Defining hydrops and indications for open fetal surgery for fetuses with lung masses and vascular tumors

doi:10.1016/j.jpedsurg.2011.10.019

Journal of Pediatric Surgery

Volume 47, Issue 1, January 2012, Pages 40-45

https://doi.org/10.1016/j.jpedsurg.2011.10.019 Get rights and content

Abstract

Purpose

The aim of this study was to identify the most accurate prenatal predictors of outcomes and need for fetal surgery for fetuses with high-risk lung masses and vascular tumors.

Methods

The records of all fetuses with high-risk lung mass (congenital cystic adenomatoid malformation-volume ratio > 1.6 or findings of hydrops) and vascular tumor evaluated between July 2001 and March 2011 were reviewed retrospectively. Hydrops was defined as accumulation of fluid in 2 or more compartments.

Results

Of fetuses with high-risk lung mass, hydrops was identified in 46% (11/24). Fetuses with hydrops and an abnormal echocardiogram (n = 8) demonstrated poor survival without fetal surgery (13%) compared with 100% survival in fetuses with hydrops and a normal echocardiogram (n = 3; P = .02). Of 21 fetuses with vascular tumor (11 sacrococcygeal and 8 cervical teratomas; 2 hemangioendotheliomas), hydrops was identified in 29% and an abnormal echocardiogram in 57%. All fetuses with hydrops had an abnormal echocardiogram and either died (n = 5) or required fetal surgery (n = 1). However, all fetuses with abnormal echocardiograms alone (n = 7) survived without fetal intervention.

Conclusions

For fetuses with lung mass, an abnormal echocardiogram in the setting of hydrops is the best predictor of mortality and need for fetal surgery. For fetuses with vascular tumor, hydrops in the setting of high-output physiology best predicts demise and need for fetal surgery.

Section snippets

Patient population

After approval from the Institutional Review Board of the Baylor College of Medicine (protocols H-20983, H-27447, and H-26009), the case records and diagnostic imaging studies of all fetuses referred to the Texas Children's Fetal Center between July 1, 2001, and March 1, 2011, with a diagnosis of lung mass, sacrococcygeal teratoma, or neck mass were reviewed retrospectively. For all patients with fetal lung mass, a cystic adenomatoid malformation-volume ratio (CVR) was calculated as previously

High-risk lung masses

During the study period, 95 fetuses were evaluated for a confirmed fetal lung mass of whom 26 (27%) were classified as high-risk lesions because of CVR greater than 1.6 (n = 25) or findings of fetal hydrops with unknown CVR (n = 1). Two fetuses diagnosed with mainstem bronchial atresia after comprehensive evaluation were excluded from analysis, leaving 24 fetuses (13 males) with high-risk lung masses (14 left sided, 10 right sided). One fetus, who underwent evaluation in 2001 and had no

Discussion

The findings in the present study suggest that fetal echocardiographic findings may be the best predictors of the need for fetal surgical resection for fetuses with high-risk lung masses. Although findings of early hydrops and echocardiographic changes are equally sensitive in detecting poor outcome for fetuses with these lesions, an abnormal echocardiogram and findings of fetal heart failure are more specific and thus superior indicators of impending fetal demise. These findings are consistent

References (26)

N.S. Adzick
Open fetal surgery for life-threatening fetal anomalies
Semin Fetal Neonatal Med
(2010)
N.S. Adzick
Management of fetal lung lesions
Clin Perinatol
(2009)
H.L. Hedrick et al.
Sacrococcygeal teratoma: prenatal assessment, fetal intervention, and outcome
J Pediatr Surg
(2004)
S. Hirose et al.
Spectrum of intrapartum management strategies for giant fetal cervical teratoma
J Pediatr Surg
(2003)
A. Holterman et al.
The natural history of sacrococcygeal teratomas diagnosed through routine obstetric sonogram: a single institution experience
J Pediatr Surg
(1998)
O.O. Olutoye et al.
Abnormal umbilical cord Doppler sonograms may predict impending demise in fetuses with sacrococcygeal teratoma
Fetal Diagn Ther
(2004)
S.J. Bond et al.
Death due to high-output cardiac failure in fetal sacrococcygeal teratoma
J Pediatr Surg
(1990)
R.D. Wilson et al.
Sacrococcygeal teratomas: prenatal surveillance, growth and pregnancy outcome
Fetal Diagn Ther
(2009)
J. Rychik et al.
Acute cardiovascular effects of fetal surgery in the human
Circulation
(2004)
H.E. Rice et al.
Congenital cystic adenomatoid malformation: a sheep model of fetal hydrops
J Pediatr Surg
(1994)

W.T. Mahle et al.

Echocardiographic evaluation of the fetus with congenital cystic adenomatoid malformation

Ultrasound Obstet Gynecol

(2000)

L.M. Morris et al.

