How to treat hepatic pulmonary fusion: case report with review of literature

Kerkeni, Yosra; Farhani, Rabeb; Sassi, Nizar; Hamzaoui, M.

doi:10.1080/00015458.2018.1496568

Cited by 5 publications

(2 citation statements)

References 13 publications

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“…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.…”

Section: Discussionmentioning

confidence: 87%

Right-Sided Congenital Diaphragmatic Hernia Caused by Hepatopulmonary Fusion

Patel

Rael

2020

Case Reports in Pediatrics

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Introduction. Hepatopulmonary fusion is a very rare finding associated with right-sided congenital diaphragmatic hernia. With less than 50 reported cases, management and outcomes of hepatopulmonary fusion are poorly understood. This report highlights that clinical presentation is not a reliable indicator of outcomes in this rare disease. Case Presentation. A term neonate admitted for tachypnea and complete opacification of the right hemithorax was diagnosed with right-sided congenital diaphragmatic hernia. Preoperative respiratory support was minimal, and the only symptom exhibited was tachypnea. During surgical repair, fusion of the lung and liver were noted, consistent with a diagnosis of hepatopulmonary fusion. Postoperatively, the patient’s pulmonary hypertension worsened and required extracorporeal membrane oxygenation. Conclusions. Many patients with hepatopulmonary fusion and only mild symptoms die postoperatively from severe pulmonary hypertension and progressive respiratory failure. Preoperative clinical status is not indicative of postoperative outcomes, and literature suggests that patients who require less support preoperatively have high mortality rates. The availability of ECMO for postoperative complications may be necessary in patients requiring repair of hepatopulmonary fusion.

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Section: Discussionmentioning

confidence: 87%

Right-Sided Congenital Diaphragmatic Hernia Caused by Hepatopulmonary Fusion

Patel

Rael

2020

Case Reports in Pediatrics

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“…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.…”

Section: Discussionmentioning

confidence: 99%

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

Terp

Roberts²,

Alonso

et al. 2022

Fetal Diagn Ther

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Introduction: Hepatopulmonary fusion (HPF) is a rare anomaly specifically associated with right-sided congenital diaphragmatic hernia (CDH). Fewer than 50 cases of HPF have been reported, most at the time of surgery or postmortem with an associated high morbidity and mortality rate. Prenatal diagnosis and optimal management of these rare cases has not been established. Case Presentation: We present a case of HPF diagnosed prenatally by fetal ultrasound and magnetic resonance imaging allowing postnatal and intraoperative planning leading to successful HPF separation and repair of the CDH. Discussion: The prenatal recognition allowed the use of preoperative computed tomography angiogram and right cardiac catheterization to plan the surgical approach. Intraoperative ultrasound confirmed abnormal vasculature associated with the HPF facilitating a successful complete HPF separation and CDH repair was performed. The patient survived and continues to do well on long term follow-up. Prenatal recognition may help reduce the high morbidity and mortality associated with HPF.

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