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.

Congenital pulmonary malformations in children in a pediatric hospital in Peru, 2010-2020.

BACKGROUND: Congenital pulmonary malformations (CPMs) are rare in children. This study aimed to describe the clinical, imaging characteristics, and treatment of patients with this pathology. METHODS: We conducted a descriptive and retrospective study with data from patients with CPMs diagnosed at Instituto Nacional de Salud del Niño-Breña (Lima-Peru), from January 2010 to December 2020. We described CPM clinical and imaging characteristics, type and treatment. RESULTS: The sample was formed of 70 patients. The median age was 29 months (range 15 days-14 years) and the male/female ratio was 1.4. The chest tomography found parenchymal involvement in 50 (71.4%) cases and mixed involvement (parenchymal and vascular) in 18 (25.7%) cases. Congenital malformation of the pulmonary airway was present in 39 (55.7%) cases, followed by bronchogenic cyst in 10 (14.3%), intralobar pulmonary sequestration in 9 (12.9%), and extralobar pulmonary sequestration in 7 (10%). Lobectomy was performed in 61 (87.1%) cases, cystectomy in 5 (7.1%), segmentectomy in 2 (2.9%), and embolization in 2 (2.9%). The most frequent post-operative complication was pneumonia, found in 9 (12.9%) cases. The median hospital stay was 26 days. No patient died during hospitalization. CONCLUSIONS: In our institution, the most frequent CPM was congenital malformation of the pulmonary airway, and lobectomy was the most frequently performed surgical procedure. CPMs represent a diverse group of disorders of lung development with varied imaging patterns and clinical manifestations.

INTRODUCCIÓN: Las malformaciones pulmonares congénitas son poco frecuentes en niños. El objetivo de este estudio fue describir las características clínicas, imagenológicas y tratamiento de los pacientes con esta patología. MÉTODOS: Se llevó a cabo un estudio descriptivo y retrospectivo con datos de los pacientes con malformaciones pulmonares congénitas diagnosticados en el Instituto Nacional de Salud del Niño-Breña (Lima-Perú) entre enero 2010 y diciembre 2020. Se describieron las características clínicas, imagenológicas, el tipo de malformación pulmonar congénita y el tratamiento. RESULTADOS: La muestra fue de 70 pacientes. La mediana de edad fue 29 meses, la relación masculino/femenino fue 1.4. En la tomografía de tórax se encontró compromiso parenquimal en 50 (71.4%) casos, y compromiso mixto (parenquimal y vascular) en 18 (25.7%). La malformación congénita de la vía aérea pulmonar se observó en 39 (55.7%) casos, seguida del quiste broncogénico en 10 (14.3%), secuestro pulmonar intralobar en 9 (12.9%) y secuestro pulmonar extralobar en 7 (10%). La lobectomía fue realizada en 61 (87.1%) casos, la quistectomía en 5 (7.1%), segmentectomía en 2 (2.9%) y embolización en 2 (2.9%). La complicación posquirúrgica más frecuente fue la neumonía en 9 (12.9%) casos. La mediana de estancia hospitalaria fue de 26 días. Ningún paciente falleció durante la hospitalización. CONCLUSIONES: En nuestra institución, la malformación pulmonar congénita más frecuente fue la malformación congénita de la vía aérea pulmonar, y la lobectomía el procedimiento quirúrgico más comúnmente realizado. Las malformaciones pulmonares congénitas representan un grupo diverso de trastornos del desarrollo pulmonar con variados patrones imagenológicos y manifestaciones clínicas.

Epidemiology and prevalence of pulmonary sequestration in Chinese population, 2010-2019.

