Funnel Chest
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.
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].
Entire Body System
Chills
- I am hoping my ds is as chilled about it all as he seems to be. He is one of dh best mates so hopefully he will still be around when ds is older and perhaps more bothered by it.[mumsnet.com]
Sepsis
- Along with above complications without statistical revisions, mediastinitis, sepsis osteomyelitis of sternum, migration locks, secondary bleeding, skin necrosis, intestinal paresis, hemopericardium, pericarditis, myocarditis, keloid scars are distinguished[iliveok.com]
Cardiovascular
Heart Murmur
- Other notable findings include a pot-belly posture, forward-drifted shoulders, a heart murmur on cardiac auscultation and arrhythmia in severe cases.[symptoma.com]
- 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]
- Some common symptoms may include: lower tolerance of exercise palpitations or a fast heartbeat recurring respiratory infections coughing or wheezing chest pain heart murmur fatigue and tiredness The precise cause of pectus excavatum is not yet known ,[leading-medicine-guide.com]
- Out of 90 patients who had heart murmurs before the surgery, 74 improved so much that the murmurs could no longer be heard.[webmd.com]
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]
Musculoskeletal
Osteophyte
- Kondrashin, distinguish following forms in funnel-shaped deformation of sternum - flat, sternum with a hook and sternum with osteophytes.[iliveok.com]
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]
Psychiatrical
Negativism
- Especially indicators grow with age, adversely affecting development and growth of child; apathy, shyness and alienation in relationships with peers, negativism and indifference in relation to parents.[iliveok.com]
Neurologic
Screaming
- To open the tendon is a matter of the primal scream, best induced through rebirthing / holotropic breath work / whatever it’s being called these days. You need a guide for that, preferably experienced. -Tom I found the tendon in Netters.[anatomytrains.com]
- Characteristic feature in infants is symptom of "paradox of inspiration": during inspiration and especially when child is crying or screaming, retraction of sternum and ribs increases. G.I.[iliveok.com]
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.
X-Ray
Pericardial Effusion
- It reduces pleural secretion and pain, and seems to reduce pneumothorax, pulmonary bar adhesions, and pericardial effusions.[ncbi.nlm.nih.gov]
- In two patients we had pericardial effusion six months after the Nuss procedure, requiring pericardiocentesis. In one patient we had fracture of the sternum. There were no complications following bar removal.[ncbi.nlm.nih.gov]
- These include pneumothorax, wound seroma, bar displacement, pericarditis and pericardial effusion, pleural effusion, hemothorax [3].[doi.org]
Pulmonary Infiltrate
- This sign is due to compression of the lungs by the bent costal cartilages of the pectus and should not be interpreted as evidence of pulmonary infiltrate.[ncbi.nlm.nih.gov]
Treatment
- There is no standard treatment protocol, but early treatment is necessary to improve airway functions and infant feeding, and to support proper nutrition for the growth of maxillofacial region.[ncbi.nlm.nih.gov]
- Treatment time was similar for 3D-CRT (3 min) and VMAT (4 min) but substantially increased for IMRT (13 min). VMAT is considered the best treatment option for the presented case of a patient with funnel chest.[ncbi.nlm.nih.gov]
- When it occurs concomitantly with esophageal cancer, it hinders intrathoracic surgery that is necessary for treatment.[ncbi.nlm.nih.gov]
- This retrospective study confirms that our treatment strategy of objective criteria for operation (functional compression index 0.2, % vital capacity[ncbi.nlm.nih.gov]
- We conclude that a VI of more than 27 and/or a FSI of less than 29 are indications for surgical treatment based on the mean VI 3SD and FSI - 3SD of normal children over 3 years of age.[ncbi.nlm.nih.gov]
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]
- 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]
- Outlook (Prognosis) The surgery usually leads to improvements in appearance, breathing, and ability to exercise. References Gottlieb LJ, Reid RR, Lee JC. Pediatric chest and trunk defects. In: Neligan PC, ed. Plastic Surgery . 3rd ed.[stlukes-stl.com]
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]
- The etiology, as well as risk factors, remain unclear.[symptoma.com]
- The etiology of pectus excavatum is uncertain, but a familial tendency has been found in clinical experience, where it may be seen in more than one sibling.[ncbi.nlm.nih.gov]
Epidemiology
- 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]
- Epidemiology Frequency United States Pectus excavatum occurs in an estimated 1 in 300-400 births, with male predominance (male-to-female ratio of 3:1). Pectus excavatum comprises approximately 90% of all chest wall deformities.[emedicine.com]
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 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
- This time is sufficient for formation of a solid callus that prevents recurrence of the deformity.[ncbi.nlm.nih.gov]
- […] second costal cartilages to allow a repositioning of the vascular pedicle onto the presternal surface; (3) turning the sternum over, placing one end on the other, and attaching one end to the other; and (4) making a groove in the turned-over sternum to prevent[ncbi.nlm.nih.gov]
- Early correction of this condition can prevent mediastinal shift.[medical-dictionary.thefreedictionary.com]
- 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]
- Overview Recommended specialists Further information Overview of pectus excavatum (funnel chest) Symptoms of pectus excavatum Causes of pectus excavatum Diagnosis of pectus excavatum Treatment of pectus excavatum Chances of recovery from pectus excavatum Prevention[leading-medicine-guide.com]
References
Article
- Brochhausen C, Turial S, Müller FKP, et al. Pectus excavatum: history, hypotheses and treatment options. Interact Cardiovasc Thorac Surg. 2012;14(6):801-806.
- 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.
- Robbins LP. Pectus excavatum. Radiol Case Rep 2011;6(1):460
- Kelly RE Jr. Pecus excavatum: historical background, clinical picture, preoperative evaluation and criteria for operation. Semin Pediatr Surg. 2008;17(3):181-193.
- Tocchioni F, Ghionzoli M, Messineo A, Romagnoli P. Pectus Excavatum and Heritable Disorders of the Connective Tissue. Pediatr Rep. 2013;5(3):e15.
- 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.
- Harris C, Croce B, Cao C. Pectus excavatum. Annals of Cardiothoracic Surgery. 2016;5(5):528.
- 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.