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CLINICAL PROFILE AND SPECTRUM OF CONGENITAL LUNG MALFORMATIONS IN A TERTIARY CARE HOSPITAL- AN

OBSERVATIONAL STUDY Dissertation submitted to

THE TAMILNADU DR .M.G.R.MEDICAL UNIVERSITY, CHENNAI

with Partial fulfillment of the regulations for the award of the Degree of

MD PAEDIATRICS (BRANCH VII)

INSTITUTE OF CHILD HEALTH AND HOSPITAL FOR CHILDREN MADRAS MEDICAL COLLEGE

CHENNAI MAY2020

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CERTIFICATE

This is to certify that the dissertation titled “CLINICAL PROFILE AND SPECTRUM OF CONGENITAL LUNG MALFORMATIONS IN A TERTIARY CARE HOSPITAL -AN OBSERVATIONAL STUDY” submitted by Dr.N.G.KAVYA KEERTHANA to the Faculty of Pediatrics, THE TAMILNADU DR.M.G.R. MEDICAL UNIVERSITY, CHENNAI in partial fulfillment of the requirements for the award of M.D.,DEGREE (PEDIATRICS) is a bonafide research work carried out by her under our direct supervision and guidance.

PROF.Dr.R.JAYANTHI.

M.D.FRCP(Glasg), The DEAN,

Madras Medical College &

Rajiv Gandhi Govt. General Hospital, Chennai – 600 003.

PROF.Dr.S.ELILARASI MD., DCH,

Director & Superintendent, Institute of Child Health &

Hospital for Children, Chennai – 600 008.

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CERTIFICATE BY THE GUIDE

This dissertation entitled “CLINICAL PROFILE AND SPECTRUM OF CONGENITAL LUNG MALFORMATIONS IN A TERTIARY CARE HOSPITAL- AN OBSERVATIONAL STUDY" is a bonafide work done by Dr.N.G.KAVYA KEERTHANA at Institute of Child health Madras medical college Chennai during the academic year 2017-2020 under the guidance of Prof.Dr.S.ELILARASI, MD., DCH, Professor of Pediatric Pulmonology, Director and Superintendent, Institute of Child Health & Hospital for children, Chennai – 600 008. This dissertation submitted to The Tamil Nadu Dr.M.G.R. Medical University, Chennai towards partial fulfillment of the rules and regulations for the award of M.D., Degree in Paediatrics, Branch(VII).

PROF.Dr.S.ELILARASI, MD.,DCH Professor of pediatric Pulmonology,

Director & Superintendent,

Institute of child health & Hospital for children, Chennai- 600 008.

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DECLARATION

I Dr.N.G.KAVYA KEERTHANA solemnly declare that the dissertation titled

“CLINICAL PROFILE AND SPECTRUM OF CONGENITAL LUNG MALFORMATIONS IN A TERTIARY CARE HOSPITAL- AN OBSERVATIONAL STUDY” has been prepared by me.

This is submitted to the Tamil Nadu DR.M.G.R Medical University, in partial fulfillment of the rules and regulations for the M.D Degree examination in paediatrics.

Place : Chennai Dr.N.G.KAVYA KEERTHANA Date :

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SPECIAL ACKNOWLEDGEMENT

My sincere thanks to PROF. Dr.R.JAYANTHI. M.D.FRCP (Glasg), Dean, Madras Medical College, for allowing me to do this dissertation, utilizing the institutional facilities.

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ACKNOWLEDGEMENT

It is with immense pleasure and privilege, I express my heartful gratitude, admiration and sincere thanks to PROF. Dr. S.ELILARASI M.D., DCH., Director &

Superintendent, Institute of Child Health &Hospital for Children,

who has supported me throughout my thesis with her patience and knowledge.

I gratefully acknowledge and sincerely thank PROF.Dr.J.RUKMANI MD,Dch., Professor of paediatrics and PROF.Dr.K.KUMARASAMY MD.DCH,DNB ., Professor of paediatrics for guidance and encouragement while undertaking this study.

I am greatly indebted to my research supervisor and teacher, Dr.B.SARATH BALAJI,MD.,DCH., Assistant Professor of Paediatric pulmonology for his advice, supervision, and crucial contribution, which made him a backbone of this thesis.

I would like to thank to my Assistant Professors Dr.V.ARUNAGIRINATHAN MD, DTCD., Dr.V.PRABU, M.D, DCH., Dr.S.PERUMAL PILLAI M.D, DCH., Dr.HARSHITHA CHANDRAMOULI, M.D, DCH., for their valuable suggestions and support.

I would like to thank my Paediatric Surgery Department, Radiology Department and Pathology Department for their contribution in the thesis.

I also thank all the members of the Dissertation Committee for their valuable suggestions.

I gratefully acknowledge the help and guidance received from Dr.S.SRINIVASAN, DCH., Registrar at every stage of this study.

I also express my gratitude to all my fellow postgraduates for their kind cooperation in carrying out this study and for their critical analysis.

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I thank the Dean and the members of Ethical Committee, Rajiv Gandhi Government General Hospital and Madras Medical College, Chennai for permitting me to perform this study. I thank all the parents and children who have ungrudgingly lent themselves to undergo this study without whom, this study would not have seen the light of the day.

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CERTIFICATE – II

This is to certify that the dissertation work titled “. CLINICAL PROFILE AND SPECTRUM OF CONGENITAL LUNG MALFORMATIONS IN A TERTIARY CARE HOSPITAL- AN OBSERVATIONAL STUDY” of the candidate Dr.N.G.KAVYA KEERTHANA with registration number 201717007 for the award of M.D. PAEDIATRICS in the branch of VII. I personally verified the urkund.com website for the purpose of plagiarism check. I found that the uploaded thesis file contains from introduction to conclusion pages and the result shows 18 percentage of plagiarism in the dissertation.

Guide And Supervisor Sign With Seal

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CONTENTS

SL.NO. TITLES PAGE.NO.

1 INTRODUCTION 1

2 REVIEW OF LITERATURE 27

3 STUDY JUSTIFICATION 40

4 AIM & OBJECTIVE OF THE STUDY 41

5 MATERIALS & METHODS 42

6 RESULTS 45

7 DISCUSSION 70

8 LIMITATIONS 72

9 SUMMARY 73

10 CONCLUSION 75

11 BIBLIOGRAPHY 77

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ANNEXURES

 Proforma

 Patient Information sheet

 Informed Consent form

 Patient Information Sheet (Tamil)

 Informed Consent Form (Tamil)

 Master Chart

84

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ABBREVIATIONS

1.CTM- Congenital thoracic malformation 2.CLM -Congenital lung malformation

3.CCAM- Congenital Cystic adenomatoid malformation 4.CPAM -Congenital Pulmonary Airway Malformation 5.CLE -Congenital lobar emphysema

6.BPS -Broncho Pulmonary Sequestration 7.BC -Bronchogenic cyst

8.VATS- Video assisted thoracoscopic surgery 9.CT-Computed tomography

10.MRI-Magnetic resonance imaging 11.USG-Ultrasonogram

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INTRODUCTION

Congenital Thoracic Malformations are uncommon and varied in their presentation.(1) However, all those who are concerned about infants and children must have an appreciation for the diagnosis and treatment of these abnormalities because the potential consequences can be life threatening. In order to understand the pathophysiology of these malformations, a basic understanding of lung development, respiratory physiology and anatomy is required and these will be discussed.