BACKGROUND: Pulmonary sequestration (PS) is the second common congenital lung malformation and has been known for over 150 years. However, there is a scarcity of epidemiological studies on it. This study aimed to characterize the epidemiology of pulmonary sequestration in Chinese population in the recent decade by using a nationwide database. METHODS: Using data from the Chinese Birth Defects Monitoring Network during 2010-2019, the prevalence rates for PS were calculated by birth year, maternal age, residence area, geographical region, and infant sex. Variations in prevalence and changes over time were further examined. Other variables of interest for analysis included the pregnancy outcomes of affected infants, the prenatal diagnosis, and the co-occurring anomalies of PS cases. RESULTS: During the study period, we identified an average prevalence rate of 0.31, 0.11, and 0.42 per 10,000 live and still births for the isolated, non-isolated, and overall PS, respectively. An upward trend was observed for each category of PS. The prevalence rates varied significantly by maternal age (< 20 years, 0.34/10,000; 20-24 years, 0.33/10,000; 25-29 years, 0.45/10,000; 30-34 years, 0.46/10,000; ≥ 35 years, 0.36/10,000), residence area (urban vs. rural, 0.51/10,000 vs. 0.30/10,000), geographical region (western, 0.33/10,000; eastern, 0.49/10,000; central, 0.43/10,000), and by infant sex (male vs. female, 0.45/10,000 vs. 0.38/10,000). Non-isolated PS cases were more likely born prematurely than isolated cases (15.29% vs. 7.83%). 40.28% and 33.80% of non-isolated cases were accompanied by additional respiratory, and circulatory system malformations, respectively. CONCLUSIONS: The study presents for the first time the prevalence of pulmonary sequestration in Chinese population. The rising prevalence and relatively poor perinatal outcome of affected fetuses or newborns indicate the necessity to improve perinatal management of PS.

Incidence and outcomes of patients with congenital diaphragmatic hernia and pulmonary sequestration.

BACKGROUND: Bronchopulmonary sequestration (BPS) has long been identified in patients with congenital diaphragmatic hernia (CDH), however the reported incidence in the literature varies widely and is not based on large series. METHODS: Version 4 of the Congenital Diaphragmatic Hernia Study Group (CDHSG) included questions specifically identifying BPS-associated cases. The data were prospectively collected between 2015 and 2020. Clinical characteristics and outcomes for CDH+BPS patients were compared to patients without BPS using univariate and multiple regression analyses. RESULTS: Out of 2118 total patients, 72 had a pulmonary sequestration (3.4%). The amount of CDH+BPS with high-risk (CDHSG type C and D) defects was significantly greater than those without BPS (68.0% vs 49.3%, respectively; p = 0.001). The need for ECLS (35/72, 48.6%) and overall mortality (21/72, 29.2%) was significantly higher in CDH+BPS. When corrected for hernia size, cardiac and chromosomal anomalies, the need for ECLS (OR 2.2, p = 0.004) and mortality (OR 2.0, p = 0.015) was significantly greater in CDH+BPS. CONCLUSIONS: This is the largest series to look at the incidence of BPS in children with CDH. They are associated with larger defect sizes, a greater need for ECLS and higher mortality than those patients without BPS.

Congenital lung malformations : analysis of 27 cases.

INTRODUCTION: Congenital lung malformations (CLM) include a complex range of developmental abnormalities. Currently, most are diagnosed prenatally or during early childhood. OBJECTIVE: to investigate clinical and imaging findings of congenital lung malformations in children. METHODS: Retrospective study of CLM diagnosed between 2000 and 2017 at the pediatric and neonatology department of Hedi Chaker Hospital. Analysis of clinical spectrum, diagnosis tools, and radiological appearances. RESULTS: Twenty seven cases of CLM have been investigated: 8 congenital lobar emphysema, 8 congenital cystic adenomatoid malformation, 8 pulmonary sequestrations, 2 bronchogenic cysts, and 1 hybrid lesion. Five (18,5%) patients were diagnosed antenatally and 22 (81,4%) postnatally. Symptoms occurred at a mean age of 9 months: Respiratory distress (11 cases), wheezing (4 cases), and pneumonia (7 cases). Antenatal ultrasound features include echogenic masses within the chest (2cases), the presence of cysts (2cases), unilateral pleural effusion (1 case) and hydramnios (2cases). A computed tomography scan was performed in all patients with a radio-histological concordance of 96%.

[Pulmonary intralobar sequestration in adults: Evolution of surgical treatment]. / Séquestrations pulmonaires intralobaires de l'adulte : évolution du traitement chirurgical.

