Balela Naisya, Fauzi Aditya Rifqi, Dwihantoro Andi
Pediatric Surgery Division, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada/Dr. Sardjito Hospital, Yogyakarta, Indonesia.
Ann Med Surg (Lond). 2020 Jul 27;57:179-182. doi: 10.1016/j.amsu.2020.07.034. eCollection 2020 Sep.
Congenital diaphragmatic eventration is characterized by the elevation of the diaphragm, causing a protrusion of the intraabdominal viscera into the affected hemithorax and resulting in respiratory distress. Diaphragmatic eventration with an intrathoracic ectopic kidney is a very rare disorder with the incidence of 0.25% of all ectopias.
A 16-day-old male presented with chief complaint of respiratory distress. His plain chest X-ray showed intestinal gases in the right diaphragm and elevation of the right diaphragm. Intraoperative findings revealed elevation of the right diaphragmatic dome and visceral displacement, including the ileum, transverse colon, and right lobe of the liver. Subsequently, hemidiaphragm plication was conducted. Two weeks after surgery, the patient suffered from respiratory distress again. Computed tomography (CT) scanning revealed right diaphragmatic elevation and an ectopic kidney inside the right hemithorax. During the second operation, there were no longer elevation of the right diaphragmatic dome nor any other organ displacement. Moreover, we decided to let the intrathoracic kidney remain in place. The outcome was good during the postoperative period and six months after surgery.
Eventration of diaphragm with an intrathoracic ectopic kidney should be considered as a differential diagnosis in neonate patients with respiratory distress accompanied by a thoracic mass.
Congenital diaphragmatic eventration with an intrathoracic ectopic kidney is a very rare disorder, requiring a personalized surgical repair to achieve a good outcome. CT scanning may help confirm the diagnosis, particularly to define the dome elevation and the intrathoracic organ precisely.
先天性膈膨升的特征是膈肌抬高,导致腹腔内脏器突入患侧胸腔,从而引起呼吸窘迫。合并胸腔内异位肾的膈膨升是一种非常罕见的疾病,在所有异位症中的发生率为0.25%。
一名16日龄男婴,主要症状为呼吸窘迫。其胸部X线平片显示右侧膈肌有肠气,右侧膈肌抬高。术中发现右侧膈穹窿抬高及内脏移位,包括回肠、横结肠和肝脏右叶。随后进行了半膈肌折叠术。术后两周,患者再次出现呼吸窘迫。计算机断层扫描(CT)显示右侧膈肌抬高,右胸腔内有一个异位肾。在第二次手术中,右侧膈穹窿不再抬高,也没有其他器官移位。此外,我们决定让胸腔内的肾脏留在原位。术后及术后六个月结果良好。
合并胸腔内异位肾的膈膨升应作为伴有胸腔肿块的新生儿呼吸窘迫患者的鉴别诊断之一。
合并胸腔内异位肾的先天性膈膨升是一种非常罕见的疾病,需要个性化的手术修复以取得良好的效果。CT扫描有助于确诊,特别是能精确界定膈穹窿抬高及胸腔内器官情况。