EMBRYOLOGY OF LUNG DEVELOPMENT

When the embryo is approximately 4 weeks old, the respiratory diverticulum develops as an outgrowth from ventral wall of foregut, which forms the primordium of the respiratory system.(2)This is mainly endodermal in origin; though, cartilaginous and muscular elements will be derived from the splanchnic mesoderm that surrounds the primitive foregut. As the respiratory diverticulum grows caudad, it becomes separated from the foregut by the lateral esophageal ridges , which fuse to form a septum at the end of fourth gestational week. Thus, the dorsal esophagus and the more ventral trachea and lung buds are defined. The larynx, formed from the fourth and sixth branchial arches, maintains communication between the pharynx and trachea.

The lung buds penetrate the coelomic residual spaces becoming the primitive pleural cavities.

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Fetal lung development is divided into five stages; embryonic, pseudoglandular, canalicular, saccular and alveolar

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EMBRYONIC STAGE

The embryonic phase of lung development begins with the formation of a groove in the ventral lower pharynx, the sulcus laryngotrachealis. . After a couple of days - from the lower part - a bud forms, the true lung primordium. In the further subdivision into the two main bronchi the smaller bud on the left is directed more laterally than the on the right which is directed more caudally. The subsequent divisions also take place unequally in that on the right three further buds form and, on the left, only two, corresponding to the later pulmonary lobes. In the next division step, the segments of the individual pulmonary lobes arise.

PSEUDO GLANDULAR STAGE

Pseudo glandular stage occurs between 7 and 16 weeks. This stage is called airway phase because bronchial air ways develop during this time. The entire air-conducting bronchial tree up to the terminal bronchioli are set down in this phase. The primordial system of passages, the air-conducting bronchial tree, is initially coated by cubic epithelium. These are the precursor cells of the ciliated epithelium and of the secretory cells.

1 2 3

Lung mesenchyma Type II pneumocytes Capillaries

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CANALICULAR STAGE

Canalicular stage occurs between 16-24 weeks. This stage is called airspace phase, because alveolar air spaces develop. The canaliculi branch out of the terminal bronchioli. The canaliculi compose the proper respiratory part of the lungs, the pulmonary parenchyma. All of the air spaces that derive from a terminal bronchiolus form an acinus. Each one comprises respiratory bronchioli and the alveolar ducts and later the alveolar sacculi. Along the acinus, which develops from the terminal bronchiolus, an invasion of capillaries into the mesenchyma occurs. The capillaries surround the acini and thus form the foundation for the later exchange of gases. From the cubic type II pneumocytes develop the flattened type I pneumocytes.

1 2 3

Type I pneumocytes Type II pneumocytes Capillaries

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A sufficient differentiation of the type II pneumocytes into the type I pneumocytes and the proliferation of the capillaries into the mesenchyma marks an important step towards the fetus being able to survive outside the uterus after roughly the 24th week of pregnancy.

SACCULAR STAGE

Saccular stage occurs between 24-36 weeks, whole clusters of sacs form on the terminal bronchioli, which represent the last subdivision of passages that supply air.

At the end of each respiratory tract passage smooth walled sacculi form, coated with type1 and type 2 pneumocytes.

1 2 3

Type I pneumocyte Type II pneumocyte Capillaries

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ALVEOLAR STAGE

Alveolar stage occurs from birth up to 8 years of age. Extensive alveolar maturation and multiplication then takes place from birth until approximately 8 years of age, with a 10- fold increase in the number of functioning alveoli.

1 2 3 4 5 6

Alveolar duct Primary septum Alveolar sac

Type I pneumocyte Type II pneumocyte Capillaries

1 2 3 4 5 6

Alveolar duct Secondary septum Alveoli

Type I pneumocyte Type II pneumocyte Capillaries

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Pulmonary vascular development follows the stages of airway and cavity by caudal growth ,resulting in the formation of pleuroperitoneal canals on either side of the foregut. The term acinus describes the functional unit of the lung that includes the respiratory bronchioli, alveolar ducts, and alveoli- all structures that evolve during or after the canalicular phase of lung development. Vascular development in this region proceeds concurrently with alveolar growth and multiplication. The preacinar structures include the trachea, major bronchi, and lobar bronchi up to the terminal bronchioles. Preacinar vascular development is completed by 16 weeks’ gestational age(3)

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FACTORS GOVERNING LUNG DEVELOPMENT

It is now recognized that pulmonary development is marked by a series of programmed events regulated by master genes such as the homeobox genes, nuclear transcription factors, hormones, and growth 6factors. These processes involve genes regulating epithelial and endothelial interactions as well as temporal and spatial interactions if several hormones and growth factors. Early development transcription factors such as hepatocyte nuclear factor-3β and thyroid transcription factor-I regulate pulmonary development from the foregut mesenchyme. Additional stimuli of pulmonary development involve the transforming growth factor- β pathway, sonic hedgehog pathway, Notch-delta pathway, Wingless-Int pathway, and cytokine receptor pathways.

Subsequent signal transduction control of organogenesis includes the apoptotic pathways, nuclear receptor pathways, and interleukin pathways. Hormones such as the glucocorticoids, thyroid hormone, and retinoic acid have been shown to regulate several of the crucial cellular interactions required for proper pulmonary organogenesis and differentiation(4)(5)(6)

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PRENATAL DIAGNOSIS

Improvements of ultrasound technology and the extensive use of antenatal ultrasound have led to an increased detection of CTMs in utero, resulting in a better perceptive of the natural history of some of these anomalies and allowing arrangements to be made for delivery and postnatal management, or in severe cases for even prenatal treatment.

Depending on its nature and size, a congenital abnormality may show increased or decreased echogenicity or have a cystic appearance; a common manifestation is a unilateral bright, echogenic lesion, due to either a CCAM, a pulmonary sequestration, a CLE, or airway atresia. However, CLE represents one of the few CTMs that are not readily diagnosed antenatally. Exact prenatal diagnosis of a pulmonary sequestration requires visualization of a systemic feeding artery, which may be identified by Doppler ultrasound.[7] Larger lesions may compress the ipsilateral lung or via mediastinal shift even the contralateral lung, thus causing lung hypoplasia. Compression of the esophagus may cause polyhydramnios; the resulting uterine distension may induce premature labor. Impairment of cardiac return due to caval vein and cardiac compression leads to fetal hydrops with ascites, pleural and pericardial effusions, and skin and scalp edema. Sometimes CCAM can be hard to distinguish from congenital diaphragmatic hernia in a newborn with respiratory distress.