BACKGROUND: Until now, the traditional procedure to treat intralobar pulmonary sequestration (ILS) in adults has been a lobectomy performed by open thoracotomy. We have reviewed our data to observe if the surgical management of these lesions has evolved over the last years. METHODS: We retrospectively reviewed the records of the patients who were operated for an ILS either by posterolateral thoracotomy (PLT group), or by thoracoscopy (TS group) between 2000 and 2016. RESULTS: Eighteen patients were operated for a SIL during this period. Prior to 2011, all resections were performed by thoracotomy (n=6) and after 2011 the surgical approach was either a thoracotomy (n=5) or a thoracoscopy (n=7). There was one conversion because of dense pleural adhesions and this patient was integrated in the PLT group for further analysis. ILS were more frequently encountered on the left side (n=12, 66.6 %) than on the right one (n=6, 33.3 %) and exclusively in the lower lobes. All patients of the PLT group underwent a lobectomy. In the TS group, 5 patients underwent a sublobar resection (2 segmentectomiesS9+10, 1 basilar segmentectomy and 2 atypical resections). There was no mortality. In the PLT group, 5 patients (45 %) had complications versus one patient (14 %) in the TS group. The mean hospital stay was 7.4 days in the PLT group versus 5.4 days in the TS group. CONCLUSIONS: These data confirm that ILS can be safely treated by a sublobar resection that should be performed, whenever possible, thoracoscopically.

Pulmonary sequestration in adults: a retrospective review of resected and unresected cases.

BACKGROUND: Pulmonary sequestration (PS) is a form of congenital pulmonary malformation that is generally diagnosed in childhood or adolescence and usually resected when diagnosed. We aim to identify the clinical presentation and course of patients diagnosed to have PS during adulthood. METHODS: Using a computer-assisted search of Mayo clinic medical records, we identified adult patients with PS diagnosed between 1997 and 2016. Clinical and radiological data were collected including postoperative course for those who underwent surgical resection. RESULTS: We identified 32 adult patients with PS; median age at diagnosis was 42 years (IQR 28-53); 17 patients (53%) were men. The median sequestration size was 6.6 cm (IQR 4.4-9.3). The type of sequestration was intralobar in 81% and extralobar in 19%. The most common location was left lower lobe posteromedially (56%). Forty-seven percent of the patients presented with no relevant symptoms. The most common radiographic finding was mass/consolidation in 61% and the most common feeding artery origin was the thoracic aorta (54%). Surgical resection was performed in 18 patients (56%) and postoperative complication was reported in 5 patients (28%). There was no surgical mortality. Median duration of follow-up after diagnosis for unresected cases, most of whom were asymptomatic, was 19 months (IQR 4-26) with no complications related to the PS reported. CONCLUSIONS: Nearly one-half of adult patients with PS present with no relevant symptoms. The decision regarding surgical resection needs to weigh various factors including clinical manifestations related to PS, risk of surgical complications, comorbidities, and individual patient preferences.

Bronchopulmonary sequestrations in a paediatric centre: ongoing practices and debated management.

OBJECTIVES: Bronchopulmonary sequestration (BPS) is the second most common congenital lung malformation, with an estimated incidence ranging from 0.15% to 1.8%. Surgical treatment is elective in patients with symptoms, but the management of asymptomatic patients remains controversial. METHODS: We retrospectively reviewed the medical records of 99 patients treated for BPS in our institution from January 2000 to December 2015. BPS was diagnosed prenatally in 86 (87%) cases. Management throughout this 16-year period was based on 3 interventions: resection by open surgery, resection by thoracoscopy and embolization. RESULTS: Among the 86 patients with a prenatal diagnosis of BPS, 14% had symptoms at birth and 10% had delayed symptoms at a median delay of 8 months (4.5-42 months). For the other 13 patients, symptoms occurred at a median age of 34 months (range 3-96 months). Embolization of the feeding vessel was performed in 46 patients with 6 secondary surgical resections (13%). A total of 59 patients were operated on: 23 cases by open surgery and 36 cases by thoracoscopy. The mean hospitalization stay was significantly longer for open surgery: 4.8 ± 1.3 days vs 4.1 ±1.5 days, respectively (P = 0.03). Differences in hospitalization stay were also found between asymptomatic and symptomatic patients: 3.5 ± 1.2 vs 5.1 ±1.6 days, respectively (P = 0.002). Two of the operated patients died. CONCLUSIONS: When surgery is chosen, thoracoscopy appears to be a valuable procedure. A better understanding of the natural history of BPS is still needed to define the optimal management and the respective roles of surgery, embolization or non-interventional follow-up.

