CHEST WALL DEFORMITY

1. JAMIA MILLIA ISLAMIA
CENTRE FOR PHYSIOTHERAPY AND REHABILITATION SCIENCES
SUBJECT: PHYSIOTHERAPY IN CARDIOPULMONARY CONDITIONS
TOPIC: CHEST WALL DEFORMITY
SUBJECT CODE: BPT(402)
CLASS: BPT 4TH YEAR
PRESENTATION DATE: 31.12.2020
SUBMITTED TO: DR. JAMALALI MOIZ
SUBMITTED BY: SAMEERA FAIZVI

2. INTRODUCTION:
 Chest wall deformities are a set of congenital diseases that span a broad spectrum of disorders.
 It encompass a wide range of anomalies which extend from the sternum to the vertebral column.
 These can manifest as isolated conditions or can correlate with extra-thoracic anomalies and
genetic syndromes.
 Deleterious effects can occur from birth to adolescence, ranging from life-threatening conditions
to psychosocial cosmetic concerns.
 Regardless, the severity of the malformation usually progresses rapidly during puberty.
 Chest wall deformity is associated with cardiac and respiratory problems and connective tissue
disorders.

3. CLASSIFICATION:

4. STERNUM DEFORMITIES:
 Cleft sternum is a rare congenital defect of the anterior chest wall and is the result of a failed
midline fusion of the sternum.
 A classification proposed by Shamberger and Welch in 1990 divided sternal clefts into 4 types:
 Thoracic ectopia cordis: In this form of sternal cleft the heart is ectopic and devoid of a skin
covering. Ectopia cordis is defined as complete or partial displacement of the heart outside the
thoracic cavity. It is a rare congenital defect in fusion of the anterior chest wall resulting in extra
thoracic location of the heart.
 Cervical ectopia cordis: In these rare defects the heart is located more cranially . Fusion between
the apex of the heart and the mouth is often present, as are severe craniofacial anomalies.
 Thoracoabdominal ectopia cordis: In this condition the heart is covered by a thin membranous or
cutaneous layer within a caudal located defect. The heart which may be located either in the thorax
or in the abdomen, is not rotated as in the previously types described, but is accompanied by
intrinsic anomalies. This anomaly is a part of the Pentalogy of Cantrell.
 Sternum cleft: The partial deformities could have a cranial or caudal location. The caudal forms
are often associated with a thoraco-abdominal ectopia cordis, whereas the cranial partial clefts exist
as isolated malformations. Sternal clefts result from failure of fusion of the mesenchymal plate
during the eighth embryonic week. The defect can be partial (superior or inferior) or complete.

7. TYPE 2- COSTAL CARTILAGE DEFORMITIES
 Pectus excavatum (PE): Pectus excavatum (PE), or funnel chest, describes a posterior depression
of the lower sternum and costal cartilages into the thoracic cavity. Cosmetic appearance is the main
presenting reason in asymptomatic patients.
 Kuhn et al catergorised excavatum types of deformity into 4 type in terms of their morphological
appearance.
 cup shaped (localized deep depression)
 Saucer shaped(diffuse superficial depression)
 Grand canyon(depression in the form of assymetrical long pipe)
 Curarino silverman deformity(pouter pigeon deformity, mixed carinatum)
 Aetiology: The sternal depression is thought to result from asymmetrical growth of the
costochondral cartilages. However, the exact aetiology is unknown. There is an association with
connective tissue disorders such as Marfan’s syndrome (21.5%) and Ehlers-Danlos syndrome
(2%).
 Clinical Presentation: Common symptoms attributed to PE include exercise intolerance,
dyspnoea, chest pain with and without exercise, and palpitations. Patients often have a slouched
posture, and young children have an associated protuberant abdomen. Almost a quarter of PE cases
are associated with scoliosis, and hence the spine should be investigated in all cases.

