Morbidity after thoracoscopic resection of congenital pulmonary airway malformations (CPAM): single center experience over a decade.

PURPOSE: Video-assisted thoracoscopic (VATS) resection of CPAM in children is an established, albeit controversial strategy for its management. We report a 10-year single center experience. METHODS: All children underwent VATS (2008-2017) and their current status was reviewed. Patients were grouped: 'symptomatic-P' (if parents reported recurrent lower respiratory tract infections etc.) or 'symptomatic-S' (neonates presenting with respiratory distress/difficulty) or 'asymptomatic'. RESULTS: 73 children, aged 10 m (4d-14yrs) underwent VATS; a neonate as an emergency ('symptomatic-S') and all others electively. The lesion was unilateral in all but one case. Histologically none were malignant. Of the elective 72 cases, 7 (10%) required conversion to open thoracotomy. Twenty (27.7%) were 'symptomatic-P' and the duration of surgery when compared to 'asymptomatic' children was longer 269 (range 129-689) versus 178 (range 69-575) minutes (P = 0.01). Post operatively, 8 children (11%) had a grade III/IV (Clavien-Dindo) complication; persistent air leak/pneumothorax (n = 5), chylothorax (n = 1), pleural effusion (n = 1) and seizure/middle cerebral artery thrombosis (n = 1). There was no mortality. Twenty-four children (33.3%) were reported 'symptomatic-P' post-surgery after a median follow up of 2.18 years. The surgical intervention had no impact on 'symptomatic-P' status (P = 0.46). CONCLUSION: The risks of surgery may outweigh benefit in asymptomatic children. CLINICALTRIALS. GOV IDENTIFIER: NCT04449614.

Long-Term Neurodevelopmental and Respiratory Outcome after Intrauterine Therapy for Fetal Thoracic Abnormalities.

INTRODUCTION: The aim of this study is to evaluate long-term neurodevelopmental and respiratory outcome after fetal therapy for fetal pleural effusion, congenital cystic adenomatoid malformation, and bronchopulmonary sequestration. METHODS: Children ≥18 months of age underwent an assessment of neurologic, motor, and cognitive development. Medical records were reviewed to determine respiratory outcome. Behavioral outcome was assessed using the Child Behavioral Checklist. RESULTS: Between 2001 and 2016, 63 fetuses with fetal hydrops secondary to thoracic abnormalities were treated at our center. Overall perinatal survival was 64% (40/63). Twenty-six children were included for follow-up (median age 55 months). Severe neurodevelopmental impairment (NDI) was detected in 15% (4/26). Three out of 4 children with severe NDI had associated causes contributing to the impairment. Overall adverse outcome, including perinatal mortality or NDI, was 55% (27/49). Fifteen percent (4/26) had severe respiratory sequelae. Parents did not report more behavioral problems than Dutch norms. DISCUSSION: Our results suggest that severe NDI in this specific high-risk cohort occurs in 15%, which is above the range of the incidence of NDI reported in case series treated with other fetal therapies (5-10%). Large multicenter studies and an international web-based registry are warranted to prospectively gather outcome data at fixed time points.

Effect of single and multiple courses of maternal betamethasone on prenatal congenital lung lesion growth and fetal survival.

PURPOSE: Administration of maternal betamethasone (BMZ) is a therapeutic option for fetuses with large microcystic congenital lung lesions at risk for, or causing, hydrops. Not all fetuses respond to a single course of BMZ. We review our experience with the use of single and multiple courses of maternal BMZ for the management of these patients. METHODS: A retrospective review of fetuses with congenital lung lesions managed with maternal BMZ from 2003 to 2014 was performed. RESULTS: Forty-three patients were managed with prenatal steroids (28 single course, 15 multiple courses). Single course recipients demonstrated a reduction in lesion size and resolution of hydrops in 82% and 88% of patients respectively compared to 47% and 56% in recipients of multiple steroid courses. Survival of multiple course patients (86%) was comparable to that of single course patients (93%) and improved compared to non-treated historical controls. Multiple course recipients demonstrated an increased need for open fetal surgery and postnatal surgery at a younger age. CONCLUSION: Fetuses who fail to respond to a single course of BMZ may benefit, as indicated by hydrops resolution and improved survival, from additional courses. However, failure to respond is indicative of a lesion which may require fetal or immediate neonatal resection.

Clinical Features of Congenital Cystic Lung Diseases: A Report on a Nationwide Multicenter Study in Japan.

