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1.
Kunisaki SM Barnewolt CE Estroff JA Ward VL Nemes LP Fauza DO Jennings RW 《Journal of pediatric surgery》2007,42(2):404-410
Purpose
The prognosis for fetuses with large congenital cystic adenomatoid malformations (CCAMs) remains uncertain. This study examined the natural history of large fetal CCAMs managed expectantly at a major referral center.Methods
A 5-year retrospective review was conducted on fetuses diagnosed with a thoracic lesion (n = 59). Large CCAMs were identified on prenatal imaging and followed longitudinally. Perinatal outcomes were assessed.Results
Twelve (20.3%) fetuses had large CCAMs in the absence of other congenital anomalies. Peak CCAM size occurred at 25.3 ± 3.6 weeks' gestation. Serial magnetic resonance volumetry demonstrated a trend toward decreasing CCAM mass volume relative to thoracic cavity volume over time. Overall, 6 patients, including 3 with signs of early hydrops, showed a marked regression of their lesions relative to estimated fetal weight. Five fetuses required an emergent intervention postnatally, including extracorporeal membrane oxygenation support (n = 1), cyst aspiration (n = 1), and lung resection (n = 5). Overall survival was 75%, with severe hydrops before 30 weeks seen in all 3 deaths.Conclusion
Large fetal CCAMs tend to peak in size at 25 weeks' gestation and are characterized by in utero diminution relative to overall fetal growth. The prognosis for most fetuses with large CCAMs remains quite favorable under careful perinatal management. 相似文献2.
Patrick F. Curran Eric B. Jelin Larry Rand Shinjiro Hirose Vickie A. Feldstein Ruth B. Goldstein Hanmin Lee 《Journal of pediatric surgery》2010,45(1):145-150
Objective
The purpose of this study is to evaluate the effect of prenatal steroid treatment in fetuses with sonographically diagnosed congenital cystic adenomatoid malformations (CCAMs).Methods
This was an institutional review board-approved retrospective review of 372 patients referred to the University of California, San Francisco (UCSF), for fetal CCAM. Inclusion criteria were (1) a predominately microcystic CCAM lesion sonographically diagnosed at our institution, (2) maternal administration of a single course of prenatal corticosteroids (betamethasone), and (3) no fetal surgery. CCAM volume-to-head ratio (CVR), presence of hydrops, mediastinal shift, and diaphragm eversion were assessed before and after administration of betamethasone. The primary end points were survival to birth and neonatal discharge.Results
Sixteen patients with predominantly microcystic CCAMs were treated with prenatal steroids. Three were excluded because of lack of follow-up information. All remaining fetuses (13/13) survived to delivery and 11/13 (84.6%) survived to neonatal discharge. At the time of steroid administration, all patients had CVR greater than 1.6, and 9 (69.2%) also had nonimmune hydrops fetalis. After a course of steroids, CVR decreased in 8 (61.5%) of the 13 patients, and hydrops resolved in 7 (77.8%) of the 9 patients with hydrops. The 2 patients whose hydrops did not resolve with steroid treatment did not survive to discharge.Conclusion
In high-risk fetal patients with predominantly microcystic CCAM lesions, betamethasone is an effective treatment. This series is a pilot study for a prospective randomized trial comparing treatment of CCAM with betamethasone to placebo. 相似文献3.
Grethel EJ Wagner AJ Clifton MS Cortes RA Farmer DL Harrison MR Nobuhara KK Lee H 《Journal of pediatric surgery》2007,42(1):117-123
Purpose
The natural history of certain prenatally diagnosed masses is well known. Large thoracic mass lesions can evolve one of 2 ways, either to regress and cause minimal morbidity, or to progress and enlarge, often resulting in hydropic changes in the fetus. This nonimmune hydrops carries a dismal prognosis, with nearly all fetuses expiring before or shortly after birth. However, hydrops associated with fetal mass lesions can be halted and even reversed with fetal intervention and treatment of the underlying defect. We examined our patients with fetal mass lesions to evaluate survival after intervention.Methods
Institutional approval was obtained by the Committee on Human Research. A retrospective review was performed of 294 fetuses evaluated over 15 years with large mass lesions. All patients were evaluated for evidence of fetal hydrops using ultrasound criteria. Patients were divided according to type of intervention. Primary outcome measure was 30-day survival after birth.Results
- (1)
- Patients without fetal hydrops did not undergo fetal intervention and survived to 30 days after birth (167/172, 97%).
