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Panduranga S, V H, Mehta RM. An unique case of isolated right upper lobe lung agenesis with abnormal middle lobe segmentation. THE EGYPTIAN JOURNAL OF BRONCHOLOGY 2022. [DOI: 10.1186/s43168-022-00146-6] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/10/2022] Open
Abstract
Abstract
Background
Isolated right upper lobe pulmonary agenesis is a highly uncommon congenital anomaly, usually detected at adulthood when the patient is evaluated for an incidental abnormal chest radiograph. Chest radiography findings are non-specific. Chest computed tomography with pulmonary angiography is the modality of choice for diagnosing pulmonary agenesis.
Case presentation
We describe a case of isolated right upper lobe agenesis in a young man who presented with mild shortness of breath and an abnormal chest radiograph. High-resolution computed tomography chest showed right upper lobe pulmonary agenesis which was initially erroneously diagnosed as right lower lobe collapse on chest X-ray. Abnormal segmentation of the middle lobe was also seen which has not been described earlier according to our literature search.
Conclusions
There should be a high index of suspicion for congenital anomalies on chest X-ray to recommend further imaging studies. This case highlights the importance of computed tomography with pulmonary angiography to adequately assess and characterize the congenital lung anomalies.
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Long term outcomes in CDH: Cardiopulmonary outcomes and health related quality of life. J Pediatr Surg 2022; 57:501-509. [PMID: 35508437 DOI: 10.1016/j.jpedsurg.2022.03.020] [Citation(s) in RCA: 14] [Impact Index Per Article: 4.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/21/2021] [Revised: 02/27/2022] [Accepted: 03/18/2022] [Indexed: 02/07/2023]
Abstract
BACKGROUND With improvements in clinical management and an increase in CDH survivorship there is a crucial need for better understanding of long-term health outcomes in CDH. AIM To investigate the prevalence of cardiopulmonary health morbidity and health related quality of life (HRQoL) in CDH survivors. METHODS We included all studies (n = 65) investigating long-term cardiopulmonary outcomes in CDH patients more than 2 years published in the last 30 years. The Newcastle-Ottawa Scale and the CASP checklist for cohort studies were utilized to assess study quality. Results were reported descriptively and collated by age group where possible. RESULTS The incidence of pulmonary hypertension was highly variable (4.5-38%), though rates (%) appeared to diminish after 5 years of age. Lung function indices and radiological outcomes were frequently abnormal, and Health Related Quality of Life (HRQoL) reduced also. Long term diseases notably emphysema and COPD are not yet fully described in the contemporary literature. CONCLUSION This study underscores cardiopulmonary health morbidity and a reduced HRQoL among CDH survivors. Where not already available dedicated multidisciplinary follow-up clinics should be established to support these vulnerable patients transition safely into adulthood. Future research is therefore needed to investigate the risk factors for cardiopulmonary ill health and morbidity in CDH survivors. TYPE OF STUDY Systematic review of case control and cohort studies.
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Willers C, Maager L, Bauman G, Cholewa D, Stranzinger E, Raio L, Casaulta C, Latzin P. School-age structural and functional MRI and lung function in children following lung resection for congenital lung malformation in infancy. Pediatr Radiol 2022; 52:1255-1265. [PMID: 35305121 PMCID: PMC9192451 DOI: 10.1007/s00247-022-05317-7] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/23/2021] [Revised: 10/21/2021] [Accepted: 02/03/2022] [Indexed: 10/31/2022]
Abstract
BACKGROUND The management of asymptomatic congenital lung malformations is debated. Particularly, there is a lack of information regarding long-term growth and development of the remaining lung in children following lung resection for congenital lung malformations. In addition to conventional pulmonary function tests, we used novel functional magnetic resonance imaging (MRI) methods to measure perfusion and ventilation. OBJECTIVE To assess functionality of the remaining lung expanded into the thoracic cavity after resection of congenital lung malformations. MATERIALS AND METHODS A prospective, cross-sectional pilot study in five children who had surgery for congenital lung malformations during infancy. Participants had structural and functional MRI as well as spirometry, body plethysmography and multiple breath washout at school age. RESULTS Structural MRI showed an expansion of the remaining lung in all cases. Fractional ventilation and relative perfusion of the expanded lung were locally decreased in functional MRI. In all other parts of the lungs, fractional ventilation and relative perfusion were normal in all children. There was an association between overall impairment of perfusion and elevated lung clearance index. The results of spirometry and body plethysmography varied between patients, including normal lung function, restriction and obstruction. CONCLUSION Fractional ventilation and relative perfusion maps from functional MRI specifically locate impairment of the remaining lung after lung resection. These changes are not captured by conventional measures such as structural MRI and standard pulmonary function tests. Therefore, following lung resection for congenital lung malformation, children should be investigated more systematically with functional lung MRI.
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Affiliation(s)
- Corin Willers
- grid.5734.50000 0001 0726 5157Division of Pediatric Respiratory Medicine, Department of Pediatrics, Inselspital, Bern University Hospital, University of Bern, Freiburgstrasse 8, 3010 Bern, Switzerland
| | - Lukas Maager
- grid.5734.50000 0001 0726 5157Division of Pediatric Respiratory Medicine, Department of Pediatrics, Inselspital, Bern University Hospital, University of Bern, Freiburgstrasse 8, 3010 Bern, Switzerland
| | - Grzegorz Bauman
- grid.410567.1Division of Radiological Physics, Department of Radiology, University of Basel Hospital, Basel, Switzerland ,grid.6612.30000 0004 1937 0642Department of Biomedical Engineering, University of Basel, Allschwil, Switzerland
| | - Dietmar Cholewa
- grid.5734.50000 0001 0726 5157Department of Pediatric Surgery, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland
| | - Enno Stranzinger
- grid.5734.50000 0001 0726 5157Institute of Diagnostic, Interventional and Pediatric Radiology, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland
| | - Luigi Raio
- grid.5734.50000 0001 0726 5157Department of Obstetrics and Gynecology, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland
| | - Carmen Casaulta
- grid.5734.50000 0001 0726 5157Division of Pediatric Respiratory Medicine, Department of Pediatrics, Inselspital, Bern University Hospital, University of Bern, Freiburgstrasse 8, 3010 Bern, Switzerland
| | - Philipp Latzin
- Division of Pediatric Respiratory Medicine, Department of Pediatrics, Inselspital, Bern University Hospital, University of Bern, Freiburgstrasse 8, 3010, Bern, Switzerland.
