The ten-year evaluation of clinical characteristics in congenital lung anomaly in pediatrics; a retrospective study in North of Iran.

INTRODUCTION: Congenital lung anomalies (CLA) are a group of anomalies, including congenital cystic adenomatoid malformation (CCAM), bronchopulmonary sequestrations (BPS), congenital lobar emphysema (CLE), and bronchogenic cysts (BC). The prevalence of these rare anomalies has risen in recent years, according to various population-based studies due to advances in fetal ultrasound technology. METHOD: This retrospective study examines the diagnosis of CLA, and was conducted on 72 patients between March 2014 and March 2024 at Taleghani Pediatric Hospital in Gorgan, Iran. RESULT: The average age was 18.8 ± 30.3 months, with the majority being boys (62.5%). Most participants had CCAM (41.7%), followed by CLE (18.1%), BPS (16.7%), pulmonary hypoplasia (9.7%), BC (8.3%), and hybrid lesion (5.6%). The majority of patients were Fars (62.5%), and the average hospitalization days was 9.4 ± 4.5 days. Cardiac anomalies were observed in 19.4% of the patients. 62 patients (86.1%) exhibited respiratory symptoms, and prenatal screening during pregnancy led to the diagnosis in 51 patients (70.8%). Most patients had left lung anomalies (43; 59.7%), and the majority (90.3%) survived. There is a statistically significant relation between needed for surgical treatment and patients' type of pulmonary lesions (p-value: 0.02). In addition, there was a significant relation between the Fars ethnicity and the presence of cardiac anomalies (p-value: 0.04). CONCLUSION: Some CLAs remain undiagnosed or untreated due to the rare nature of congenital lung anomalies. Nevertheless, improvements in ultrasound and other imaging methods will make diagnosing and managing these anomalies during the prenatal period more prevalent, resulting in enhanced understanding.

Complications of Convex-Probe Endobronchial Ultrasound-Guided Transbronchial Needle Aspiration: A Multi-Center Retrospective Study.

BACKGROUND: Infrequent serious complications of convex-probe endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) have been reported. The aim of this study was to assess serious complications related to convex-probe EBUS-TBNA and to determine the complication rate in a large group of subjects. METHODS: In this retrospective study, a 15-item questionnaire on features of cases with EBUS-TBNA complications was sent to experienced bronchoscopists performing convex-probe EBUS-TBNA at 3 pulmonary centers. The medical records were then reviewed by these bronchoscopists to complete the questionnaire. Hemorrhage responsive to topical treatment, temporary laryngospasm/bronchospasm, transient oxygen desaturation, and fever lasting <24 h were excluded. Only complications requiring further treatment/intervention were considered serious. The rate of serious complications was calculated from the obtained data. RESULTS: In a total of 3,123 cases within a 5-y period, EBUS-TBNA was performed for staging lung cancer in 15.8%, diagnosis in 67.5%, and diagnosis and staging in 16.3%. Of the 3,123, 11.6% had parenchymal lesions adjacent to major airways. EBUS-TBNA was performed 11,753 times (3.76/case) at 6,115 lymph node stations and lesions (1.92/station or lesion). Five serious complications were recorded (0.16%): fever lasting >24 h, infection of bronchogenic cyst, mediastinal abscess, pericarditis, and pneumomediastinitis with empyema, each in one case. Four complications occurred in cases diagnosed with benign disease by EBUS-TBNA. All complications were treated with broad-spectrum antibiotics. Four subjects were hospitalized for 21.7 ± 20.7 d. CONCLUSIONS: Convex-probe EBUS-TBNA is a safe method in general. However, serious complications, including infections, can be encountered rarely. All precautions should be taken for complications before and during the procedure.

Differences in the distribution and presentation of bronchogenic cysts between adults and children.

