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Funnel Chest
Chest Funnel

A funnel chest, more commonly known as pectus excavatum, is a congenital deformity characterized by a depression of the sternal bone. The etiology, as well as risk factors, remain unclear. Signs and symptoms may not be present initially, but the eventual compression of the heart in adulthood can cause marked symptoms, especially in the absence of clinical suspicion about funnel chest being the underlying cause.

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Presentation

Funnel chest (medically termed pectus excavatum) is considered to be a rather common congenital disease, seen in 1 in 1,000 live births. In the United States, 1 in 300 Caucasian children is diagnosed with a funnel chest [1] [2]. A significant predilection toward male gender has been reported, with the male-to-female ratio ranging from 2:1 to even 9:1 [2] [3]. Pectus excavatum rarely causes symptoms in childhood and infancy (which is why physicians often inform parents that there is no reason for concern and that the problem is cosmetic in nature only). But many reports have illustrated the importance of this condition in the pathogenesis of debilitating cardiopulmonary symptoms [1] [2] [4] [5] [6]. The reason why symptoms do not appear until adulthood, however, is unexplained as yet. The most probable theory suggests the loss of chest wall elasticity in adulthood, which leads to reduced compliance and direct compression of the depressed sternum on the cardiac structures, specifically the right ventricle [2]. As a result, previously healthy patients may complain of dyspnea, shortness of breath, palpitations (particularly after exercise), persistent fatigue and asthma-like symptoms [1] [3] [6] [7]. If the diagnosis is not made, prolonged compression of the chest can cause reduced lung volume, cardiac compression and valvular heart disease (mitral regurgitation or prolapse of the mitral valve) [2] [7]. Funnel chest is often associated with scoliosis (in about 15% of cases), while connective tissue disorders, such as Marfan syndrome and Ehlers Danlos syndrome may also be associated with it [2] [6] [8].

Cardiovascular

  • Heart Murmur

    Many have heart murmurs and breathe noisily during sleep. Called also funnel breast or chest and koilosternia. Copyright 2007. An Elsevier publication. All rights reserved. [web.archive.org]

    Other notable findings include a pot-belly posture, forward-drifted shoulders, a heart murmur on cardiac auscultation and arrhythmia in severe cases. [symptoma.com]

    Except in mild cases, it interferes with sustained exercise, delays upper respiratory infection recover, reduces the ability to eat a full meal, and may produce a functional heart murmur CONTINUE SCROLLING OR CLICK HERE FOR RELATED ARTICLE Reviewed on [medicinenet.com]

    In moderate and severe cases, the sunken breastbone can press on the heart or lungs, causing: Shortness of breath during exercise Rapid heartbeat or palpitations Wheezing or coughing Chest and back pain Heart murmur Decreased stamina/increased fatigue [uchicagomedicine.org]

  • Systolic Murmur

    In one series, systolic murmurs were auscultated in 46% of cases not associated with an underlying connective tissue disorder. [5] The prevalence of murmurs was independent of the degree of deformity in this study. [medscape.com]

    murmur is heard in about half of the patients due to compression of the right ventricle by the chest wall [17, 22]. [sages.org]

Skin

  • Disfiguring Scar

    We suggested that disfiguring scars with keloid formation cause less psychological distress than persistent congenital malformation of the chest. [ncbi.nlm.nih.gov]

Workup

The initial diagnosis of a funnel chest is made during a physical examination as early as infancy when inspection and palpation of the chest wall confirms a cup-shaped concavity in the sternum [1] [2]. The concavity is well defined, deep and more prominently involves the lower portion of the sternal bone [1] [2]. Depression of 4th-7th costal cartilages can be observed in a significant number of individuals, in which case the concavity might be broader [2]. Other notable findings include a pot-belly posture, forward-drifted shoulders, a heart murmur on cardiac auscultation and arrhythmia in severe cases [1] [2]. On the other hand, when previously healthy patients describe cardiopulmonary signs and symptoms during history taking, an earlier finding of a funnel chest must raise clinical suspicion toward this disorder as the underlying cause. Family history, an integral part of the patient interview, is also an important feature, as up to 43% of pectus excavatum cases have a positive family history for the condition [1]. Imaging studies, however, are the gold standard in confirming congenital abnormalities of the chest wall, and either computed tomography (CT) or magnetic resonance imaging (MRI) can be used [2] [3] [8], which both show the abnormal site of the sternum and its compression of the heart. Pulmonary function tests, electrocardiography (ECG) and echocardiography (either transthoracic or transesophageal) are recommended during workup, in order to assess the function and structure of the heart.

