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
- Pain
Care in the hospital following surgery The surgical repair of pectus excavatum is a painful procedure. Length of stay in the hospital is determined primarily by pain control. [pedsurg.ucsf.edu]
Pain medications are injected through this route, which the patient may also be able to control with a button. PCA: Patient controls when pain medication is given. A syringe of pain medication is connected to the patient’s IV. [eapsa.org]
Pain Management Our team uses patient-controlled anesthesia and works very closely with our pain management team to help control postoperative pain. [hopkinsallchildrens.org]
Pain Control: Pain control service at Phoenix Children’s Hospital will help manage and keep the patient comfortable post-operatively. [surgery4children.com]
- Weakness
There is only one report about CPR in a patient with a sternal Nuss bar, in which paramedics reported difficulty in performing CPR because of high resistance against compressions, but they had been able to obtain a weak pulse while performing compressions [emergency-live.com]
The sunken chest appearance is further emphasised by poor posture and weak and tight chest muscles associated with the condition. [pectuspt.com]
There is general muscle weakness and pain, extreme neck weakness, head tremor, a stiff stilted gait and affected kittens are reluctant to walk. [messybeast.com]
These include Mongoloid eye shape, arachnodactyly, and high palate, hyperelasticity of skin, ears dysplasia, dolichostenomelia, scoliosis, mitral valve prolapse, umbilical hernia, and weakness of sphincters. [iliveok.com]
- Inflammation
Antifungal drugs applied to the skin or sometimes taken by mouth Antifungal drugs taken by mouth (such as itraconazole or terbinafine) may be needed in people who have infections that are widespread or cause inflammation or infections that are difficult [msdmanuals.com]
The steroid reduces inflammation and may ease itch and redness quickly. However, the steroid does not kill the fungus and so a steroid cream alone should not be used. [patient.info]
It is also possible that this generally decreases the movement of the pectus bar in the tunnel created in the anterior part of the thoracic cavity causing less inflammation and reduced problems with seroma. [doi.org]
Inflammation raises the temperature, leading to temporary or permanent lightening of the fur in that area. [messybeast.com]
- Trisomy 21
It may occur as a solitary congenital abnormality or in association with other genetic disorders or syndromes : Marfan syndrome, Ehlers Danlos Syndrome, Morquio syndrome, Noonan syndrome, Trisomy 18, Trisomy 21, homocystinuria, osteogenesis imperfecta [health.ucdavis.edu]
Respiratoric
- Dyspnea
Preoperatively dyspnea functional grades in group I were 15% in grade IV, 32.5% in grade III and 22.5% in grade II, while in group II preoperatively, dyspnea were 8.7% in grade IV, 28.26% in grade III and 13.04% in grade II (Table 2). [cardiothoracicsurgery.biomedcentral.com]
A 5.5-month-old female domestic longhair cat was examined because of dorsal deviation of the caudal aspect of the sternum and progressively worsening dyspnea during play activities. [ncbi.nlm.nih.gov]
The reduced exercise capacity is primarily related to exertional dyspnea caused by a diminished stroke volume and ventilatory restrictions. [medscape.com]
(de Rooster, Polis) Case Description —A 5.5-month-old female domestic longhair cat was examined because of dorsal deviation of the caudal aspect of the sternum and progressively worsening dyspnea during play activities. [doi.org]
- Barrel Chest
chest [ chest ] barrel chest a rounded, bulging chest with abnormal increase in the anteroposterior diameter, showing little movement on respiration; seen in emphysema, kyphosis, and chronic airflow limitation. Barrel chest. [medical-dictionary.thefreedictionary.com]
This index better addresses the phenomena of “barrel” and “pancake” shaped chests with deformity than the Haller index. [annalscts.com]
In some patients with emphysema, the posterior AP diameter is increased and the two measurements may approximate ( barrel chest ). The degree of chest deformity in emphysema is not a reliable guide to the severity of the functional defect. [78stepshealth.us]
The rationale was that it seemed unnecessary to perform an extensive and radical resection when the malleability of the thoracic cage is well demonstrated clinically by the observation that a characteristic ‘barrel chest’ develops even in adult patients [doi.org]
- Aspiration
Three complications were encountered: in one patient hemothorax occurred that was cured by a single punction aspiration, in other patient one of the Kirschner's pins slipped into the thoracic cavity. [ncbi.nlm.nih.gov]
Tatum, a senior at Glendale’s Mountain Ridge High School and an aspiring make-up artist, had pectus excavatum surgery in mid-June. “Right now I find it easier to stay awake during the day and my naps have reduced immensely,” says Tatum. [raisingarizonakids.com]
The nature of skin and subcutaneous nodules may have to be determined by aspiration or biopsy. [78stepshealth.us]
Many surgeons consider mandatory drainage of retrosternal space with active aspiration by Redon for 3 days. Retrosternal space is drained by polyethylene tubing. [iliveok.com]