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Role of Endobronchial Ultrasound-guided Transbronchial Needle Aspiration in the Diagnosis and Management of Mediastinal Cyst. J Bronchology Interv Pulmonol 2020; 27:142-146. [PMID: 31855882 DOI: 10.1097/lbr.0000000000000640] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
Abstract
BACKGROUND Foregut cysts account for >50% of cystic lesions in the mediastinum, of which bronchogenic cysts are most common. Surgical resection is the most definitive approach for its diagnosis and treatment. A recent systematic review, however, suggests that endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) has a role in the management of bronchogenic cyst. We report our experience with EBUS-TBNA in the diagnosis and management of bronchogenic cysts. METHODS Medical records of patients with evidence of mediastinal cysts who underwent EBUS-TBNA between 2008 and 2016 were reviewed.The primary aims of this study were to assess EBUS-TBNA diagnostic yield of peri-bronchial cysts and their specific type/origin and to determine its short-term and long-term drainage efficacy. RESULTS A total of 26 patients met the inclusion criteria. The cytopathology diagnosis was compatible with bronchogenic cyst in 4 cases, pleural-pericardial cyst in 3 cases, and 19 were indeterminate cysts. Successful long-term treatment occurred in 5.5% of the subjects. One patient developed inflammatory pericarditis after EBUS-TBNA. CONCLUSION Diagnostic and therapeutic yield of EBUS-TBNA for mediastinal cysts is limited and surgical resection remains the treatment of choice.
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Zhang Q, Shan KS. A Mysterious Paratracheal Mass: Pulmonary Agenesis. Cureus 2020; 12:e8738. [PMID: 32714677 PMCID: PMC7377013 DOI: 10.7759/cureus.8738] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/03/2022] Open
Abstract
A 35-year-old lady with a history of possible tuberculosis infection 15 years ago presented to the clinic with the chief complaint of cough. Incidental chest CT showed a right paratracheal and medial right apical heterogeneous soft tissue mass with central areas of calcification that warranted further investigation. A routine endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) discovered isolated lobar pulmonary agenesis as the underlying cause of the mass without findings of malignancy on pathology reports.
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Affiliation(s)
- Qian Zhang
- Internal Medicine, Abington Hospital - Jefferson Health, Abington, USA
| | - Khine S Shan
- Internal Medicine, University of Maryland Medical Center, Baltimore, USA
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6
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Paes B, Kim D, Saleem M, Wong S, Mitchell I, Lanctot KL. Respiratory syncytial virus prophylaxis in infants with congenital airway anomalies compared to standard indications and complex medical disorders. Eur J Pediatr 2019; 178:377-385. [PMID: 30610419 DOI: 10.1007/s00431-018-03308-1] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/31/2018] [Revised: 10/23/2018] [Accepted: 12/14/2018] [Indexed: 10/27/2022]
Abstract
An observational study was conducted of children < 2 years who received ≥ 1 dose of palivizumab in 32 Canadian institutions from 2005 to 2017. We compared respiratory illness (RIH) and respiratory syncytial virus-related hospitalization (RSVH) hazards in children with a congenital airway anomaly (CAA) versus those prophylaxed for standard indications (SI) and serious medical disorders (SMD). Data were assembled on neonatal course, demographics, palivizumab utilization and adherence, and respiratory illness events, and analyzed using ANOVA, chi-square tests and Cox proportional hazards. Twenty-five thousand three children (1219 CAA, 3538 SMD, and 20,246 SI) were enrolled. Palivizumab adherence was 74.8% overall and similar across groups. For 2054 respiratory-related events, 1724 children were hospitalized. RIH rates were 13.6% (CAA), 9.6% (SMD), and 6.0% (SI). RSVH rates were 2.4% (CAA), 1.6% (SMD), and 1.5% (SI). After adjustment for demographic and neonatal differences, children with a CAA had a significantly increased RIH and RSVH hazard relative to SI (RIH, HR = 1.6, 95% CI 1.2-2.2, p = 0.002; RSVH, HR = 2.1, 95% CI 1.0-4.4, p = 0.037) but similar to SMD (RIH, HR = 1.3, 95% CI 0.9-1.9, p = 0.190; RSVH, HR = 1.7, 95% CI 0.7-4.1, p = 0.277).Conclusion: Children with a CAA experience higher RIH risk. RSVH hazard was similar between CAA and SMD but higher for CAA compared to SI, implying that this population requires surveillance for RSV prophylaxis. What is Known: • Children with congenital airway anomalies (CAA) are at risk for respiratory tract illness and respiratory syncytial virus-related hospitalization (RSVH) with accompanying morbidity and mortality • RSV prophylaxis may be useful in children with a CAA, but is not routinely recommended What is New: • Children with a CAA had a 1.6-2.3 fold greater risk of respiratory-related hospitalization and RSVH compared to those prophylaxed for standard, approved indications and serious medical disorders. • RSVH risk in children aged < 2 years with either upper or lower airway anomalies is similar. Children with a CAA require careful surveillance during the RSV season and prophylaxis may be appropriate.
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Affiliation(s)
- Bosco Paes
- Department of Pediatrics - HSC-3A, McMaster University, 1280 Main Street West, Hamilton, Ontario, L8S 4K1, Canada.
| | - Doyoung Kim
- Medical Outcomes and Research in Economics (MORE®) Research Group, Sunnybrook Health Sciences Centre, University of Toronto, Toronto, Ontario, Canada
| | - Mahwesh Saleem
- Medical Outcomes and Research in Economics (MORE®) Research Group, Sunnybrook Health Sciences Centre, University of Toronto, Toronto, Ontario, Canada
| | - Sophie Wong
- Medical Outcomes and Research in Economics (MORE®) Research Group, Sunnybrook Health Sciences Centre, University of Toronto, Toronto, Ontario, Canada
| | - Ian Mitchell
- Department of Pediatrics, University of Calgary, Calgary, Alberta, Canada
| | - Krista L Lanctot
- Medical Outcomes and Research in Economics (MORE®) Research Group, Sunnybrook Health Sciences Centre, University of Toronto, Toronto, Ontario, Canada
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Abstract
To survive the transition to extrauterine life, newborn infants must have lungs that provide an adequate surface area and volume to allow for gas exchange. The dynamic activities of fetal breathing movements and accumulation of lung luminal fluid are key to fetal lung development throughout the various phases of lung development and growth, first by branching morphogenesis, and later by septation. Because effective gas exchange is essential to survival, pulmonary hypoplasia is among the leading findings on autopsies of children dying in the newborn period. Management of infants born prematurely who had disrupted lung development, especially at the pre-glandular or canalicular periods, may be challenging, but limited success has been reported. Growing understanding of stem cell biology and mechanical development of the lung, and how to apply them clinically, may lead to new approaches that will lead to better outcomes for these patients.