Quite the reverse, the diagnosis of smaller lesions may be delayed until infancy, school age, or even adolescence; though, many of them are diagnosed within the first 2 years of life.[8] Differential diagnoses also include potentially lethal diseases such as thoracic neuroblastoma and pleuropulmonary blastoma.[9] [10] Unfortunately, with similar appearances of different congenital lung and non pulmonary malformations, a definitive

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diagnosis cannot usually be established antenatally with absolute certainty by ultrasound (11)

Antenatal ultrafast magnetic resonance imaging is a valuable adjunct to prenatal ultrasound in the evaluation of chest masses. It may provide further information on the nature of CTMs with similar ultrasound appearances, may help to differentiate lung lesions from extra thoracic abnormalities such as congenital diaphragmatic hernia, and is most useful in the evaluation of large or atypical masses. Magnetic resonance imaging may also be helpful in determining lung volume, which has a prognostic implication for postnatal pulmonary function. In addition, this technique may be useful in planning both in utero interventional procedures and immediate postnatal surgery. (12) (13)

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PATHOPHYSIOLOGY OF FETAL LUNG LESIONS

Huge fetal lung lesions have reproducible pathophysiological effects on the developing fetus. Esophageal compression by the thoracic mass causes interference with fetal swallowing of amniotic fluid and results in polyhydromnios. Polyhydromnios is common maternal condition for pre natal ultrasonography .So, a prenatal diagnostic marker exists for large fetal lung tumors. Support for this concept comes from the absence of fluid in the fetal stomach in some of these cases and the alleviation of fetal hydramnios after effective fetal treatment. The hydrops is secondary to venacaval obstruction and cardiac compression from large tumors causing extreme mediastinal shift. Like CCAMs, a fetal BPS can also cause fetal hydrops, either from mass effect or from tension hydrothorax that is the result of fluid or lymph secretion from BPS(14).CCAM and BPS form important causes of non-immune hydrops fetalis.

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CONGENITAL PULMONARY AIRWAY MALFORMATIONS

After antenatal detection of a malformation serial ultrasound monitoring is recommended. CTMs may change in size and/or form during pregnancy, but the growth pattern is quite unpredictable. There is an ample spectrum of clinical severity, with a relationship between the size of the malformation and attendant lung hypoplasia and the occurrence of pulmonary hypertension in the newborn period. The majority of fetuses with CTM have a good outcome, and an initially large lesion does not necessarily correlate with a poor prognosis. However, development of hydrops is linked with an extremely high risk of fetal or neonatal death. Fetuses at a high risk of hydrops need close ultrasound monitoring. To better understand the natural history of CCAM, some authors developed a sonographic measure of CCAM volume normalized for gestational age ( “CCAM volume ratio”) and reported it to be useful as an predictor of the risk of hydrops. The fastest rate of growth was noted between 20 and 25 weeks, with a peak of the CCAM volume ratio at 25 weeks. Others recommended a combination of polyhydramnios, fetal hydrops, and a low “normal lung to thorax transverse area ratio”

as predictor of an increased probability of mortality or severe respiratory difficulty. In contrast to CCAM, pulmonary sequestrations appear to have a more favorable outlook, causing hydrops only due of a tension hydrothorax. Because diverse congenital lesions differ in their natural histories and prognoses, a correct diagnosis is a prerequisite for providing appropriate management and parental counseling. However, despite the use of serial ultrasound scans and magnetic resonance imaging this may not be possible in several cases. In the presence of an associated life-threatening anomaly termination of the pregnancy will frequently be chosen. Many lesions (both CAM and pulmonary

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sequestrations) show a decrease in size over time and some even entirely disappear on serial prenatal ultrasound scans toward the end of pregnancy. Thus all patients with antenatally detected CTM require postnatal evaluation, and documentation of true resolution be confirmed, preferably by CT scans.

CCAM VOLUME RATIO:

A CCAM volume ratio(CVR) is obtained by dividing CCAM volume by head circumference to correct for fetal size. ACVR greater than 1.6 is predictive of increased risk of hydrops, with 80% of these fetuses developing hydrops. The CVR may be useful in selecting fetuses at risk of hydrops and thus needing close ultrasound observation and possible fetal intervention(15). Serial CVR measurements have shown that CCAM growth usually reaches a plateau by 28weeks of gestation. For fetuses at less than 28 weeks gestation, the recommendation is twice weekly ultrasound surveillance if the CVR is greater than 1.6 and initially weekly surveillance for fetuses with smaller CVR values.

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CLASSIFICATION

Major distinctive pathological (and radiological) features allow to classify CCAM into following broad categories.CCAM have been divided by Stocker into 3 types.

Classification of CCAM

By Stocker et al.1977

Type –I Type –II Type - III

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1. STOCKER TYPE I :large cyst (>2cm)

Currently referred to as the macrocystic CCAMs based on gross anatomy and ultrasound findings .These consist of large sometimes multiple or multiloculated cysts.

They are not true cysts and always communicate with proximal airway and distal lung parenchyma. Histologically they are lined with respiratory ciliated cuboidal or columnar epithelium.

2. STOCKER TYPE II

Currently referred to as microcystic CCAM.These consist of small uniform multiple or multiloculated cysts. They are not true cysts and are lined by ciliated columnar or cuboidal epithelium.

3. STOCKER TYPE III:( solid lesion )

These are macroscopically and microscopically solid lesions without cysts.

Pathologically these are grouped into pulmonary hyperplasia group.

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REVISED CLASSIFICATION OF STOCKER(2002)

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CCAM receives its blood supply from the pulmonary circulation and is not sequestered from the tracheo bronchial tree. However, type II and III lesions can occasionally coexist with extralobar sequestration(hybrid lesions), and in such cases, they may receive systemic arterial supply

HISTOLOGY

CCAM is differentiated from other congenital cystic diseases by 4 characteristics : (I) Polypoid projections of the mucosa

(II) An increase in smooth muscle and elastic tissue in cyst walls

(III) Absence of bronchial cartilage (unless it is trapped within the lesion) (IV) Presence of tall columnar mucinous epithelium

(V) Absence of inflammation

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CONGENITAL LOBAR EMPHYSEMA

Congenital lobar emphysema, is identified as hyperinflation of one or more pulmonary lobes and leads to compression of nearby structures. The principal cause of congenital lobar emphysema is developmental deficiency of bronchial cartilage, which occurs in approximately 25% of cases. Obstruction of a lobar bronchus occurs in another 25% of cases which is intrinsic as in idiopathic bronchial stenosis ,inspissated mucous, bronchial torsion, extensive mucosal proliferation or extrinsic compression of bronchi by cardiopulmonary vessels, lymph nodes, cysts. However, in approximately 50%, the cause is unknown. CLE is characterized by hyperinflation of a lung lobe caused by air trapping, resulting in distension of the lobe, causing a mass effect that compresses the remaining lobes and causes mediastinal shift , leading to hemodynamic change(16)(17)