AIM: The current study aimed to assess the perinatal risk of congenital cystic lung diseases (CCLD) and to establish a suitable surgical strategy in consideration of postoperative lung growth and problems during adulthood. METHODS: Among 874 CCLD patients identified in a nationwide survey, 428 patients were born between 1992 and 2012 and treated at 10 high-volume centers were retrospectively reviewed with statistical analysis. RESULTS: In the 194 patients who were prenatally diagnosed to have CCLD, 16.7% presented with fetal hydrops as observed using ultrasonography. The Apgar score (5 minutes) was lower than 5 in 5.4%. As of postnatal day 30, 14.0% of the neonatal patients required respiratory support, and 3.3% (8/243) had died because of pulmonary hypoplasia. Among those who were asymptomatic immediately after birth, 33.6% of the patients developed the respiratory infectious symptoms during their first year of life, and 22.1% did so between the age of 1 and 2 years. The postoperative percent vital capacity among the prenatally diagnosed patients was significantly higher than that among the postnatally diagnosed patients (98.3 ± 11.9 vs. 81.7 ± 9.7, p < 0.0222). Late complications included thoracic deformity in 30 patients and persistent lung cyst in 4 patients, whereas malignancy was not observed in the present series. CONCLUSIONS: Approximately 10 to 15% of prenatally diagnosed CCLD patients may carry a high risk of perinatal respiratory distress. Early operation before developing episodes of lung infection, seem to be associated with a better development of the reserved lung during later life. The incidence of carcinogenesis among patients with CCLD may be extremely low.

Prenatal imaging and postnatal presentation, diagnosis and management of congenital lung malformations.

PURPOSE OF REVIEW: Congenital lung malformations (CLMs) vary in their clinical presentation and severity. Increases in prenatal diagnosis, observed regression of certain lesions, and prognostic uncertainty are driving an evolution in management. RECENT FINDINGS: There has been an increase in the early diagnosis of these malformations, a change that is attributable to the routine use of prenatal ultrasound. Although prenatal diagnosis of CLMs using ultrasound and MRI has increased, chest radiography and computed tomography still play important roles in diagnosis. The management of these lesions depends on the type of malformation and symptoms. The treatment of asymptomatic patients with lung malformations is controversial, because the prognosis of these lesions is largely unknown. Proponents of early intervention argue that the complications of CLM, which may include infection, pneumothorax, bleeding and malignant transformation, justify surgery. Advocates of conservative management note that some CLMs disappear postnatally, and that the long-term complication rate following surgery is unknown. There is a need to obtain natural history data regardless of the therapeutic recommendations. SUMMARY: This article reviews the prenatal radiographic features and postnatal clinical findings of various CLMs and the dilemmas regarding treatment.

Thoracoscopy in the management of congenital lung diseases in infancy.

BACKGROUND: This study aimed to compare the results of thoracoscopic surgery for congenital lung diseases between infants younger than 6 months and those older than 6 months at the time of surgery in terms of operation duration, surgical complications, chest tube duration, and hospital stay. METHODS: The charts of 30 thoracoscopic resections for congenital lung diseases were retrospectively reviewed. This study compared 17 children younger than 6 months (mean, 3.94 months; range, 0.37-5.7 months; group 1) with 13 children older than 6 months (mean, 12.05 months; range, 6.2-24.63 months; group 2) at the time of surgery. The median follow-up period was 9 months (range, 1-41 months). RESULTS: Lobectomy was performed in 27 cases, bilobectomy in 1 case, and nonanatomic excision in 2 cases. The mean operating time for group 1 (176±54 min) was similar to that for group 2 (160±46 min). The difference is not significant. The mean duration of chest tube drainage was similar in the two groups (4.4 days; range, 1-9 days for group 1 vs. 4.1 days; range, 3-8 days for group 2). The complications included 1 major and 10 minor complications, with no statistically significant difference between the two groups. Three surgical procedures in each group were converted. The hospital stay was not statistically different between the two groups (8 days; range, 3-20 days for group 1 vs. 6 days; range, 4-10 days for group 2). CONCLUSIONS: The study findings showed no statistically significant difference between the two groups in terms of operation time, complication rate, conversion rate, or hospital stay. Lobectomy can be safely and successfully performed by thoracoscopy even for children younger than 6 months.

Congenital cystic adenomatoid malformation: monitoring the antenatal and short-term neonatal outcome.