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Kandil S, Hassan RH, Fouda A, Zedan M. Unusual presentations of lung lesions in children: difficult to diagnose case series. THE EGYPTIAN JOURNAL OF BRONCHOLOGY 2016. [DOI: 10.4103/1687-8426.193644] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
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Gorospe L, Muñoz-Molina GM, Ayala-Carbonero AM, Fernández-Méndez MÁ, Arribas-Marcos Á, Castro-Acosta P, Arrieta P, García-Gómez-Muriel I, Gómez-Barbosa CF, Barrios-Barreto D. Cystic adenomatoid malformation of the lung in adult patients: clinicoradiological features and management. Clin Imaging 2016; 40:517-22. [DOI: 10.1016/j.clinimag.2015.11.014] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/01/2015] [Accepted: 11/05/2015] [Indexed: 01/13/2023]
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David M, Lamas-Pinheiro R, Henriques-Coelho T. Prenatal and Postnatal Management of Congenital Pulmonary Airway Malformation. Neonatology 2016; 110:101-15. [PMID: 27070354 DOI: 10.1159/000440894] [Citation(s) in RCA: 63] [Impact Index Per Article: 7.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/08/2015] [Accepted: 09/06/2015] [Indexed: 11/19/2022]
Abstract
Congenital pulmonary airway malformation (CPAM) is one of the most common lung lesions detected prenatally. Despite the research efforts made in the past few years, controversy and lack of clarity in the literature still exist regarding nomenclature, classification, pathogenesis and the management of CPAM. Therefore, it is of greatest importance to delineate the natural history of CPAMs and to create a consensus to guide the management and follow-up of these lesions. This review will focus on classification systems, highlighting the most recent advancements in pathogenesis, and current practice in the prenatal diagnosis of CPAM. Strategies of prenatal management and postnatal management will be reviewed. Long-term follow-up, including lung cancer risk, is discussed and an outcome perspective is presented.
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Affiliation(s)
- Mafalda David
- Pediatric Surgery Department, Centro Hospitalar Sx00E3;o Jox00E3;o, Porto, Portugal
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11
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Abstract
Antenatal detection of congenital pulmonary airway malformations (CPAM) has improved immeasurably from its initial application in the 1980s and probably encompasses >80% of all such lesions. Accurate diagnosis still remains less reliable and definitive diagnosis requires detailed anatomical imaging (typically with CT scan) in the post-natal period. About 10% of all lesions will present with symptoms during the neonatal period and the choice of surgical intervention is then easy. For those that remain asymptomatic then there is still a degree of controversy about elective surgical resection. This article presents the case for elective surgery within the first year of life and aims to quantify the risks of non-intervention such as abscess, empyema, recurrent pneumonia, air-leak, and pneumothorax and various types of malignancy in such cases. The current surgical approach now includes both open muscle-sparing thoracotomy and thoracoscopic resection.
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Affiliation(s)
- Rashmi Singh
- Department of Paediatric Surgery, King׳s College Hospital, Denmark Hill, London SE5 9RS, UK
| | - Mark Davenport
- Department of Paediatric Surgery, King׳s College Hospital, Denmark Hill, London SE5 9RS, UK.
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12
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Abstract
Antenatal diagnosis of lung lesion has become more accurate resulting in dilemma and controversies of its antenatal and postnatal management. Majority of antenatally diagnosed congenital lung lesions are asymptomatic in the neonatal age group. Large lung lesions cause respiratory compromise and inevitably require urgent investigations and surgery. The congenital lung lesion presenting with hydrops requires careful postnatal management of lung hypoplasia and persistent pulmonary hypertension. Preoperative stabilization with gentle ventilation with permissive hypercapnia and delayed surgery similar to congenital diaphragmatic hernia management has been shown to result in good outcome. The diagnostic investigations and surgical management of the asymptomatic lung lesions remain controversial. Postnatal management and outcome of congenital cystic lung lesions are discussed.
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Affiliation(s)
- Dakshesh H Parikh
- Department of Paediatric Surgery, Birmingham Children׳s Hospital NHS Foundation Trust, Steelhouse Lane, Birmingham B4 6NH, UK.
| | - Shree Vishna Rasiah
- Southern West Midlands Newborn Network and Birmingham Women's Health Care NHS Trust, Mindelsohn Way, Edgbaston Birmingham, B15 2TG UK
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13
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Kuo CP, Lu YT, Lin RL. Agenesis of right upper lobe of lung. Respirol Case Rep 2015; 3:51-3. [PMID: 26090110 PMCID: PMC4469139 DOI: 10.1002/rcr2.98] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/19/2015] [Revised: 02/07/2015] [Accepted: 02/10/2015] [Indexed: 11/28/2022] Open
Abstract
Agenesis of the right upper lobe of the lung is a very uncommon congenital anomaly and may be referred to chest clinics in adulthood for an incidental finding of abnormal chest radiograph. The presentations of chest radiograph may imitate many common situations such as right upper lobe collapse presenting as an ipsilateral shifting of the mediastinum or elevation of the right hemidiaphragm due to eventration or subdiaphragmatic lesions. A chest computed tomography is considered the most conclusive examination used to diagnose lung agenesis. Three-dimensional reconstructed images can be particularly helpful in delineating abnormalities of the bronchi and associated arterial and venous structures. We describe here a young woman with allergic rhinitis and bronchial asthma since her early childhood. She was referred to our clinic for an incidental finding of abnormal chest radiograph after a school health checkup. Right upper lobe atelectasis or intra-abdominal lesions were initially suspected. After a thorough image study, she was diagnosed as a case of agenesis of the right upper lobe. Our report emphasizes the importance that a high index of suspicion and adequate image investigation are necessary to diagnose congenital lung anomalies.
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Affiliation(s)
- Chiu-Ping Kuo
- Division of Chest Medicine, Department of Internal Medicine, MacKay Memorial Hospital Taipei, Taiwan
| | - Yen-Ta Lu
- Division of Chest Medicine, Department of Internal Medicine, MacKay Memorial Hospital Taipei, Taiwan
| | - Rong-Luh Lin
- Division of Chest Medicine, Department of Internal Medicine, MacKay Memorial Hospital Taipei, Taiwan
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Congenital lobar hyperinflation: Conservative management as an alternative therapy. ANALES DE PEDIATRÍA (ENGLISH EDITION) 2014. [DOI: 10.1016/j.anpede.2013.07.001] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022] Open
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15
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[Congenital lobar hyperinflation: conservative management as an alternative therapy]. An Pediatr (Barc) 2013; 81:45-8. [PMID: 24269550 DOI: 10.1016/j.anpedi.2013.07.001] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/01/2012] [Revised: 07/03/2013] [Accepted: 07/29/2013] [Indexed: 01/26/2023] Open
Abstract
Congenital lobar emphysema used to be treated surgically. Congenital lobar hyperinflation is the currently recommended term, as it involves pathologically healthy lung tissue, which is why conservative management may be an option. Four cases of diagnosed congenital lobar hyperinflation are presented in which conservative treatment was chosen due to their clinical stability. Their outcome has been satisfactory with progressively normal radiology.
