介绍一种少见膈疝

Todaet al. Surgical Case Reports (2019) 5:81

Right-sided Bochdalek hernia containing retroperitoneal fat in the elderly: report of acase

老年病人内容物为腹膜后脂肪的右侧Bochdalek疝一例报告

MichihitoToda* , Aya Yamamoto and Takashi Iwata

翻译：清远市第二人民医院普外科刘为民

审校：广州医科大学附属肿瘤医院胸外科黄渊锋博士

本文翻译过程中得到中山大学附属第一医院放射科余深平教授指导，在此深表谢意！

Abstract

摘要

Background: Most cases of Bochdalek hernias are diagnosed during the neonatal period and arise on the left side. We report a rare case of a right-sided Bochdalek hernia in an elderly patient.

背景：多数Bochdalek疝多见于新生儿期，左侧发生居多。现报道一例发生在右侧老年患者的少见病例。

Case presentation: A 72-year-old man presented with chest tightness and nausea. He had no history of thoracic and abdominal trauma. Preoperative CT scan showed a well-circumscribed mass in the right thoracic cavity of 28-cm diameter compressing the right lower lobe.The mass was mostly fat component and seemed to connect with retroperitoneal fat. We made some diagnoses: lipoma, liposarcoma, and diaphragmatic hernia.Surgical resection was performed by thoracotomy so as to resect the mass and repair the defect of the diaphragm. The mass seemed to be retroperitoneal fat which escaped from the hernia orifice.The neck of the mass was separated by a vessel-sealing device immediately above the hernia orifice. The defect of the diaphragm was repaired by direct suturing after completion of resection.Microscopic pathologic examination showed that the mass was maturated fat tissue. Four months postoperatively, there was no evidence of recurrence of the hernia.

病例介绍：。一72岁老年男性因 胸部紧束感和恶心就诊。无胸腹部创伤史。手术前CT显示右侧胸腔内有一直径为28cm界限清楚肿块，肿块压迫右肺下叶。肿块大部分为脂肪组织，似乎与腹膜后脂肪相连续。我们做了如下诊断：脂肪瘤、脂肪肉瘤和膈疝。采用开胸手术切除肿物，修复膈肌缺损。术中发现肿块似乎是来自疝环的腹膜后脂肪。使用血管闭合系统在疝环上方分离肿块颈部。完成切除后直接缝合法修补膈肌缺损。组织病理学检查显示肿块为成熟的脂肪组织。手术后数月4月随访未发现复发证据。

Conclusions: The diagnosis of an adult Bochdalek hernia is often difficult, so it is important to consider the examination carefully and to determine the better surgical procedure.

结论：成人Bochdalek疝的诊断一般比较困难。所以重要的是要仔细考虑检查方法、确定更好的手术方法。

Background

Bochdalek hernias which are most common types of diaphragmatic hernia comprise 90% of congenital diaphragmatic hernias [1]. Because most cases of Bochdalek hernias are diagnosed during the neonatal period, diagnosis in adults is rare [2]. An adult Bochdalek hernia (ABH) is usually caused by a state of increased intra-abdominal pressure, such as pregnancy and operations under the pneumoperitoneum [3].Around 80–90% of Bochdalek hernias arise on the left side [4]. There are currently fewer than 100 cases of Bochdalek hernias reported in adults in the literature,and only about 20 cases involving right-sided hernias [5, 6]. We here present arare case of a right-sided Bochdalek hernia in an elderly patient that was resected via a posterior lateral incision and discuss the clinical presentation and management of ABH.

背景

Bochdalek疝是最常见的膈疝，构成先天性膈疝的90% ^[1]。由于大多数膈疝在新生儿期得到确诊，所以在成人中诊断为此种疾病的情况比较少见^[2]。成人的Bochdalek疝通常由腹腔压力升高诱发，比如妊娠和气腹下手术^[3]。约80–90%的Bochdalek疝发生在左侧^[4]。当前文献中记载的成人Bochdalek疝不足100例，仅仅约20例发生在右侧^[5,6]。我们现报道一例通过后侧方切口予以的治疗发生在老年人右侧少见的Bochdalek疝，并讨论这类疾病的临床表现和治疗方法。