OBJECTIVE: To determine the antenatal and short-term neonatal outcome of antenatally detected congenital cystic adenomatoid malformation (CCAM). METHODS: A retrospective review was conducted on all women with an antenatal diagnosis of CCAM who attended the Royal Women's Hospital, Melbourne, between January 1995 and December 2005. RESULTS: An antenatal diagnosis of CCAM was made in 38 singleton pregnancies. Serial ultrasounds were performed in 34 cases. Thirteen lesions (38%) appeared to resolve, ten lesions (29%) appeared to decrease, four lesions (12%) appeared to remain unchanged and seven lesions (21%) appeared to increase in size. Four pregnancies (10.5%) were complicated by hydrops fetalis which was associated with a poor outcome. Thirty-seven babies were liveborn. Seven babies (18.9%) developed respiratory distress. Two of these babies died within two days of birth resulting in three deaths in total. Two babies were lost to follow up (n=33). Sixty per cent of babies in whom the CCAM appeared to resolve on antenatal ultrasound had an abnormal chest X-ray (CXR). All computed tomography (CT) scans (eight of eight) in this group were abnormal. Of the surviving babies, 27.3% (nine of 33) have had surgery to date. CONCLUSIONS: The antenatal diagnosis of CCAM is associated with a good short-term prognosis. The pregnancy should initially be managed at a tertiary centre with serial ultrasound. Asymptomatic babies should have a postnatal CT even if the CCAM appears to have resolved or decreased on antenatal ultrasound.

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 x 10(-4); 95% CI, 8.0 x 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.

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.

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.

Outcome of the prenatally diagnosed congenital cystic adenomatoid lung malformation: a Canadian experience.

UNLABELLED: Congenital cystic adenomatoid malformation of the lung (CCAM) is diagnosed by prenatal ultrasonography with an increasing frequency but controversy persists as to its prognosis and prenatal management. METHOD: A multi-institutional study of cases of CCAM diagnosed antenatally identified by ultrasonographers and by a review of hospital charts. RESULTS: We obtained 48 cases from five centers. We estimate the incidence of CCAM at 1:25,000 to 1:35,000 pregnancies. The incidence of voluntary abortions was 15% (7/48), of spontaneous abortions 2% (1/41) and of postnatal death 10% (4/40). One of the postnatal deaths was from trisomy 18. Of the 7 aborted fetuses, 2 had multiple malformations and 1 had severe hydrops and oligohydramnios; the other 4 had a large mass with mediastinal displacement but without hydrops. When pregnancy was allowed to continue, 56% of the lesions regressed spontaneously, even though one third of these had initial progression. In 17 cases (42%) the mediastinal shift corrected itself, sometimes by simple growth of the fetus but most often by a decrease in the size of the lung mass. In 1 fetus, repeated needle decompressions followed by double-pigtail catheter drainage of large cysts allowed regression of hydrops. Despite this, neonatal death occurred from pulmonary hypoplasia. CONCLUSION: CCAM can lead to fetal or neonatal demise from hydrops, lung hypoplasia, prematurity or severe associated malformations, but has a good prognosis in the majority of cases.

Prenatal ultrasound diagnosis of congenital cystic adenomatoid malformation of the lung: a report of 26 cases and review of the literature.

OBJECTIVES: To evaluate the sonographic appearances and prenatal natural history of congenital cystic adenomatoid malformation of the lung. METHODS: In each case a detailed examination of the thoracic lesion and a complete fetal survey was performed. The pregnancies that elected to continue were followed to term. RESULTS: A total of 26 cases were identified. The pregnancy was electively terminated in nine cases (35%). All the remaining 17 pregnancies ended in liveborn infants (100%). The lesion disappeared completely in three fetuses (18%). Of the 14 infants in whom the lesion was confirmed at birth nine required surgery in the neonatal or post-natal period. Five children did not undergo surgery. CONCLUSIONS: Conservative management appears to be an adequate medical practice in cases of isolated congenital unilateral cystic adenomatoid malformation of the lung, in the absence of hydrops and/or acute polyhydramnios.

Prognostic factors associated with congenital cystic adenomatoid malformation of the lung.

This study presents 18 cases of prenatally diagnosed congenital cystic adenomatoid malformation (CCAM) to identify potential factors that could predict prognosis. Comparisons of prenatal parameters were made between fetuses that survived and those that died perinatally. It was found that microcystic lesion, bilateral lung involvement and hydrops were each highly correlated with poor prognosis, while neither polyhydramnios nor mediastinal shift was significantly associated with had outcome. Fetal interventions were indicated only in two of the surviving cases: a thoracocentesis and a cysto-amniotic shunt. A therapeutic amniocentesis was performed in one case of polyhydramnios. The diagnosis of CCAM was histologically confirmed in all cases by necropsy or by postnatal lobectomy.

