDH was diagnosed in 177 infants. Sixty six (37%) underwent ECLS, of which, 11 died prior to repair, 33 were repaired post-decannulation, and 22 were repaired on ECLS. Repair was delayed in patients on ECLS (19 versus 10 days, p < 0.001). Patients repaired on ECLS had longer ECLS runs (22 versus 12 days, p < 0.001) and higher rates of bleeding and mortality than those repaired post-decannulation. Survival was 54% in infants undergoing ECLS, 65% in those who underwent repair, 36% in those repaired during ECLS, and 85% in those who were decannulated prior to repair. Eighteen percent (N = 4) of deaths after repair on ECLS were attributable to surgical bleeding. The remainder was due to pulmonary hypertension or sepsis.

Conclusion

Infants who underwent CDH repair post-decannulation had excellent outcomes and no mortalities attributable to repair. Neonates who underwent repair on ECLS late on bypass had the lowest survival rate with only 18% of mortality in this cohort attributable to surgical bleeding.

     

Anterior fundoplication at the time of congenital diaphragmatic hernia repair

Purpose  The loss of normal anatomic barriers in neonates with congenital diaphragmatic hernia (CDH) can predispose children to gastroesophageal reflux (GER). In an attempt to improve post-operative feeding, we have added a modified anterior fundoplication to restore natural gastric and esophageal positioning. Methods  The institutional review board of both participating centers approved this study. Between 1997 and 2008, 13 neonates with high-risk anatomy underwent repair of CDH combined with an anterior fundoplication (Boix-Ochoa). The anatomic indications for concomitant fundoplication were absence of an intra-abdominal esophagus, an obtuse angle of His, and a small, vertically oriented stomach. Results  Ten patients survived to discharge and eight were on full oral nourishment. One required partial gastrostomy feedings for an improving oral aversion and quickly progressed to full oral feedings. One patient with chromosomal anomalies and swallowing dysfunction remained on long-term bolus gastrostomy feedings. Two with progressive symptoms of GER and failure to thrive required conversion to a 360° wrap after 18 months of medical management. This was performed in conjunction with a planned, staged muscle flap reconstruction in one patient. There were no complications related to the fundoplication. Conclusion  Anatomic predictors of severe GER can be efficiently countered at the time of CDH repair. A modified fundoplication should be considered in the operative management of high-risk infants. Presented at the 41st Annual Pacific Association of Pediatric Surgeons Conference, Jackson Lake Lodge Wyoming, 30 June 2008.     

Indications for thoracoscopic repair of congenital diaphragmatic hernia in neonates

Purpose  

We reviewed 26 consecutive cases of congenital diaphragmatic hernia (CDH) prospectively to establish selection criteria for successful thoracoscopic CDH repair (TR).