Perinatal management of congenital cystic lung lesions in the age of minimally invasive surgery

Background

Most congenital cystic lung lesions (CCLLs) do not require in utero or perinatal intervention. The management of asymptomatic lesions is controversial: the theoretical risk of infection and malignancy is offset by whether thoracotomy in asymptomatic children is justified. We examined our recent experience and the role of minimally invasive surgery.

Methods

We analyzed the pre-, peri-, and postnatal findings of all consecutive CCLLs diagnosed between 1997 and 2005. We reviewed records for pre-, and postnatal imaging, management, and outcome.

Results

Thirty-five CCLL were diagnosed prenatally. Since 2000, all asymptomatic lesions were removed endoscopically at 6 to 18 months (thoracoscopy for 6 extralobar sequestrations, 3 intralobar sequestrations/congenital cystic adenomatoid malformations, 5 bronchogenic cysts, and retroperitoneal laparoscopy for 2 intraabdominal sequestrations). Congenital cystic adenomatoid malformation elements were present in more than 70%. Two abdominal lesions have regressed, and 2 patients are awaiting intervention. One symptomatic infant underwent thoracotomy for congenital lobar emphysema.

Conclusions

It has been argued that the risks associated with congenital lung lesions (infection and malignancy) justify intervention in the asymptomatic patient. In our experience, all these lesions could be safely removed using endosurgical techniques. Counseling of (future) parents should be updated to include minimally invasive surgery in the management algorithm.     

Video-assisted thoracoscopic surgical excision of cystic lung disease in children

Purpose

Video-assisted thoracoscopic surgical (VATS) technique for resection of cystic lung disease (CLD) may offer some advantages when compared with thoracotomy in children.

Methods

From September 1999 to August 2004, 6 pediatric patients underwent VATS for CLD. Patients were chosen for VATS based upon surgeon's choice. Data are expressed as mean ± SD. The Children's Healthcare of Atlanta institutional review board approved this study.

Results

The types of lesions included congenital cystic adenomatoid malformations (n = 1), extrapulmonary sequestrations (n = 3), congenital lobar emphysema (n = 1), and bronchogenic cyst (n = 1). The extent of resection included lobectomy (n = 2) and excision (n = 4). Age and weight were 11.8 ± 18 months (range 6 days to 4 years) and 7.5 ± 3.6 (range 4.0-14.0) kg, respectively. Operating time was 103 ± 70 (range 38-223) minutes. Chest tube duration was 1.2 ± 0.8 (range 0-2) days. Morphine use on the first postoperative day was 0.2 ± 0.3(range 0.05-0.20) mg/kg. Length of stay was 2.5 ± 1.9 (range 1-6) days. There were no conversions to thoracotomy and no complications.

Conclusion

VATS technique appears to be a safe and effective technique in managing CLD in children of all ages. More patients, however, need to be studied.     

Is congenital cystic adenomatoid malformation a premalignant lesion for pleuropulmonary blastoma?

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.     

Thoracoscopic versus open resection of congenital lung lesions: a case-matched study

Purpose

The purpose of the study was to compare the outcomes in children undergoing thoracoscopic versus open resection of congenital lung lesions.

Methods

Retrospective review of 12 consecutive children (<3 years of age) undergoing thoracoscopic resection of a congenital lung lesion between 2004 and 2005 was performed. Intraoperative and early postoperative results were compared with randomly selected age- and sex-matched (2:1) patients undergoing thoracotomy between 2000 and 2005.

Results

Twelve children underwent thoracoscopic resection and were compared with 24 that underwent thoracotomy. Seventy five percent of the lesions in both groups were congenital cystic adenomatoid malformations. There were no major intraoperative complications. Two thoracoscopic procedures were converted to a thoracotomy. Perioperative outcomes including operative time, length of stay, duration and volume of chest tube drainage, and dose and duration of intravenous opioids were similar for the procedures. However, children undergoing thoracoscopic procedures were less likely (odds ratio = 0.07) to have received adjunctive regional anesthesia. Overall morbidity was 33% thoracoscopic and 25% open (P = .70).

Conclusion

Thoracoscopic resection is a safe and feasible alternative to open resection of congenital lung lesions. Examination of long-term advantages of the thoracoscopic approach such as decreased risk of chest wall deformity and scoliosis and improved cosmesis will require longer follow-up.     

Long-term outcome in patients with prenatally diagnosed cystic lung disease: special reference to ventilation and perfusion scan in the affected lung

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.     

