Congenital Cystic Lung Malformation Research Articles

Mediastinal and retroperitoneal transdiaphragmatic bronchogenic cyst with gastric mucosa

Congenital cystic lung lesions are rare lung malformations. They are mostly diagnosed by a routine antenatal ultrasound scanning. Clinical symptoms ranging from asymptomatic condition to life-threatening course. We presented a case of congenital cystic lung malformation, the case demonstrated a 20- month- old boy who was reported to our hospital because he was diagnosed prenatally with a right-sided cystic lung mass. He was asymptomatic. A diagnosis of mediastinal retroperitoneal bronchogenic cyst was made radiologically. He underwent a successful right thoracotomy and the transdiaphragmatic cystic mass was completely resected. Histologic examination revealed bronchogenic cyst with gastric mucosa. Early diagnosis improves outcomes in infants with congenital cystic lung anomalies. Surgical management is the treatment of choice for congenital cystic lung malformations.

Type I regressed pleuropulmonary blastoma in a 10-year-old boy.

Pleuropulmonary blastoma (PPB) is a rare, malignant tumor of the lung and is the most common primary pulmonary malignancy in children. Here, we report a case of a boy who was diagnosed with type I regressed PPB after being mislabeled with congenital pulmonary malformation. A 10-year-old boy presented to our hospital with a history of worsening dyspnea. Since birth, his clinical status and radiographic images were concerning for congenital lobar emphysema that was managed conservatively. A chest computed tomography (CT) scan confirmed the persistence of a large cystic lesion and a diagnostic and therapeutic cystectomy was performed. Microscopic examination confirmed the presence of PPB type Ir. Patient was managed surgically alone with no added chemotherapy, as there was no overall survival benefit. PPB Ir has an overall favorable clinical outcome. Limited follow-up data are available due to the rarity of the lesion and the overlap with other congenital cystic lung malformations.

Врожденная кистозная мальформация легких у новорожденных

Врожденная кистозная мальформация легких у новорожденных

Congenital Cystic Lung Malformation Presenting in a Previously Asymptomatic Thirty-Year-Old Patient

This case is a rare presentation in an adult in his thirties of an uncommon disease normally detected in utero or shortly thereafter. It posed a diagnostic dilemma for our surgical and medical teams initially.

Pathology of asymptomatic, prenatally diagnosed cystic lung malformations

AimThe management of asymptomatic congenital cystic lung malformations is controversial. Arguments for excision of asymptomatic lesions are the potential for infection and malignancy. Following antenatal detection, our institute performs a CT at 1month, clinic follow-up by 3months to discuss the controversial management, and offers surgery by 6months of age. We investigated the histopathology of asymptomatic lesions to determine whether there was evidence of subclinical infection or malignancy. MethodsA retrospective review of prospectively collected antenatal congenital cystic lung malformations more than a 10year period (2005–2014) was conducted. Information was gathered from the antenatal registry and histopathology reports. Infection was defined by the presence of microabscesses or neutrophil/macrophage infiltration, as per histopathological criteria. Main resultsFrom the cohort of 99 patients, the study focused on 69 asymptomatic lesions. These cases comprised 34 congenital pulmonary airway malformations (CPAM), 15 pulmonary sequestrations (PS), and 20 hybrid lesions. Eighteen cases (26%) had microscopic disease — 16 cases of infection and 2 tumors. The infectious cases comprised 7 with microabscesses and 9 with neutrophil/macrophage infiltration. There were two cases of tumors, namely pleuropulmonary blastoma. These tumors were followed up by the oncology team with regular imaging until 3years of age and clinical review thereafter. ConclusionTwenty-six percent of antenatally detected, asymptomatic cystic lung malformations demonstrated either subclinical infection or malignancy. This information can be used for counseling parents and determining the method of treatment.

OC25.05: Percutaneous laser ablation under ultrasound guidance for fetal hyperechogenic microcystic lung lesions with hydrops

Objectives: To determine the perinatal outcome of fetuses with congenital cystic lung lesions complicated by hydrops, who underwent thoracoamniotic shunting. Methods: A retrospective study of 7 hydropic fetuses with prenatally diagnosed congenital cystic lung malformation (4) or bronchopulmonary sequestration (3), who underwent thoracoamniotic shunting at a single tertiary center. Results: The median gestational age at diagnosis was 23 weeks (15–29) and the median gestational age at shunt insertion was 25 weeks (19–31). In 6 cases, the hydrops resolved following shunt insertion, whereas in one fetus, who underwent shunting at 19 weeks, the hydrops persisted despite the shunting and the patient elected to terminate the pregnancy. All 6 fetuses whose hydrops resolved following shunting were born alive at a median gestational age of 36.5 weeks (33–38) resulting in perinatal survival of 86%. None of the patients delivered prior to 32 weeks of gestation. Conclusions: Hydropic fetuses with congenital cystic lung lesions can benefit from thoraco-amniotic shunting resulting in perinatal survival of 86%. Therefore, hydropic fetuses with macrocystic CCAM or BPS should be offered thoraco-amniotic shunting as the treatment of choice.

OC25.04: Should fetuses with congenital cystic lung lesions complicated by hydrops be treated by thoraco‐amniotic shunting?