Case presentation

A 72-year-old slightly obese man with a body mass index of 28.4 presented to our hospital forchest tightness and nausea for 2 weeks. There was no history of thoracic and abdominal trauma. A chestX-ray film revealed a double line on the right diaphragm (Fig. 1). The mass comprised mostly fatty tissue, and any other organs such as intestinal tract were not included in the mass (Fig. 2b). The results of blood chemistry studies,including tumor markers, were within normal ranges.Thus, the following differential diagnosis were considered: lipoma,liposarcoma,and diaphragmatic hernia. Surgery was performed for diagnosis and treatment via a small lateral thoracotomy via the seventh intercostal space with thoracoscopic assistance.

 病例介绍

一72岁老年男性因胸部紧张感和恶心两周来我院就诊，患者轻度肥胖，BMI为28.4。无胸腹部外伤病史。胸部X-ray检查发现右侧膈肌双线影(Fig.1)。肿块大部分由脂肪组织构成，其中未发现其他器官比如肠管(Fig. 2b)。血液化学检查显示肿瘤标志物在正常范围之内。因此，考虑于下列疾病相鉴别：脂肪瘤、脂肪肉瘤和膈疝。为了确定诊断和治疗，我们采取胸腔经辅助经第7肋间隙小切口进入胸腔探查手术。

A retroperitoneal fat pad of 28cm in size was slid into the thoracic cavity from the right lumbocostal triangle,as the hernia orifice (Fig. 3a,b). The size of orifice was about 8 ×5 cm.We transected the neck of the fat pad above the orifice, because the hernia content in the thoracic cavity was larger than the orifice and difficult to reduce.Several feeding arteries contained in the stem were dissected by avessel-sealing device. The orifice was closed by suturing the surrounding diaphragmatic muscle and the chest wall. The collapsed lung could be re-expanded by positive pressure ventilation without developing acute lung edema. The operation time was 112 min and the total blood loss 220 g. The fat pad measured 28 ×9.7× 9.5cm(Fig.4a). Histological examination revealed maturated fat tissue (Fig. 4b). The chest drain was removed on the first postoperative day. Postoperative chest X-ray films demonstrated progressive re-expansionof the right lower lobes, which had been collapsed preoperatively. The patientwas discharged on the third postoperative day.Notonly his dyspnea on effort but also his stiff neck improved considerably.When last seen at his 4-month follow-up visit, he reported a good quality of life and there was no evidence of recurrence.

一28cm腹膜后脂肪垫在疝缺损处从右侧腰肋三角滑入胸腔(Fig. 3a, b)。疝孔大小约为8 ×5 cm。由于胸腔内的疝内容物体积大于疝孔直径以致还纳困难，所以我们在疝孔上方切断脂肪垫的颈部。通过血管闭合系统切断颈部的数支营养血管。通过缝合疝孔周围膈肌组织和胸壁关闭缺损。通过正压通气复张萎陷的肺组织，未形成急性肺水肿。手术时间112分钟，出血量220克。脂肪垫大小为28×9.7× 9.5cm (4a)。组织学检查提示为成熟的脂肪组织(Fig. 4b)。术后第一天拔出胸腔引流管。手术后胸部X-ray发现手术前萎陷塌陷右下肺进行性复张，术后第三天出院。呼吸困难和颈部僵硬感均明显改善。术后四个月时随访患者自述生活质量良好，无复发征象。

Discussion

Diagnosis of ABH is not easy. A misdiagnosis rate has been reported as 38% by Thomas and Kapur[7]. ABH onset is considered to be due to trauma or carbon dioxide during laparoscopic surgery which raises abdominal pressure, usually without a history in the neonatal period [8]. Further,ABH is said to have a deep relationship with body mass index [9]. In this case,the patient did not have a history of trauma or abdominal surgery. Obesity was considered to be one of the causes of hernia. Unlike infants who lapse into severe dyspnea soon after birth, the most frequent symptoms in ABH patients are mild discomfort such as chest tightness, abdominal discomfort, and dyspnea on effort [10]. Twenty-five percent of ABH patients do not complain about any symptoms [10]. Sagittal and coronal scanof enhanced CT with contrast media is useful for diagnosis. This provides detailed information about herniated viscera and diaphragm defects. In addition,the chest CT reveals the filled intestinal segments or the presence of soft tissues on the diaphragm and helps in making a definitive diagnosis of ABH.MRI was also reported as useful for depicting hernia and diaphragm defects[11].