- (2)
- Patients with fetal mass lesions that developed hydrops fared poorly with no intervention (1/33 survival, 3%), whereas fetuses undergoing prenatal intervention fared much better (15/30 open, 50%; 3/10 percutaneous, 30%).
- (3)
- Four patients with hydropic congenital cystic adenomatoid malformation (n = 3) or pulmonary sequestration (n = 1) received steroids in preparation for surgery but underwent no intervention, and the patients survived the neonatal period.
Conclusion
Fetuses with prenatal diagnoses of masses not associated with hydrops have excellent prognosis with survival higher than 95%. Nonimmune hydrops associated with prenatal diagnosis of a fetal mass is a devastating complication with less than 5% survival. Open resection of a mass causing hydrops resulted in 50% survival, with reversal of hydrops in a group with near-uniform fatality. Further investigation is warranted regarding the use of minimally invasive prenatal therapies including steroid administration for hydropic fetuses. 相似文献4.
Cass DL Olutoye OO Cassady CI Moise KJ Johnson A Papanna R Lazar DA Ayres NA Belleza-Bascon B 《Journal of pediatric surgery》2011,46(2):292-298
Aim
The purpose of this study is to evaluate the accuracy of prenatal diagnostic features, particularly congenital cystic adenomatoid malformation volume ratio (CVR), in predicting outcomes for fetuses with lung masses.Methods
The records and imaging features of all fetuses referred to the Texas Children's Fetal Center with a fetal lung mass between July 2001 and May 2010 were reviewed retrospectively. Data collected included gestational age (GA) at diagnosis, fetal magnetic resonance imaging findings, CVR, mass size, nature of fetal treatment, surgical findings, pathology, and outcome. Data were analyzed for predicting development of hydrops or the need for fetal therapy using receiver operating characteristic curves.Results
Of 82 fetuses (41 male) evaluated for a lung mass, 53 (65%) were left-sided (1 bilateral), and the mean (SD) GA at diagnosis was 21.5 (4.3) weeks. Seventy-three fetuses underwent fetal magnetic resonance imaging at a mean (SD) GA of 26.1 (4.6) weeks. Thirteen fetuses (16%) had fetal treatment. Four fetuses with hydrops underwent open fetal surgical resection, and 3 survived. Six fetuses with large lung masses and persistent mediastinal compression near term underwent ex-utero intrapartum therapy-to-resection procedures, and 3 fetuses with hydrops underwent serial thoracentesis. Congenital cystic adenomatoid malformation volume ratio correlated strongly with the development of hydrops and the need for fetal therapy with an area under the receiver operating characteristic curve of 0.96 (P < .0001) and 0.88 (P < .0001), respectively. Of 18 fetuses with a CVR greater than 2.0 compared with 2 (3%) of 60 with a CVR of 2.0 or less, 10 (56%) required fetal intervention (P < .0001).Conclusion
Congenital cystic adenomatoid malformation volume ratio correlates strongly with the development of fetal hydrops and the need for fetal intervention. A threshold value of 2.0 yields the most powerful statistical results. 相似文献5.
