Survival after CCAM associated with ascites: a report of a case and review of the literature

A congenital cystic adenomatoid malformation (CCAM) is a congenital lung lesion that, when associated with hydrops, is thought to have a dismal prognosis without fetal intervention. The authors report a case of a fetus with a CCAM associated with ascites as the only manifestation of hydrops, which was diagnosed at 19 weeks gestation. The CCAM regressed without intervention between 23 and 29 weeks of gestation with resolution of the ascites. The baby was born asymptomatic at term, and the infant underwent elective resection of the CCAM at 1 year of age. A review of the literature from 1980 to 2000 yielded 9 other cases of spontaneous resolution of hydrops associated with CCAM, suggesting that although rare, this outcome is a possibility in fetuses diagnosed with the condition. Because most fetuses diagnosed with hydrops undergo termination of pregnancy, the true incidence of hydrops resolution is potentially higher than predicted. This has important implications for prenatal counseling particularly in centers in which fetal intervention is not available.     

Anesthetic management for neonates with congenital cystic adenomatoid malformation

We experienced two cases of congenital cystic adenomatoid malformation (CCAM) diagnosed by prenatal ultrasonography. The first case with type I CCAM underwent the resection of the right upper lobe 8 days after birth. Postoperative course was uneventful. The second case with type III CCAM developed non-immune hydrops, severe mediastinal shift, and polyhydroamnios at 29 weeks' gestation. At 30 weeks' gestation, cesarean section was performed. Immediately after birth, the resection of lung tumor for the neonate was performed. However, the neonate died on the 1st postoperative day.     

Resolution of hydrops fetalis in congenital cystic adenomatoid malformation after prenatal steroid therapy

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.     

Fetal cystic adenomatoid malformation: prenatal diagnosis and natural history

We studied the natural history and pathophysiology of congenital cystic adenomatoid malformation (CCAM) detected prenatally by ultrasound in twelve fetuses. Two types of fetal CCAM can be distinguished by gross anatomy, ultrasound findings, and prognosis. Microcystic lesions are usually associated with fetal hydrops and have a poor prognosis (five cases with one survivor). Antenatal diagnosis, maternal transport, and immediate thoracotomy after birth allowed the first reported survival of a newborn with a large microcystic CCAM. Macrocystic lesions are not usually associated with hydrops and have a favorable prognosis (five of seven survived). We conclude that fetuses with hydrops are at high risk for fetal or neonatal demise without intervention. Fetuses with CCAM but without hydrops have a good chance for survival with maternal transport, planned delivery, and immediate neonatal resuscitation and surgery.     

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.     

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.     

Intraabdominal pulmonary sequestration.

A left upper quadrant fetal abdominal mass was detected at 24 weeks gestation. The mass was again confirmed in a postnatal ultrasound. Pathological analysis of the excised mass demonstrated an intraabdominal lung sequestration with Stocker type II congenital cystic adenomatoid malformation (CCAM). The sonographic characteristic of these lesions are those of a homogeneous echogenic mass with variable shape passing through or arising from the diaphragm. Surgical excision is recommended because of the uncertainty of the preoperative diagnosis and the possibility of malignant changes in CCAM.     

Congenital cystic adenomatoid malformation detected by prenatal sonography; report of 2 cases

We report 2 cases of congenital cystic adenomatoid malformation (CCAM) detected by prenatal sonography. The first CCAM was diagnosed by fetal sonography in a female fetus at 30 weeks' gestation. The infant was born at 37 weeks' gestation, with a body weight of 2,770 g. After birth, chest computed tomography (CT) showed a multicystic mass in the middle lobe of the lung. She remained asymptomatic until age 21 months, when she suffered pneumonia. Two months later, middle lobectomy was performed. The second CCAM was diagnosed by fetal sonography in a female fetus at 25 weeks' gestation. She was born at 39 weeks' gestation, with a body weight of 3,292 g. Four days after birth, CCAM type II was diagnosed by chest CT. The infant was asymptomatic, and left lower lobectomy was performed 11 months after birth.     

Fetal lung interstitial tumor: a cause of late gestation fetal hydrops

Most fetal lung masses present by mid gestation, grow during the canalicular phase of lung development (18-26 weeks of gestation), and plateau in growth or shrink after 26 weeks of gestation. We describe the unique case of a fetal lung mass presenting at 37 weeks of gestation with hydrops and fetal heart failure. The late growth of this lesion and resultant hydrops prompted resection as part of the ex utero intrapartum treatment. Histopathology revealed a rare, recently described fetal lung interstitial tumor. This case demonstrates that a subset of fetal lung masses may continue to grow later in gestation and emphasizes the need for late gestation imaging and close follow-up in this patient cohort.     