CLE is twice as common in males as it is in females and affects most commonly the left upper lobe (in 40-50% of cases), followed by the middle lobe (in 30-40%), and the right upper lobe (in 20%), although bilateral involvement has also been described. Upto 10%

of patients with congenital lobar emphysema present with associated abnormalities, the most common being cardiac abnormalities. Congenital lobar emphysema is generally diagnosed at birth, on the basis of a profile of respiratory failure, or through routine chest X-rays taken during routine medical visits; a routine chest X-ray reveals a hyperinflated lung lobe, sometimes accompanied by lung herniation across the midline, mediastinal shift, and areas of atelectasis in the adjacent lung parenchyma. The affected

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lobe can appear opacified because of amniotic fluid retention. The acquisition of CT scans of the chest plays an essential role in the diagnosis of CLE because it allows the evaluation of bronchial obstructions and anatomical changes, thereby defining the limits of the affected lobe and indicating the location of its blood vessels. The differential diagnosis of congenital lobar emphysema includes pneumatoceles, pneumothorax, pulmonary atelectasis, and pulmonary hypoplasia. CLEs are generally treated surgically, and lobectomy through thoracotomy or video-assisted lobectomy is the surgical treatment of choice in symptomatic children. In patients with CLE, pulmonary lobectomy results in a minimal loss of lung volume in the remaining parenchyma, and, because of compensatory lung growth during childhood, there is no long-term difference in respiratory function.(18)(19)

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SEQUESTRATION

In 1946, Pryce used the term “sequestration” to explain the abnormal lung that was

“disconnected” or “secluded” from the normal bronchial tree and had anomalous systemic arterial supply. In 1974, Sade et al., coined the term “sequestration spectrum”

to try and include all the various combinations of lung and vascular anomalies. In 1987, Clements and Warner coined a new term, “malinosculation” to describe this spectrum of abnormalities where there is an anomalous communication between the different components of lung tissue, namely, the lung parenchyma, tracheobronchial tree, arteries, and veins. Presence of disruptions of normal communication was also encompassed in this terminology by Clements and Warne. Lee et al., further refined the concept of malinosculation and classified it as Broncho pulmonary malinosculations .This classification is a systematic approach for the evaluation of BPVMs, taking into account isolated and concurrent abnormalities of airway, arteries, and veins.

Pulmonary sequestration accounts for 0.15-6.45% of all lung malformations and is characterized by normal, nonfunctioning lung tissue that has no connection with the bronchial tree and receives its blood supply from the systemic circulation. Pulmonary sequestrations can be classified as extralobar or intralobar depending on their location in relation to the adjacent normal lung and on their visceral pleural covering. There is a third, rarer type of pulmonary sequestration designated broncho pulmonary foregut malformation, in which an abnormal lung is connected to the gastrointestinal tract. (20)(21) Extralobar pulmonary sequestration, also known as accessory lung, is covered by visceral pleura and separated from the functioning lung, whereas intralobar pulmonary

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sequestration can be completely covered by normal lung tissue or by a segment of the visceral pleura of the lung lobe within which the sequestration occurs. The etiology of BPS is not fully known. The ratio between intralobar pulmonary sequestration and extralobar pulmonary sequestration is 3:1; intralobar pulmonary sequestration is equally distributed between the genders and extralobar pulmonary sequestration is more prevalent in the male gender (80% of cases).

Extralobar pulmonary sequestrations are generally located in the lower lobes of the left lung (80%), in close proximity to the costophrenic sulcus , and are less common than are intralobar pulmonary sequestrations, which account for approximately 75% of all cases of pulmonary sequestrations. About 10% of all extralobar pulmonary sequestrations are located below the diaphragm. In intralobar pulmonary sequestrations, there is no lung segment predominance; though some have reported a greater frequency of intra lobar pulmonary sequestrations in the posterior basal segment of the left lower lobe. Such sequestrations can go undiagnosed throughout the lifetime of the individual or can manifest as recurrent lower lobe infections.

Both types of pulmonary sequestration receive their blood supply from the systemic circulation, generally via the descending thoracic aorta or the abdominal aorta. In intralobar pulmonary sequestration, venous drainage is into the pulmonary veins, whereas it is into the systemic venous system (azygos vein or portal vein) in extralobar pulmonary sequestration. The diagnosis of pulmonary sequestration can be recognized in the prenatal period by ultrasound, which reveals a homogeneous, echodense, well- defined mass with anomalous vascularization.

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After birth, a chest X-ray finding of an elongated or cystic lesion, adjacent or posterior to the cardiac silhouette and described as a triangular, well-defined mass, may suggest the diagnosis. In the prenatal period, it can be difficult to diagnose pulmonary sequestration by ultrasound, because extralobar sequestration can be mistaken for a normal lung (the tissues having similar characteristics), whereas intralobar sequestration can be incorrectly diagnosed as congenital cystic adenomatoid malformation.

Nuclear magnetic resonance imaging can be useful in clarifying the diagnosis in such cases. A CT scan of the chest reveals the sequestered lung tissue and its vascularization, as well as other associated malformations, if present. The test of choice for the postnatal diagnosis is CT angiography, CT angiography is used instead of conventional radiography because the former is less invasive and more capable of diagnosing such lesions, showing the vascularization of the sequestered tissue in detail and therefore allowing safer surgical planning.

Patients who are asymptomatic at birth can subsequently develop recurrent pneumonia or can remain asymptomatic and be diagnosed incidentally. Other patients can develop complications, such as hemoptysis, massive hemothorax, cardiovascular complications, fungal/bacterial infections, benign tumors, or malignant degeneration. Intralobar pulmonary sequestration is generally not accompanied by other congenital lung malformations. However, extralobar pulmonary sequestrations can be accompanied by changes in the heart, diaphragm, and chest wall in up to 50% of cases or by other types of lung malformation, such as type II CCAM.

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The treatment of choice is surgical resection of the lesion through sequestrectomy or pulmonary lobectomy at the time of diagnosis principally due to the possibility of recurrent infections, hemorrhage, malignant transformation, and other complications.

The surgical treatment of choice in cases of extralobar pulmonary sequestration is sequestrectomy in which the pedicle is carefully resected and ligated; sequestrectomy can also be performed through video-assisted thoracoscopy, a procedure that has been shown to be safe and to have a low rate of postoperative complications. Sequestrectomy is also the resection of choice in cases of intralobar pulmonary sequestration, in order to preserve normal lung tissue that continues to develop until approximately 8 years of age. Though, it is not always possible to delineate the sequestered tissue within the affected lobe, in which case pulmonary lobectomy is required. (22)(23)

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HYPOPLASIA LUNG

Pulmonary hypoplasia almost always occurs secondary to other intrauterine disorders that produce an impairment of normal lung development. Conditions such as pleural effusions with fetal hydrops, deformities of the thoracic spine and rib cage (thoracic dystrophy)cystic adenomatoid malformation, and congenital diaphragmatic hernia physically constrain the developing lung. Any condition that produces oligohydramnios (e.g., prolonged premature rupture of membranes or fetal renal insufficiency) may also lead to diminished lung growth.

