Development of a multi-institutional registry for children with operative congenital lung malformations

Kunisaki, Shaun M.; Saito, Jacqueline M.; Fallat, Mary E.; Peter, Shawn D. St.; Lal, Dave R.; Johnson, K. Norman; Mon, Rodrigo; Adams, Cheryl; Aladegbami, Bola; Bence, Christina M.; Burns, R. Cartland; Corkum, Kristine S.; Deans, Katherine J.; Downard, Cynthia D.; Fraser, Jason D.; Gadepalli, Samir K.; Helmrath, Michael A.; Kabre, Rashmi; Landman, Matthew P.; Leys, Charles M.; Linden, Allison F.; López, Joseph J.; Mak, Grace Z.; Minneci, Peter C.; Rademacher, Brooks L.; Shaaban, Aimen F.; Walker, Scott G.; Wright, Tiffany; Hirschl, Ronald B.

doi:10.1016/j.jpedsurg.2019.01.058

Cited by 23 publications

(24 citation statements)

References 57 publications

(76 reference statements)

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“…Since CPAMs usually have abnormal alveoli, gas exchange is poor, and larger lesions can expand, causing mass effect to adjacent normal lung tissue. In three-fourths of cases, affects one of the lower lobes (1). Bilateral and multi-lobar disease is uncommon.…”

Section: Cpammentioning

confidence: 99%

“…The type major types of BPS are intralobar (75%) and extralobar (25%) (1). Intralobar sequestrations are at increased risk for bacterial seeding with subsequent pneumonia because they are contained within a common visceral pleura with normal aerated lung.…”

Section: Bpsmentioning

confidence: 99%

“…Mass effect on the heart can lead to compromised venous return to the right atrium and ultimately in utero heart failure (30,31). Fortunately, hydrops probably occurs in fewer than 10% of cases based on a multi-institutional report (1). Fetuses at increased hydrops risk are those with an initial CVR of at least 1.4 (20,24).…”

Section: Fetal Diagnosismentioning

confidence: 99%

“…Congenital lung lesions represent a group of anomalies that include congenital pulmonary airway malformations (CPAM), bronchopulmonary sequestrations (BPS), congenital lobar emphysema (CLE) (Figure 1), and bronchogenic cysts (1). Their incidence has increased in recent years based on several population-based studies, which suggest a frequency of 1 in 2,500 live births (2,3).…”

Section: Introductionmentioning

confidence: 99%

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Narrative review of congenital lung lesions

Kunisaki¹

2021

Transl Pediatr

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This article reviews the contemporary diagnosis and management of antenatally diagnosed congenital lung lesions. These anomalies, which include congenital pulmonary airway malformation (CPAM) (formerly congenital cystic adenomatoid malformation), bronchopulmonary sequestration (BPS), bronchogenic cyst, and congenital lobar emphysema (CLE), are relatively rare but are increasingly encountered by clinicians because of the improved resolution and enhanced sensitivity of fetal ultrasound. Serial assessment of these lesions throughout pregnancy remains the norm rather than the exception.Perinatal management strategies may differ based on initial size and growth patterns of these masses until delivery. Fetal magnetic resonance imaging and other diagnostic testing can sometimes be helpful in providing additional prognostic information. Over the last decade, maternal steroids have become standard of care in the management of larger lesions at risk for nonimmune hydrops. As a result, fetal surgical procedures, including open resection, thoracoamniotic shunting, and ex utero intrapartum treatment (EXIT), are less uncommonly performed. Decisions regarding whether delivery of these fetuses should occur in a tertiary care center with pediatric surgery coverage versus delivery at a local community hospital are now highly relevant in most prenatal counseling discussions with families. Large lung malformations may require urgent surgical removal in the early postnatal period because of respiratory distress. Other complications, such as recurrent pneumonia, pneumothorax, and cancer, are indications for postnatal lung resection on an elective basis. Many children are good candidates for minimally invasive (thoracoscopic) surgical approaches as an alternative to resection by thoracotomy. In the vast majority of cases, the overall prognosis remains excellent.