BACKGROUND: Bronchogenic cysts are rare congenital malformations that occur in adults and children, with differences in distribution and presentation. METHODS: The study population comprised 16 infants and children (aged 7 days to 18 years) and 23 adults (aged 20-78 years) who received pathological diagnoses of bronchogenic cysts over a 14-year period (1999-2012). Cyst distribution and presentation were reviewed. RESULTS: Half (8/16) of the infants and children presented with palpable masses in the neck (n=6) or on the skin (n=2), and only one (12.5%) presented with symptoms of mild stridor. Another eight pediatric patients had mediastinal (n=7) or pulmonary (n=1) bronchogenic cysts, and respiratory symptoms were present in six (75%) patients. Thirteen of 23 (56.5%) adult patients had asymptomatic cysts (neck, n=1; mediastinum, n=11; lung, n=1). Symptomatic presentations occurred in 10/23 (43.5%) patients, including 2 with palpable mass in the neck, 3 in the mediastinum, 4 in the lung, and 1 in the retroperitoneum. Among the 13 asymptomatic patients, 6 were identified during regular health screening, 5 during routine chest computed tomographic surveys for cancer, and 2 incidentally found during thyroid and parathyroid surgery. Bronchogenic cysts tended to be larger in symptomatic than in asymptomatic adults. CONCLUSION: The clinical spectra of bronchogenic cysts differ between adults and children and are closely related to cyst location and, probably, size.

[Benign cysts of the mediastinum: series of 28 cases]. / Les kystes bénins du médiastin : à propos de 28 cas.

INTRODUCTION: Benign cystic mediastinal mass form a group of heterogeneous and uncommon lesions. Surgical resection is the gold standard in these conditions. We reported our institutional experience in management of these benign tumours. In this review, cardiac and ganglionary benign mass were excluded. METHODS: We retrospectively reviewed the records of 28 patients with benign cysts of the mediastinum, who are operated in our department between January 2003 and December 2009 (7years period). RESULTS: There were 13 females (46.4%) and 15 males (53.5%), with a mean age of 36.8years (range: 13-63years). Most lesions (n=22) were equally in the anterior and middle mediastinum, only six were in the posterior mediastinum. Seventeen patients (60.7%) were symptomatic, with chest pain and cough as the most common symptoms. The diagnosis of mediastinal cyst was fortuitous in 11 patients with the waning of an assessment made for another reason. Surgery was indicated in all patients in order to both diagnostic and therapeutic. Posterolateral thoracotomy incision is the most common. Complete resection of the cyst was possible only in 18 cases (64.3%), whereas in ten (35. 7%) other cases part of the cyst was left in place due to tight adhesions to vital structures. After histological study, there were: nine bronchogenic cysts (32.1%), seven hydatid cysts (25%), four cystic lymphangiomas (14.3%), three mature cystic teratomas (10.7%) and three pleuropericardial cysts, one thymic cyst and one parathyroid cyst. The postoperative course was uneventful and no recurrence has been observed until now. CONCLUSION: The benign cysts of the mediastinum is a rare entity, the hydatid etiology remains common in our context. Surgical treatment remains the treatment of choice for mediastinal cysts, when the patient is operable, to save the risk of complications or degeneration.

[Benign primary cystic lesions of mediastinum in adult: the clinical spectrum and surgical treatment]. / Lesiones quísticas primarias y benignas del mediastino en el adulto: espectro clínico y tratamiento quirúrgico.

BACKGROUND AND OBJECTIVE: The mediastinal cysts form a group of heterogeneous and uncommon benign lesions of neoplastic, congenital, or inflammatory conditions. The forgoing controversy is how to manage them; surgical removal or observation. We reviewed our experience including some rare conditions, emphasizing the clinical spectrum and surgical treatment. PATIENTES AND METHODS: This is a retrospective review between 2000 and 2007 included 34 cases of primary mediastinal cystic lesions. Clinical features, imaging techniques, surgical operation, morbidity, mortality and follow-up were analyzed. RESULTS: There were 18 females (53%) and 16 males (47%), with a mean age+/-standard deviation of 45.3+/-14.1 years (range: 22-74). Most of cysts were congenital (94%), except patients with hydatid disease (6%). 24% of cysts (n=8) were detected in anterior mediastinum. Rest of them (n=26) were located in visceral mediastinum. Patients usually were symptomatic (61%). Chest pain and discomfort was most common symptom, others were dyspnea, cough and hemoptysis, respectively. Cysts excision was performed in all cases with an uneventful recovery and with no recurrence in long term follow up. CONCLUSIONS: Asymptomatic mediastinal cysts are not rare. Surgery is a reliable method of treatment of mediastinal cysts with acceptable mortality and morbidity.