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.     

Importance of fetal fluid imbalance in congenital cystic adenomatoid malformation of the lung.

Congenital cystic adenomatoid malformation (CCAM) is a regional overgrowth of bronchioles with suppression of alveolar development in fetal and newborn lung. Twelve patients with CCAM were treated. Six premature infants had acute respiratory distress. Prenatal ultrasound was performed in only five patients and was abnormal in all five. Five premature neonates also had patent ductus arteriosus complicating their courses. Six older children presented with recurrent pneumonitis. Radiographs showed asymmetry of the chest and radiolucent masses in all 12 patients. Seven had type I lesions, two had type II lesions, and three had type III lesions. Hydramnios and hydrops were present in three, and hydrops alone was present in one of the six neonates. These four patients died. One other neonate died of respiratory failure and persistent fetal circulation. Seven patients survived for an extended period. Ultrasound makes the prenatal diagnosis of hydramnios and hydrops possible. It is in this group that fetal interventions can be considered. For the remaining patients, surgical intervention is indicated at the time of diagnosis.     

Persistent pulmonary hypertension complicating cystic adenomatoid malformation in neonates.

Neonates with congenital diaphragmatic hernia (CDH) are known to be susceptible to stress-induced persistent pulmonary hypertension (PPHN). Congenital cystic adenomatoid malformations (CCAMs) may also present as respiratory distress in the newborn. Intubation and mechanical ventilation cause clinical deterioration because of air trapping within cystic spaces; these patients require prompt lobectomy. PPHN has not been commonly associated with CCAM. Three patients with CCAM were encountered who developed PPHN postlobectomy. Three newborns, 36 to 38 weeks' gestation, presented with respiratory distress. Two had diagnosis of thoracic tumors on fetal ultrasound (22 and 33 weeks). Chest x-ray at birth confirmed cystic intrathoracic tumors in all and they underwent immediate thoracotomy and lobectomy (1 right upper, 1 left lower, 1 left upper). The patients were stable for 4 hours to 5 days postoperatively and then developed findings consistent with PPHN by cardiac echocardiography and required extracorporeal membrane oxygen (ECMO) support. ECMO was required for 66.5 to 120 hours. Each patient was successfully weaned to conventional ventilatory support. The clinical course of these patients was similar to those with CDH who undergo immediate surgery. The stress of surgical intervention combined with hypoxia and hypercarbia stimulates a hyperactive pulmonary vasculature and the development of PPHN. ECMO provides an effective adjunct to support patients with PPHN on the basis of congenital cystic adenomatoid malformations.     

A case of thoraco-amniotic shunt for congenital cystic adenomatoid malformation

Congenital cystic adenomatoid malformation (CCAM) is a congenital pulmonary anomaly, which may lead to fetal hydrops, pulmonary hypoplasia, and fetal or neonatal death. Recently, diagnosis and surgery for fetus have been improved. We experienced a case of CCAM, classified in Stocker class I, with a single cyst of about 5 cm in diameter. A 32 year-old pregnant woman had a fetus diagnosed as CCAM at 25 th gestational week. The thoraco-amniotic shunt placement using a modified double pig tail catheter was performed at 27 th gestational week under general anesthesia with 1% isoflurane in oxygen 2 l x min(-1) and nitrous oxide 4 l x min(-1), and 100 microg of fentanyl. Fetal movement was suppressed during surgery. This fetal therapy was effective and the cyst disappeared after surgery. The baby was delivered with caesarian section under spinal anesthesia with 0.5% bupivacaine 2.5 ml. On the next day, right lung lobectomy was accomplished under general anesthesia with fentanyl 0.2 mg and pancuronium 6.0 mg. Anesthesia with isoflurane, nitrous oxide, and fentanyl was useful for the fetal surgery of thoraco-amniotic shunt placement. Consequently, caesarian section and lung lobectomy were completed successfully.     

Postnatal spontaneous resolution of congenital cystic adenomatoid malformations

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.     

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.     