First decade's experience with thoracoscopic lobectomy in infants and children

Purpose

This study evaluates the safety and efficacy of thoracoscopic lobectomy in infants and children.

Methods

From January 1995 to March 2007, 97 patients underwent video-assisted thoracoscopic lobe resection. Ages ranged from 2 days to 18 years and weights from 2.8 to 78 kg. Preoperative diagnosis included sequestration/congenital adenomatoid malformation (65), severe bronchiectasis (21), congenital lobar emphysema (9), and malignancy (2).

Results

Of 97 procedures, 93 were completed thoracoscopically. Operative times ranged from 35 minutes to 210 minutes (average, 115 minutes). There were 19 upper, 11 middle, and 67 lower lobe resections. There were 3 intraoperative complications (3.1%) requiring conversion to an open thoracotomy. Chest tubes were left in 88 of 97 procedures for 1 to 3 days (average, 2.1 days). Hospital stay ranged from 1 to 12 days (average, 2.4 days).

Conclusions

Thoracoscopic lung resection is a safe and efficacious technique. It avoids the inherent morbidity of a major thoracotomy incision and is associated with the same decrease in postoperative pain, recovery, and hospital stay as seen in minimally invasive procedures.     

Large fetal congenital cystic adenomatoid malformations: growth trends and patient survival

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.     

Outcomes after postnatal resection of prenatally diagnosed asymptomatic cystic lung lesions

Background

Symptomatic congenital lung lesions require surgical resection, but the management of asymptomatic lung lesions is controversial. Some surgeons advocate observation because of concerns about potential operative morbidity and mortality, as well as a lack of long-term follow-up information. On the other hand, malignant degeneration, pneumonia, and pneumothorax are known consequences of cystic lung lesions. This study aims to assess the safety of resection for asymptomatic lung lesions that were diagnosed before birth.

Methods

A retrospective review of all patients with prenatally diagnosed lung lesions at Children's Hospital of Philadelphia (Philadelphia, Penn) was performed from 1996 to 2005. The perioperative course of patients who were asymptomatic was analyzed.

Results

One hundred five complete records of children with asymptomatic lesions were reviewed. Overall mortality was 0% and morbidity was 6.7% including 2.9% significant postoperative air leak and 3.8% transfusion requirement. Nine patients had a pathologic diagnosis that differed from preoperative radiological findings, and 9 patients had additional pathologic findings.

Conclusion

This series demonstrates that surgery can be performed safely on patients who were asymptomatic with congenital cystic adenomatoid malformation of the lung and other types of lung lesions with no mortality and minimal morbidity. The frequency of disparate pathologic diagnoses and the potential for development of malignancy and other complications support the argument for early resection.     

Evolving experience with video-assisted thoracic surgery in congenital cystic lung lesions in a British pediatric center

Background/Purpose

Video-assisted thoracic surgery (VATS) is increasingly used for the resection of congenital cystic lung lesions (CLLs). This study aimed to evaluate the efficacy of VATS and its outcome in both antenatally and postnatally detected CLLs.

Methods

Forty-six patients managed during 2000-2005 were studied. Demographics, investigations, operative details, and outcome data were collected and evaluated. Patients were divided into 3 groups for analysis.

Results

Antenatally diagnosed (groups I and II, n = 35): group I (20) had VATS at 20 months median (range, 16-35 months). Video-assisted thoracic surgery was successful in 14 of 20 (70%), notably in all cases of extralobar sequestrations and foregut duplication cysts. Inadequate vision/lung collapse and technical difficulties were the main reasons for conversion to open thoracotomy. Group II (n = 15) was considered unsuitable for VATS because of neonatal symptoms (6 congenital cystic adenomatoid malformations of the lung [CCAMs]) and/or large size/inexperience (5 CCAMs, 4 sequestrations) and had elective thoracotomy at 8 months median (range, 6 days-20 months).Postnatally diagnosed (group III, n = 11): 3 CCAMs, 6 duplications, and 2 sequestrations were diagnosed because of recurrent chest infection (8) or stridor (2), or incidentally (1) at 8 years median (range, 1.2-14 years). Video-assisted thoracic surgery was successful in 3 foregut duplications. A duplication and an intralobar sequestration were converted; open thoracotomy was performed in others because of previous recurrent pneumonic episodes.Postoperative pain and hospital stay were significantly less (P < .001) in successful VATS resection: median of 2 days (range, 1-7 days) compared with thoracotomy median of 6 days (range, 4-20 days).