High-risk fetal congenital pulmonary airway malformations have a variable response to steroids

J Pediatr Surg

(2009)

W.H. Peranteau et al.

Effect of maternal betamethasone administration on prenatal congenital cystic adenomatoid malformation growth and fetal survival

Fetal Diagn Ther

(2007)

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This finding suggests that in our study population, ultrasound findings are more likely to be associated with postnatal respiratory outcomes when compared to the MRI measurement O/E NFLV. Additionally, although CVR is the most common ultrasound measurement used to predict outcomes in patients with CLM, absolute cyst volume should also be considered as an important tool to guide prenatal counseling and postnatal treatment [18–21]. The use of cyst volume is supported by Girsen et al. who reported maximal cyst volume > 34.0 cm3 as the strongest ultrasound predictor of respiratory distress in patients with CLM (100% sensitivity, 85% specificity, 54% positive predictive value, and 100% negative predictive value) [15].
Congenital lung malformations (CLM) are rare developmental anomalies of the fetal lung with a minority of patients exhibiting symptoms around the time of birth. Although ultrasound remains the gold standard, fetal MRI has recently been incorporated as an adjunct imaging modality in the workup and prenatal counseling of patients with CLM as it is thought to more accurately delineate lesion boundaries and diagnose lesion type. We evaluate what prenatal variables correlate with postnatal respiratory symptoms.
We performed a retrospective review of patients with prenatal diagnosis of CLM treated at our institution between 2006-2020. Fetal ultrasound and magnetic resonance imaging (MRI) parameters including maximal congenital pulmonary airway malformation volume ratio (CVR), absolute cyst volume, and observed to expected normal fetal lung volume (O/E NFLV) were correlated with outcomes including postnatal respiratory symptoms, need for supplementary oxygen or mechanical ventilation, delay in tolerating full feeds, resection in the neonatal period.
Our study included 111 patients, all of whom underwent fetal ultrasound with 64 patients additionally undergoing fetal MRI. Postnatal respiratory symptoms were noted in 22.5% of patients, 19.8% required supplemental oxygen, 2.7% mechanical ventilation and two patients requiring urgent resection. Ultrasound parameters including absolute cyst volume and maximal CVR correlated with need for mechanical ventilation (p=0.034 and p=0.024, respectively) and for urgent resection (p=0.018 and p=0.023, respectively) and had a marginal association with postnatal respiratory symptoms (p=0.050 and p=0.052). Absolute cyst volume became associated with postnatal respiratory symptoms (p=0.017) after multivariable analysis controlling for maternal steroid administration and gestational age. O/E NFLV did not correlate with perinatal outcomes.
We have found that ultrasound-based measurements correlate with postnatal respiratory symptoms, while MRI derived O/E NFLV does not. Further studies are needed to elucidate the role of MRI in the prenatal workup of congenital lung malformations.
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Placentomegaly and polyhydramnios are also commonly seen. Untreated hydrops from severe mediastinal compression compromise cardiac venous return and ultimately lead to in utero heart failure [24,25]. Fortunately, hydrops is a relatively rare event, occurring in less than 10% of fetuses based on recent reports using multi-institutional data [13].
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Recent advances in noninvasive imaging and molecular testing have made it possible to safely diagnose many disorders relatively early in gestation. These advances should soon allow widespread prenatal screening for the majority of human genetic diseases, opening the door to the possibility of treatment/correction prior to birth. In addition to the obvious psychological and financial benefits of curing a disease in utero, and thereby enabling the birth of a healthy infant, there are multiple biological advantages that are unique to the fetal recipient, which provide compelling rationale for performing potentially curative treatments, such as stem-cell transplantation or gene therapy, prior to birth. Herein, we provide an overview of the exciting field of fetal therapy, touching briefly on prenatal surgical interventions and then focusing the majority of the chapter on in utero transplantation (IUTx) and in utero gene therapy (IUGT). We also discuss some of the biological hurdles that have thus far limited the success of IUTx to patients with immunodeficiencies, some of the unique risks associated with IUGT, and finish the chapter by highlighting several recent experimental breakthroughs in immunology, hematopoietic/marrow ontogeny, gene transfer, and in utero cell delivery, which have collectively provided means of overcoming these barriers, thus setting the stage for clinical application of these highly promising therapies.
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An accurate prenatal diagnostic assessment enables a correct risk-related patient stratification, on which to individually base on the parental counseling regarding fetal monitoring extent, maternity clinic selection and planning of pre- and postnatal surgical options [13,16,18,33]. A prenatal intervention is considered in a fetus <28–30 weeks at high-risk for preterm delivery and fetal demise due to e.g. an high-output cardiac failure (hydrops) or placentomegaly if left untreated [2,15,31,39–48]. Intrauterine therapy consists of closure of the feeding vessels with laser technology or open fetal surgery.
We report a case series of two newborns with a sacrococcygeal teratoma (SCT) with intraspinal involvement. In one case, the SCT was detected prenatally and presented postnatally as a large mass of 8 × 10 × 10 cm. In the second case the prenatal ultrasound was unremarkable and the SCT presented postnatally as a rather flat one-sided gluteal mass. MRI diagnostics led to the diagnosis of extradural intraspinal involvement. In an interdisciplinary operation between pediatric surgeons and pediatric neurosurgeons a complete complications-free tumor resection was possible in both cases with a favorable postoperative course. Due to the complete resection and benign histology (Gonzales-Crussi grade 0 and 1 with exclusion of yolk sac tumor components), adjuvant measures such as chemotherapy were not necessary. At 4 months no morbidity or mortality occurred, micturition and defecation were unremarkable in both cases and neurological examinations were age appropriate.
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The CPAM volume ratio (CVR) was prospectively measured throughout the pregnancy as previously detailed [17]. Hydrops was defined as the presence of fluid in two or more spaces, including ascites, pleural effusion, pericardial effusion, or skin edema, with fetal echocardiographic evidence to support heart dysfunction as reported elsewhere [25]. The pathologic diagnosis was confirmed in all resected specimens.
The purpose of this study was to evaluate short-term respiratory outcomes in neonates with symptomatic congenital lung malformations (CLM).
Consecutive newborns who underwent surgical resection of a CLM were retrospectively reviewed. Demographic, prenatal, and outcomes data were analyzed as appropriate (p < 0.05).
Twenty-one neonates were managed at a median gestational age of 36.2 weeks [interquartile range (IQR), 33.8–39.0]. Endotracheal intubation was required in 14 (66.7%) for a median of 7.5 days [interquartile range (IQR), 3.0–25.8]. Three (14.3%) children underwent ex utero intrapartum treatment-to-resection, and another 14 (66.7%) had neonatal lung resections performed at a median age of 2.0 days (IQR, 0.08–19.5 days). Excluding one patient who received comfort care at birth, all neonates survived to hospital discharge with a median length of hospitalization of 36.5 days (IQR, 23.8–56.5). More than one-quarter were discharged on supplemental oxygen by nasal cannula. Based on a median follow up of 35.5 months (IQR, 19.0–80.8), CLM-related morbidity was still evident in 55.0%.
Our study suggests a high incidence of complications and chronic respiratory morbidity after neonatal lung resection for symptomatic CLMs. These data highlight the need to provide realistic expectations in perinatal counseling discussions with families and the importance of coordinating appropriate multidisciplinary follow up for these children.
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Diagnosis and management of congenital lung malformations has evolved dramatically over the past several decades. Advancement in imaging technology has enabled earlier, more definitive diagnoses and, consequently, more timely intervention in utero or after birth, when indicated. These advancements have increased overall survival rates to around 95% from historical rates of 60%. However, further refinement of diagnostic technique and standardization of treatment is needed, particularly as the increased sensitivity of diagnostic imaging results in more frequent diagnoses. In this article, we provide an updated review of the diagnostic strategies, management, and prognosis of congenital lung malformations.