John S. Riley John W. Urwin Edward R. Oliver Beverly G. Coleman Nahla Khalek Julie S. Moldenhauer Susan S. Spinner Holly L. Hedrick N. Scott Adzick William H. Peranteau 《Journal of pediatric surgery》2018,53(2):265-269
Purpose
The prenatal natural history of intralobar and extralobar bronchopulmonary sequestrations (BPSs), including lesion growth patterns and need for prenatal intervention, have not been fully characterized. We review our series of BPSs to determine their natural history and outcomes in the context of the need for prenatal intervention.Methods
A retrospective review of the pre/postnatal course of 103 fetuses with an intralobar (n = 44) or extralobar BPS (n = 59) managed at a single institution between 2008 and 2015 was performed. Outcomes included prenatal lesion growth trajectory, presence of hydrops, need for prenatal intervention, survival, and postnatal surgical management.Results
Most extralobar (71%) and intralobar BPSs (94%) decreased in size or became isoechoic from initial to final evaluation. Peak lesion size occurred at 26–28 weeks gestation. Eight fetuses developed hydrothorax, four of which (all extralobar BPSs) also developed hydrops. All four hydropic fetuses received maternal betamethasone, and three hydropic fetuses underwent thoracentesis and/or thoracoamniotic shunt placement with subsequent hydrops resolution. All fetuses survived. Forty-one intralobar (93%) and 35 extralobar BPSs (59%) were resected after birth.Conclusions
BPSs tend to decrease in size after 26–28 weeks gestation and rarely require fetal intervention. Lesions resulting in hydrothorax ± hydrops can be effectively managed with maternal steroids and/or drainage of the hydrothorax.Level of evidence
IV 相似文献6.
Purpose
Although antenatal resolution of congenital cystic adenomatoid malformations (CCAMs) is well documented, complete spontaneous postnatal resolution is rare, its existence even questioned by some.Methods
All cases of antenatally diagnosed CCAMs over 7 years were retrospectively reviewed. Inclusion criteria were the following: (1) antenatal diagnosis of CCAM, (2) persistence on postnatal imaging, and (3) subsequent spontaneous resolution on postnatal imaging.Results
Of 56 antenatally diagnosed CCAMs, 2 patients were identified. Both had macrocystic lesions. In case 1, the CCAM that filled the hemithorax on antenatal ultrasound was smaller on postnatal chest radiograph and disappeared by age 37 months on computed tomography. Case 2 had significant reduction of the CCAM at birth (persistence of the lesion on initial radiographs and ultrasound was documented). By 5 months, the lesion was not evident on computed tomography. Neither case was associated with symptoms, polyhydramnios, hydrops, or other abnormalities.Conclusions
In patients with an antenatal diagnosis of CCAM, spontaneous resolution may occur postnatally in 4% of cases. Significant reduction in CCAM size may portend possible disappearance and therefore warrants an observational period before resection. 相似文献7.
Jessica L. Roybal 《Journal of pediatric surgery》2010,45(8):1633-1639
Background
Congenital high airway obstruction syndrome (CHAOS) is caused by complete or near-complete obstruction of the fetal airway. Obstruction sets in motion a sequence of events that can ultimately lead to fetal demise. However, on rare occasions in utero airway decompression occurs, reversing syndromic findings and improving the prognosis. In our relatively large series of CHAOS patients, we have observed a spectrum of clinical severity. The aim of this study was to identify the prenatal characteristics of CHAOS predictive of a milder postnatal course.Methods
The medical charts of all fetuses observed at our institution with the diagnosis of CHAOS were reviewed for radiologic findings, delivery information, perinatal course, autopsy or discharge report, and long-term follow-up.Results
Between 1996 and 2008, 12 fetuses with CHAOS were identified. Four fetuses had no evidence of hydrops on initial imaging. Of the 8 fetuses displaying hydrops, 3 were terminated, 2 died in utero, and 1 with multiple anomalies died at birth. Six fetuses were delivered via the ex utero intrapartum therapy procedure for attempted salvage, and 5 of the 6 survived the neonatal period including all 4 fetuses without hydrops. Serial prenatal imaging demonstrated less severe signs of CHAOS in 3 fetuses, and in 2 of them, direct laryngoscopy revealed a tiny opening in the airway. All 3 fetuses that showed improvement on serial imaging survived the neonatal period and were discharged home by 2-10 weeks of age.Conclusions
Although the natural history of CHAOS is variable, trends in prenatal ultrasound findings are highly predictive of postnatal outcome and are a valuable guide to prenatal counseling. 相似文献8.