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Abstract
Several population-based birth cohort studies documented that 30% of children suffer from wheezing during respiratory infections before their third birthday. Infants are prone to wheeze because of anatomic factors related to the lung and chest wall in addition to immunologic and molecular influences in comparison to older children. Viral infections lead to immunologic derangements that cause wheezing both in immunocompetent and immunodeficient infants. Anatomic causes of wheeze may be extrinsic or intrinsic to the airway. Not every wheeze is indicative of asthma but prediction of asthma in persistent wheezers is possible. Testing for allergy in these infants is worthwhile and can be of significant value in avoidable allergens. Treatment of an infant with wheezing depends on the underlying etiology. Response to bronchodilators is unpredictable and a trial of inhaled steroids may be warranted in a patient who has responded to multiple courses of oral steroids, has moderate to severe wheezing, or a significant history of atopy including food allergy or eczema. Ribavirin administered by aerosol, hyper-immune respiratory syncytial virus immunoglobulin (RSV IVIG), and intramuscular monoclonal antibody to an RSV protein have been used for RSV bronchiolitis in infants with congenital heart disease or chronic lung disease.
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Affiliation(s)
- Yehia M El-Gamal
- From the Pediatric Allergy and Immunology Unit, Children's Hospital, Ain Shams University, Cairo, Egypt
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17
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Al Marri M, Mahufouz A, Darwish A, Al Marri N. Intralobar Pulmonary Sequestration in 22 year-old Male: Case report and review of literature. Qatar Med J 2012. [DOI: 10.5339/qmj.2012.1.16] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/03/2022] Open
Abstract
Pulmonary sequestration is a rare congenital malformation of the lower respiratory tract characterized by a non-functioning mass of lung tissue that is not communicating with the normal tracheo-bronchial tree and receives its vascular supply from a systemic artery. We report the first case of intrapulmonary sequestration in Qatar, of a 22 year-old Qatari male who presented with recurrent chest infection. This case study includes a literature review.
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Affiliation(s)
- M.R. Al Marri
- *Pulmonary and Internal Medicine, Department of Medicine, Doha, Qatar
| | - A. Mahufouz
- **Radiology Department, Hamad Medical Corporation, Doha, Qatar
| | - A. Darwish
- **Radiology Department, Hamad Medical Corporation, Doha, Qatar
| | - N. Al Marri
- *Pulmonary and Internal Medicine, Department of Medicine, Doha, Qatar
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Naito Y, Beres A, Lapidus-Krol E, Ratjen F, Langer JC. Does earlier lobectomy result in better long-term pulmonary function in children with congenital lung anomalies? A prospective study. J Pediatr Surg 2012; 47:852-6. [PMID: 22595560 DOI: 10.1016/j.jpedsurg.2012.01.037] [Citation(s) in RCA: 32] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/06/2012] [Accepted: 01/26/2012] [Indexed: 11/25/2022]
Abstract
BACKGROUND Management of asymptomatic congenital pulmonary airway malformations remains controversial when addressing the optimal timing of surgical resection. Neonatal resection is advocated by some based on the theory that earlier lobectomy results in greater compensatory lung growth. We examined whether age at lobectomy is correlated with better pulmonary outcomes as reflected by pulmonary function and exercise testing. METHODS Patients who had lobectomy for congenital pulmonary airway malformation between 1985 and 2002 were identified and underwent detailed clinical history, physical examination, pulmonary function testing (total lung capacity, forced vital capacity, forced expiratory volume in 1 second), and exercise testing (power, maximal oxygen uptake [Vo(2)max]). RESULTS Of 87 patients identified, 47 met the inclusion criteria, and 28 were tested prospectively. Age at the time of lobectomy ranged from 3 days to 56 months. There was no correlation between age at lobectomy and pulmonary function (total lung capacity, P = .408; forced vital capacity, P = .319; forced expiratory volume in 1 second, P = .174) or maximal work capacity (power, P = .280). There was a trend toward lower Vo(2)max in patients who had undergone lobectomy at an older age (Vo(2)max, P = .055). CONCLUSION Most children undergoing lobectomy have normal long-term pulmonary function. We found no correlation between age at lobectomy and future pulmonary function. Cardiopulmonary exercise testing should be considered in evaluating functional outcome in these patients.
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Affiliation(s)
- Yoko Naito
- Division of Respirology, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
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19
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Di Prima FAF, Bellia A, Inclimona G, Grasso F, Teresa M, Cassaro MN. Antenatally diagnosed congenital cystic adenomatoid malformations (CCAM): Research Review. J Prenat Med 2012; 6:22-30. [PMID: 22905308 PMCID: PMC3421952] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 06/01/2023]
Abstract
Prenatal identification of lung abnormalities has increased with prenatal surveillance. With the advent of improved antenatal imaging over the past ten years, the diagnosis, assessment and management of congenital cystic lung abnormalities have changed. These were once considered the exclusive domain of the surgeon, who had the authority to operate on all congenital cystic lung abnormalities regardless of size or clinical signs in order to avoid the risk of cancer and improve lung growth in even asymptomatic infants. Clinicians are reconsidering this approach in the light of the spontaneous improvement and possible resolution that occurs over months to years with many of these lesions, thinking about the opportunity to take a more conservative approach in many minimally symptomatic or asymptomatic infants in the early months of life. The risks of subsequent cancer are poorly understood and probably overstated. Many centers advocate surgery only in cases of symptomatic or significant lesions, although there is little consensus as to what constitutes a significant lesion.This article will review current knowledge (classification, pathogenesis, genetics, prenatal evaluation, clinical implications) on congenital cystic adenomatoid malformations (CCAM) and discuss management options for young children with these lung abnormalities.
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20
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Kwak HJ, Moon JY, Kim SI, Kim TH, Sohn JW, Kim SH, Shin DH, Park SS, Chung WS, Yoon HJ. Congenital Cystic Adenomatoid Malformation with Bronchial Atresia in Elderly Patients. Tuberc Respir Dis (Seoul) 2012; 72:501-6. [PMID: 23101017 PMCID: PMC3475458 DOI: 10.4046/trd.2012.72.6.501] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/20/2011] [Revised: 09/30/2011] [Accepted: 11/06/2011] [Indexed: 11/24/2022] Open
Abstract
Congenital cystic adenomatoid malformation (CCAM) is an uncommon, nonhereditary anomaly caused by arrest of lung. Patients with CCAM may present with respiratory distress as newborns, or may remain asymptomatic until later in life. CCAM type I is rarely found in association with bronchial atresia (BA) in adults; we present such a case. Case: A 54-year-old female presented with chronic cough and blood-tinged sputum. Physical examination and laboratory tests were unremarkable. Chest radiographs and a CT scan of the chest showed multiple large air-filled cysts consistent with a CCAM in the right lower lobe, and an oval-shaped opacity in the distal right middle lobal bronchus. Based on the radiologic findings, right middle lobectomy and a medial basal segmentectomy of the right lower lobe were performed via a thoracotomy. These lesions were consistent with Stocker's Type I CCAM and BA in the different lobes.