In these conditions, there will be limiting of the capacity for capillary and gas exchange surface area due to airway and arterial branching being inhibited.

Pulmonary hypoplasia involves a decrease in both the number of airway generations (up to 50%) and the number of alveoli (up to 67%) . In some conditions, like in oligohydramnios or thoracic dystrophy, the hypoplasia is bilateral because of the presence of bilateral lung constraint. However, large, unilateral lesions, such as cystic adenomatoid malformation and congenital diaphragmatic hernia , may displace the mediastinum and thereby produce a contralateral hypoplasia, although usually not as severe as that seen on the ipsilateral side.(24) Pulmonary hypoplasia is recognized in the newborn period either because of the presentation of persistent pulmonary hypertension or because the degree of hypoplasia is quite severe. In either case, oxygen and mechanical ventilation may be required to support gas exchange because of hypoxia and respiratory distress.

Specific therapy to control the pulmonary hypertension, such as inhaled nitric oxide, may be useful. However, in cases of severe hypoplasia the limited capacity of the pulmonary circulation may be inadequate to sustain life. Occasionally, in circumstances that are not

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amenable to less invasive treatment and support, extracorporeal membrane oxygenation (ECMO)may provide gas exchange for a critical period of time and permit survival.(25)

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BRONCHOGENIC CYST

Bronchogenic cysts are usually single unilocular cyst filled with mucous or fluid but can also be multiple when located inside the lung parenchyma. They are lined by pseudostratified ciliated columnar respiratory epithelium and contain hyaline cartilage plates. They most commonly occur in mediastinum although they can occur anywhere in the thoracic cavity or elsewhere in retroperitoneum, tongue, neck and subcutaneous tissue. Children typically present with either infection of the cyst or from mass effect caused by the cyst, presenting as respiratory distress, dyspnea, recurrent pneumonia, lobar emphysema or hemorrhage unless detected on antenatal ultrasound. Bronchogenic cysts also pose a rare risk of malignant transformation. There have been reports of rhabdomyosarcoma, pulmonary blastoma, and malignant mesenchymoma found in resected bronchogenic cysts.

Treatment includes complete surgical resection, enucleation or lobectomy due to potential complications and risk of malignant change. The use of Video assisted thoracoscopic surgery has revolutioned the treatment of bronchogenic cyst. The advantages of VATS over thoracotomy include smaller incisions, less pain, shorter hospital stays and rapid return to physical activity.

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REVIEW OF LITERATURE

(1) A study was conducted by Jean Marie E.Jamero, at Philippine heart Centre between 1975-2005.

A 30 year retrospective review of 20 patients with congenital cystic lung malformations was done. The study had 45% congenital cystic adenomatoid malformations,25% Congenital lobar emphysema,15% bronchogenic cysts and 15% of pulmonary sequestration. The common symptoms at presentation were dyspnea, acute respiratory tract infection, fever, tachypnea, retractions, decreased breath sounds and tachycardia. Lobectomy was done in 70%.Poor postoperative outcome was not associated with the types of congenital lung malformations. There was no significant correlation between survival and number of lobes resected.

(2) A study was conducted by Jonathan Durell1 December 2014 in Oxford Children's Hospital, University of Oxford, United Kingdom.

The objective of the study is to understand the etiology, Classification, natural history, investigations and treatment of congenital lung lesions. Majority of these cystic lesions comprise a spectrum of CCAM, Sequesteration, CLE, bronchogenic cysts.

The conclusion of the study is that pulmonary sequestration, CCAM, Congenital lobar emphysema, bronchogenic cysts represent a spectrum of cystic congenital lung lesions which manifests with wide variety of symptoms from fetal demise to chronic cough to incidental detection in asymptomatic children. though with

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the use of antenatal ultrasound and postnatal imaging, the detection of the lesions have dramatically improved, still there remains controversy over pathophysiology and management of these lesions.

(3) In a study done by Altair da silva costa junior et al1 at sao Paulo/escolapaulista de medicina, brazil

The objective is to analyze retrospectively the medical sheets of patients with pulmonary malformations submitted to surgical management and to investigate the evaluation which is done clinically before the final diagnosis is made.

The medical charts of patients with pulmonary malformations operated on at the São Paulo Hospital-Federal University of São Paulo/Paulista School of Medicine-from 1969 to 2004. Each medical chart was analyzed as to the following: clinical profile, previous treatment, diagnosis, nosocomial complications and surgical treatment. The child having received a diagnosis of pulmonary malformation, having undergone pulmonary resection, and chart data being complete were included as an inclusion criteria.

In this study, the medical charts revealed 60 patients diagnosed with pulmonary malformations-27 cases of bronchogenic cyst, 10 cases of pulmonary sequestration, 14 cases of congenital lobar emphysema and 9 cases of cystic adenomatoid malformation-underwent surgery. Ages ranged from 4 days to 62 years (mean was 17.9 years). Ninety-two percent of the patients presented symptoms (mean duration, 15.37 months). There was a predominance of males (55%). Of the 60 patients undergoing surgery, 27 (45%) received preoperative

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29

home or hospital treatment with antibiotics. Regarding complications, we observed that mortality was 3.3% and morbidity was 23%.

The conclusion of the study was delayed diagnosis or misdiagnosis of pulmonary malformations resulted infrequent, recurrent infectious complications as well as unnecessary hospitalizations and treatments.

(4) A study was conducted by Moti M.Chowdhury Department of Radiology, Royal Hospital for Sick Children, Edinburgh, UK

The objective is to review the use of radiological modalities for diagnostic evaluation of lung malformations both antenatally and postnatally, common radiological findings of most prevalent congenital lung malformations and to discuss the use of diagnostic facilities in clinical management of these conditions.

Conclusion of the study is advances in antenatal and postnatal detection by imaging modalities has increased detection of both asymptomatic and clinically symptomatic lung malformations which overall has facilitated antenatal counselling, planning and surgical management whenever appropriate.

(5) A review by Federica Annunziata1 June 2019 Division of Paediatrics, Department of Translational Medical Sciences, Federico II University, Naples, Italy.

The study aimed to provide information on a number of unresolved issues arising on the management of congenital thoracic malformations. They presented an

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30

algorithm for assessment and follow-up of CTM diagnosed in antenatal and postnatal period.

(6) A study was conducted by Tawil MI1 Department of Radiology, Royal Liverpool Children's Hospital Alder Hey, Eaton Road, UK.