Congenital malformations of the lung.

Congenital malformations of the lung are rare and vary widely in their presentation and severity. The most common manifestation of the congenital cystic disease of the lung at newborn and early infancy is respiratory distress. Later on in life, cysts usually lose this compressive character and may remain asymptomatic until infection occurs, while producing cough, dyspnea and thoracic pain. The purpose of this study is to review authors institutional experience of congenital cystic lung disease, with specific reference to diagnosis, treatment, as well as outcome, furthermore, to present some cases with unusual clinical manifestations.

Lung cysts, spontaneous pneumothorax, and genetic associations in 89 families with Birt-Hogg-Dubé syndrome.

RATIONALE: Birt-Hogg-Dubé syndrome (BHDS) is an autosomal, dominantly inherited genodermatosis that predisposes to fibrofolliculomas, kidney neoplasms, lung cysts, and spontaneous pneumothorax. OBJECTIVES: We evaluated 198 patients from 89 families with BHDS to characterize the risk factors for pneumothorax and genotype-pulmonary associations. METHODS: Helical computed tomography scans of the chest were used to screen for pulmonary abnormalities. BHD mutation data were used for genotype-pulmonary associations. We examined the relationship of pneumothorax with categorical parameters (sex, smoking history, and lung cysts) and continuous parameters (number of cysts, lung cyst volume, and largest cyst diameter and volume). Logistic regression analyses were used to identify the risk factors associated with pneumothorax. MEASUREMENTS AND MAIN RESULTS: Twenty-four percent (48/198) of patients with BHDS had a history of pneumothorax. The presence of lung cysts was significantly associated with pneumothorax (p = 0.006). Total lung cyst volume, largest cyst diameter and volume, and every parameter related to the number of lung cysts were significantly associated (p < 0.0001) with pneumothorax. A logistic regression analysis showed that only the total number of cysts in the right parenchymal lower lobe and the total number of cysts located on the pleural surface in the right middle lobe were needed to classify a patient as to whether or not he or she was likely to have a pneumothorax. Exon location of the BHD mutation was associated with the numbers of cysts (p = 0.0002). CONCLUSIONS: This study indicates that patients with BHDS have a significant association between lung cysts and spontaneous pneumothorax.

Surgical management of mediastinal lesions.

Mediastinal tumors and cysts are relatively uncommon lesions requiring histologic confirmation. This retrospective study reports the experience of our department in the diagnosis and treatment of mediastinal lesions. Mediastinal lesions that were surgically treated in 200 patients aged 6-84 years, during a period of 28 years, were included in this series. Sixty patients had an apparently non-resectable lesion or lymphadenopathy of the anterior superior mediastinum. They had an anterior mediastinotomy and biopsy of the mediastinal lesion. No perioperative deaths were recorded in those patients. There were recorded 5 (8.3%) complications. Histological diagnosis was established in all patients: lymphoma (n = 21), metastatic carcinoma (n = 16), thymic lesions (n = 10), germ cell tumor (n = 3), other lesions (n = 10). The remainder 140 patients underwent a resection of the mediastinal lesion. One (0.7%) perioperative death and 21 (15%) complications were recorded. The histological diagnosis of the excised lesions was: thymic lesions (n = 60), neural tumors (n = 21), thyroid lesions (n = 14), bronchial cysts (n = 12), pericardial cysts (n = 10), germ cell tumors (n = 6), other lesions (n = 17). Our results are compared favorably with those reported in international literature. Surgery is the management of choice for patients with mediastinal lesions. It allows for establishing certain histological diagnosis and curative excision of the lesion, when it is necessary, with low operative risk.

