Management of right-sided fetal congenital diaphragmatic hernia with hepatopulmonary fusion: a report of three cases and literature review / 中华围产医学杂志

ABSTRACT

Objective:To investigate the clinical features and management of right-sided congenital diaphragmatic hernia (RCDH) with hepatopulmonary fusion (HPF).Methods:This retrospective study analyzed the clinical characteristics of three cases of RCDH complicated by HPF that were treated in Guangzhou Women and Children's Medical Center from June to December 2022. Diaphragm defects in the three cases were classified according to the international standard of diaphragm defect classification. Besides, an extensive search of publications was performed including domestic and foreign databases, including CNKI, Wanfang Database, Yiigle, VIP Chinese journals, PubMed, Embase and UpToDate databases from January 1997 to April 2023 using terms including "congenital diaphragmatic hernia" and "hepatopulmonary fusion". Clinical features and prognosis of RCDH complicated by HPF were summarized.Results:(1) Cases in the present study: RCDH was found in case 1 and case 2 during routine prenatal ultrasound examination; antenatal fetal MRI showed partial displacement of the hepatocele into the right hemithorax, right lung hypoplasia, a normal-sized left lung and without left shift of the mediastinum in both cases. Postnatal chest radiographs of case 1 and case 3 showed dense shadow in the left lung and mediastinum shifted to the right. Case 2 had a D-type defect and a slight shift of the mediastinum to the left was observed on the postnatal chest radiograph. Preoperative imaging findings indicated highly suspected HPF in the three cases. Case 1 and case 2 had complete separation of liver and lung and underwent diaphragmatic herniorrhaphy with patch. Partial lung resection was performed in case 2. Both case 1 and case 2 survived (length of hospital stay was 22 d and 23 d, respectively). Case 3 did not undergo hepatopulmonary separation or herniorrhaphy after exploratory operation and died of persistent pulmonary hypertension. (2) Literature review: Only 40 cases of CDH with HPF were retrieved from PubMed. Among the 43 cases including the above three cases, 27 (62.8%) had a right shift or no deviation of the mediastinum before surgery and nine (20.9%) had a left shift of the mediastinum, while the condition of seven patients (16.3%) were not described. There were 26 patients undergoing complete separation of liver and lung and 19 (73.1%) of them survived. Thirteen patients underwent partial separation of liver and lung and six of them survived. Four patients died without receiving separation.Conclusions:HPF should be considered in patients with RCDH, especially in cases with no left shift in the mediastinum in the imaging. Preoperative evaluation for surgery in such cases needs to be managed as if it were a major operation that may require hepatectomy or partial pneumonectomy.