Congenital cystic adenomatoid malformation of lung type 1

Congenital cystic adenomatoid malformation, a rare developmental, hamartomatous abnormality of the lung, usually is unilateral, is localized, and presents in early infancy. Delayed occurrence in older children and multilobar involvement are rare. We describe a case of congenital cystic adenomatoid malformation type 1 with multilobar involvement, associated emphysema, and coexistent tracheobronchopathia osteochondroplastica in an adolescent girl for whom the correct diagnosis was achieved only on histologic examination. The importance of an accurate diagnosis of this entity enables proper subtyping, management to minimize the risk of infections and malignancy, and exclusion of associated malformations.     

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.     

Bronchioloalveolar carcinoma in congenital cystic adenomatoid malformation of lung

Congenital cystic adenomatoid malformation (CCAM) of lung is a rare condition with the potential for malignant transformation. We report a patient who underwent lobectomy for a cystic lung lesion, which was found to be a type 1 CCAM associated with a mucinous bronchioloalveolar carcinoma. Retrospective review of a biopsy specimen from the same lobe excised during an ipsilateral empyema drainage 11 years previously showed similar foci of bronchioloalveolar carcinoma. The patient remains well 3 years after surgery. This case demonstrates the indolent nature of malignancies seen in association with type 1 CCAMs and also that complete excision, probably by lobectomy, is the treatment of choice to avoid recurrence.     

Residual congenital cystic adenomatoid malformation and thoracic scar deformation after fetal surgery: a case report

Fetal surgery is a treatment option for fetuses with congenital cystic adenomatoid malformation (CCAM) of the lung who develop hydrops before 32 weeks of gestation. We report on a fetus with CCAM and hydrops who underwent subtotal resection of a huge right, lower lobe CCAM at 20 weeks of gestation. Postnatally, the infant developed a thoracic scar deformation and was suspected to have residual CCAM. The residual CCAM was resected and the chest wall deformity was corrected at 3 1/2 years of age.     

Aberrant arterial supply in congenital cystic adenomatoid malformation of the lung

We report on a newborn girl with cystic adenomatoid malformation of the lungs supplied by an aberrant artery arising from the aorta at the level of the diaphragm. To the best of our knowledge, it is the first case diagnosed by prenatal ultrasound in which an arterial digital subtraction angiography (DSA) after birth showed the aberrant vessel.     

Congenital cystic adenomatoid malformation of the lung

Nine cases of congenital cystic adenomatoid malformation of the lung are described. One was stillborn: two presented in the newborn period (one of them surviving after a lobectomy), and the remaining six were older children all of whom survived after lobectomy. There have been only three cases previously reported in children outside the newborn period. The pathological, clinical, and radiological features are discussed and compared with previously reported cases.     

Limited pulmonary resections for congenital cystic adenomatoid malformation of the lung.

Congenital cystic adenomatoid malformations (CAMs) are rare lung lesions characterized by the presence of a multicystic mass of pulmonary tissue. To clarify the surgical management of CAM of the lung, we reviewed our institutional experience with 34 patients with histologically documented CAM. Symptoms developed in the first 2 days of life in 20 patients; 14 patients developed symptoms a median of 69 days (range, 22 days to 15 years) after birth. A diagnosis of CAM was confirmed at thoracotomy in all patients. Four patients were treated with a limited pulmonary resection for disease confined to one lobe. Seven patients underwent a composite resection involving a lobectomy plus limited resection of the second lobe. One patient underwent pneumonectomy for a severely malformed lung. The remainder of the patients were treated with anatomic lobectomy. The only recurrence was a patient who had a partial cystectomy performed at the initial operation. We conclude that in the appropriate malformation subtype, limited pulmonary reactions can preserve lung tissue and may prevent subsequent complications of CAM.     

Congenital cystic adenomatoid malformation of the lung

Twenty cases of cystic adenomatoid malformation of the lung were observed: 2 had died in utero; the diagnosis was made at birth in 13 infants of which 3 were premature. A Bochdalek's hernia had been diagnosed before birth in 2 cases by echo-tomography. The correct antenatal diagnosis had been made in 2 cases. Two infants had no symptoms, 3 were dyspneic, 8 were in respiratory distress and had to be intubated and ventilated. Two had a prune belly syndrome. Eight infants had a thoracotomy during their first week of life. Pulmonary resections concerning those 13 patients comprised 10 lower lobectomies, 1 of which was associated with a lingulectomy, and 2 upper lobectomies, 1 of which was associated with a middle lobe resection. Five patients were diagnosed and operated upon between 10 months and 8 years of age; 4 had recurrent bronchitis and 1 was diagnosed during the treatment of a gastroenteritis. They had 3 lower and 2 upper lobectomies. Recovery was uneventful in all patients except for 1 who was reoperated upon for intestinal obstruction. Antenatal diagnosis of cystic adenomatoid malformation should become standard. The malformation may be mistaken for a pulmonary sequestration or bronchogenic cyst. Differential diagnosis of a congenital hernia is important.     

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.     

先天性肺囊性腺瘤样畸形的诊断和治疗

目的 总结小儿先天性肺囊性腺瘤样畸形的诊断及手术治疗，提高对该病的认识。方法 对24例术前均摄胸部X线平片、CT并经手术、病理证实的先天性肺囊性腺瘤样畸形病儿的临床资料进行回顾性总结。其中男13例，女11例，平均年龄4．75岁，主要症状是呼吸道感染及呼吸困难，病史ld至8年。结果 影像学术前确诊率79．17％。24例行开胸术，证实病变均为单侧，侵犯1个肺叶22例，其中左叶15例、右叶7例；侵犯2个以上肺叶者2例。14例与支气管无交通，l例合并胸主动脉的异常血供，l例合并巨大实性肿物。20例行肺叶切除、3例行肺段切除、l例因胸膜肺母细胞瘤行全肺切除。全组无术后并发症。按Stocker分型：I型13例，Ⅱ型9例，IⅡ型2例。结论 影像学检查是诊断先天性肺囊性腺瘤样畸形的主要方法。对有症状者，手术切除病变侵犯的肺组织是治疗该病安全、有效的方法。     

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.     