Kamata S Usui N Kamiyama M Nose K Sawai T Fukuzawa M 《Journal of pediatric surgery》2006,41(12):2023-2027
Background/Purpose
The natural history of cystic lung disease (CLD) such as congenital cystic adenomatoid malformation (CCAM) and pulmonary sequestration has been altered by the advent of prenatal diagnosis. Although recent advances including fetal therapy have gradually improved outcome, the long-term course and the function of the residual lung have not been well clarified.Methods
Twenty-two patients with CLD who had been prenatally diagnosed and treated between 1990 and 2004 were reviewed. The clinical outcome and growth measurements were established, and, where possible, all infants underwent ventilation and perfusion lung scan.Results
Mediastinal shift was present in 14 fetuses. Fetal hydrops was present in 5 fetuses. Antenatal intervention was performed for hydrops in 2 fetuses (cyst-amniotic shunt and aspiration). Twenty-one infants underwent appropriate excisional surgery. Final diagnosis included CCAM (n = 12) and pulmonary sequestration (n = 7). No late death was observed. Common complications were failure to thrive (n = 5), frequent respiratory tract infection (n = 4), and asthmatic attack (n = 4). A significant decrease in lung ventilation and perfusion on the affected side was observed in patients with hydrops, lobectomy, and CCAM.Conclusion
Long-term follow-up including respiratory care and growth assessment should be performed in prenatally diagnosed patients with CLD, especially those who present with hydrops. 相似文献9.
Lee M. Morris Jeffrey C. Livingston Timothy M. Crombleholme 《Journal of pediatric surgery》2009,44(1):60-65
Background/Purpose
Anecdotal reports suggest that maternal steroids may arrest the growth of congenital pulmonary airway malformations (CPAMs), preventing or reversing hydrops. We reviewed our experience with CPAMs to determine the fetal response to steroid therapy.Methods
This study is a retrospective review of all fetal CPAMs from 2004 to 2008. Fetuses with high-risk CPAMs that received at least one course of steroids were identified. Fetal magnetic resonance imaging and ultrasound data were used to classify the CPAMs, identify hydrops fetalis and follow the fetuses poststeroid dosing.Results
Forty-four fetuses with CPAM were identified. Fifteen patients were found to have received at least one course of steroids. Thirteen were hydropic and 2 were nonhydropic. Seven of the 13 hydropic fetuses (54%) showed an initial response to steroid administration, whereas the 2 nonhydropic high-risk fetuses progressed to birth without developing hydrops. Seven of the 15 patients, however, resulted in fetal demise or early postnatal death, giving a survival rate of 53%.Conclusions
High-risk CPAMs have a variable response to steroids. This variable response demonstrates the need for a placebo-controlled randomized study to more accurately determine the effect of steroids on hydrops and CPAM growth rates. Repeated steroid courses may not be helpful, and progression in CPAM volume to head circumference ratio (CVR) or hydrops should prompt open fetal surgery to prevent irreversible fetal insult. 相似文献10.
Background
The relationship between congenital cystic adenomatoid malformation (CCAM) and pleuropulmonary blastoma (PPB), whether causal, correlational, or coincidental, remains controversial. There is a lack of consensus as to the optimal treatment of patients with asymptomatic CCAM.Method
We reviewed all cases of CCAM and PPB seen at our institution from 1999 to 2008. Institutional Research Ethics Board approval was obtained. The incidence of CCAM and PPB, respectively, was calculated based on birth numbers during the study period.Results
Seventy-four CCAMs were resected over the study period in 129 children diagnosed with CCAM. Five PPBs were diagnosed during the study period. Three of the 5 PPB cases were initially diagnosed as CCAMs. These PPBs were not clinically or radiologically distinguishable from CCAMs. In our referral area, the incidence of CCAM was 1 in 12,000; and the incidence of PPB was 1 in 250,000 live births. The mortality rate for PPB in this cohort was 20%.Conclusion
Asymptomatic cystic lung malformations represent a therapeutic dilemma. In this cohort, the incidence of PPB among apparently benign lung lesions was 4%. No clinical or radiological markers differentiated benign CCAMs from PPBs. Our experience provides further justification for resection of all CCAMs. This should be discussed with parents until CCAMs and PPBs can be clearly distinguished preoperatively. 相似文献11.
