Prenatally diagnosed, right-sided congenital diaphragmatic hernia complicated by hepatic pulmonary fusion and intrathoracic kidney

Abstract

Right-sided, congenital diaphragmatic hernia is not common but accounts for about 20% of all congenital diaphragmatic hernia. It sometimes has serious complications, such as intrathoracic kidney and hepatic pulmonary fusion.Here we report a rare case of right-sided diaphragmatic hernia, complicated by intrathoracic kidney and hepatic pulmonary fusion. Repair of the diaphragm via the abdominal approach was difficult and the disease recurred. The second operation was through a right thoracotomy and was successful. The biopsied specimen of the diaphragm contained hepatocytes and bile duct cells which strongly suggested the close relationship between liver and the diaphragm in this case.Right-sided, congenital diaphragmatic hernia (R-CDH) is rare compared to left-sided CDH and accounts for about 20% of the disease. R-CDH is sometimes difficult to diagnose prenatally and Duess et al. reported that prenatal diagnosis and patch closure may be risk factors for increased mortality.Furthermore, R-CDH may be complicated by some specific pathophysiological states, such as intrathoracic kidney and hepatic pulmonary fusion, which is rare but causes complicated and difficult problems for the surgical repair of the diaphragm.In this report, we describe a rare case with the combination of intrathoracic kidney and hepatic pulmonary fusion in R-CDH and discuss the etiology of the disease and surgical treatment.Correspondence to: Yutaka Kanamori, Division of Surgery, Department of Surgical Specialties, National Center for Child Health and Development, Tokyo, Jap2-10-1 Okura, Setagaya, Tokyo 157-8535, Japan, Tel; +81-3-3416-0181, Fax; +81-3-3416-2222; E-mail: kanamori-y@ncchd.go.jp. Case reportThe patient's mother was referred to our institute at 33 weeks of gestation because her baby was suspected to have a right congenital diaphragmatic hernia. Fetal ultrasonography and fetal MRI ( Figure 1a, b) were taken; R-CDH was confirmed with right intrathoracic kidney and careful follow-up was initiated. At 38 weeks and 4 days of gestational age the female patient was born at 2,780 g birth weight and intensive care was started by the neonatologists in the neonatal care unit. A plain chest X-ray showed that the right thoracic space was occupied by the right lobe of the liver and the right lung was severely compressed (Figure 1c). Ultrasound examination revealed that the right lobe of the liver was prolapsed into the right thoracic space, the hepatic veins were draining directly into the right atrium and the inferior vena cave was defective at the retro-hepatic part. The first operation was performed at 1 day old, using an abdominal approach with a right upper transverse incision. The ventral rim of the diaphragm was formed by muscle but the postero-lateral rim was composed of a very thin membrane. This part of the membrane was biopsied, the right lobe of the liver was maneuvered into the abdominal cavity and the membranous diaphragm was taken in by non-absorbable sutures. The severe pulmonary hypertension gradually subsided but art...

Hepatopulmonary fusion associated with right-sided congenital diaphragmatic hernia: management of this rare anomaly and a review of the literature

“…Only 22 cases of HPF were identified (Table 1). [4][5][6][7][8][9][10][11][12][13][14][15][16][17][18] The embryological basis for development of HPF is not yet understood. One theory for this development is that failure in formation of the diaphragm during early gestation allows hepatic tissue to herniate into the chest during the 10th week of gestation.…”

Right-Sided Congenital Diaphragmatic Hernia Caused by Hepatopulmonary Fusion

“…To our knowledge, there are 37 reported cases in 21 published reports of HPF in the English language; this excludes two cases published in 1977 and called “pseudosequestration” [ 4 ]. Of those, 9 cases are similar to the one reported here [ 5 – 13 ], 10 cases are not similar in patient presentation, comorbidities, and/or management [ 14 – 21 ], 17 cases do not include enough information for comparison [ 3 , 22 , 23 ], and 1 case is a secondary HPF after initial repair of right-sided CDH [ 24 ]. Of these 37 cases, there were 19 deaths and 18 survivors.…”

Prenatal Recognition of Hepatopulmonary Fusion in Right-Sided Congenital Diaphragmatic Hernia for Successful Operative Planning

“…Similarly, a case of a stepwise approach including an initial surgery to repair the CDH, and a subsequent surgery after respiratory decline to partially separate the HPF has been described [16]. Varying surgical approaches have also been described in cases of attempted complete separation including electrocautery, partial hepatectomy, partial pulmonary lobectomy, and sharp dissection [2,6,[17][18][19][20][21][22][23]. Previous case reports demonstrate poor postoperative outcomes with complete separation of liver and lung and partial separa- tion has been suggested.…”