Malformaciones congénitas pulmonares diagnosticadas en un periodo de 10 años / Congenital Pulmonary Malformations Diagnosed Over a Period of 10 Years

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A case of concomitant intralobar bronchopulmonary sequestration and situs inversus totalis.

BACKGROUND AND AIMS: Situs inversus is a rare congenital abnormality involving partial or complete transposition of the thoracic or abdominal viscera. In situs inversus totalis, both the thoracic and abdominal viscera are transposed. The incidence of this condition is 0.01% to 0.02%. Bronchopulmonary sequestration (BPS) is a rare congenital abnormality of the respiratory tract with an incidence of 0.15% to 1.80%. Intralobar sequestration is uncommonly associated with congenital anomalies. METHODS: A routine chest X-ray of a 41-year-old asymptomatic man showed dextrocardia, a left-sided liver, right-sided stomach, and left paracardiac opacity. RESULTS: Computed tomography (CT) and CT pulmonary angiography revealed dextrocardia with situs inversus totalis and left paracardiac intralobar BPS in the lingular segment of the left upper lobe. CONCLUSION: We present a rare case of combined situs inversus totalis and intralobar BPS with an atypical location and feeder artery.

Secuestro pulmonar: serie clínica de 16 pacientes / Pulmonary sequestration: clinical report of 16 patients

Objetivo: Describir los hallazgos clínicos de pacientes tratados por secuestro pulmonar en el Hospital de Niños Roberto del Río, entre los años 2000 y 2012. Métodos: Estudio descriptivo retrospectivo. Se revisaron las fichas clínicas de todos los pacientes atendidos en el Hospital entre el año 2000 y 2012 con diagnóstico de secuestro pulmonar confirmado por anatomía patológica. Resultados: Se registraron 16pacientes, 8 niños y 8 niñas. Del total 9 fueron secuestros extralobares y 7 intralobares. La sospecha diagnóstica fue por imagen de neumonía persistente en el 44 por ciento de los casos, distrés respiratorio neonatal en el 25 por ciento, ecografía antenatal en el 19 por ciento y neumonía repetida en el 12 por ciento. El 75 por ciento de los secuestros estaba ubicado en el lóbulo inferior izquierdo, el 19 por ciento en lóbulo inferior derecho y el 6 por ciento en lóbulo superior derecho. La técnica quirúrgica de elección fue la toracotomía abierta. El 56 por ciento de los pacientes se operó antes del año de vida (31,5 por ciento antes de las 2 semanas de vida). La evolución postoperatoria fue buena en el 69 por ciento de los casos, mientras que un 12,5 por ciento presentó atelectasia, 12,5 por ciento fístula broncopleural y 6 por ciento neumotórax residual. La mediana de estadía hospitalaria fue de 9 días. No se registró mortalidad. Conclusiones: Se presenta la experiencia de esta patología en el Hospital Roberto del Río enfatizándose la importancia de la sospecha diagnóstica.

Objective: To describe our experience with sixteen children treated for pulmonary sequestration at the Children 's Hospital Roberto del Rio, between 2000 and 2012. Method: We reviewed retrospectively the clinical charts of all those patients up to 15 years old, who were treated for pathologically proven pulmonary sequestration in our hospital, between 2000 and 2012. Results: Sixteen patients were recorded, 8 boys and 8 girls that were diagnosed and/or treated between the newborn period and 14 years old. Nine of them had extralobar sequestration and 7 intralobar. The diagnosis was suspected for persisting pneumonia in 44%, neonatal respiratory distress in 25%, prenatal ultrasound in 19%, and recurrent pneumonia in 12%. Seventy-five percent of sequestrations were located in the left lower lobe, 19% in the right lower lobe and 6% in the right upper lobe and all of them were operated via thoracotomy. Fifty-six percent underwent surgery before one year of age (31.5% before 2 weeks of life) and 69% of patients did well postoperatively. Postoperative atelectasis were detected in 12.5%, bronchopleural fistula in 12.5% and residual pneumothorax in 6%. The average hospital stay was 9 days and no mortality was registered. Conclusions: Our experience is similar to the previously reported series and emphasizes the importance of early diagnosis in the treatment of pulmonary sequestration in children.

Atypical mycobacterial infection in sequestrated lung in an infant presenting with chronic pneumonitis and recurrent wheezing.