Davenport M Warne SA Cacciaguerra S Patel S Greenough A Nicolaides K 《Journal of pediatric surgery》2004,39(4):549-556
Background/purpose
The natural history of parenchymal lung lesions such as congenital cystic adenomatoid malformation (CCAM) and pulmonary sequestration (PS) has been altered by the advent of antenatal ultrasonography. Initial reports were characterized by a high (about 30%) incidence of adverse features (eg, hydrops) and a poor outcome and did not accord with our recent experience. The authors have reviewed the outcome of fetuses that had been diagnosed in a large tertiary referral fetal medicine unit with the aim of delineating current experience. The term cystic lung disease was used throughout to avoid unjustifiable histologic precision.Methods
The scans of all fetuses that had been diagnosed with cystic lung disease between January 1995 and July 2001 were reviewed. The outcome of each pregnancy was established, and, where possible, all infants underwent appropriate investigations, including thoracic computed tomography (CT) scans.Results
Sixty-seven fetuses had a cystic lung abnormality diagnosed from January 1995 to July 2001. The median (range) age at diagnosis was 21 (19 to 28) weeks. The lesion was right sided in 29 (43%), left in 36 (54%), and bilateral in 2 (3%); it was characterized as dominantly macrocystic in 27 (40%), microcystic in 35 (52%), and mixed in 5 (8%). Mediastinal shift was present in 30 fetuses (45%). Severe signs of fetal distress (eg, hydrops) were present in 5 fetuses (7%). Antenatal intervention was performed in 4 fetuses (thoraco-amniotic shunts [n = 3] and percutaneous intrauterine laser therapy [n = 1]). Sixty-four (96%) of the fetuses were born alive. There was 1 termination of pregnancy and 2 intrauterine deaths (all severe microcystic lesions). Forty-two infants (63%) underwent thoracotomy and appropriate excisional surgery at a median of 7.5 months (range, 1 day to 34 months). Two infants (which included the fetus having intrauterine laser therapy) died early in the postnatal period. Both were large microcystic lesions and had antenatal features of severe fetal distress. Twelve infants were investigated in the postnatal period but did not undergo surgery. Ten infants were not appropriately investigated or were lost to follow-up. Histologic examination showed definitive diagnostic features of CCAM (n = 25) or PS (n = 6). Other lesions with hybrid features of both were also seen (n = 11). There was a degree of correlation between antenatal ultrasound features (size of cyst [P = .03], in-utero behavior [P = .06], mediastinal shift [P = 0.05]) and the need for surgery but not with the final histologic diagnosis. Surgical excision was required in 45% of lesions showing late-gestation “resolution.”Conclusions
Antenatally diagnosed “cystic lung disease” has an excellent prognosis in the absence of signs of severe fetal distress. The need for surgery should be based on appropriate postnatal investigations (eg, CT scans), rather than on antenatal behavior. 相似文献12.
