Right Congenital Diaphragmatic Hernia Associated With Hepatic Pulmonary Fusion: A Case Report

Laamiri, R.; Belhassen, S.; Ksia, Amine; Salem, A. Ben; Kechiche, Nahla; Mosbahi, Sana; Sahnoun, Lassâad; Mongi, Mekki; Belghith, Mohsen; Nouri, Abdellatif

doi:10.21699/jns.v5i3.370

Cited by 13 publications

(10 citation statements)

References 7 publications

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“…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.…”

Section: Discussionmentioning

confidence: 99%

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

Almaramhy

2018

J Int Med Res

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Hepatopulmonary fusion is a rare malformation that is often discovered during operative repair of right-sided congenital diaphragmatic defects. Based on a search using medical search engines, we only found 22 cases of hepatopulmonary fusion in the English literature worldwide to date. We describe herein a case of hepatopulmonary fusion with right-sided congenital diaphragmatic hernia in a female neonate who presented with respiratory distress. We discuss management of this case and review the relevant literature.

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Section: Discussionmentioning

confidence: 99%

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

Almaramhy

2018

J Int Med Res

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“…A redoubtable complication, which appears in right-sided hernias, is hepatic pulmonary fusion, an extremely rare condition (only 14 cases in litera-ture, most of them in the neonatal period/ infancy). Hepatic and pulmonary tissue fuse by a fibrous band, broncho-biliary fistula or anomalous drainage from the right lung to the intrahepatic inferior vena cava are findings related to HPF (9).…”

Section: Discussionmentioning

confidence: 99%

Right congenital diaphragmatic hernia – a diagnosis to consider

Darie¹,

Vlad²

2019

Ro J Pediatr

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Objectives. Diaphragmatic hernia is not a common disease. The severity of symptoms may vary depending on the size of defect and the organs involved. A defect on the right side is quite rare. This congenital condition may not be clinically obvious for a long time. Material and method. We present the case of a patient hospitalised in the Pediatrics Department of "Grigore Alexandrescu" Hospital for unrelated pathology, incidentally diagnosed with right congenital diaphragmatic hernia after the age of three. Results (case presentation). 3 years 6 months old girl is admitted for persistent cough. An antenatal diagnosis of agenesis of the aortic arch had been set. She was born premature, required intensive care during the neonatal period, one diagnosis being right pulmonary hemorrhage. Postnatal ultrasound examination diagnosed venous duct anomaly. She had a good clinical course with normal growth, normal cardiac and liver functions. The clinical examination was unremarkable. A thoracic X-ray described homogeneous, well-defined opacity in the right lower pulmonary lobe. Abdominal ultrasound confirmed azygos continuation of the inferior vena cava. She underwent antibiotic therapy for pneumonia (7 days) with no change of the thoracic image. Thoracic computed tomography demonstrated Morgagni's hernia with liver thoracic dislocation. Conclusions. Diaphragmatic hernia is a challenging diagnosis to be made. Although a congenital condition, it may be asymptomatic for a long time and a high degree of suspicion is required.

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“…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

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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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Right Congenital Diaphragmatic Hernia Associated With Hepatic Pulmonary Fusion: A Case Report

Abstract: We present a case of male newborn presented with respiratory distress at 21 hours of life. The patient was operated for right congenital diaphragmatic hernia (CDH). Hepatic pulmonary fusion (HPF) was found at surgery.

Cited by 13 publications

References 7 publications

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

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

Right congenital diaphragmatic hernia – a diagnosis to consider

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

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