Congenital cystic adenomatoid malformation in the fetus: natural history and predictors of outcome.

Cystic adenomatoid malformation (CCAM) is a rare lesion that often is diagnosed by prenatal ultrasonography. Outcome varies from hydrops and fetal death to resolution before birth. The authors reviewed their 7-year experience with 17 fetuses diagnosed with CCAM by prenatal ultrasonography to determine the natural history of the lesion and to identify factors that might predict outcome. Of the 17 fetuses, five died during intrauterine life (3 terminations, 2 fetal deaths); four of them had hydrops. Twelve fetuses were carried to term. Only one had prenatal intervention: a thoracoamniotic shunt at 24 weeks. All 12 infants survived and underwent resection. Only four required neonatal support (1 extracorporeal membrane oxygenation, 2 ventilator, 1 oxygen). The initial CCAM:chest ratio, degree of mediastinal shift at time of diagnosis, location of CCAM, and age at time of diagnosis did not correlate with outcome. Sonographically predicted pathological type did not correlate with pathological diagnosis after surgery, or with outcome. The only accurate predictors of outcome were presence of hydrops (all died) and decrease in size of CCAM during gestation (all survived). The outcome for fetuses with CCAM may be better than previously recognized. Many of the lesions decrease in size, despite significant mediastinal shift and lung compression at the time of diagnosis. Fetal intervention should be considered only for fetuses with hydrops. Others should be monitored with serial ultrasonography. Parental counseling, especially regarding pregnancy termination, should reflect the positive outcome noted in most cases.

Congenital cystic adenomatoid malformation of the lung: prenatal management and prognosis.

Thirty-two cases of congenital cystic adenomatoid malformation of the lung diagnosed antenatally are reported. Antenatal diagnosis has made it possible to document the progress of the condition in utero and the postnatal prognosis. It has been possible to advise on termination and intrauterine intervention, to counsel the parents, and to plan arrangements for delivery and postnatal care among obstetricians, neonatologists, and pediatric surgeons. According to Stocker's classification there were 12 cases of type I, 15 of type II, and 5 of type III. Five pregnancies were terminated. Antenatal drainage of a cyst was performed in four patients with two survivors. Thirteen babies showed relative regression of the cyst as pregnancy progressed. After delivery the extent of the cystic malformation was assessed by chest x-rays and computed tomography scanning in 25 and angiography in 6. Treatment consisted of observation in 4, embolization in 2, operation as an emergency in 3, and electively around 4 months in 15.

Fetal thoracic lesions.

Prenatal detection and serial sonographic study of fetuses with lung lesions now make it possible to define the natural history of these lesions, determine the pathophysiologic features that affect clinical outcome, and formulate management based on prognosis. The prenatal diagnostic hallmarks and natural history of congenital cystic adenomatoid malformation of the lung and pulmonary sequestration are presented. The finding that fetuses with hydrops are at very high risk for fetal or neonatal death led to successful fetal surgical resection of the massively enlarged pulmonary lobe (fetal lobectomy) in several cases. The fetus with a lung mass but without hydrops has a good chance for survival with maternal transport, planned delivery, and immediate neonatal resuscitation and surgery. Large fetal lung tumors may partially disappear on serial prenatal sonography, suggesting that improvement can occasionally occur during fetal life.

Prenatal diagnosis of congenital cystic adenomatoid lung malformation: a report of seven cases.

Six cases of macrocystic and one case of microcystic congenital adenomatoid lung malformation were diagnosed by ultrasound between 20 and 31 weeks of gestation. Combined polyhydramnios and fetal hydrops was present in three cases, polyhydramnios alone in one case, and isolated fetal hydrops also in one case. In the remaining two cases, both polyhydramnios and fetal hydrops were absent. Fetal outcome was poor, i.e., two terminations of pregnancy, three early neonatal deaths, and two survivors.

[Congenital cystic adenomatoid malformation of the lung. A report of 12 cases]. / Malformación adenomatoidea quística pulmonar congénita. Presentación de 12 casos.

The congenital cystic adenomatoid malformation of the lung is uncommon. In the Hospital Infantil de México Federico Gómez, 12 cases have been seen over a period of 31 years, which points out how rare this malformation is. There was a male predominance, and 75% of the patients had symptoms by 8 months of age. The main complaints were progressive respiratory failure in the newborn period and recurrent lung infection in older children. Treatment was surgical in all cases. There was only one fatality due to cardiac failure.