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Affiliation(s)
- Hyun Jung Kwak
- Department of Internal Medicine, Hanyang University College of Medicine, Seoul, Korea
| | - Ji-Yong Moon
- Department of Internal Medicine, KEPCO, Medical Foundation Hanil General Hospital, Seoul, Korea
| | - Sa-Il Kim
- Department of Internal Medicine, Hanyang University College of Medicine, Seoul, Korea
| | - Tae Hyung Kim
- Department of Internal Medicine, Hanyang University College of Medicine, Seoul, Korea
| | - Jang Won Sohn
- Department of Internal Medicine, Hanyang University College of Medicine, Seoul, Korea
| | - Sang-Heon Kim
- Department of Internal Medicine, Hanyang University College of Medicine, Seoul, Korea
| | - Dong Ho Shin
- Department of Internal Medicine, Hanyang University College of Medicine, Seoul, Korea
| | - Sung Soo Park
- Department of Internal Medicine, Hanyang University College of Medicine, Seoul, Korea
| | - Won Sang Chung
- Department of Thoracic and Cardiovascular Surgery, Hanyang University College of Medicine, Seoul, Korea
| | - Ho Joo Yoon
- Department of Internal Medicine, Hanyang University College of Medicine, Seoul, Korea
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Abstract
BACKGROUND/PURPOSE The management of asymptomatic congenital lung lesions is controversial. It is unclear whether elective resection provides a significant benefit. We sought to determine whether early vs delayed resection of asymptomatic congenital lung malformations resulted in complications. METHODS Institutional billing records were queried for patients with lung malformations over a 10-year period. Medical records were reviewed for demographics, type of anomaly, symptoms, management, and procedural or disease-related complications. RESULTS Eighty-seven patients were identified. The diagnoses included congenital cystic adenomatoid malformation (41%), bronchogenic cyst (19.3%), sequestration (13.2%), and congenital lobar emphysema (12.0%). Fifty patients were observed for some period. Eleven became symptomatic, and 47 underwent resection at a mean age of 11 months. There was no difference in the type of resection, length of hospitalization, or complication rate between patients who underwent early vs delayed resection. There were no occurrences of malignancy or death. CONCLUSIONS In our series, there was no difference in measurable outcomes between early and delayed resection of congenital lung lesions. These data provide some support for a management strategy that might include observation with delayed resection for asymptomatic patients.
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22
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Zhang BS, Zhou NK, Yu CH. Congenital bronchoesophageal fistula in adults. World J Gastroenterol 2011; 17:1358-61. [PMID: 21455337 PMCID: PMC3068273 DOI: 10.3748/wjg.v17.i10.1358] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/11/2010] [Revised: 01/04/2011] [Accepted: 01/11/2011] [Indexed: 02/06/2023] Open
Abstract
AIM: To study the clinical characteristics, diagnosis and surgical treatment of congenital bronchoesophageal fistulae in adults.
METHODS: Eleven adult cases of congenital bronchoesophageal fistula diagnosed and treated in our hospital between May 1990 and August 2010 were reviewed. Its clinical presentations, diagnostic methods, anatomic type, treatment, and follow-up were recorded.
RESULTS: Of the chief clinical presentations, nonspecific cough and sputum were found in 10 (90.9%), recurrent bouts of cough after drinking liquid food in 6 (54.6%), hemoptysis in 6 (54.6%), low fever in 4 (36.4%), and chest pain in 3 (27.3%) of the 11 cases, respectively. The duration of symptoms before diagnosis ranged 5-36.5 years. The diagnosis of congenital bronchoesophageal fistulae was established in 9 patients by barium esophagography, in 1 patient by esophagoscopy and in 1 patient by bronchoscopy, respectively. The congenital bronchoesophageal fistulae communicated with a segmental bronchus, a main bronchus, and an intermediate bronchus in 8, 2 and 1 patients, respectively. The treatment of congenital bronchoesophageal fistulae involved excision of the fistula in 10 patients or division and suturing in 1 patient. The associated lung lesion was removed in all patients. No long-term sequelae were found during the postoperative follow-up except in 1 patient with bronchial fistula who accepted reoperation before recovery.
CONCLUSION: Congenital bronchoesophageal fistula is rare in adults. Its most useful diagnostic method is esophagography. It must be treated surgically as soon as the diagnosis is established.
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23
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Hoey ETD, Bhatnagar P, Mankad K, Gopalan D, Darby M, Robertson R. Imaging appearances of congenital thoracic lesions presenting in adulthood. Can Assoc Radiol J 2009; 60:172-81. [PMID: 19631492 DOI: 10.1016/j.carj.2009.06.005] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/15/2023] Open
Abstract
Many congenital lesions of the thorax are detected for the first time in adulthood when they can simulate a wide range of pathologies, including infection and neoplasia. They can be broadly classified into tracheobronchial, parenchymal, vascular, and combined parenchymal/vascular abnormalities. An awareness of their typical imaging features enables a confident diagnosis and helps direct appropriate patient management.
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24
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Wong A, Vieten D, Singh S, Harvey JG, Holland AJA. Long-term outcome of asymptomatic patients with congenital cystic adenomatoid malformation. Pediatr Surg Int 2009; 25:479-85. [PMID: 19404649 DOI: 10.1007/s00383-009-2371-5] [Citation(s) in RCA: 77] [Impact Index Per Article: 4.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 04/17/2009] [Indexed: 10/20/2022]
Abstract
PURPOSE Congenital cystic adenomatoid malformation (CCAM) represents a rare congenital anomaly of the lung. It remains controversial whether patients with asymptomatic lesions warrant early surgical intervention. Our aim was to review the outcome of asymptomatic CCAM patients at a paediatric tertiary centre. METHODS The medical case notes of all children with CCAM presenting to our institution between 1986 and 2007 were reviewed. Data on pre- and post-natal investigations, clinical presentation, lesion site, type of surgical procedure, timing, and outcomes of surgery were reviewed. RESULTS A total of 35 patients were diagnosed with CCAM during the 21-year study period (1986-2007). Sixty percent (n = 21) were asymptomatic at birth including eight patients with prenatal ultrasound scan confirming CCAM. In this group, 18 patients (86%) subsequently developed symptoms (median age 2 years, range 1 month-13 years) and required surgery. Symptoms included pneumonia with or without infected CCAM (43%), respiratory distress (14%) and spontaneous pneumothorax (14%). Eight patients underwent multiple hospital presentations with complications related to CCAM. Of the 21 initially asymptomatic patients, 17 (81%) underwent surgical resection. Only one of these patients was completely asymptomatic prior to surgery. There were eight post-operative complications and no mortality. One patient underwent a second thoracotomy for residual CCAM. The median length of hospital stay was 9 days (range 3-32 days). CONCLUSION This study suggests patients who present with asymptomatic CCAM will subsequently become symptomatic. Early surgical referral and intervention may be beneficial to avoid the development of complications.