An infant was referred to us for persistent cough and recurrent wheezing. Chest X-ray showed an abnormal shadow in the right lower lobe. A volumetric contrast-enhanced computerized tomography (CT) scan of the chest revealed an abnormal blood vessel extending from the descending aorta to the right lower lobe and anomalous venous drainage from the right lower lobe into inferior vena cava. Angiography confirmed the CT scan findings. Pulmonary sequestration associated with scimitar syndrome was subsequently diagnosed. Patient underwent right lower lobectomy and right inferior pulmonary vein was anastomosed into the left atrium. Pathological examination after surgery revealed epitheloid granuloma in the sequestrated lung parenchyma and mycobacteria in the draining lymph nodes. This is a rare case of pulmonary sequestration associated with scimitar syndrome and complicated by mycobacterial infection.

Imaging of extralobar pulmonary sequestration in a patient with tetralogy of Fallot with pulmonary atresia, ventricular septal defect and multiple aorto-pulmonary collateral arteries with multidetector computed tomography.

Complex pulmonary vascular blood supply is common in patients with tetralogy of Fallot with pulmonary atresia, major systemic to pulmonary collateral arteries and hypoplastic or deficient central pulmonary arteries. An extralobar lung sequestration, which has not been described previously in these patients, was imaged in a 6-week-old infant with multidetector computed tomography with sub-millimeter resolution. Arterial and venous vessels were analyzed using three-dimensional vascular exploration tools and results were confirmed with cardiac catheterization.

Malformación adenomatoidea quística pulmonar: actualización de la entidad a propósito de cinco casos / Cystic adenomatoid malformations of the lung: presentation of 5 cases and review

Presentamos una serie de cinco casos de malformación adenomatoideaquística pulmonar, recogidos en el período 1994-2006. Es una entidad poco frecuente (aproximadamente 400 casos publicados)que tiene características morfológicas de inmadurez pulmonar. Todos los pacientes estudiados fueron tratados quirúrgicamente en nuestro centro. Revisamos la evolución del proceso, los hallazgos histológicos, la clasificación actual de la entidad y los aspectos más novedosos del diagnóstico y tratamiento quirúrgico prenatal. Tres de nuestros pacientes eran varones, recién nacidos a término, y los otros dos pacientes eran niñas, de 3 y 9 años. En todos ellos los hallazgos radiológicos mostraban imágenes quísticas limitadas a un lóbulo, ocupados por aire o por líquido. Tras la lobectomía en el estudio histológico, cuatro de ellas fueron clasificadas como tipo 2 de la clasificación de Stoker y una (la paciente de más edad) como tipo 1 (AU)

We describe five cases of cystic adenomatoid malformations of the lung collected from our files from 1994 to 2006. It is an unusual malformation and has features of immaturity with less than 400cases previously published. All patients were surgically treated in our institution. We review the clinical outcome, microscopic findings, current classification schemes and prenatal surgical treatment. Our study includes 3 new-born males and two girls, 3 and 9 years old. All of them radiographically showed air or fluid filled cystic masses involving a single lobe. After lobectomy, four lung specimens were classified as Stoker type 2 lesions and the older one as type 1 (AU)

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.

Secuestro pulmonar: 8 casos / Bronchopulmonary sequestration: 8 cases

Introducción: El secuestro pulmonar, malformación congénita caracterizada por tejido pulmonar embrionario quístico no funcionantecon vascularización de una arteria sistémica anómala, es poco conocido por su baja incidencia. Se clasifican en intralobares (75 por ciento) y extralobares (25 por ciento). Son frecuentes a izquierda y en lóbulos inferiores (60 por ciento- 90 por ciento). La mayoría presenta neumopatías inflamatorias repetidas en un mismo lugar. Objetivo: Describir características de secuestros pulmonares operados. Material y Método: Estudio descriptivo-retrospectivo. Período enero 1988 - junio 2003. La información se obtuvo de fichas clínicas y registro de biopsias. Se describen edad, sexo, características clínicas, métodos diagnósticos, tratamiento quirúrgico, evolución y hallazgos anatomopatológicos. Resultados: Total 8 pacientes, 6 mujeres y 2 hombres. Edad promedio 22 años, rango: 2-63, mediana: 14. La presentación fue procesos infecciosos en 5 casos, desgarro hemoptoico en 1 y 2 pacientes asintomáticos. Los síntomas y signos son inespecíficos. Uno presento otra malformación congénita. La radiografía y la tomografía axial computada presentaron imágenes inespecíficas. En 1 caso se realizó aortografía. Se planteó diagnóstico preoperatorio en 3 pacientes. el pulmón y los lóbulos más afectados fueron derechos e inferiores respectivamente. el intralobar fue más frecuente en 7 casos. Se realizó lobectomía con identificación del vaso sistémico, que se originó en aorta torácica con más frecuencia y fue único en todos. No hubo morbimortalidad. En el seguimiento están todos asintomáticos. Conclusión y Discusión: Los secuestros pulmonares son infrecuentes, se presentan generalmente en jóvenes como neumopatías inflamatorias a repetición y tienen excelente pronóstico postoperatorio.