Objective of the study was to v review the clinical and radiological spectrum of CCAM, comparing the antenatally with the postnatally diagnosed cases.

In this study, Fifteen cases of antenatally and/or postnatally diagnosed and histopathologically proven CCAM were retrospectively identified over a period of 4 years

Conclusion of the study is there is no considerable difference found between two groups. Early detection of these lesions antenatally would help patients by avoiding delay in making the diagnosis, which can lead to serious complications.

CT was successful in accurately diagnosing and grading CCAM lesions.

(7) In a study done by HylasPaiva da Costa Ferreira et al1 at Universidade Federal do Rio Grande do Norte - UFRN, Federal University of Rio Grande do Norte - Natal, Brazil

The objective is to study the main congenital lung malformations treated and its principal diagnostic methods employed, the indications for surgical treatment and the results which was obtained, at a referral facility for pediatric thoracic surgery.

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31

The medical charts of 52 patients who were diagnosed with congenital lung malformations and who had been submitted to pulmonary resection between January of 1997 and December of 2006 were reviewed. Age > 12 years and incomplete clinical data were taken as exclusion criteria. The final sample after exclusion criteria comprised 35 patients.

In this study, the mean age was 31 months, and there was a predominance of males (n = 21). The anatomopathological findings were cystic adenomatoid malformation (n = 14), congenital lobar emphysema (n = 13), pulmonary sequestration (n = 8) and arteriovenous malformation (n = 1). The most common type of lung resection was left lower lobectomy (in 25.71%) followed by left upper lobectomy (in 22.85%), different types of segmentectomy (in 22.85%), right upper lobectomy (in 14.28%), right lower lobectomy (in 8.57%) and middle lobectomy (in 5.71%). Of the 35 patients, Ten patients (28.5%) presented with postoperative complications, 34 (97.14%) were submitted to closed pleural drainage, with a mean duration of 3.9 days for thoracic drainage. No deaths were there in our sample.

The conclusion of the study was pulmonary resection for the treatment of congenital lung malformations is a safe procedure, presenting low morbidity and no mortality at a referral facility for pediatric thoracic surgery.

(8) In a study done by shin-ichi-takedaet al1 which was published in European Journal of Cardio-Thoracic Surgery, Volume 15, Issue 1, January 1999

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32

The objective is to review their institutional experience of congenital cystic lung disease, reviewing the clinical spectrum of the disease related to age, and present some cases with abnormal clinical manifestations. Between 1962 and 1996, 26 patients (9 females and 17 males) under 15 years old underwent evaluation and surgical treatment for congenital cystic lung disease were taken. 7 patients were under 1 year old, and 19 were over 1 year of age. There were 13 bronchogenic pulmonary cysts, 6 pulmonary sequestrations, 4 congenital cystic adenomatoid malformations (CCAM), and 3 congenital lobar emphysemas.

All patients under 1 year old showed respiratory distress with mediastinal shift but no episodes of infection. In contrast, 13 of the 19 patients over 1 year old had symptoms of recurrent infection without respiratory distress. Five patients over 1 year old were entirely asymptomatic from birth. There were significant differences (P<0.05) in the frequencies of respiratory distress and infection between the two groups (χ2-test). Lobectomy was performed in 21 patients,

segmentectomy in one patient, excision in 3 patients, and exploration in one patient. There was no incident of postoperative mortality or morbidity except for one patient with CCAM complicated by re-expansion lung edema.

The conclusion of the study was in patients under 1 year old, cystic lesions were discovered by respiratory distress and in patients over 1 year old signs of infection were the most important clinical features. Early recognition of these relatively rare congenital cystic lung lesions would lead to proper and immediate surgical intervention.

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33

(9) In a study done by Lima Met al1 from January 1975 and October 2007at Department of Pediatric Surgery, Bologna University

The objective is to review their institutional experience with congenital cystic lung disease emphasizing on diagnosis and management. Between January 1975 and October 2007, 109 patients have been treated, of which 52 were females and 57 were males, the age ranged from the birth to 13 years. 104 patients presented with solitary lesions: CCAM (47), CLE (16), PS (22), BC (19). The remainders 5 patients presented two simultaneous lesions: intralobar PS and CCAM (2), CLE and CCAM (3).All the lesions have been treated surgically: in the first cases, only symptomatic patients underwent surgery, while in the last years, patients have systematically been submitted operated.

The conclusion of the study was the treatment of these lesions is surgical: CCAM (type I-II) and CLE should be treated promptly in newborns for respiratory distress and pneumothorax; CCAM (type II) and BC generally become symptomatic gradually and expose to degenerative risk; intralobar PS generally becomes symptomatic and surgery prevents the risk of infections.so a proper clinical evaluation of the congenital cystic lung diseases allow a precocious and effective surgical timing.

10) In a study done by Evrard V et al1 at Department of Thoracic Surgery, University Hospital Gasthuisberg, KatholiekeUniversiteit, Herestraat 49, B-3000 Leuven, Belgium.

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34

The objective is to study the relation in terms of clinical presentation and embryology of congenital parenchymatous pulmonary malformations and to evaluate their experience with the surgical management and diagnosis of congenital parenchymatous Broncho pulmonary malformations and to compare their data with the literature

In this study, From January 1979 to December 1996 a series of 48 patients, 18 females (37.5%) and 30 males (62.5%) were operated on for congenital Broncho pulmonary malformations. It included pulmonary sequestration (16 patients), congenital cystic adenomatoid malformation (14 patients), bronchogenic cysts (13 patients) and congenital lobar emphysema (5 patients). The mean age at which the first clinical symptoms occurred was 8.8 years (1 day to 62 years), and the mean age at the time of surgical intervention was 9.3 years (1 day to 62 years). In that lobectomy was performed in 22 cases; in other patients more lung- preserving surgery such as sequestrectomy or enucleation was performed.one postoperative death occurred following lobectomy for pulmonary sequestration,.

Eleven other patients presented with postsurgical complications: pneumothorax (n = 5), pleural effusion (n = 3), portal vein thrombosis (n = 1), prolonged air leak (n = 2) and hemorrhage requiring re-intervention (n = 1).

The conclusion of the study was that even if asymptomatic any thoracic cystic lesion expanding on chest radiography should be an indication for surgical resection, because of the risk of infection, pulmonary compression, or malignant degeneration. In cases of fetal intrathoracic mass, prenatal diagnosis and intrauterine intervention may be indicated.

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35

11) In a study done by Shanmugam G et al1 at Department of Paediatric Cardio Thoracic Surgery, Royal Hospital for Sick Children, Dalnair Street, Glasgow G3 8SJ, Scotland, UK.