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Affiliation(s)
- A Wong
- Department of Academic Surgery, The Children's Hospital at Westmead, The University of Sydney, Locked Bay 4001, Westmead, NSW, 2145, Australia
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25
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Treatment of Congenital Cystic Adenomatoid Malformation: Should Lobectomy Always Be Performed? Ann Thorac Surg 2008; 86:249-53. [DOI: 10.1016/j.athoracsur.2008.01.036] [Citation(s) in RCA: 48] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/15/2007] [Revised: 01/13/2008] [Accepted: 01/14/2008] [Indexed: 11/18/2022]
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26
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Tsai AY, Liechty KW, Hedrick HL, Bebbington M, Wilson RD, Johnson MP, Howell LJ, Flake AW, Adzick NS. Outcomes after postnatal resection of prenatally diagnosed asymptomatic cystic lung lesions. J Pediatr Surg 2008; 43:513-7. [PMID: 18358291 DOI: 10.1016/j.jpedsurg.2007.10.032] [Citation(s) in RCA: 71] [Impact Index Per Article: 4.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 10/22/2022]
Abstract
BACKGROUND Symptomatic congenital lung lesions require surgical resection, but the management of asymptomatic lung lesions is controversial. Some surgeons advocate observation because of concerns about potential operative morbidity and mortality, as well as a lack of long-term follow-up information. On the other hand, malignant degeneration, pneumonia, and pneumothorax are known consequences of cystic lung lesions. This study aims to assess the safety of resection for asymptomatic lung lesions that were diagnosed before birth. METHODS A retrospective review of all patients with prenatally diagnosed lung lesions at Children's Hospital of Philadelphia (Philadelphia, Penn) was performed from 1996 to 2005. The perioperative course of patients who were asymptomatic was analyzed. RESULTS One hundred five complete records of children with asymptomatic lesions were reviewed. Overall mortality was 0% and morbidity was 6.7% including 2.9% significant postoperative air leak and 3.8% transfusion requirement. Nine patients had a pathologic diagnosis that differed from preoperative radiological findings, and 9 patients had additional pathologic findings. CONCLUSION This series demonstrates that surgery can be performed safely on patients who were asymptomatic with congenital cystic adenomatoid malformation of the lung and other types of lung lesions with no mortality and minimal morbidity. The frequency of disparate pathologic diagnoses and the potential for development of malignancy and other complications support the argument for early resection.
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Affiliation(s)
- Anthony Y Tsai
- The Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA
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27
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Oliveira DRCD, Rodrigues Jr. AJ. Hérnia de bochdalek em adulto. Rev Col Bras Cir 2008. [DOI: 10.1590/s0100-69912008000100010] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/21/2022] Open
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28
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Abstract
Pulmonary vascular malformations have historically been diagnosed in a wide range of age groups, but the extensive use of prenatal imaging studies has resulted in the majority of lesions being diagnosed in utero. Among this group of lesions, bronchopulmonary sequestrations (BPS), hybrid lesions with both congenital cystic adenomatoid malformation (CCAM) and BPS, aberrant systemic vascular anastomoses, and pulmonary arteriovenous malformations (PAVM), are the most common. The biologic behavior of these lesions and the subsequent therapy is, in large part, determined by the age of the patient at diagnosis. In the fetus, large BPS or hybrid lesions can result in fetal hydrops and in utero fetal demise. In the perinatal period, pulmonary hypoplasia from the mass effect or air trapping within the cystic component of hybrid lesions can result in life-threatening respiratory distress. In the postnatal period, communication of the lesion with the aero-digestive system can result in recurrent pneumonia. Alternatively, increased pulmonary blood flow from the systemic arterial supply can result in hemorrhage, hemoptysis, or high output cardiac failure. In addition, there have been several reports of malignant degeneration. Finally, the broad spectrum encompassed by these lesions makes classification and subsequent communication of the lesions confusing and difficult. This paper will review the components of these lesions, their associated anomalies, the diagnosis and natural history, and finally, current concepts in the management of pulmonary vascular malformations.
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Affiliation(s)
- Kenneth W Liechty
- Center for Fetal Research, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA.
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29
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Abstract
Bronchoesophageal fistulas are usually diagnosed in the neonatal period. As such, the condition is rare in adults. We present a case of a congenital bronchoesophageal fistula in a 62-year-old man with the complaint of severe bouts of cough and choking after swallowing liquid. His workup included a barium esophagogram that revealed a fistula between the esophagus and a right lower lobe bronchus. The diagnosis should be considered in certain individuals with suggestive symptomatology and unexplained respiratory pathology. The fistula was divided and resected, The patient had an uneventful recovery.
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Affiliation(s)
- Lei Su
- Department of Thoracic Surgery, Xuan Wu Hospital of Capital University of Medical Science, No. 45 Changchun Street, Beijing 100053, China.
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30
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Abstract
With the advent of improved antenatal imaging over the past 10 years, the diagnosis, assessment and management of congenital cystic lung abnormalities have changed. These were once considered the exclusive domain of the surgeon, who had the authority to operate on all congenital cystic lung abnormalities regardless of size or clinical signs in order to avoid the risk of cancer and improve lung growth in even asymptomatic infants. Clinicians are reconsidering this approach in the light of an appreciation of the spontaneous improvement and possible resolution that occurs over months to years with many of these lesions. The risks of subsequent cancer are poorly understood and probably overstated, whereas the magnitude of compensatory lung growth is poorly defined in the majority of children with small unilateral congenital cystic abnormalities. The evolution of regional, national and ideally international databases will provide much-needed longitudinal data to better inform clinicians of the optimal way in which to manage these children.
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31
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Vieira J, Rego A, Oliveira A, Ferreira DS, Furtado A, Couceiro A, Miranda JA, Leal F, Vouga L. Sequestro broncopulmonar – Experiência de 12 anos. REVISTA PORTUGUESA DE PNEUMOLOGIA 2006. [DOI: 10.1016/s0873-2159(15)30455-4] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/23/2022] Open
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32
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Nagata K, Kamio Y, Ichikawa T, Kadokura M, Kitami A, Endo S, Inoue H, Kudo SE. Congenital tracheoesophageal fistula successfully diagnosed by CT esophagography. World J Gastroenterol 2006; 12:1476-8. [PMID: 16552825 PMCID: PMC4124334 DOI: 10.3748/wjg.v12.i9.1476] [Citation(s) in RCA: 16] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
Tracheoesophageal fistula (TEF) or bronchoesophageal fistula may be congenital, inflammatory, neoplastic, or secondary to trauma. Congenital TEF or bron-choesophageal fistula is usually associated with eso-phageal atresia and is readily diagnosed in infancy. But if it is not associated with esophageal atresia, it may persist until adulthood. Some theories have been proposed to explain this delay in diagnosis. We present a case of a 70-year-old man with congenital TEF. The TEF was successfully diagnosed by multidetector-row CT esophagography.