Conclusions

Video-assisted thoracic surgery is a safe and effective option for asymptomatic congenital CLLs. It is anticipated that more successful CCAM resections using VATS will occur in the future as our technical ability improves.     

Thoracoscopic versus open resection for congenital cystic adenomatoid malformations of the lung

Purpose

This study evaluated the potential advantages of thoracoscopy compared to thoracotomy for resection of congenital cystic adenomatoid malformations (CCAM).

Methods

We conducted a retrospective chart review of consecutive cases of CCAM resection at University of California San Francisco Children's Hospital from January 1996 to December 2006.

Results

Thirty-six cases of postnatal CCAM resections were done over the past 10 years; 12 patients had thoracoscopic resections, whereas 24 patients had open resections. Patients in the thoracoscopic group had significantly longer operative time (mean difference of 61.3 minutes; 95% confidence interval [CI], 30.5-92.1) but shorter postoperative hospital stay (mean difference of 5.7 days; 95% CI, 0.9-10.4) and duration of tube thoracostomy (mean difference of 2.6 days; 95% CI, 0.7-4.5) and lower odds of postoperative complications (odds ratio of 9.0 × 10⁻⁴; 95% CI, 8.0 × 10⁻⁶-0.1). In the subgroup analysis of only asymptomatic patients, the thoracoscopy group still had a significantly shorter hospital stay (mean difference of 2.8 days; 95% CI, 0.7-4.8). There was also a pattern for reduced complications in the thoracoscopy group (OR, 0.13; 95% CI, 0.02-1.0; P = .05). The average hospital costs were similar in both groups. With a conversion rate of 33% (6/18), patients with a history of preoperative respiratory symptoms had a higher incidence of conversion than those who were asymptomatic (66.7% vs 0%, P = .005). These four patients had a history of pneumonia.

Conclusion

Minimally invasive resection of CCAM results in longer operative time but shorter hospital stay, potentially reduced complications, and no additional hospital costs. Thoracoscopic lobectomy in patients with a history of pneumonia is challenging and a risk factor for conversion to thoracotomy.     

Clinicopathologic assessment of prenatally diagnosed lung diseases

Purpose

To investigate the clinical features and pathologic diagnosis of prenatally diagnosed lung diseases.

Materials and Methods

The medical records of 28 fetuses with prenatally diagnosed lung diseases were reviewed with regard to perinatal courses, ultrasonographic measurement of the lesion volume ratio, and the histopathology of lung tissue.

Results

Of the 23 fetuses with a prenatal diagnosis of congenital cystic adenomatoid malformation (CCAM), 4 required an emergency lobectomy (1 prenatally and 3 postnatally), whereas the other 14 showed uneventful courses after birth. The pathologic diagnosis of the resected lungs included CCAM (n = 4), lobar emphysema (n = 2), intralobar sequestration (n = 2), and bronchial atresia (n = 1). The peak value of the lesion volume ratio was significantly higher in the CCAM cases that required urgent surgery than in the non-CCAM cases (2.29 ± 0.46 vs 1.20 ± 0.21, P < .001). The ratio decreased after the 25th to the 30th week to less than 1.0 in the 5 non-CCAM cases, but remained higher than 2.0 in the 4 CCAM cases. Prenatally diagnosed extralobar pulmonary sequestration caused massive pleural effusion requiring a fetal intervention in 4 of the 5 fetuses.

Conclusions

The prenatally diagnosed lung lesions include various kinds of pulmonary diseases. Regardless of the diseases, sequential ultrasonographic assessment may predict perinatal risks.     

Does the use of fentanyl in epidural solutions for postthoracotomy pain management in neonates affect surgical outcome?

Background/Purpose

Continuous epidural analgesia is routinely used to manage pain in infants undergoing resection of a congenital cystic adenomatoid malformation (CCAM) of the lung. Our aim was to determine if there is a difference in the length of stay (LOS), supplemental analgesic requirements, pain control, and the incidence of adverse respiratory events in infants receiving the 2 standard epidural solutions commonly used: bupivacaine 0.1% and bupivacaine 0.1% with fentanyl 2 to 5 μg/mL.

Methods

We retrospectively reviewed the charts of infants who received epidural infusions containing bupivacaine 0.1% (n = 18) and bupivacaine 0.1% with fentanyl 2 to 5 μg/mL (n = 10) after CCAM resection during a 12-month period. LOS, rescue opioid, and nonopioid analgesic use, incidence of respiratory depression, and pain scores were recorded.