The objective is to review their institutional experience with pulmonary resection for congenital Broncho pulmonary malformations and analysis of outcome and management of pregnancies with a diagnosis of congenital lung malformations prenatally

Between January 1993 and December 2003, 31 patients underwent evaluation and pulmonary resection for Broncho pulmonary malformations. Common clinical presentations were respiratory infections/pneumonias (22), respiratory distress (9), and dyspnoea (9). Diagnostic modalities included chest radiography, CT scan (22), MRI scan (7), bronchoscopy (5) and arteriography (1).There were congenital cystic adenomatoid malformations (CCAM) (13), pulmonary sequestrations (6), bronchogenic cysts (3), and congenital lobar emphysemas (CLE) (9). 15 patients who underwent resection were diagnosed by ultrasound antenatally. No pregnancy was terminated. There was no fetal demise. In 6 cases Regression of the sonographic appearance was observed. 8 emergency resections were performed (CCAM 4; CLE 3; Bronchogenic cyst 1). Surgical procedures included 24 lobectomies.no deaths were there. Postoperative complications included: pneumothorax (1) and persistent air leak (n=2; one requiring completion lobectomy).In 5 patients there were Persistent mild symptoms at long-term follow-up

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36

The conclusion of the study was congenital lobar emphysema and Congenital cystic adenomatoid malformation were the commonest congenital anomalies.

Congenital lung malformations are increasingly diagnosed antenatally, sometimes necessitating emergency surgical resection. Postnatal evaluation is required in all infants with a prenatal diagnosis. Therapeutic decompression is indicated in the presence of mass effects .Even in asymptomatic patients the risk of infection, pulmonary compression and malignant degeneration makes resection imperative. The procedure of choice is lobectomy and it is well tolerated, and leads to excellent outcomes.

12) In a study done by Bailey PVet al1 at Department of Surgery, Cardinal Glennon Children's Hospital, St. Louis, Mo.

In this study between 1970 and 1988, 45 patients from birth to thirteen years of age (22 girls and 23 boys) underwent evaluation and treatment for Broncho pulmonary malformations. 37 had solitary lesions: cystic adenomatoid malformation (n = 9), bronchogenic cyst (n = 13), pulmonary sequestration (n = 6), congenital lobar emphysema (n = 6), arteriovenous malformation (n = 2), and bronchial atresia (n = 1). 8 additional patients had 2 simultaneous abnormalities and 3 patients had congenital diaphragmatic hernias. 21 patients presented with respiratory symptoms, in which 7 had severe symptoms. 12 presented with pulmonary infection and 10 patients were completely asymptomatic. Plain chest roentgenogram was the only diagnostic imaging performed in 11 patients. 13 patients underwent computed tomographic scan, but in only 4 it was essential for diagnosis. Prenatal ultrasonography was done in 3 patients in which two

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37

demonstrated cystic adenomatoid malformation, with one false negative study.

Thoracotomy with excision of the lesion by pneumonectomy or lobectomy resulted in survival of 42 patients (93%). 3 deaths in neonates were due to pulmonary hypertension and hypoplasia.

The conclusion of the study was that congenital Broncho pulmonary malformations usually can be diagnosed by plain chest x-ray films. Occasionally ancillary studies such as computed tomography or ultrasonography may be necessary. Combinations of the different types of Broncho pulmonary malformations occurred frequently. Soon after diagnosis, All lesions, including symptomatic lesions in neonates, can be managed surgically.

13) In a study done by TurkyilmazA et al1 at Ataturk University, Faculty of Medicine, Department of Thoracic Surgery, Erzurum, Turkey.

The objective is to present their experience with Congenital Pulmonary Malformation patients who were surgically treated in their clinic and to discuss their findings along with those from the literature.

In this study, under the age of sixteen years surgical treatment was performed on 19 patients who were diagnosed with CPM in their clinic between January 1995 and December 2008. The age, gender, symptoms, diagnoses, locations of the lesions, hospitalization times and surgical method used, complications, and the results of all patients were evaluated retrospectively.

There were pulmonary sequestration (PS) - 6, bronchogenic cyst(s) (BC) – 5, congenital lobar emphysema (CLE) - 4, congenital cystic adenomatoid

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38

malformation (CCAM) Type I - 3, and PS and CCAM coexistence - 1 patient.

Resection was done in all patients. No death related to surgery occurred.

The conclusion of the study was CCAM, CLE, and PS may lead to respiratory distress which will be life-threatening in infants. To improve severe symptoms, prevent fatal complications, and establish a histopathological diagnosis surgery is needed.

14) In a study done by KhemiriMet al1 at Service médecine infantile A, hôpitald'Enfants Bab Saadoun-Jabbary, CP 1007 Tunis, Tunisie

The objective is to report different clinical pictures of cystic pulmonary malformation (CPM) and its problems in diagnosis.

In this study, Cases of CPM between 01 January 1994 and 31 December 2004 diagnosed in their institution were reviewed.

30 children with CPM were studied. They consisted of 13 girls and 17 boys ranging from 20 days to 16 years of age at the time of the diagnosis. There were 17 cases of congenital lobar emphysema (CLE), 5 cystic adenomatoid malformations (CAM), 7 bronchogenic cysts (BC), and 4 pulmonary sequestrations (PS). 3 patients presented two associated lung malformations. The mean ages at the time of diagnosis was from 2 to 88 months. The symptoms consisted of recurrent attacks of respiratory embarrassment (n=6, 20%);

respiratory distress (n=14, 46.6%), pulmonary infection (n=8, 26.6%) associated with haemoptysis in two cases, haemothorax (n=1) and a chance discovery (n=1). Radiological investigations led to the diagnosis in all cases of CLE and

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CAM although it contributed less to the diagnosis of BC and PS. 29 patients required chirurgical treatment involving lobectomy (n=22), cystectomy (n=8) and pneumonectomy (n=2) In all cases the histopathological examinations confirmed the diagnosis and rectified the preoperative diagnosis in 4 cases. The postoperative period was uneventful in 26 children with a mean of follow-up of 24 months (4 months to 7 years) except for 1 patient with CLE, who died a few days after a lobectomy due to acute nosocomial pneumonia. 3 patients had transient and episodic attacks of dyspnoea.

The conclusion of the study was CPM may be responsible for many radiological and clinical pictures that present difficulties in their diagnosis. Polymorphism is related to its topography, the type of malformation, and the evolutive complications.

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40

STUDY JUSTIFICATION

 To know the incidence and prevalence of various congenital lung malformations, age of onset, mode of presentation, their complications, diagnostic modalities and management plans.

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41

AIM & OBJECTIVES

To determine the clinical profile and spectrum of Congenital lung malformations in a tertiary care Hospital.

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42

MATERIALS & METHODS Study design-Observational study

Study setting- Children in study age group attending general OP and admitted in medical and surgical ward, satisfying the inclusion criteria in Institute of child health, Egmore, Chennai.

Study period -March 2018 to September 2019 Study population-

Inclusion criteria: All children from birth till 12years of age presenting with respiratory symptoms or radiological abnormalities suggestive of congenital lung malformations attending the hospital.