讨论

Bochdalek疝诊断不易。Thomas 和 Kapur报道误诊率为38%^[7]。Bochdalek疝的发生通常被认为是由于创伤或者腹腔镜手术时升高腹压所致。通常没有新生儿期病史^[8]。在本例中病人不具备创伤史和腹部手术史。肥胖被认为是疝的原因之一。不像婴儿期出生后迅速出现呼吸困难，成人最常见的症状为轻度不适，比如胸部紧张感、腹部不适和用力时呼吸困难^[10]。25%的Bochdalek疝病人没有任何症状^[10]。增强CT矢状位和冠状位对于诊断非常有用。他可以提供关于疝内容物和缺损的详细资料。另外，胸部CT可以揭示充填的肠段和横膈上的软组织，帮助确定Bochdalek疝的诊断。据报道MRI对于描述疝和膈肌缺损也是非常有用的方法^[11]。

For surgical treatment of Bochdalek hernias, both transabdominal and transthoracic approaches have been reported[2, 4, 12, 13]. If the patient had signs of intestinal obstruction or strangulation, an abdominal approach might be preferable to reintroduce the intestinal tract, resect ischemic organs,and reconstruct [2]. Meanwhile,if the protruded organs are suspected fatty tumors, a transthoracic approach might be an easier procedure for separating adhesions, resecting tumor, and repairing the diaphragm, especially if it is right-sided. Minimal invasive approaches by complete thoracoscopic surgery were also reported [14]. Proper surgical procedures should be selected due to the result of preoperative image examination.In many cases, the hernia sac is returned to the abdomen to avoid pleural injury by incising the hernia sac [15].

就Bochdalek疝的外科治疗选择，经腹腔和经胸腔均有报道^{[2, 4, 12,13]}。如果病人显示有肠道梗阻和绞窄的症状，应该首先选择经腹腔方法以还纳肠管、切除缺血器官，重建正常结构^[2]。同时，如果怀疑突出器官为脂肪瘤，经胸腔的方法容易分离黏连、切除肿瘤、修补膈肌，特别是在右侧。也有报道通过胸腔镜微创的方法完成手术^[14]。根据术前结果选择适当的手术方式。很多情况下，将疝囊还纳回腹腔，一般通过切开疝囊避免胸部损伤^[15]。

Although the riskof seroma was reported in the remnant sac, it had been reported that the remnant sac disappeared after surgery [16]. Moreover, several reports said that surgeons tried to reduce theremnant sacs [15,16]. In ourcase, there was no hernia sac because it was just a sliding hernia of the retroperitoneal fat pad through the Bochdalek foramen into the thoracic cavity.Many surgeons prefer to construct a repair that is reinforced with a prosthetic graft because of the continuing stress on the diaphragm that results from respiratory movements [17]. However, if the diaphragm defect is not so large, it may be better to construct the diaphragm by direct suturing to avoid infection and postoperative adhesions.

尽管有报道称残余疝囊有可能形成血清肿，但是残余疝囊在手术后会逐渐消失^[16]。而且，也有数篇报道声称一些外科医生试图减少残余疝囊^[15,16]。本例中，由于仅仅是腹膜后脂肪垫通过Bochdalek裂孔形成滑动性疝进入胸腔，因此没有疝囊存在。因为存在持续的呼吸运动对膈肌的压力，很多外科医生愿意应用人工合成材料重建修补^[17]。然而，如果膈肌缺损较小，为了避免术后感染和粘连形成，直接缝合修补或许更为合适。

Conclusions

ABH is a relatively rare disease, and the diagnosis is often difficult because of its poor symptoms. Careful examination is essential to determine the best surgical procedure. A transthoracic approach is useful if the ABH is right-sided.

讨论

Bochdalek疝是一个相对少见疾病，由于缺乏症状常常使诊断发生困难。对于确定行外科手术治疗来说，仔细检查病人是必要的。对右侧病变经胸腔是一条实用的手术途径。

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