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

Almaramhy, Hamdi H.

doi:10.1177/0300060518759892

Cited by 13 publications

(14 citation statements)

References 25 publications

(43 reference statements)

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“…Patients often die in the perioperative period due to respiratory failure, pulmonary hypertension, right heart failure, congenital heart diseases, and thrombosis of the inferior vena cava. 30 In this review, the survival rate among the cases reported in literature was 58.8 and 50% in the report of the CDG Study Group. The overall survival was 56.8%.…”

Section: Prognosismentioning

confidence: 70%

“…The demonstration of hepatic veins draining directly into the right atrium also suggests HPF. 30 Whenever there is a right CDH with mediastinum deviation to the right, an advanced imaging study should be conducted to exclude HPF. A routine ultrasound with color Doppler should be performed in all cases of right CDH.…”

Section: Clinical Presentationmentioning

confidence: 99%

“…3 However, there may be contralateral deviation of the mediastinum when the diaphragmatic defect is large and the bowel and other organs are herniated. 3,9,30 Ultrasonography is useful in the diagnosis of HDC but it is not a reliable image examination to diagnose FHP. An ultrasound with color Doppler may show portal branches above the diaphragm and can help in the diagnosis of HPF.…”

Section: Clinical Presentationmentioning

confidence: 99%

“…An ultrasound with color Doppler may show portal branches above the diaphragm and can help in the diagnosis of HPF. 30 Advanced imaging modalities, such as computed tomography (►Fig. 2) and magnetic resonance imaging (MRI), are most likely to demonstrate HPF, particularly when intravenous contrast is used.…”

Section: Clinical Presentationmentioning

confidence: 99%

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Congenital Hepatopulmonary Fusion

Rocha

2022

Eur J Pediatr Surg

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Congenital hepatopulmonary fusion (HPF) is a rare anomaly characterized by a fusion between the liver and lung parenchyma. HPF cases have been scarcely reported in the literature. An extensive search of publications was performed in the PubMed and Google Scholar databases without a time limit. In total, 34 clinical case reports were found in the literature, and a study by the Congenital Diaphragmatic Hernia (CDH) Study Group reported data on 10 patients with HPF. Of these 44 infants, 20 were male, 20 were female, and four were reported without gender specification. Nineteen (43.2%) patients required intubation on the first day of life. Six (13.6%) patients had their clinical presentation during the first year of life, and four (9%) clinically presented with HPF between 2.5 and 11 years of age. In these patients, cough, asthma-like symptoms, dyspnea, hemoptysis, right-side chest pain, respiratory infections, and pneumonia were the relevant clinical signs. Right-lung vascular anomalies were present in 18 (40.9%) patients. A complete liver and lung separation was successful in 17 (38.6%) patients. The overall survival was 56.8%. Congenital HPF has no gender predominance. In most cases, it behaves similar to a right CDH in need of resuscitation and intubation after birth. The majority of the cases are discovered during the surgical procedure for CDH. The best surgical approach has not been established and depends on the degree of fusion and vascular anomalies. An advanced imaging assessment is necessary before a surgical approach is attempted. The prognosis is ominous.

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Section: Prognosismentioning

confidence: 70%

Section: Clinical Presentationmentioning

confidence: 99%

Section: Clinical Presentationmentioning

confidence: 99%

Section: Clinical Presentationmentioning

confidence: 99%

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Congenital Hepatopulmonary Fusion

Rocha

2022

Eur J Pediatr Surg

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“…8 In a review of literature, the combination of right CDH, HPF, and anomalous venous drainage of the right lung was reported in one case, 9 and the association of HPF and sequestration was reported in 3 cases. 10 However, the association of bilateral CDH, HPF, partial anomalous right pulmonary venous drainage and sequestrated lobe was not reported before.…”

Section: Follow-up and The Outcomesmentioning

confidence: 93%

Surgical repair of bilateral congenital diaphragmatic hernia associated with hepatopulmonary fusion

Bawazir

2019

SMJ

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Bilateral congenital diaphragmatic hernia)CDH(is a rare congenital anomaly with high neonatal mortality. The condition is rarely associated with hepatopulmonary fusion)HPF(. We reported the surgical repair of bilateral CDH in a male infant aged 6 weeks. The patient had herniation of the liver on the right side with HPF in association with anomalous drainage of the right pulmonary vein into the hepatic vein and ventricular septal defect. We approached both sides thoracoscopically; however, the right side required a subcostal incision. We separated the liver from the right lower lung lobe laterally using electrocautery, and the defect was closed with a mesh. The medial portion was left intact to avoid injury of the anomalous pulmonary venous drainage. After 2 weeks, the patients had a surgical repair of the cardiac defects, and the postoperative course was complicated by bleeding and prolonged ventilation. No hernia recurrence was observed after 9 months.

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Chest ultrasound findings in hepatopulmonary fusion (HPF)

Morello,

Buonsenso

2023

J Ultrasound

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

Cited by 13 publications

References 25 publications

Congenital Hepatopulmonary Fusion

Congenital Hepatopulmonary Fusion

Surgical repair of bilateral congenital diaphragmatic hernia associated with hepatopulmonary fusion

Chest ultrasound findings in hepatopulmonary fusion (HPF)

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