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.

Pulmonary sequestration: a review of 26 cases.

OBJECTIVES: Pulmonary sequestration is a continuum of lung anomalies for which no single embryonic hypothesis is yet available. The aim of this study was to assess the diagnostic tools and treatment for the rare condition, pulmonary sequestration, in an unspecialised centre. METHODS: We performed an analysis of 26 cases of pulmonary sequestration (paediatric and adult) operated at the Centre Hospitalier Universitaire Vaudois between May 1959 and May 1997. A review of the extralobar and intralobar types of sequestrations is discussed. Angiography is compared to other diagnostic tools in this condition, and treatment is discussed. RESULTS: Twenty-six cases of pulmonary sequestrations, a rare congenital pulmonary malformation, were operated on in the defined time period. Seventy-three percent (19) of the cases were intralobar and 27% (seven) extralobar. Extralobar localisation was basal in 71% and situated between the upper and the lower lobe in 29%. In six cases, the diagnosis was made by exploratory thoracotomy. In the other 20 cases, diagnosis was evoked on chest X-ray and confirmed by angiography. Lobectomy (46%) was the most common treatment procedure. Segmental resection was performed in 30% of the cases and bilobectomy in 4%. Post-operative morbidity was low. The most significant complications were pleural empyema, haemothorax and haemopneumoperitoneum in case of extralobar sequestration. There was no evidence of metaplasia or pre-neoplastic changes. CONCLUSIONS: Despite its rarity, some radiological features are sufficiently suggestive of diagnosis of pulmonary sequestration. Investigations are necessary in order to avoid unexpected pathology at the time of operation. Resection of the involved lung leads to excellent results and the long-term outcome is highly favourable.

Congenital malformations of the lung and mediastinum--a quarter century of experience from a single institution.

Congenital malformations of the lung are rare and vary widely in their presentation and severity. The authors reviewed 25 years of experience with specific reference to diagnosis, treatment, and outcome. From July 1970 to June 1995, 70 patients were diagnosed with congenital malformations of the lung, which included sequestration (n = 20), foregut anomalies (n = 20), congenital lobar emphysema (CLE; n = 10), congenital cystic adenomatoid malformation (CCAM; n = 5), benign lung cysts (n = 6), lung aplasia/ hypoplasia (n = 4), and other miscellaneous disorders (n = 5). All patients with pulmonary hypoplasia presented at birth. With the exception of one patient, infants with CCAM and CLE presented before 5 months of age. All other patients presented from birth to 16 years of age. A prenatal diagnosis was accurate in two patients. Although prompt surgical management is the rule, the exceptions were children with bilateral lung involvement. Corrective surgery was delayed in some patients in whom extended respiratory support was required or in whom the delay led to an increase in pulmonary reserve. Extracorporeal membrane oxygenation (ECMO) was used in two patients pre- and postoperatively to manage persistent pulmonary hypertension. This review, representing the largest series of congenital lung lesions, showed that (1) prenatal diagnosis is useful but generally does not change the outcome; (2) computerized tomography (CT) is the optimum postnatal diagnostic imaging modality if chest radiography is not definitive; (3) ECMO can be an adjunct in treating associated pulmonary hypertension; (4) pulmonary resection (lobectomy) is the surgical procedure of choice for most lesions; (5) expected survival is good; and (6) pulmonary hypertension is the most common cause of mortality.