Fibrous fusion between the liver and the lung: An unusual complication of right congenital diaphragmatic hernia

Katz, Schmuel; Kidron, D.; Litmanovitz, Ita; Erez, Ilan; Dolfin, Zippora

doi:10.1016/s0022-3468(98)90214-7

Cited by 31 publications

(24 citation statements)

References 3 publications

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“…The absence of a mediastinal shift or ipsilateral mediastinal shift was reported as a characteristic feature of some cases [8,9] but this depends on the right diaphragmatic formation and right pulmonary hypoplasia and is not always seen [17]. Airway connection of the affected lung tissue to the right bronchus or trachea was reported in some papers [7,8,10] and, in our case, airway injury occurred in the second operation. In these complicated cases, lobectomy of the right lung or total right lung resection is mandatory to prevent postoperative complications.…”

Section: Discussionmentioning

confidence: 51%

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

Takezoe¹,

Nomura²,

Ogawa³

et al. 2017

Birth Defect

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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...

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Section: Discussionmentioning

confidence: 51%

“…Hepatic pulmonary fusion was first reported by Katz et al in 1998[7] and 17 cases have been reported in the English literature [7][8][9][10][11][12][13][14][15][16][17][18]. Among these, 6 patients died and the prognosis is poor [7,8,10,13,18].…”

Section: Discussionmentioning

confidence: 99%

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

Takezoe¹,

Nomura²,

Ogawa³

et al. 2017

Birth Defect

View full text Add to dashboard Cite

show abstract

“…In previous reports [7][8][9] of the adhesion between the liver and the lung, the herniated liver was not easily reduced, and in such cases, operations had to be repeatedly performed [7][8][9]. In another report, the patient died of respiratory failure [8]. In our case, the CDH repair was initially attempted through a thoracic approach, but the herniated liver cannot be reduced.…”

Section: Discussionmentioning

confidence: 76%

“…Tsugawa et al [7] first reported 2 right-sided CDH cases with the hepatopulmonary adhesion in 1987. Recently, 2 cases of right-sided CDH with fusion between the liver and the lung were also reported in 1998 [8] and 2003 [9]. Furthermore, bronchobiliary fistula has also been reported [10].…”

Section: Discussionmentioning

confidence: 99%