Exclusion criteria: Congenital diaphragmatic hernia, Eventration, Vascular ring, Airway anomalies, already operated cases of lung malformations.

Sample size: 35(convenient sample)

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MANOEUVRE

After obtaining informed consent from parents, demographic data like age, sex and locality of the family was collected. History regarding the complaints for which the child was admitted to the hospital was noted. History regarding Antenatal ultrasound scans and detection of any anomalies was asked and noted. History regarding the neonatal period like birth weight of the child and hospitalization for any illness was noted. History regarding developmental milestones was asked and noted. Immunization status of the child whether completely or partially immunized was also asked.

Detailed clinical examination including dysmorphic facies, mediastinal shift, Clubbing, cyanosis seen as per prerequisites. Cardiovascular system, respiratory system, abdominal system and neurological examination was done. All relevant blood investigations done were noted down. Chest x-ray done for all patients and was further proceeded with HRCT if chest x-ray is abnormal. Bronchoscopy and CT pulmonary angiography was done in relevant cases. Echocardiography done in all patients to look for associated anomalies.

All children were followed up during the course of hospital stay till discharge and was also followed up after surgery.

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STATISTICAL ANALYSIS

Data was entered in excel sheet. Statistical analysis of data performed by statistical software SPSS version 20.Outcome variables were expressed as proportion.

Mean value of quantitative data were analyzed using One way ANOVA and a P value of less than 0.05 was considered as significant.

ETHICAL CONSIDERATIONS

The study was commenced after obtaining ethical committee clearance.

Informed consent was obtained from parent. Strict confidentiality was maintained while analysing and presenting the data.

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RESULTS

AGE

Of the total 35 congenital lung malformation children, mean age of presentation (SD) was 5 months. Majority of children were less than 1 year of age(84%).

AGE DISTRIBUTION AMONG THE DISEASE GROUPS AGE

<3 months

3-6 months 6-12 months 1-2 years

>2 years

CPAM 2 3 4 2 1

CLE 5 3 3 1 0

Pulmonary sequestration 1 2 0 0 0

Hypoplasia lung 2 2 1 0 1

Scimitar syndrome 0 0 1 0 1

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46 2

3

4

2

1 5

3 3

1

0 1

2

0 0 0

2 2

1

0

1

0 0

1

0

1 0

2 4 6

<3 months 3-6 months 6-12 months 1-2 years >2 years

No of patients

Age distribution

CPAM CLE

Pulmonary sequestration Hypoplasia lung Schimitar syndrome

Among 35 children,84% of CCAM patients in our study are below one year of age .High degree of suspicion is needed to detect CCAM in infants if they are not antenatally diagnosed.

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SEX DISTRIBUTION

Among 36 children, 18(51%) were females and 17(49%) were male.

SEX DISTRIBUTION AMONG THE DISEASE GROUPS Gender

Male Female

CPAM 6 6

CLE 8 4

Pulmonary sequestration

1 2

Hypoplasia lung 1 5

Scimitar syndrome 1 1

Males Females 49%

51%

Sex distribution

Males Females

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48

6

8 1

1 1

6 4

2

5 1

0 1 2 3 4 5 6 7 8 9

CPAM CLE Pulmonary sequestration Hypoplasia lung Schimitar syndrome

NO of patients

GENDER DISTRIBUTION

Female Male

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PRESENTING COMPLAINTS

Congenital lung malformations have varied clinical presentation from Respiratory distress, fever, cough, recurrent pneumonia feeding difficulties in the decreasing order of frequency. Given the wide variability in clinical presentation, accurate diagnosis is critical for counselling and for prompt treatment.

Cough Fever Resp.Distress Fast breathing Rec. pneumonia Hemoptysis Feeding Difficulty Other congenital anomalies

CPAM (12) 9 9 10 10 2 0 0 1

CLE (12) 9 4 12 12 0 0 0 0

Pulmonary sequestration (3)

1 1 2 2 0 0 0 0

Hypoplasia lung (6)

3 0* 3 4 1 1 0 1

Scimitar syndrome (2)

1 1 1 1 0 0 1* 1

Chi-square P

value 0.52 0.03 0.09 0.22 0.54 0.29 0.002 0.17

Inference: There is a significantly greater number of patients WITHOUT fever in Hypoplasia lung group, and with feeding difficulty in Scimitar syndrome group.

(Numbers in column one within parenthesis denote the total number of patients among the 35 with that particular disease)

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COUGH

Among 35 children 9 children of Congenital pulmonary airway malformations,9 children of Congenital lobar emphysema,1 pulmonary sequesteration child,3 children with lung hypoplasia and 1 child with scimitar syndrome presented with cough as their presenting complaint.

FEVER

Among 35 children of congenital lung malformations,9 children with CPAM, 4 children with CLE, 1 Pulmonary sequestration child,1 child with Scimitar syndrome presented with fever.

9 9

1 3

1

COUGH

0 2 4 6 8 10

CPAM CLE Pulmonary

sequestration

Hypoplasia lung

Schimitar syndrome

9

4

1 0 1

FEVER

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CPAM CLE Pulmonary sequestration Hypoplasia lung Schimitar syndrome

10

12 2

3 1

RESPIRATORY DISTRESS RESPIRATORY DISTRESS

Out of 35 children with Congenital lung malformations,10 children with CPAM,12 children with CLE,3 children with lung hypoplasia,2 children with Pulmonary sequestration and 1 child with scimitar syndrome had respiratory distress at the time of initial presentation.

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FAST BREATHING

Among 35 children with Congenital lung malformations,12 children (34.2%) with CLE,10 children (28.57%) with CPAM,4 children (11.4%) with hypoplasia lung,2 children (5.7%) with Pulmonary sequestration and 1 child (2.85%) with scimitar syndrome had fast breathing at the time of presentation.

CPAM CLE PULMONARY SEQUESTRATION HYPOPLASIA LUNG SCHIMITAR SYNDROME

10

12 2

4 1

FAST BREATHING

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Other presenting complaints in congenital lung malformation children include Recurrent pneumonia, Feeding difficulty and hemoptysis.

0 0.5 1 1.5 2 2.5 3 3.5

CPAM CLE Pulmonary

sequestration

Hypoplasia lung Schimitar syndrome

OTHER PRESENTING COMPLAINTS

Rec. pneumonia Hemoptysis Feeding Difficulty Others

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ANTENATAL DETECTION AND STEROID USE

Ultrasound forms the first imaging in detection of CTMs that are identified during antenatal period. Close monitoring with serial fetal usg is performed to assess its size, location, its characteristics and blood supply. In our study antenatal detection accounts for 40 % (6 cases) of total CCAM and Sequestration cases diagnosed.

Antenatal detection

CPAM 4

Pulmonary sequestration

2

Chi-square P value 0.25

Out of 15 CCAM and Sequestration cases 6 cases (40%) were detected antenatally using antenatal USG. Antenatally diagnosed cases presented earlier to the hospital.

References

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