8.  Investigation: Computerized Tomography (CT) scan are necessary to access the severity of the
deformity and to calculate the Haller index to estimate the severity of chest depression in patients
with PE. (Haller index is the ratio of the transverse diameter and the anteroposterior diameter of the
rib-cage. Haller index in a normal chest is around 2.5, but in pectus excavatum the index can reach
3.25 or even as high as 5.5) ; Chest x-rays, both anteroposterior (AP) and lateral, are routinely per-
formed, which may help to define the severity of the deformity as well as help to evaluate the
thoracic spine.
 Conservative treatment: physical exercise has an important role in conservative pectus
excavatum treatment though is not seen as a means to resolve the condition on its own. It is used in
order to halt or slow the progression of mild or moderate excavatum condition and as
supplementary treatment to improve a poor posture to prevent secondary complications, and to
prevent relapse after treatment.
 Exercises are aimed at improving posture, strengthening back and chest muscles, and enhancing
exercise capacity, ideally also increasing chest expansion. Pectus exercises include deep breathing
and breath holding exercises as well as strength training for the back and chest muscles.
Additionally, aerobic exercises to improve cardiopulmonary function are employed.

10. PECTUS CARINATUM ( PIGEON CHEST )
 Pectus carinatum: pigeon chest, is a spectrum of anterior chest wall anomalies characterized by
protrusion of the sternum and adjoining costal cartilages.
 The deformity may be classified as either chondrogladiolar or chondromanubrial, depending on the
site of greatest prominence.
 Type 1- Chondrogladiolar: The symmetric sternal protrusion is located in the inferior or mid
sternum region. The lower ribs may be slightly or severely depressed on lateral side.
 Type 2-Chondromanubrial: These have been frequently referred to as Currarino- Silverman
syndrome or Pouter Pigeon Breast. Currarino-Silverman syndrome is a rare disorder characterized
by premature fusion of manubrio-sternal joint and the sternal segments, resulting in a high carinate
chest deformity.
 Aetiology: The underlying aetiology for PC is unknown and thought to be related to overgrowth of
the costal cartilages. A familial incidence of PC is seen in up to 26% of patients. There is an
association with connective tissue disorders, such as Marfan’s syndrome, scoliosis (34%), and
congenital heart disease (6%).
 Clinical presentation: Symptoms include exertional dyspnea, decreased exercise tolerance, and
precordial chest pain. The majority of patients, however, present because of the cosmetic
deformity. Some children with pectus carinatum also have scoliosis (i.e. curvature of the spine).

11.  Investigation: Either a PA and lateral chest x-ray or a CT scan will allow good visualization
of the extent of the abnormality. Any spinal abnormality should also be evaluated. The respiratory
and cardiac functions should be assessed.
 Management / Interventions:
 External bracing technique: the use of a customized chest-wall brace that applies direct pressure
on the protruding area of the chest produces excellent outcomes. The brace consists of front and
back compression plates that are anchored to aluminum bars. These bars are bound together by a
tightening mechanism which varies from brace to brace. This device is easily hidden under
clothing and must be worn from 14 to 24 hours a day. Regular supervision during the bracing
period is required for optimal results. Adjustments may be needed to the brace as the child grows
and the pectus improves.

12. PHYSIOTHERAPY MANAGEMENT:
 Postural correction and strengthening chest and back muscles may help with pectus excavatum as
bad posture is implicated in the severity and prominence of the condition.
 The aim of exercises in this condition is to:
a. Loose and lengthen tight or contractured tissues.
b. Improve chest wall and spinal mobility
c. Strengthen muscles needed for chest expansion and elevation
d. Restore and maintain normal posture
e. Improve and maintain lung compliance
 Exercises such as the use of dumbbells to strengthen the latissimus dorsi and rhomboids.

14. TYPE 3- RIB DEFORMITIES
 Based on the radiological overview on pediatric rib deformities presented in 2002, the deformities
in ribs are based on their origin- congenital or acquired.
 Congenital deformity
a. Cervical ribs: Cervical ribs arise from the seventh cervical vertebra. Cervical ribs are rarely
symptomatic in early childhood
b. Abnormal Number of Ribs: An alteration in the number of ribs has been found in trisomy 21
syndrome and in patients with the VATER association
c. Abnormal Size or Shortening of Ribs: Short ribs do not extend as far anteriorly as the sternum.
The resultant diminished volume of the chest restricts respiratory motion and causes respiratory
insufficiency. Short ribs constitute an integral part of several syndromes.