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APSA paperDefining hydrops and indications for open fetal surgery for fetuses with lung masses and vascular tumors

Abstract

Purpose

Methods

Results

Conclusions

Section snippets

Patient population

High-risk lung masses

Discussion

Open fetal surgery for life-threatening fetal anomalies

Semin Fetal Neonatal Med

Management of fetal lung lesions

Clin Perinatol

Sacrococcygeal teratoma: prenatal assessment, fetal intervention, and outcome

J Pediatr Surg

Spectrum of intrapartum management strategies for giant fetal cervical teratoma

J Pediatr Surg

The natural history of sacrococcygeal teratomas diagnosed through routine obstetric sonogram: a single institution experience

J Pediatr Surg

Abnormal umbilical cord Doppler sonograms may predict impending demise in fetuses with sacrococcygeal teratoma

Fetal Diagn Ther

Death due to high-output cardiac failure in fetal sacrococcygeal teratoma

J Pediatr Surg

Sacrococcygeal teratomas: prenatal surveillance, growth and pregnancy outcome

Fetal Diagn Ther

Acute cardiovascular effects of fetal surgery in the human

Circulation

Congenital cystic adenomatoid malformation: a sheep model of fetal hydrops

J Pediatr Surg

Echocardiographic evaluation of the fetus with congenital cystic adenomatoid malformation

Ultrasound Obstet Gynecol

High-risk fetal congenital pulmonary airway malformations have a variable response to steroids

J Pediatr Surg

Effect of maternal betamethasone administration on prenatal congenital cystic adenomatoid malformation growth and fetal survival

Fetal Diagn Ther

APSA paper
Defining hydrops and indications for open fetal surgery for fetuses with lung masses and vascular tumors