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Affiliation(s)
- Koichi Nagata
- Digestive Disease Center, Showa University Northern Yokohama Hospital, 35-1 Chigasaki-chuo, Yokohama 224-8503, Japan.
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Kaplan KA, Beierle EA, Faro A, Eskin TA, Flotte TR. Recurrent pneumonia in children: a case report and approach to diagnosis. Clin Pediatr (Phila) 2006; 45:15-22. [PMID: 16429211 DOI: 10.1177/000992280604500103] [Citation(s) in RCA: 15] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Affiliation(s)
- Karly A Kaplan
- Department of Pediatrics, University of Florida, Gainesville 32610-0296, USA
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34
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Kulwa E, Tharakan T, Baxi L. Congenital Cystic Adenomatoid Malformation in the Fetus: A Hypothesis of Its Development. Fetal Diagn Ther 2005; 20:472-4. [PMID: 16113577 DOI: 10.1159/000086836] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/20/2004] [Accepted: 12/20/2004] [Indexed: 11/19/2022]
Abstract
We present a case of congenital cystic adenomatoid malformation of the lung diagnosed at 34 weeks of gestation in the setting of polyhydramnios. The fetus had CCAM in the L lung, with mediastinal shift to the right and ascites. The neonate underwent drainage of cysts and subsequent left lung lobar resection with improvement in respiratory function. The pathology of CCAM is reviewed in detail. We speculate the role of alcohol as a teratogen through retinoic acid at 8-10 weeks of gestation when fetal lungs are actively developing.
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Affiliation(s)
- Ema Kulwa
- Department of Obstetrics and Gynecology, Sloane Hospital for Women, Columbia University Medical Center of New York Presbyterian Hospital and College of Physicians and Surgeons of Columbia University, New York, N.Y. 10032, USA
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35
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Abstract
Congenital lung malformations are often discovered incidentally on routine prenatal sonography or postnatal imaging. Lesions such as congenital cystic adenomatoid malformations (CCAM), sequestrations, bronchogenic cysts and congenital lobar emphysema may be asymptomatic at birth or at the time of discovery later in life. Some authors advocate simple observation because of the lack of data on the incidence of long-term complications. However, there are very few described cases where CCAM and intralobar sequestration have remained asymptomatic throughout life; complications eventually develop in virtually all patients. The most common complication is pneumonia, which may respond poorly to medical treatment. Other complications include the development of malignancies (carcinomas and pleuropulmonary blastomas), pneumothorax and hemoptysis or hemothorax. Since lung resection will be required sooner or later for CCAM, intralobar sequestration and intrapulmonary bronchogenic cysts it is best not to wait for complications to occur. For patients diagnosed prenatally, we recommend surgery at 3 to 6 months of life at the latest, so that compensatory lung growth can occur. At this age the postoperative course is usually smooth and long-term follow-up has shown normal respiratory function. Mediastinal bronchogenic cysts also tend to become symptomatic and elective resection is recommended. On the other hand, asymptomatic congenital lobar emphysema may regress spontaneously and observation is warranted. The management of small noncommunicating extralobar sequestrations is more controversial; it is known that these lesions can remain asymptomatic throughout life but complications may develop and they are sometimes difficult to differentiate from neuroblastoma.
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Affiliation(s)
- Jean-Martin Laberge
- Division of Pediatric Surgery, The Montreal Children's Hospital, McGill University Health Center, Montreal, Quebec, Canada.
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36
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Herrero Y, Pinilla I, Torres I, Nistal M, Pardo M, Gómez N. Cystic Adenomatoid Malformation of the Lung Presenting in Adulthood. Ann Thorac Surg 2005; 79:326-9. [PMID: 15620971 DOI: 10.1016/s0003-4975(03)01655-2] [Citation(s) in RCA: 27] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 08/06/2003] [Indexed: 11/30/2022]
Abstract
Cystic adenomatoid malformation is an uncommon embryonic developmental abnormality usually diagnosed in neonates and infants. Its presentation in adulthood is rare, with only 27 cases reported up to now. Due to its rarity, it is seldom suspected and adult physicians are not familiar with its clinical and radiologic features. We report two cases of cystic adenomatoid malformation presenting in adults, one as a recurrent pneumonia, and another as a coincidental finding on a chest roentgenogram. We describe the clinical features, radiologic and computed tomographic findings, and the histopathologic characteristics in this article, along with a review of the literature.
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Affiliation(s)
- Yolanda Herrero
- Department of Radiology, Hospital Universitario La Paz, Madrid, Spain.
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37
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Stefanutti G, Filippone M, Tommasoni N, Midrio P, Zucchetta P, Moreolo GS, Toffolutti T, Baraldi E, Gamba P. Cardiopulmonary anatomy and function in long-term survivors of mild to moderate congenital diaphragmatic hernia. J Pediatr Surg 2004; 39:526-31. [PMID: 15065021 DOI: 10.1016/j.jpedsurg.2003.12.006] [Citation(s) in RCA: 62] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/30/2022]
Abstract
BACKGROUND/PURPOSE In the last decades, several studies regarding cardiopulmonary sequelae in survivors of congenital diaphragmatic hernia (CDH) have been published, but results often are conflicting, and controversies still exist. The aim of this study was to assess cardiopulmonary anatomic and functional outcome in a group of long-term survivors of CDH of mild to moderate degree. METHODS Twenty-four children aged 8.15 +/- 2.80 years underwent clinical examination with growth assessment, chest radiographs, echocardiography, pulmonary perfusion scintigraphy, static lung volumes measurement, and spirometry. RESULTS Mean Z scores of weight for age and height for age were within normal values. Echocardiography showed normal anatomy and function in all but 3 patients with isolated CDH, in whom minor alterations were detected. Mean perfusion to the affected side was significantly lower (45.16 +/- 5.30%; P <.0001) but still within normal range. Four children showed a substantial impairment of perfusion to the hernia side. The mean spirometric values and pulmonary volumes were normal. However, a mild restrictive pattern was evident in 6 children (27.3%), an obstructive pattern in 3 (13.6%), and a mixed obstructive and restrictive impairment in 1. CONCLUSIONS Hypoplastic lungs of mild to moderate CDH survivors continue to cause pulmonary morbidity in some children many years after the correction of the defect. In particular, lung perfusion appears to be impaired in 20% of the patients and pulmonary function in 45%, without any significant cardiac or developmental sequelae. The negative correlation between FEV1 and duration of ventilation at presentation (r = -0.49; P =.026) may be caused by the consequences of lung hypoplasia, but initial ventilatory management may contribute to increased pulmonary morbidity. Relationship between perfusion and FEF25-75 (r = 0.61; p = 0.004) could reflect an equivalent degree of reduction in the caliber of distal airways and pulmonary vascular tree.