Systematic review and meta-analysis of the postnatal management of congenital cystic lung lesions

Background

Antenatally detected asymptomatic congenital cystic lung lesions may be managed conservatively or by surgical resection. We undertook a systematic review and meta-analysis to quantify the risks of elective surgery, emergency surgery, and observation.

Methods

All series published between 1996 and 2008, where the postnatal management of congenital cystic lung lesions was described, were reviewed. A meta-analysis was performed to determine whether elective or emergency surgery was associated with a higher risk of adverse outcomes.

Results

There were 41 reports describing 1070 patients (of whom 79% were antenatally detected). Five hundred five neonates survived without surgery into infancy, of whom only 16 (3.2%) became symptomatic. For all ages, elective surgery was associated with significantly less complications than emergency surgery. The risk ratio was 2.8 (95% confidence interval, 1.4-5.5; P < .005) when comparing complications after elective surgery with emergency surgery.

Conclusions

The risk of asymptomatic cases developing symptoms is small. However, elective surgery is associated with a better outcome than emergency surgery. If elective surgery is undertaken, it should be performed before 10 months. Although no prognostic indicators have so far been identified in the literature, a conservative approach may be appropriate for small lesions.     

Neonatal transthoracic puncture in a case of congenital cystic adenomatoid malformation of the lung

A patient with congenital cystic adenomatoid malformation of the lung (CCAM) is presented who showed severe respiratory deterioration in the neonatal period caused by hyperinflation of the cystic component and compression of the contralateral lung. Transthoracic drainage of the cyst was performed to avoid preoperative artificial ventilation. On aspiration, air and liquid was removed from the cyst. After stabilization, surgical intervention was performed within 24 hours. Postoperative recovery was uneventful, and the child was discharged at day 13. J Pediatr Surg 37:1495-1497.     

Congenital cystic adenomatoid malformation of the lung in adults

Congenital cystic adenomatoid malformation of the lung presents mainly in neonates, is rare in children beyond infancy, and has not been reported in adults. Two adult males (aged 24 and 35) had congenital cystic adenomatoid malformation of the right and left lower lobes respectively. A third case, that of a 7-year-old girl, provided the link between the typical neonatal and these adult cases. All three lesions consisted of single or multiple macroscopic cysts, as well as a network of interconnecting spaces (the adenomatoid component). The lining varied from pseudostratified columnar ciliated, to simple mucinous and cuboidal epithelium. Abundant smooth muscle was present in two cases. Cartilage was absent in all three cases. The absence of inflammation is typical of the lesion in neonates. By contrast, all three of our patients had clinical and pathologic evidence of chronic inflammation. We postulate that congenital cystic adenomatoid malformation, when confined to a lobe or segment, may be clinically silent in infancy and may present as pneumonia associated with a cystic lesion on chest x-ray in childhood or later life.     

A case of congenital cystic adenomatoid malformation of the lung in a 4-year-old child

A case of congenital cystic adenomatoid malformation (CCAM) of the lung is reported. A 3-year-old boy was admitted for elevation of infiltrative shadow in the right lower lung field on a chest X-ray. The clinical diagnosis was emphysematous bullae with inflammation. After 6 months, he had recurrent infected bullae, and was admitted. He received chemotherapy with antibiotics prior to the operation. Right lower lobectomy was performed on February 2, 1998. The resected lung was composed of multiple cysts with thin wall measuring 10-60 mm in diameter. The histological examination of specimen revealed CCAM (Stocker type I), which is rare in this age. The patient has been well for 16 months postoperatively.     

A case of congenital cystic adenomatoid malformation of the lung in 18-year-old male]

A case of congenital cystic adenomatoid malformation (CCAM) of the lung is reported. The patient had recurrent pneumonia after the second day of delivery, and was admitted to our hospital, at the age of 18. He received right pneumonectomy under the diagnosis of CCAM. The resected lung was composed of multiple cysts with thin wall measuring 20-30 mm in diameter. Microscopic examination showed that the cyst wall was lined by ciliated columnar epithelium, and infiltration of chronic inflammatory cells was found in the submucosal layer. Bronchial cartilage was absent through the specimen. These microscopic findings are identical with those of CCAM, which is very rare in this age.     

Diagnosis of congenital cystic adenomatoid malformation of the lung in newborn infants and children.

Seventeen patients were diagnosed as having congenital cystic adenomatoid malformation of the lung during 1970-88. One case was associated with congenital diaphragmatic hernia and the child died before operation. The other 16 children underwent successful surgery. The patients presented in one of three ways--with neonatal respiratory impairment (12 cases) requiring urgent diagnosis and treatment, with recurrent respiratory tract infections (3), and with pneumothorax (1). Diagnostic problems arose with the neonatal presentation. In four of the children laparotomy was performed for presumed diaphragmatic hernia; three of these children had right sided lesions. Congenital diaphragmatic hernia was excluded by contrast studies in a further two patients. Histological examination confirmed the diagnosis in all cases. The postoperative course was uneventful in all 16 patients. No long term impairment of pulmonary function was noted.