Subdiaphragmatic bronchogenic cysts: a comprehensive review of the literature.

Bronchogenic cysts are foregut-derived developmental anomalies most commonly encountered in the mediastinum and rarely in the abdomen or retroperitoneally. A comprehensive review of the English-language literature of subdiaphragmatic bronchogenic cysts (sBCs) revealed only 48 reported cases of sBC. Although most cases are incidentally discovered, preoperative differential diagnosis often includes tumors with malignant potential and necessitates surgical resection to obtain a definitive diagnosis. Herein, we describe a case of a 46-year-old female presenting with intermittent left flank pain, upon which computed tomography demonstrated a retroperitoneal mass. Upon resection, histopathology revealed the mass to be a thin-walled cystic mass lined by ciliated columnar cells and cartilage, consistent with a subdiaphragmatic bronchogenic cyst. A comprehensive literature review of sBC was also performed.

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.

Lung cysts in subacute hypersensitivity pneumonitis.

PURPOSE: To determine the frequency of cystic lesions on high-resolution computed tomography (CT) in patients with subacute hypersensitivity pneumonitis. METHODS: High-resolution CT scans in 182 patients with proven subacute hypersensitivity pneumonitis were retrospectively evaluated for the presence of lung cysts. Patients with CT evidence of emphysema or interstitial fibrosis and patients with connective tissue disease were excluded from the study. Two thoracic radiologists reviewed the thoracic CT scans for the presence and location of lung cysts. RESULTS: Thin-walled lung cysts were identified in 24 (13%) of 182 patients and ranged in size from 3 to 25 mm in maximal diameter. The patients included 16 men and 8 women (age range, 30-79 years; mean age, 48 years). The cysts ranged from 1 to 15 in number (mean, 4 cysts) and had a random distribution. Sixteen of the 24 patients also had areas of decreased attenuation and vascularity consistent with air trapping. CONCLUSIONS: Thin-walled cysts can be seen in a small percentage of patients with subacute hypersensitivity pneumonitis. The cysts resemble those seen in lymphocytic interstitial pneumonia, and their pathogenesis is uncertain.

[Air-filled cysts of tracheal origin: nosological problems and actual frequency]. / Kystes aériens d'origine trachéale: problèmes nosologiques et fréquence réelle.

Air-filled paratracheal bronchogenic cysts are extremely rare. They are lined by respiratory epithelium. We have operated on 2 patients whose cysts were diagnosed by CT scan: one presenting with bloody sputum and the other with dysphagia. Both patients had a history of neck surgery. Because such cases are rare in the literature, their exact origin, whether tracheocele, diverticulum or other form of air-filled cyst, remains a matter of debate. However, their frequency is probably underestimated because most cause few symptoms and are well tolerated. In a recent radiologic study their prevalence was found to be 2% in patients undergoing CT scan.

Complications of the naevoid basal cell carcinoma syndrome: results of a population based study.

There are many potential complications which have been reported in association with the naevoid basal cell carcinoma syndrome. We have been able to show the relative frequencies of these problems in a population based study of 84 cases in the north west of England. The major complications of basal cell carcinomas and jaw cysts occur in over 90% of patients by 40 years of age, but may both occur before 10 years of age. Less well described complications are ovarian calcification or fibroma (24%), medulloblastoma (5%), cardiac fibroma (3%), cleft palate (5%), and ophthalmic abnormalities such as squint or cataract (26%). This study more clearly defines the possible complications of the syndrome and gives clearer guidelines for counselling and screening affected and at risk persons.

[A case of paraesophageal bronchogenic cyst with esophageal communication].