Objectives: To determine the perinatal outcome of fetuses with congenital cystic lung lesions complicated by hydrops, who underwent thoracoamniotic shunting. Methods: A retrospective study of 7 hydropic fetuses with prenatally diagnosed congenital cystic lung malformation (4) or bronchopulmonary sequestration (3), who underwent thoracoamniotic shunting at a single tertiary center. Results: The median gestational age at diagnosis was 23 weeks (15–29) and the median gestational age at shunt insertion was 25 weeks (19–31). In 6 cases, the hydrops resolved following shunt insertion, whereas in one fetus, who underwent shunting at 19 weeks, the hydrops persisted despite the shunting and the patient elected to terminate the pregnancy. All 6 fetuses whose hydrops resolved following shunting were born alive at a median gestational age of 36.5 weeks (33–38) resulting in perinatal survival of 86%. None of the patients delivered prior to 32 weeks of gestation. Conclusions: Hydropic fetuses with congenital cystic lung lesions can benefit from thoraco-amniotic shunting resulting in perinatal survival of 86%. Therefore, hydropic fetuses with macrocystic CCAM or BPS should be offered thoraco-amniotic shunting as the treatment of choice.

Surgical Management of Congenital Cystic Lung Malformations in Older Patients

Congenital cystic malformations of the lung often go unrecognized in childhood. It is controversial whether surgical management in older patients requires more extensive lung resection. The aim of our study was to analyze the outcomes of surgical management in older patients. A retrospective review was made of all patients treated surgically for congenital cystic lung lesion between 1994 and 2009. One hundred two patients (65 women, 37 men) underwent surgery for a congenital cystic lung lesion. Median age was 47 years (range, 4 to 80 years). Surgical approach was through a thoracotomy in 70 patients, thoracoscopy in 21, sternotomy in 5, mediastinoscopy in 4, and laparoscopy and cervical in 1 each. Simple enucleation of the cyst was done in 67 patients, but 35 required more extensive pulmonary resection: lobectomy in 20, segmentectomy in 8, wedge in 6, and bilobectomy in 1. There was no significant difference between these groups with respect to age, sex, or postoperative outcomes. Univariate analysis revealed pneumonia, complex cysts, male sex, left-sided lesions, and bronchogenic cysts as predictors of a concomitant pulmonary resection being necessary during removal of the lesions. In a multivariable model, pneumonia and bronchogenic cysts persisted as predictors of the need for a more extensive pulmonary resection. A cystic malformation can be resected by simple resection, but in approximately one third of cases, a more extensive pulmonary resection is required. Patients with bronchogenic cysts who present with recurrent pneumonia are more likely to require a more extensive pulmonary resection.

Fetal Pulmonary Malformations: Defining Histopathology

Although classification schemes have sought to categorize congenital cystic lung malformations, studies including the pathology of pulmonary malformations occurring specifically during the fetal period are limited. To better characterize such histopathology, we reviewed a total of 23 fetal lung malformations seen at the Children's Hospital of Philadelphia from 1996 to 2004. Twenty-one of the 23 fetal pulmonary malformations could be categorized into 1 of 3 groups based upon the predominant histologic features present within each lesion. Group 1 (9/21) demonstrated tubular airspaces lined by columnar epithelium. Group 2 (6/21) contained airspaces lined by cuboidal epithelium and surrounded by smooth muscle with abundant interstitial mesenchyme. Group 3 (6/21) showed a mixture of relatively mature-appearing airspaces lined by flattened epithelium and scattered dilated bronchiole-like structures. Cysts were of variable size but in all cases showed a respiratory-type lining. Gestational ages ranged from 21 5/7 to 38 2/7 weeks. Patients in groups 1 and 2 were generally younger than those in group 3; however, morphology did not seem to correlate entirely with normal stages of fetal lung development, and group 2 lesions in particular were the least akin to normal fetal lung. In 4 cases a systemic vascular supply to a lobe of lung was identified, providing evidence that such vasculature is embryonic in origin. The histopathology of fetal lung malformations highlights the variability seen in such lesions at all ages, and it is hoped that continued investigations will provide further insight into these enigmatic lesions.

Konnatale zystische Lungenfehlbildungen

The aim of the study concerning congenital cystic lung malformations was to evaluate prenatal diagnoses postnatally to determine prognostic factors as well as to define optimized perinatal management. The study is based on 45 prenatal ultrasound examinations depicting fetal cystic lung lesions. 32 of the mothers had follow-up examinations. 5 pregnancies were terminated due to CCAM and additional malformations. Complete regression of the lesions was seen prenatally in 8 cases and postnatally in 5 children. Surgical intervention due to respiratory insufficiency was necessary in 4 neonates. According to the imaging results, CCAM was present in 4 cases and sequestration in 7 patients. No correlation between the imaging findings and the surgical results was found in 3 children: One child suffered from rhadomyoid dysplasia, and in the case of the second child, a left-sided hernia of the diaphragm and additional sequestration were detected. The third child showed AV malformation. The cystic lesions of the 14 children operated upon were proven histologically. The degree of accuracy in the present study was high. Precise perinatal management is warranted in order to determine according to the clinical relevance surgical intervention and to prevent complications after the first year of life. This is performed during the neonatal period for respiratory insufficient neonates and within the first year of life for clinically stable children.