16. TYPE 4- COMBINED COSTAL CARTILAGE AND RIB DEFORMITIES
 Poland Syndrome (PS): It is a rare congenital anomaly characterized by hypoplasia of the breast
and nipple, scarcity of subcutaneous tissue, absence of the costosternal portion of the pectoralis
major muscle, lack of the pectoralis minor muscle, aplasia or deformity of the costal cartilages or
ribs II to IV or III to V, alopecia of the axillary and mammary region, and unilateral
brachysyndactyly.
 Many variations of the syndrome have been described, from mild hypoplasia of the pectoralis
major musculature to severe hypoplasia of the thoracic wall.
 Etiology: The exact etiology is unknown, the proposed etiology id the disruption of subclavican
arterial blood supply of limb bud during 6th foetal week.
 Clinical features: anatomical abnormalities are usually unilateral. Clinically, patient have absent
anterior axillary fold with posterior axillary fold being easily visible from front. The nipple and
aerola may be hypoplastic or absent with deficient subcutaneous tissues. The chest is depressed on
affescted side due to hypoplasia or absence of underlying 2-4 or 3-5 ribs and cartilages. Rarely
lung may herniate through defect in chest wall giving flail segment. This may cause respiratory
distress in newborn period.

17.  Investigation:
 CT scan will show absent perctoralis major
 Prenatal sonographic evaluation can demonstrate unilateral limb defects and unilateral chest wall
asymmetry and other associated anatomical defect
 Mammogram, hypoplasia of the unilateral breast and hypoplasia of the pectoralis major
 Chest radiograph findings (unilateral hyperlucent thorax).
 Ultrasound of the chest wall (defects of the pectoralis major and minor musculature).
 Management: surgical interventions

20. ASPHYXIATING THORACIC DYSPLASIA
 Asphyxiating thoracic dysplasia (ATD), also known as Jeune syndrome, is a rare inherited bone
growth disorder that primarily affects the thoracic region.
 Common signs and symptoms can include a narrow chest, short ribs, shortened bones in the arms
and legs, short stature, and extra fingers and toes.

21. REFERENCES:
 Chest wall deformity by amulya k saxena
 Chest wall deformity by Michael singh, dakshesh parikh and brian kenney
 https://en.wikipedia.org/wiki/Asphyxiating_thoracic_dysplasia
 https://en.wikipedia.org/wiki/Pectus_excavatum
 Review article on congenital chest wall deformity by Yedikule Göğüs Hastalıkları Hastanesi Ve Göğüs Cerrahisi Merkezi, İstanbul. 2 Okan Üniversitesi Tip Fakültesi Göğüs Cerrahisi A.D.
 Shad, J., Budhwani, K., & Biswas, R. (2012). Thoracic ectopia cordis. BMJ case reports, 2012, bcr1120115241. https://doi.org/10.1136/bcr.11.2011.5241
• Article on Poland syndrome Chandra Madhur Sharma, Shrawan Kumar,1 Manoj K. Meghwani,2 and Ravi P. Agrawal Indian J Hum Genet. 2014 Jan-Mar; 20(1): 82–84.doi: 10.4103/0971-
6866.132764
 https://www.sciencedirect.com/topics/medicine-and-dentistry/ectopia-cordis
 https://thoracickey.com/other-chest-wall-deformities/
 https://studyres.com/doc/3318151/chapter-53-chest-wall-deformities
 https://obgynkey.com/congenital-chest-wall-deformities/
 https://physio-pedia.com/Pectus_carinatum_(_pigeon_chest_)?utm_source=physiopedia&utm_medium=search&utm_campaign=ongoing_internal
 Poland’s Syndrome Revisited Alexander A. Fokin, MD, PhD, and Francis Robicsek, MD, PhD The Department of Thoracic and Cardiovascular Surgery, Carolinas Heart Institute, Carolinas Medical
Center, Charlotte, North Carolina
 Joshua, S.A., Shetty, L., Pare, V., & Sebastian, R. (2012). Chondro-manubrial deformity and bifid rib, rare variations seen in pectus carinatum: a radiological finding. Surgical and Radiologic
Anatomy, 35, 443-447.
 Pediatric Ribs: A Spectrum of Abnormalities by Ronald B. J. Glass, MD ● Karen I. Norton, MD ● Sandra A. Mitre, MD Eugene Kang, MD
 Tafti D, Cecava ND. Poland Syndrome. [Updated 2020 Nov 20]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020 Jan