A case of mediastinal bronchogenic cyst communicating with the esophagus was reported. Previously, only 2 cases have been reported in the available literature. A 34-year-old man was admitted with a cystic mass communicating with the esophagus which was demonstrated on a barium study. Operation was performed with a suspect of esophageal diverticulum or congenital cyst with esophageal communication. At right thoracotomy, a mass measuring 6.0 x 5.0 cm with a well-defined patent communication to the esophagus was resected. It was a monolocular cyst containing a small amount of viscous mucus. Histologically, the cyst lined by a ciliated columnar epithelium, and it was diagnosed as a bronchogenic cyst because of the presence of the mucous glands, smooth muscle tissue and cartilage. This is the first case report of mediastinal bronchogenic cyst with esophageal communication appeared in the Japanese literature.

[Bronchogenic cysts in the carina]. / Kystes bronchogéniques de la carène.

Between 1977 and 1990, 11 children with carinal bronchogenic cysts were operated in our institution: 8 girls and 3 boys, ranging in age from 1 month to 5 years. All were symptomatic (acute respiratory distress and recurrent bronchiolitis). Chest X-ray showed an unilateral over distension in 10/11 cases. Barium oesophagogram showed a compression in 6/10 cases. Bronchoscopy noticed an extrinsic compression in 10/11 cases and a tracheal and/or bronchial diskinesia in 5/11 cases. The computed tomography showed a low density mass in 4/4 cases. 9 cysts were left-sided and 2 right-sided. Both children underwent a second surgery for a second cyst. 2 pneumonectomies for complete parenchyma destruction were realised. 1 left pulmonary hypoplasia was noticed. A tracheal and/or bronchial diskinesia in post-operative was noticed in 5/6 cases. The clinical and functional respiratory following was good in 10/11 cases. An early surgery treatment is necessary before definitive sequelae.

[Two successful cases of paraesophageal bronchogenic cysts].

Paraesophageal bronchogenic cyst is a rare disease. Only 24 cases have been previously reported in Japan. Recently, we experienced two successful cases of this disease. Two cases (64-year-old man and 31-year-old man) were both asymptomatic and pointed out by chance on the chest X-ray at the left posterior mediastinum. The second case was an intramural esophageal cyst. Microscopic examination showed the typical findings of the bronchogenic cyst, as the inner wall of the cyst was covered with ciliated columnar epithelium and the wall consisted of cartilage, mucous glands and smooth muscle. Both postoperative courses were uneventful. Resection was recommended because of the symptoms due to the compression of the cyst and the possibility of malignancy.

[Bronchogenic esophageal cysts in the adult. Four cases]. / Kystes oesophagiens bronchogéniques de l'adulte. Quatre observations.

Bronchogenic oesophageal cysts possess a mucosa of the airway type but are unconnected to the tracheobronchial tree; they are in close anatomical relation with the oesophageal wall. These characteristics are explained by the fact that the respiratory and digestive tract share the same embryonic development. Four cases of bronchogenic oesophageal cyst are reported, and 21 well-documented cases from the literature are reviewed. The lesion is extremely rare in adults, often complicated and responsible for gastrointestinal, respiratory and sometimes even cardiac symptoms. Its features at radiology, endoscopy and computerized tomography are suggestive of the diagnosis. Treatment consists of excision by enucleation. Excision must be complete for recurrences to be avoided.

Primary mediastinal tumors in children.

From 1950-1986, a total of 159 children (age 1 day-16 years) were treated for primary mediastinal tumors at our hospital. There were 77 malignant and 82 benign tumors. Tracheal compression causing respiratory distress was a significant symptom in 45.3% (24/53) of the children under 2 years of age. The diagnosis was based on the chest X-ray and the findings at surgery. Malignant lymphoma was usually diagnosed by cervical lymph node biopsy (23/39) but the treatment protocol was non-surgical. Non-lymphatic malignant tumors were completely or partially excised in 59.0% (23/39) of the cases. There was no early or late mortality in patients with benign tumors. At follow-up (0.5-24 years; mean 6.0 years), 62.3% (48/77) of the patients with malignant tumors were alive and symptom free. About half of the mediastinal tumors in children are malignant. Mediastinal tumors in small children can cause severe respiratory symptoms demanding urgent treatment. The treatment of choice is surgery (except in lymphomas) and the results are good even in malignant tumors.