Kuroda T Kitano Y Honna T Sago H Hayashi S Saeki M 《Journal of pediatric surgery》2004,39(12):1819-1822
Purpose
The aim of this study was to investigate the prenatal courses and management of abdominal surgical diseases.Methods
Of the 327 patients registered with our fetal treatment board since March 2002, 83 fetuses referred to the surgical department were enrolled for the current study. The prenatal diagnosis, sequential fetal images, and perinatal courses of these cases were reviewed retrospectively.Results
Of the 83 cases, abdominal diseases were suspected in 34, lung and thoracic diseases in 25, genitourinary diseases in 12, and other anomalies in 12. Meconium peritonitis (MP), intestinal obstruction, and abdominal wall defects accounted for approximately 65% of the abdominal diseases. Five patients with prenatally diagnosed lung diseases underwent fetal surgical intervention, and 17 of the 22 liveborn patients survived. In contrast, none of the patients with prenatally diagnosed abdominal anomalies underwent fetal surgical intervention, yet, 23 of the 24 liveborn patients survived. However, preterm labor and hydrops were seen frequently in the patients with giant cystic MP, suggesting a fetal critical condition.Conclusions
Although the clinical outcome of abdominal diseases seemed favorable with postnatal treatment, the current results suggested the occurrence of hidden mortality in utero and the potential need for fetal intervention for some abdominal conditions, such as MP. 相似文献13.
Lim FY Crombleholme TM Hedrick HL Flake AW Johnson MP Howell LJ Adzick NS 《Journal of pediatric surgery》2003,38(6):940-945
Background
Congenital high airway obstruction syndrome (CHAOS) is a life-threatening condition with a poorly understood natural history.Methods
A retrospective review of five patients with CHAOS between 1997 and 2002 was performed.Results
All fetuses had large echogenic lungs, dilated airways, inverted diaphragms, and massive ascites. One fetus with a laryngeal cyst was terminated at 22 weeks. A twin fetus with findings suggestive of a tracheal web had progressive hydrops, which led to fetal demise. The remaining 3 patients delivered via the ex utero intrapartum treatment (EXIT) procedure survived. The first patient tolerated progressive hydrops for 12 weeks in utero. He had tracheal atresia but underwent laryngotracheoplasty successfully. He is the first long-term CHAOS survivor and is speaking at 5 years of age. The 2 patients with relatively stable lung volumes prenatally have laryngeal atresia with a pinpoint posterior laryngeal fistula. Their postnatal clinical courses were much more benign than the first survivor.Conclusions
The prenatal natural history and postnatal course of CHAOS depends on whether the airway obstruction is complete. The EXIT procedure offers the potential for salvage of this otherwise lethal condition. Hydrops may be well tolerated prenatally for weeks with potential resolution if airway fistulization is present. 相似文献14.
Jessica L. Roybal 《Journal of pediatric surgery》2011,46(7):1325-1332
Background
Large, prenatally diagnosed sacrococcygeal teratomas (SCTs) present a formidable challenge because of their unpredictable growth and propensity for complications. In our experience, even with aggressive serial imaging, many fetuses have died under a policy of “watchful waiting.” We propose “early delivery” as the best option for selected cases of high-risk fetal SCT.Methods
The medical charts of all fetuses with SCT followed up at our institution and delivered before 32 weeks of gestation were reviewed for radiologic findings, fetal interventions, delivery information, perinatal inpatient course, and autopsy or discharge report.Results
Between 1996 and 2009, excluding those that underwent fetal surgery, 9 patients with fetal SCT were delivered before 32 weeks of gestation. Four had type I tumors, and 5 had type II tumors. Of the 9 fetuses, 4 survived the neonatal period. The only surviving patient delivered before 28 weeks underwent an ex utero intrapartum therapy procedure.Conclusions
A significant number of pregnancies complicated by high-risk SCT will manifest signs of fetal or maternal decompensation, or both, between 27 and 32 weeks of gestation. In the absence of fulminant hydrops, preemptive early delivery can be associated with surprisingly good outcomes in appropriately selected fetuses with high-risk SCT. 相似文献15.