Results

The LOS in patients receiving fentanyl in their epidural solution was 1 day longer than those receiving plain bupivacaine (median 4 vs 3 days, respectively). Nonopioid analgesic and rescue opioid use was greater in patients who did not have fentanyl in their epidural solutions. Pain ratings were not significantly different. The incidence of respiratory depression was greater in patients receiving epidural infusions containing fentanyl (50% vs 17%, respectively).

Conclusion

The addition of fentanyl to epidural infusions of bupivacaine in infants undergoing thoracotomy for resection of CCAM may prolong recovery and increase the incidence of adverse respiratory events without providing a significant analgesic benefit.     

Outcome predictors for infants with cystic lung disease

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.     

Congenital lung malformations--antenatal and postnatal evaluation and management.

We reviewed our institutional experience with pulmonary resection for congenital bronchopulmonary malformations and analysed the management and outcome of pregnancies with a prenatal diagnosis of congenital lung malformations. Between January 1993 and December 2003, 31 patients underwent evaluation and pulmonary resection for bronchopulmonary malformations. Common clinical presentations were respiratory distress (9), respiratory infections/pneumonias (22), and dyspnoea (9). Diagnostic modalities included chest radiography, CT scan (22), MRI scan (7), arteriography (1), and bronchoscopy (5). There were 13 congenital cystic adenomatoid malformations (CCAM), six pulmonary sequestrations, three bronchogenic cysts, and nine congenital lobar emphysemas (CLE). Fifteen patients who underwent resection were diagnosed by antenatal ultrasound. No foetus had hydrops or associated malformations. No pregnancy was terminated. There was no foetal demise. Regression of the sonographic appearance was observed in six cases. Amniotic puncture was required for hydramnios in three cases. Eight emergency resections were performed (CCAM 4; CLE 3; Bronchogenic cyst 1). Surgical procedures included 24 lobectomies, one right middle lobectomy with a wedge resection of the right lower lobe, one completion right lower lobectomy, four sequestrectomies, one mediastinal mass excision and one wedge resection for a bronchogenic cyst. There were no deaths. Postoperative complications included: persistent air leak (n=2; one requiring completion lobectomy) and pneumothorax (1). Persistent mild symptoms were present in five patients, at long-term follow-up. Congenital cystic adenomatoid malformation and congenital lobar emphysema were the commonest congenital anomalies. Congenital lung malformations are increasingly diagnosed antenatally, sometimes necessitating emergent surgical resection. The natural history is variable. All infants with a prenatal diagnosis require postnatal evaluation. Patients should be evaluated for associated disorders. The presence of mass effects is an indication for therapeutic decompression. The risk of pulmonary compression, infection and malignant degeneration makes resection imperative, even in asymptomatic patients. Lobectomy is the procedure of choice, is well tolerated, and leads to excellent outcomes.     

Pulmonary function after early vs late lobectomy during childhood: a preliminary study

Background

One proposed reason for the early resection of asymptomatic congenital cystic adenomatoid malformations is the theoretical benefit of optimizing compensatory lung growth during infancy and early childhood. Our aim was to determine if early lobectomy is associated with better long-term pulmonary function than lobectomy later in childhood.

Methods

A retrospective chart review of children undergoing pulmonary lobectomy for benign disease from 1990 to 2006 was performed. Those having surgery before and after 2 years of age were compared. Forced vital capacity (FVC) and forced expiratory volume in 1 second (FEV₁) were used as indicators of pulmonary growth, with FVC less than 80% predicted and FEV₁ less than 80% consistent with impaired pulmonary function.

Results

Of 115 patients identified, 14 had postoperative pulmonary function testing at a mean age of 10 years. Of these, 7 had lobectomy before and 7 had lobectomy after 2 years of age. There was no significant difference between groups in mean FVC (81.5 vs 83.3) or the number of children with FVC less than 80% predicted, nor was there a difference in mean FEV₁ (87.6 vs 82.9) or the number of children with FEV₁ less than 80%.

Conclusions

Age at the time of lobectomy did not influence FVC or FEV₁. These preliminary data suggest that early lobectomy does not confer an advantage to the child with respect to long-term pulmonary function. A prospective study is necessary to confirm or refute these findings in a larger group of children.     