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Section: Cpammentioning

confidence: 99%

Section: Bpsmentioning

confidence: 99%

Section: Fetal Diagnosismentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Narrative review of congenital lung lesions

Kunisaki¹

2021

Transl Pediatr

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“…PSs account for up to one-fourth of all lesions. 3 The pathognomonic feature of sequestrations that distinguishes them from the more common CPAM is a systemic arterial supply, usually arising from the aorta. Regardless of the type of CLM, resection is advocated by many to avoid complications of infection, pneumothorax, or malignancy.…”

Section: Introductionmentioning

confidence: 99%

Preoperative Embolization Facilitates Segmental Resection of Pulmonary Sequestration in an Infant

Prasad

Pennell

Arthur

et al. 2021

European J Pediatr Surg Rep

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The most common congenital lung malformations are congenital pulmonary airway malformations and pulmonary sequestrations. Many surgeons advocate resection to prevent complications of infection, malignancy, and pneumothorax. The standard of care is lobectomy, but segmentectomy and embolization alone have been reported. These methods avoid the complications of lobectomy but are not widely practiced due to concerns about incomplete resection or involution of the lesion. We present a novel approach to the treatment of a pulmonary sequestration in a 7-month-old male using preoperative embolization followed by a sublobar pulmonary resection. The embolization clearly demarcated the affected lung intraoperatively, thereby facilitating complete removal of the lesion with a segmental lung resection rather than complete lobectomy.

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Accuracy of Chest Computed Tomography in Distinguishing Cystic Pleuropulmonary Blastoma From Benign Congenital Lung Malformations in Children

et al. 2022

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IMPORTANCEThe ability of computed tomography (CT) to distinguish between benign congenital lung malformations and malignant cystic pleuropulmonary blastomas (PPBs) is unclear.OBJECTIVE To assess whether chest CT can detect malignant tumors among postnatally detected lung lesions in children. DESIGN, SETTING, AND PARTICIPANTSThis retrospective multicenter case-control study used a consortium database of 521 pathologically confirmed primary lung lesions from January 1, 2009, through December 31, 2015, to assess diagnostic accuracy. Preoperative CT scans of children with cystic PPB (cases) were selected and age-matched with CT scans from patients with postnatally detected congenital lung malformations (controls). Statistical analysis was performed from January 18 to September 6, 2020. Preoperative CT scans were interpreted independently by 9 experienced pediatric radiologists in a blinded fashion and analyzed from January 24, 2019, to September 6, 2020.MAIN OUTCOMES AND MEASURES Accuracy, sensitivity, and specificity of CT in correctly identifying children with malignant tumors. RESULTS Among 477 CT scans identified (282 boys [59%]; median age at CT, 3.6 months [IQR, 1.2-7.2 months]; median age at resection, 6.9 months [IQR, 4.2-12.8 months]), 40 cases were extensively reviewed; 9 cases (23%) had pathologically confirmed cystic PPB. The median age at CT was 7.3 months (IQR, 2.9-22.4 months), and median age at resection was 8.7 months (IQR, 5.0-24.4 months). The sensitivity of CT for detecting PPB was 58%, and the specificity was 83%. High suspicion for malignancy correlated with PPB pathology (odds ratio, 13.5; 95% CI, 2.7-67.3; P = .002).There was poor interrater reliability (κ = 0.36 [range, 0.06-0.64]; P < .001) and no significant difference in specific imaging characteristics between PPB and benign cystic lesions. The overall accuracy rate for distinguishing benign vs malignant lesions was 81%. CONCLUSIONS AND RELEVANCEThis study suggests that chest CT, the current criterion standard imaging modality to assess the lung parenchyma, may not accurately and reliably distinguish PPB from benign congenital lung malformations in children. In any cystic lung lesion without a prenatal diagnosis, operative management to confirm pathologic diagnosis is warranted.

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Development of a multi-institutional registry for children with operative congenital lung malformations

Cited by 23 publications

References 57 publications

Narrative review of congenital lung lesions

Narrative review of congenital lung lesions

Preoperative Embolization Facilitates Segmental Resection of Pulmonary Sequestration in an Infant

Accuracy of Chest Computed Tomography in Distinguishing Cystic Pleuropulmonary Blastoma From Benign Congenital Lung Malformations in Children

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