Tsao K Hawgood S Vu L Hirose S Sydorak R Albanese CT Farmer DL Harrison MR Lee H 《Journal of pediatric surgery》2003,38(3):508-510
Background/Purpose: Development of hydrops fetalis in fetuses with congenital cystic adenomatoid malformations (CCAM) is a significant risk factor for fetal or neonatal demise. In rare cases, resolution of CCAM has occurred, presumably owing to lesion maturation or involution. Steroid therapy, utilized for lung maturity, has been postulated to accelerate this process. The natural history of hydropic fetuses with CCAM after receiving steroid therapy is presented. Methods: The authors prospectively followed up with 3 patients who had antenatally diagnosed CCAM and nonimmune hydrops fetalis with predicted mortality. All patients declined or were not candidates for fetal intervention and were treated with standard prenatal betamethasone to increase lung maturity. Results: Three fetuses had CCAM and nonimmune hydrops fetalis diagnosed prenatally. After a course of prenatal steroids during the second trimester, all 3 patients had resolution of their hydrops and were delivered at term without respiratory distress. Conclusions: Nonimmune hydrops fetalis in fetuses with congenital cystic adenomatoid malformation is a harbinger for fetal demise. The resolution of hydrops in these patients after receiving steroid therapy is an interesting and compelling observation. Because the mechanism of this process is speculative, further studies are needed to elucidate the relationship between antenatal steroids and maturation of congenital cystic adenomatoid malformation. J Pediatr Surg 38:508-510. 相似文献
16.
S. Christopher Derderian Shivika Trivedi Jody Farrell Roberta L. Keller Larry Rand Ruth Goldstein Vickie A. Feldstein Shinjiro Hirose Tippi C. MacKenzie 《Journal of pediatric surgery》2014
Objective
Primary hydrothorax is a rare congenital anomaly with outcomes ranging from spontaneous resolution to fetal demise. We reviewed our experience with fetuses diagnosed with primary hydrothorax to evaluate prenatal management strategies.Methods
We reviewed the records of patients evaluated for fetal pleural effusions at our Fetal Treatment Center between 1996 and 2013. To define fetuses with primary hydrothorax, we excluded those with structural or genetic anomalies, diffuse lymphangiectasia, immune hydrops, and monochorionic diamniotic twin gestations.Results
We identified 31 fetuses with primary hydrothorax, of whom 24 had hydrops. Hydropic fetuses were more likely to present with bilateral effusions. Of all fetuses with primary hydrothorax, 21 had fetal interventions. Survival without hydrops was 7/7 (100%), whereas survival with hydrops depended on whether or not the patient had fetal intervention: 12/19 (63%) with intervention and 1/5 (20%) without intervention. Premature delivery was common (44%) among those who had fetal intervention.Conclusions
Fetal intervention for primary hydrothorax may lead to resolution of hydrops, but preterm birth and neonatal demise still occur. Understanding the pathophysiology of hydrops may provide insights into further prenatal management strategies, including targeted therapies to prevent preterm labor. 相似文献17.
Irving J. Zamora Fariha Sheikh Christopher I. Cassady Oluyinka O. Olutoye Amy R. Mehollin-Ray Rodrigo Ruano Timothy C. Lee Stephen E. Welty Michael A. Belfort Cecilia G. Ethun Michael E. Kim Darrell L. Cass 《Journal of pediatric surgery》2014
Purpose
The purpose of this study was to evaluate fetal magnetic resonance imaging (MRI) as a modality for predicting perinatal outcomes and lung-related morbidity in fetuses with congenital lung masses (CLM).Methods
The records of all patients treated for CLM from 2002 to 2012 were reviewed retrospectively. Fetal MRI-derived lung mass volume ratio (LMVR), observed/expected normal fetal lung volume (O/E-NFLV), and lesion-to-lung volume ratio (LLV) were calculated. Multivariate regression and receiver operating characteristic analyses were applied to determine the predictive accuracy of prenatal imaging.Results
Of 128 fetuses with CLM, 93% (n = 118) survived. MRI data were available for 113 fetuses. In early gestation (< 26 weeks), MRI measurements of LMVR and LLV correlated with risk of fetal hydrops, mortality, and/or need for fetal intervention. In later gestation (> 26 weeks), LMVR, LLV, and O/E-NFLV correlated with neonatal respiratory distress, intubation, NICU admission and need for neonatal surgery. On multivariate regression, LMVR was the strongest predictor for development of fetal hydrops (OR: 6.97, 1.58–30.84; p = 0.01) and neonatal respiratory distress (OR: 12.38, 3.52–43.61; p ≤ 0.001). An LMVR > 2.0 predicted worse perinatal outcome with 83% sensitivity and 99% specificity (AUC = 0.94; p < 0.001).Conclusion
Fetal MRI volumetric measurements of lung masses and residual normal lung are predictive of perinatal outcomes in fetuses with CLM. These data may assist in perinatal risk stratification, counseling, and resource utilization. 相似文献18.