Antenatally suspected congenital cystic adenomatoid malformation of the lung: postnatal investigation and timing of surgery

Background

We have previously reported the outcome of a cohort of cases over a 10-year period with antenatally suspected congenital cystic adenomatoid malformation (CCAM) and have recommended elective surgery within the first year of life for persistent, asymptomatic cases because of the risk of long-term complications. The aims of this study were to document optimal postnatal investigation and timing of surgery.

Methods

Cases were identified using the Oxford Congenital Anomaly Register, theatre records, and histopathology reports. Nineteen cases from 2003 onwards were reviewed with respect to postnatal imaging with chest x-ray and computed tomography scan and timing of surgery.

Results

Chest x-ray was poor at detecting CCAM with only 61% sensitivity. Computed tomography scan was 100% sensitive with no false-positive diagnoses.Of the 19 cases since 2003, 13 cases were asymptomatic and had elective surgery. Eight had surgery at 6 months of age or older, and of these, 4 (50%) had evidence of chronic inflammation/infection on histology including 1 case with multiple abscesses found at surgery and 1 case with multiple adhesions. Five cases had surgery at less than 6 months of age and only 1 of these, aged 5.5 months, had evidence of infection present at histology.

Conclusions

All cases of antenatally suspected CCAM should be followed up postnatally and investigated with computed tomography scan. Elective surgery in asymptomatic cases, at around 3 to 6 months of age, is recommended for persistent lesions because of the risk of long-term complications.     

Prenatal diagnosis and outcome of fetal lung masses

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.     

Prenatal steroids for microcystic congenital cystic adenomatoid malformations

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.     

Ex utero intrapartum treatment with placement on extracorporeal membrane oxygenation for fetal thoracic masses

Purpose

We describe our experience with fetuses diagnosed with life-threatening chest masses who were delivered by ex utero intrapartum treatment with placement on extracorporeal membrane oxygenation (EXIT-to-ECMO).

Methods

The first fetus presented with a cystic mediastinal mass and enlarging echogenic lungs. Bronchoscopic evaluation during ex utero intrapartum treatment (EXIT) revealed complete airway obstruction secondary to a carinal bronchogenic cyst. The second fetus presented with a massive left congenital cystic adenomatoid malformation. The EXIT procedure was performed because of significant mediastinal shift, severe compression of the normal lung parenchyma, and signs of fetal distress.

Results

In both cases, extracorporeal membrane oxygenation (ECMO) was initiated while on placental support. The fetuses were then delivered, and a definitive resection of their thoracic lesions was successfully performed. There were no major perioperative complications. Both children made expedient recoveries without significant cardiopulmonary sequelae.

Conclusion

To our knowledge, this is the first report describing the successful use of EXIT-to-ECMO as a bridge to definitive resection of large chest masses diagnosed in utero. EXIT-to-ECMO is a novel and effective management strategy for stabilizing patients with profound respiratory compromise secondary to congenital thoracic lesions.     

Characteristics of congenital cystic adenomatoid malformations associated with nonimmune hydrops and outcome

Background/Purpose

In fetuses with congenital cystic adenomatoid malformations of the lung (CCAMs), hydrops fetalis and large masses are associated with poor outcomes. This study attempts to (1) determine sonographic features (in addition to large size) that correlate with hydrops and (2) characterize the features that correlate with outcome among hydropic fetuses.

Method

Charts and sonograms of fetuses with large, unilateral CCAMs were retrospectively reviewed. Mass features evaluated included laterality, macrocystic/microcystic, cystic/solid predominance, degree of mediastinal shift, retrocardiac component, diaphragm eversion, polyhydramnios, and mass-thorax ratio (MTR). Features of hydrops included degree of ascites, scalp and integumentary edema, pleural/pericardial effusion, and placentomegaly.

Results

Thirty-six fetuses with large CCAMs were studied: 27 with and 9 without hydrops. Three sonographic features were significantly associated with hydrops: MTR of at least 0.56, cystic predominance of mass, and eversion of hemidiaphragm. Of 27 fetuses with hydrops, 10 (37%) demonstrated all 3 features compared with none in those without hydrops (P = .04). All 9 nonhydropic fetuses were expectantly managed, and 100% survived. In the hydropic group, none of the expectantly managed fetuses survived, and 10 (43%) of the 21 fetuses who underwent fetal intervention survived.

Conclusion

Three features of large CCAMs were significantly associated with hydrops: MTR, cystic predominance, and diaphragm eversion. Identification of these features will allow clinicians to accurately predict which fetuses may warrant closer follow-up and possible treatment.