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Congenital Lung Malformation

Congenital Anomaly of Lung Unspecified


Presentation

  • Infants present with cough, wheezing, respiratory distress, and cyanosis. Older children may present with recurrent chest infections.[emedicine.medscape.com]
  • Presents important “take home concepts within each chapter to help you recall clinical pearls. Includes the most need-to-know basic science, focusing on providing clear implications for patient care.[books.google.com]
  • Strengths with possible “problems expected at birth with advice on how to manage them are presented. Logical outline of management.[books.google.com]
  • This depends on the severity of the condition, and, in the case of cysts, on the size and number present.[rbhh-specialistcare.co.uk]
  • Individuals with congenital lung malformations can present with respiratory symptoms at birth or can remain asymptomatic for long periods.[ncbi.nlm.nih.gov]
Turkish
  • This abnormal vein on the chest radiography creates a gentle curve bulging into the right chest from the mediastinum that some believe resembles the Turkish sword called a scimitar.[emedicine.medscape.com]
Increased Susceptibility to Infections
  • The presence of a malformation on the left side may therefore increase susceptibility to infection. We also observed general symptoms, such as growth impairment or eating disorders, in a minority of infants.[pediatrics.aappublications.org]
Tachypnea
  • ): Tachypnea is the most common sign encountered in the newborn period, reflecting respiratory distress.[childrensnational.org]
  • Typical presenting signs include tachypnea, respiratory distress including grunting, chest retractions, and cyanosis. Childhood presentation Thirty percent of CPAMs are diagnosed after the neonatal period.[clinicaladvisor.com]
  • The most common clinical presentation is progressive respiratory distress including tachypnea, grunting, retractions and cyanosis.2 Upon physical examination, there may be prominence of the affected side of the chest, ipsilateral hyperresonance and decreased[ispub.com]
Labored Breathing
  • Additional signs and symptoms Tachypnea Grunting respirations Labored breathing accompanied by chest wall retractions Cyanosis History A family history of tumors and/or cystic lesions should be obtained irrespective of the patient’s age, presenting signs[clinicaladvisor.com]
Contusion
  • For example, placement of a chest tube to manage suspected tension pneumothorax in a patient with congenital lobar emphysema may lead to lung contusion and ventilation through the chest tube instead of into the remaining healthy lung.[emedicine.medscape.com]
Amenorrhea
  • Case 2 A 22-year-old primigravida, with history of 40 weeks of amenorrhea sought routine antenatal check-up. Her blood and biochemical investigations were within normal limits. Routine anomalous scan revealed multiple cysts in left hemithorax.[jacpjournal.org]

Treatment

  • Early diagnosis and prompt treatment offer the possibility of absolutely normal lung development. The treatment of asymptomatic patients with lung malformations is controversial, because the prognosis of these diseases is unpredictable.[ncbi.nlm.nih.gov]
  • Treatment People with congenital lobar emphysema or pulmonary cysts may not need any treatment, while others may need surgery to remove the affected lobe (lobectomy).[rbhh-specialistcare.co.uk]
  • Treatment consists of surgical excision, sometimes as early as three months.[drranjansuri.com]
  • LEVEL OF EVIDENCE: III - Treatment Studies. Copyright 2017. Published by Elsevier Inc.[ncbi.nlm.nih.gov]
  • These limitations strongly impact the treatment of these pulmonary pathologies diagnosed during the antenatal period. The advancement of prenatal imaging has modified the treatment of congenital pulmonary malformations.[journals.lww.com]

Prognosis

  • Postnatal and in adult patients, lobectomy is the treatment of choice for symptomatic cases. [5], [8] Prognosis also depends on Stocker type, type I lesions carry overall good prognosis.[jacpjournal.org]
  • The treatment of asymptomatic patients with lung malformations is controversial, because the prognosis of these diseases is unpredictable. The management of these lesions depends on the type of malformation and symptoms.[ncbi.nlm.nih.gov]
  • Prognosis depends on the size of the lesion, and the degree of functional impairment. Small lesions may remain asymptomatic. Failure to recognize a malformation may lead to inappropriate intervention.[emedicine.medscape.com]
  • What is my baby’s prognosis? Generally, the size of a CLM can be correlated with its clinical significance. Smaller lesions rarely produce any symptoms prenatally or in the immediate post natal period and the prognosis for these babies is excellent.[childrensmn.org]
  • Type IV : 15%, large cysts up to 10 cm lined by flattened epithelium; good prognosis; similar to grade 1 pleuropulmonary blastoma although less cellular; sample generously to rule out blastoma Clinical features Associated with stillbirth, neonatal distress[pathologyoutlines.com]

Etiology

  • A consistent chapter format enables you to rapidly and effortlessly locate the most current protocols on manifestations, etiologies, triggers, approaches to treatment, complications, and preventative strategies.[books.google.com]
  • , Conflict of Interest: None DOI: 10.4103/2320-8775.158854 Congenital cystic adenomatoid malformations (CCAMs), also known as congenital pulmonary airway malformation is a developmental, non-hereditary, hamartomatous abnormality of lung with unknown etiology[jacpjournal.org]
  • […] or CT now used to evaluate for systemic artery and should replace arteriography • US prenatal / neonatal --homogeneous echogenicity (ELS) • Probably acquired in most cases – Only 50% discovered before age 20 • Sx: cough, sputum, recurrent pneumonia • Etiology[learningradiology.com]
  • The etiology remains unknown. Hamartomatous change in the terminal bronchioles or aberrant embryogenesis at or before the seventh gestational week producing cystic distortion of the lung architecture is suspected [1].[ctsnet.org]
  • Ongoing controversies regarding etiology, diagnosis, treatment Surgical management of asymptomatic lesions are controversial. Pros: Surgery may be technically more difficult and carry higher morbidity in the setting of an infected CPAM.[clinicaladvisor.com]

Pathophysiology

  • Pathophysiology Congenital cystic adenomatoid malformation is an uncommon developmental lung anomaly that is thought to occur early in fetal lung development, around the fifth to the eighth weeks of gestation.[ispub.com]
  • Wheezing is frequently associated with CPMs, potentially induced by the pathophysiological mechanisms underlying the CPM. Surgical removal of the CPM had no significant impact on these symptoms.[pediatrics.aappublications.org]

Prevention

  • A consistent chapter format enables you to rapidly and effortlessly locate the most current protocols on manifestations, etiologies, triggers, approaches to treatment, complications, and preventative strategies.[books.google.com]
  • CCAMs can also prevent the alveoli – the air sacs in the lungs – from developing normally. Some of the growths can shrink or even disappear without the need for treatment.[drranjansuri.com]
  • An analysis based on phenotypic appearance thus prevented misclassification errors. Our study has some limitations.[pediatrics.aappublications.org]
  • This is to prevent complications from the cysts such as infection. There have also been reports of growths developing in the cysts in later life.[uhs.nhs.uk]
  • This limitation must be taken into account during the prenatal consultation so as to have the most prepared discussion about the predicted evolution of the lesion and the possible need for preventative excision.[journals.lww.com]

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