Treatment

The mean treatment duration to normal reshape was achieved at 10 months (4-21). The remaining patients are improving under continuing active treatment. [ncbi.nlm.nih.gov]

During this one of our specialist clinicians will give a detailed explanation of our treatment programme, discuss your individual symptoms and assess your suitability for treatment. [londonorthotics.co.uk]

In any case, the method seems to be a valuable adjunct therapy in the treatment of PE. [doi.org]

Prognosis

Ruling out the concomitance of pectus excavatum and connective tissue disorders, therefore, may have a direct implication both on surgical outcome and long term prognosis. [ncbi.nlm.nih.gov]

However, the prevalence of several risk factors for tinea cruris, such as obesity and diabetes mellitus, is rapidly increasing among adolescents. [4] Prognosis The prognosis of tinea cruris is excellent with appropriate diagnosis and treatment; however [emedicine.medscape.com]

Treatment and prognosis Although historically most cases were not treated, and early research suggested that even in extreme cases repair does not offer significant improvement in respiratory function tests 2 more recently symptomatic improvement in ventilation [radiopaedia.org]

Etiology

Although the etiology remains unclear, the pathogenesis of PE is currently thought to involve the overgrowth of the costochondral region of the ribs. [ncbi.nlm.nih.gov]

Etiology has been discussed as it may alter the type of surgery required in various cases. The extreme hazard of tension pneumothorax and the mechanism of its production peculiar to these cases are pointed out. [pdfs.journals.lww.com]

Further studies on larger series of patients are needed in order to reveal possible common etiological factors in PE and PSP. [hindawi.com]

The etiology, as well as risk factors, remain unclear. [symptoma.com]

Epidemiology

Rural-Based Tertiary Health Care Center. 61 Pariath K...Nair PA 31543529 2019 5 Prevalence and clinical characteristics of itch in epidemic-like scenario of dermatophytoses in India: a cross-sectional study. 61 Verma S...Szepietowski JC 31419346 2019 6 Epidemiology [malacards.org]

Epidemiology [ 1 ] This is much less common than pectus excavatum. The prevalence in the USA has been assessed at 0.06% with one third of patients having a positive family history. [patient.info]

To determine whether there were differences in survival patterns, we tested whether pectus excavatum patients survived longer than controls, using a standard epidemiological method. [ncbi.nlm.nih.gov]

Epidemiology: It occurs in 1 in 400 births. Etiology (cause): Sometimes the child may have been born with a pectus. It may get worse with age. Some people think that the cartilaginous ribs grow unevenly, pushing down the breastbone. [eapsa.org]

Pathophysiology

The current article provides an in depth review of the pathophysiology and clinical characteristics of pectus excavatum, as well as an overview of the treatment options in order to help the practitioners caring of affected patients in their evaluation [prrjournal.com]

Bauhinus [ 1 ] gave the first pathophysiological hypothesis, mentioning a hypertension of the diaphragm during embryonic development as the pathophysiologic factor. [doi.org]

Pathophysiology The most common etiologic agents for tinea cruris include Trichophyton rubrum and Epidermophyton floccosum; less commonly Trichophyton mentagrophytes and Trichophyton verrucosum are involved. [emedicine.medscape.com]

References Koumbourlis, A.C. (2009) Pectus excavatum: pathophysiology and clinical characteristics. Paediatric Respiratory Reviews 10:3–6. [healthand.com]

Pathophysiology In pectus excavatum, the growth of bone and cartilage in the anterior chest wall is abnormal, typically affecting 4-5 ribs on each side of the sternum. [emedicine.com]

Prevention

Early correction of this condition can prevent mediastinal shift. [medical-dictionary.thefreedictionary.com]

This time is sufficient for formation of a solid callus that prevents recurrence of the deformity. [ncbi.nlm.nih.gov]

The means of avoiding this complication is tight closure of the skin, which will prevent air being pulled into the mediastinum during respiration. © Lippincott-Raven Publishers. [pdfs.journals.lww.com]

In the hours following the procedure, he or she will need to take deep breaths using a breathing machine (incentive spirometer) to prevent lung congestion and pneumonia. [healthcare.utah.edu]

The exercises can also prevent progression of a mild case, possibly make surgical correction easier and help prevent a recurrence after surgery. [livestrong.com]

References

  1. Brochhausen C, Turial S, Müller FKP, et al. Pectus excavatum: history, hypotheses and treatment options. Interact Cardiovasc Thorac Surg. 2012;14(6):801-806.
  2. Jaroszewski D, Notrica D, McMahon L, Steidley DE, Deschamps C. Current management of pectus excavatum: a review and update of therapy and treatment recommendations. J Am Board Fam Med. 2010;23(2):230-239.
  3. Robbins LP. Pectus excavatum. Radiol Case Rep 2011;6(1):460
  4. Kelly RE Jr. Pecus excavatum: historical background, clinical picture, preoperative evaluation and criteria for operation. Semin Pediatr Surg. 2008;17(3):181-193.
  5. Tocchioni F, Ghionzoli M, Messineo A, Romagnoli P. Pectus Excavatum and Heritable Disorders of the Connective Tissue. Pediatr Rep. 2013;5(3):e15.
  6. Winkens R, Guldemond F, Hoppener P, Kragten H, van Leeuwen Y. Pectus excavatum, not always as harmless as it seems. BMJ Case Rep. 2009;2009:bcr10.2009.2329.
  7. Harris C, Croce B, Cao C. Pectus excavatum. Annals of Cardiothoracic Surgery. 2016;5(5):528.
  8. Oncel M, Sunam GS, Tezcan B, Gurol Akyol K, Dereli Y. Clinical experience of repair of pectus excavatum and carinatum deformities. Cardiovascular Journal of Africa. 2013;24(8):318-321.
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