Usui N Kamata S Sawai T Kamiyama M Okuyama H Kubota A Okada A 《Journal of pediatric surgery》2004,39(4):603-606
Background/purpose
This study aimed at identifying characteristic features indicating congenital cystic adenomatoid malformation of the lung (CCAM) and evaluating the outcome predictors to identify prenatally subgroups of fetuses with significantly different probabilities of mortality or severe respiratory difficulty.Methods
Twenty-eight neonates who had undergone antenatal evaluation for cystic lung disease (CLD) were reviewed retrospectively. The patients were divided into 3 groups according to the severity of their clinical course; mild (n = 7), moderate (n = 13), and severe (n = 8). Ultrasonographic findings in the fetus and their pulmonary lesion were evaluated. The normal lung to thorax transverse area ratio (L/T) was measured by ultrasonography.Results
High echogenicity of the lesion throughout pregnancy and polyhydramnios were frequently seen in CCAM. All of the patients with other CLD showed isoechogenicity at the end of pregnancy. All patients in the severe group had both polyhydramnios and fetal hydrops. L/T was increased in mild and moderate groups, whereas no patient in the severe group had an increase in L/T at the final measurement. Each value of final L/T in the severe group was less than 0.25.Conclusions
The subgroup of fetuses with an increased probability of mortality or severe respiratory difficulty could be predicted from the combination of polyhydramnios, fetal hydrops, and a final L/T value of less than 0.25. 相似文献19.
Roubliova X Verbeken E Wu J Yamamoto H Lerut T Tibboel D Deprest J 《Journal of pediatric surgery》2004,39(7):1066-1072
Background/Purpose
Although the pulmonary vascular abnormalities in congenital diaphragmatic hernia (CDH) are described from a morphologic point of view, the treatment of pulmonary hypertension (PH) remains one of the main unsolved problems in clinical daily practice. For this reason, detailed studies in well-validated animal models could still be of significance in our understanding of the pathogenesis of CDH.Methods
In does of 23 days’ gestational age (GA), 39 fetuses underwent creation of diaphragmatic hernia (DH) and 15 fetuses a sham thoracotomy (SHAM). Thirty-nine nonoperated littermates served as internal controls (CTR). Fetuses were harvested by cesarean section on days 25, 27, 29, and 30 of gestation. Lung specimens were obtained and formalin fixed for further vascular morphometry studies.Results
Lung vessels from DH fetuses started to show significantly smaller internal diameter (ID), external diameter (ED), larger arterial wall thickness (WT), and, in particular, a proportionally higher medial thickness (%MT) and adventitial thickness (%AT) from day 27 onward when compared with control fetuses. SHAM fetuses, which were harvested at term, showed no differences with CTR.Conclusions
This is the first report documenting changes over time in the vascular system in a rabbit fetal model of surgically induced DH. These changes mimic pathologic findings observed in human fetuses and are also concordant with earlier observations in the surgical ovine model and the toxic nitrofen rat model. 相似文献20.
Jacob T. Stephenson Kullada O. Pichakron Tim Jancelewicz J. Kevin Grayson 《Journal of pediatric surgery》2010,45(1):65-69