Medication is possible, but there is a danger of recurrence and of death from aspiration pneumonia. MYCOBACTERIUM AVIUM-INTRACELLULARE COMPLEX (MAC) Type: Predisposition Breeds: Abyssinian, Somali, Siamese MAC is a slow-growing bacterium. [messybeast.com]
- Sighing
It will feel like a big relieving sigh when that tendon opens. But don’t be surprised if there is accompanying fear or terror as you get into the breathing. [anatomytrains.com]
Cardiovascular
- Heart Murmur
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 some patients, the proximity of the sternum and the pulmonary artery may cause a heart murmur. For ordinary everyday activities, a person with pectus excavatum may have no symptoms, but with rigorous exercise, symptoms often appear. [columbiasurgery.org]
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]
murmurs (UCLA) 16% of patients have mild or moderate scoliosis (UCLA) Though not universal, we don’t want to overstate the potential respiratory or heart problems associated with the condition; many people with a pectus deformity do not suffer from any [londonorthotics.co.uk]
- 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
- Sweating
Jock itch gets its name because it is common in people who sweat a lot, as do athletes. It also is more likely to occur in people who are overweight. Although often uncomfortable and bothersome, jock itch usually isn't serious. [mayoclinic.org]
It is more common if you have unusual heat, sweating, or friction in the groin, or are overweight. What are the symptoms of tinea cruris? Symptoms include: Red patches along the upper thigh, butt, and groin. [saintlukeskc.org]
Loose clothing can prevent sweating and the warm, moist environment in which fungus thrives. Make sure you wash any workout clothes or athletic supporters after each use. [healthline.com]
The following steps will help prevent the itchy condition: Wash groin and buttocks with soap and water after exercise and sweating. Wash workout clothes, underwear, and swimwear after each use. [medicinenet.com]
If you are overweight, try to lose weight to reduce chafing and sweating. Diagnosis of tinea cruris The diagnosis of tinea cruris is confirmed by microscopy and culture of skin scrapings. [dermnetnz.org]
Musculoskeletal
- Chest Deformity
Abstract The cases of 36 out of 62 patients who underwent surgical correction of funnel chest deformity between 1978 and 1988 with an average follow-up of 7.6 years were reviewed. [ncbi.nlm.nih.gov]
Pectus excavatum is the medical term for a chest deformity. It is sometimes also referred to as pigeon or funnel chest. The chest deformity is distinguished by a sunken sternum accompanied usually with flared ribs. [healdove.com]
According to this hypothesis, the small lung to chest volume ratio augment the negative intrapleural pressure thereby deforming the chest wall. [erj.ersjournals.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.
Treatment
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]
This experience helps us easily diagnose your child’s condition and work with you to decide on the best treatment. At Seattle Children’s, your family has a full team behind you, from diagnosis through treatment and follow-up. [seattlechildrens.org]
Pulmonary function tests may be required to assess lung capacity. [ 5 ] Management [ 2 ] Mild cases may not require treatment, as the deformity can be hidden under clothing. [patient.info]
Treatment of pectus excavatum Vacuum Bell Mild to moderate pectus excavatum may be improved by a focused exercise regimen and/or a vacuum bell approach. [stanfordchildrens.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]
Outlook (Prognosis) The surgery usually leads to improvements in appearance, breathing, and ability to exercise. References Nuss D, Kelly RE. Congenital chest wall deformities. In: Holcomb GW, Murphy JP, Ostlie DJ, eds. [leehealth.org]
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
Further studies on larger series of patients are needed in order to reveal possible common etiological factors in PE and PSP. [hindawi.com]
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 (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]
The etiology, as well as risk factors, remain unclear. [symptoma.com]
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]
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]
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]
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]
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
Day following surgery Your child will be expected to get up out of bed and walk the day following surgery and will need to practice deep breathing to keep the lungs healthy and prevent pneumonia. [pedsurg.ucsf.edu]
Early correction of this condition can prevent mediastinal shift. [medical-dictionary.thefreedictionary.com]
The exercises can also prevent progression of a mild case, possibly make surgical correction easier and help prevent a recurrence after surgery. [livestrong.com]
This time is sufficient for formation of a solid callus that prevents recurrence of the deformity. [ncbi.nlm.nih.gov]
References
- 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.