Muddle in the middle: A rare case of a hepatopulmonary fusion and lung sequestration in a neonate with a right‐sided congenital diaphragmatic hernia

Oudtshoorn, Sarah van; Gikenye, Njeri; Kikiros, Colin; Gera, Parshotam

doi:10.1111/jpc.15342

Cited by 3 publications

(2 citation statements)

References 7 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…This case demonstrates the value of prenatal recognition of HPF in right-sided CDH and the importance of imaging in developing a surgical plan. In previously reported cases of HPF, diagnosis was rarely made preop- eratively and more often recognized intraoperatively or at postmortem [2,5,9,10]. In this case, fetal MRI and prenatal US imaging played an important role in recognizing HPF, affording an opportunity to change the management.…”

Section: Discussionmentioning

confidence: 88%

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

Terp

Roberts²,

Alonso

et al. 2022

Fetal Diagn Ther

View full text Add to dashboard Cite

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.

show abstract

Section: Discussionmentioning

confidence: 88%

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

Terp

Roberts²,

Alonso

et al. 2022

Fetal Diagn Ther

View full text Add to dashboard Cite

show abstract

“…6,7 The preoperative diagnosis of HPF is difficult, and some cases have been diagnosed on magnetic resonance image or tomography scan. 8 A rightward mediastinal shift may provide information about the patient's condition, as a leftward shift is expected in right-sided CDH. 7 The diagnosis of HPF is almost always made intraoperatively.…”

Section: Introductionmentioning

confidence: 99%

Congenital Hepatopulmonary Fusion

Rocha

2022

Eur J Pediatr Surg

View full text Add to dashboard Cite

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.

show abstract