Cervical cystic hygroma in the fetus: clinical spectrum and outcome

Cervical cystic hygroma is thought by most pediatric surgeons to be an isolated, usually resectable lesion with an excellent prognosis. However, prenatal sonography has revealed a high "hidden mortality" among fetuses with this condition, and most perinatologists consider it to be uniformly fatal. In an attempt to resolve these two differing perspectives, we analyzed 29 cases seen at two centers over 4 years. Of 27 fetuses diagnosed before 30 weeks' gestation, only one survived. Twenty-five of the 27 were aborted; severe hydrops was present in 21 of these 25. Two of the 27, both with stigmata of Noonan's syndrome, underwent spontaneous regression during the second trimester: one died at 2 weeks of age, and the other survived. Successful karyotypes were obtained on 17 fetuses: nine were normal, seven were 45X, and one was trisomy 21. Fetuses with abnormal karyotypes had a lower incidence of polyhydramnios (0% v 67%), additional anomalies (12% v 67%), and consanguinity or a history of abnormal pregnancies (0% v 89%). Two fetuses were diagnosed after 30 weeks' gestation. Neither had hydrops, polyhydramnios, associated anomalies, or an abnormal karyotype. One had a completely normal sonogram at 17 weeks' gestation. Both were operated on within the first 4 days of life; one did well without complications, and the other required a permanent tracheostomy because of extensive hypopharyngeal involvement. A cystic hygroma presenting in the fetus has a different natural history and prognosis from one presenting postnatally. The vast majority of fetal cases are diagnosed before 30 weeks' gestation, and present with hydrops or diffuse lymphangiomatosis. The dismal outlook in this group justifies elective termination in most cases.(ABSTRACT TRUNCATED AT 250 WORDS)     

Thoracoscopic treatment of a neonatal traumatic pneumatocele

Traumatic pneumatoceles appear rarely after pulmonary parenchymal or bronchial disruption. Treatment is usually expectant with intervention reserved for complications, such as infection, expansion, or cardiopulmonary deterioration. A 17-day-old female was transferred to our NICU with a left-sided pneumatocele resulting from an intraparenchymal chest tube insertion. She was born at 30 weeks gestation and required a chest tube insertion for severe hydrops fetalis with respiratory failure and associated chylothoraces. An acute deterioration following several weeks of clinical improvement was unsuccessfully treated with radiologically guided drainage. In this paper, we describe the thoracoscopic management of this case.     

Management of fetal mediastinal teratoma

Background/Purpose

Mediastinal teratomas are rare congenital germ cell tumors that prenatally can compress mediastinal structures and cause hydrops. Two possible presentations of massive fetal mediastinal teratoma include hydrops leading to fetal demise, or fetal esophageal and airway compression causing late-gestation polyhydramnios and preterm labor. The authors present 2 cases of fetal mediastinal teratoma that illustrate successful strategies for either of these presentations.

Methods

A 37-year-old woman carrying a fetus with a mediastinal mass and secondary hydrops at 23 weeks of gestation underwent in utero resection of the mass. Delivery was by cesarean delivery at 25 weeks because of preterm labor. A 24-year-old mother carrying a fetus with a mediastinal mass and severe polyhydramnios at 36 weeks of gestation underwent an ex utero intrapartum therapy procedure for establishment of an airway and tumor resection on uteroplacental support.

Results

These strategies resulted in physiologic improvement in the first case and controlled resection and resuscitation in the second. The first patient had significant sequelae of prematurity including bronchopulmonary dysplasia but is currently well at 9 months of age. The second patient is well at 1 year of age.

Conclusions

Massive fetal mediastinal teratoma can result in fetal or neonatal mortality by a variety of mechanisms. Optimal prenatal and perinatal management is required to salvage fetuses compromised by this lesion.     

The use of cardiopulmonary bypass and extracorporeal membrane oxygenation for support during removal of two teratomas and hydrops fetalis

The removal of massive tissue tumors often leads to rapid blood loss and decreased lung compliance because of large volume shifts. Cardiopulmonary bypass (CPB) and extracorporeal membrane oxygenation (ECMO) have both been used as a means of support during resection of saccrococcygeal teratomas. Hydrops fetalis is the accumulation of fluid in extravascular spaces and body cavities. This leads to edema and sometimes hypoxia. ECMO has been used for support during treatment of hydrops fetalis. This patient was diagnosed, via sonogram, at 30 weeks gestation to have two teratomas and hydrops fetalis. Because of the risk of hemorrhage and poor lung compliance during removal of these types of tumors, CPB and ECMO were used. This support allowed for successful removal of the tumors. Following removal of the tumors, the patient failed to wean from CPB because of the severity of hydrops. The patient remained on extracorporeal support for treatment of hydrops fetalis. This report describes the perfusion techniques used for support during the removal of teratomas and the treatment of hydrops fetalis.