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38
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Aziz D, Langer JC, Tuuha SE, Ryan G, Ein SH, Kim PCW. Perinatally diagnosed asymptomatic congenital cystic adenomatoid malformation: to resect or not? J Pediatr Surg 2004; 39:329-34; discussion 329-34. [PMID: 15017547 DOI: 10.1016/j.jpedsurg.2003.11.021] [Citation(s) in RCA: 116] [Impact Index Per Article: 5.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
Abstract
BACKGROUND Management of asymptomatic congenital cystic adenomatoid malformation (CCAM) is controversial. The natural history of untreated asymptomatic CCAM is unknown, although most surgeons recommend resection of these lesions to prevent future infection. The aim of this study was to determine the relative surgical risk of resection compared with the risk of observation for these patients. METHODS A retrospective review of hospital records between 1996 and 2002 in a tertiary care pediatric referral center was conducted. All perinatally (prenatal or neonatal) diagnosed CCAMs were included. In addition, patients presenting with late diagnosis of CCAM were also reviewed. RESULTS Forty-eight children had CCAM diagnosed perinatally. Thirteen of these were symptomatic and required surgery within 6 months; these were excluded from the analysis. Of the 35 asymptomatic infants, 6 were operated on electively before 6 months of age (median age, 4.5 months). The other 29 asymptomatic infants were followed up for more than 6 months. Of these, 9 remained asymptomatic and were eventually operated on electively (median age, 13 months). Three (10%) had CCAM infections at 7, 8, and 11 months of age and required resection. The remaining 17 children have not undergone resection and are still asymptomatic (median follow-up, 3 years). An additional 12 patients presented with a late diagnosis of CCAM. All of these presented with complications (infection or pneumothorax) and underwent resection (median age, 6 years). Overall, the complication rate after resection of an asymptomatic CCAM was not significantly different from those of resected CCAM that had already developed infection or pneumothorax (P =.64). CONCLUSIONS Ten percent of perinatally diagnosed asymptomatic patients had 5 complications requiring surgery during follow-up. The true incidence is probably higher given the relatively short follow-up in our series. Morbidity after resection of a complicated CCAM was not statistically significantly higher than after elective resection for an asymptomatic CCAM. Although conservative management of asymptomatic CCAM may be warranted, a more extended period of follow-up is necessary before this approach can be recommended.
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Affiliation(s)
- Dalal Aziz
- Hospital for Sick Children, Toronto, Ontario, Canada
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39
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López Represa C, Parra Parra MI, Remacha Esteras MA, Tabuyo Pizarro M. Fístula traqueoesofágica congénita, una causa rara de tos crónica en el adulto. Med Clin (Barc) 2004; 122:78. [PMID: 14733863 DOI: 10.1016/s0025-7753(04)74147-0] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
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40
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Sauvat F, Michel JL, Benachi A, Emond S, Revillon Y. Management of asymptomatic neonatal cystic adenomatoid malformations. J Pediatr Surg 2003; 38:548-52. [PMID: 12677563 DOI: 10.1053/jpsu.2003.50119] [Citation(s) in RCA: 99] [Impact Index Per Article: 4.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/11/2022]
Abstract
BACKGROUND/PURPOSE Although much is known about the prenatal course of cystic adenomatoid malformations (CCAM), the postnatal course of asymptomatic lesion is less well documented. The authors studied the pre- and postnatal course and treatment of asymptomatic CCAM. METHODS The authors reviewed the files of all patients referred to Necker-Enfants Malades hospital with an antenatal diagnosis of CCAM and asymptomatic at birth. RESULTS Files of 29 patients were studied. The first x-ray film was considered normal in 12 cases (41.3%). Computed tomography was normal in 4 cases and showed cystic lung malformations in the other patients. Postnatally, clinical manifestations occurred in 3 patients (10.3%). CCAM vanished in 6 cases. Surgical resection of CCAM was performed in 17 cases (58.6%). All the patients currently are asymptomatic. CONCLUSIONS CCAM can shrink or vanish during pregnancy and antenatal ultrasound findings are not predictive of the postnatal course. Thus, all infants with prenatal diagnosis of CCAM require postnatal evaluation. Normal radiographic findings at birth do not rule out CCAM persistence on CT. The treatment of asymptomatic CCAM is controversial. Surgery may be advocated because of the low morbidity and the prevention of late complications, above all, cancer. The surgical indications of small (<3 cm) and asymptomatic lesions should be discussed on a case-by-case basis with the parents.
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Affiliation(s)
- Frédérique Sauvat
- Department of Pediatric Surgery, Hospital Necker-Enfants Malades, Paris Cedex, France
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41
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Merkus PJFM. Effects of childhood respiratory diseases on the anatomical and functional development of the respiratory system. Paediatr Respir Rev 2003; 4:28-39. [PMID: 12615030 DOI: 10.1016/s1526-0542(02)00311-1] [Citation(s) in RCA: 20] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/24/2022]
Abstract
The anatomical and functional development of the lung appears especially vulnerable to a whole range of insults during gestation and the first few years of life. A significant proportion of adult lung disease originates in utero or early infancy. Most publications on this topic are descriptive retrospective studies. An important limitation of these is that structural changes may precede abnormalities in lung function and development of symptoms. Little is known with certainty with respect to the long-term effects of early insults to the respiratory system. Furthermore, the reversibility of the functional and/or structural defects is hardly ever adequately investigated and it is probably not correct to extrapolate findings from adult studies to paediatric pulmonary diseases. Promoting or facilitating optimal lung growth in fetuses and infants and reducing the incidence of lower respiratory tract infection in infancy may reduce the incidence of adult chronic lung disease in generations to come.
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Affiliation(s)
- Peter J F M Merkus
- Department of Paediatrics/Respiratory Medicine, Sophia Children's Hospital, Erasmus University Medical Centre Rotterdam, Rotterdam, The Netherlands.
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42
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Abstract
The child with recurrent chest infections presents the clinician with a difficult diagnostic challenge. Does the child have a simply-managed cause for their symptoms, such as recurrent viral respiratory infections or asthma, or is there evidence of a more serious underlying pathology, such as bronchiectasis? Many different disorders present in this way, including cystic fibrosis, a range of immunodeficiency syndromes, and congenital abnormalities of the respiratory tract. In some affected children, lung damage follows a single severe pneumonia: in others it is the result of inhalation of food or a foreign body. The assessment of these children is demanding: it requires close attention to the history and examination, and in selected cases, extensive investigations. Early and accurate diagnosis is essential to ensure that optimal treatment is given and to minimise the risk of progressive or irreversible lung damage. The aim of this chapter is to examine the causes of recurrent chest infections and to describe how this complex group of children should be assessed and investigated.
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Affiliation(s)
- Jon Couriel
- Respiratory Unit, Royal Liverpool Children's Hospital, Liverpool, UK
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