Pectus Excavatum (Cobbler's Chest, Sunken Chest, Funnel Chest)

Clinical Features:

• Anomaly that arises from posterior depression (sunken appearance) of the sternum.
• May compress the heart against the spine, deviating the heart to the left.

Complications:

• Cardiac - apparent cardiomegaly, cardiac compromise, mitral valve prolapse
• Respiratory - lung basal collapse, respiratory compromise, restrictive airway disease
• Skeletal - back pain, chest pain
• Psychosocial - cosmetic issues

Epidemiology

• Pectus excavatum occurs in approximately 1 in 150 to 1000 births.
• There is a male predominance (male-to-female ratio of 3:1).
• Family members affected in 35% to 45% of cases.[1][2]

Associations:

• Isolated anomaly (most commonly)
• Diaphragm abnormalities
• Familial (~37% have 1st degree relative)
• Fetal alcohol syndrome
• Homocystinuria
• Intra uterine pressure
• Marfan syndrome
• Noonan syndrome
• Prematurity
• Rickets
• Spinal muscular atrophy

Chest Xray Findings:

• May mimic cardiomegaly.
• Displacement of the pulmonary vascularity & obscured right cardiac border resemble a right middle lobe process.
• Posterior ribs are horizontal.
• Anterior ribs have a more vertical course.

Management - Haller Index Calculation:

• The Haller index can be used based on CT scan measurements.
• The Haller index is the ratio between the horizontal distance of the inside of the ribcage & the shortest distance between the vertebrae & sternum.[3]
• A Haller index over 3.25 is often defined as severe.[4]

Management - Surgery:

• Surgical correction - relief of restrictive airway disease & for cosmesis.
• Techniques include - Magnetic mini-mover procedure, Nuss procedure, Ravitch technique, & Vacuum bell technique.

References:

* [1] - Andre Hebra. "eMedicine — Pectus Excavatum". [link]
* [2] - "Pectus Excavatum: Frequently Asked Questions: Surgery: UI Health Topics". Harold M. Burkhart and Joan Ricks-McGillin. [link]
* [3] - Haller JA, Kramer SS, Lietman SA (1987). "Use of CT scans in selection of patients for pectus excavatum surgery: a preliminary report". J. Pediatr. Surg. 22 (10): 904–6.
* [4] - Jeannette Diana-Zerpa; Nancy Thacz Browne; Laura M. Flanigan; Carmel A. McComiskey; Pam Pieper (2006). Nursing Care of the Pediatric Surgical Patient (Browne, Nursing Care of the Pediatric Surgical Patient). Sudbury, Mass: Jones & Bartlett Publishers. p. 253. ISBN 0-7637-4052-7.
* Shamberger RC (1996). "Congenital chest wall deformities". Curr Probl Surg 33 (6): 469–542.

Image Credit (Top): Chest Xray by Dr Donna D'Souza on RadPod (cc)

Image Credit (Top): Scar Post Surgery by Jordan Hackworth on Flickr (cc)

Tags: Back Pain - Chest Pain - Funnel Chest - Homocystinuria - Marfan Syndrome - Mitral Valve Prolapse - Noonan Syndrome - Pectus Excavatum - Prematurity - Rickets - Spinal Muscular Atrophy - Surgery
Posted by Medicalchemy
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Skeleton Of Woman From Back (Skull To Pelvis)

Skeleton Of Woman From Back

(Skull To Pelvis):

Image Credit: Plastische Anatomie des menschlichen Körpers für Künstler und freunde der kunst von Dr. Julius Kollmann o. ö. professor der anatomie zu Basel. Mit menreren hundert ..... Leipzig verlag von veit & Comp. 1910. page 514

Tags: Back - Chest - Pelvis - Skeleton - Spine - Woman
Posted by Medicalchemy
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Bifid Ribs

Bifid Rib Clinical Features:

• Bifid rib is usually unilateral.
• May occur in isolation.
• Bifid ribs are usually asymptomatic.
• May also found with multi-system malformations.
• They often discovered incidentally by chest x-ray.

Clinical Assessment:

• Examine the mouth & skin carefully.
• Physical findings such as odontogenic cysts, subcutaneous calcifications, & palmar pits should prompt more extensive radiological / radiological investigations.
• With normal physical examination, & asymptomatic patient, further radiological assessment gives a low yield for abnormalities.

Clinical Associations:

• Gorlin-Goltz (Basal cell nevus) syndrome (65-70% of patients) [2,10]
• Job's syndrome [3]
• Kindler syndrome [4]
• Malignancy in childhood (esp neuroblastoma) [5]

Complications:

• Asymptomatic (usually)
• Intercostal nerve entrapment
• Musculoskeletal pain
• Thoracic outlet syndrome [6]

Epidemiology:

• Prevalence of bifid rib ~ 0.15% to 3.4% (~ 2%). [1]
• Up to 20% of all congenital rib anomalies. [1]
• Bifid ribs occur in up to 8.4% of Samoans. [8]
• Bifid ribs on chest Xray occur in 2.8% of Koreans. [9]

Morphological Patterns:

• Two distinct morphological patterns. [7]
• 1) - long and slender (fissured) bifid spaces, with the bifid rib appearing as a two-pronged fork (fork type). [7]
• 2) - more rounded bifid spaces (hole type). [7]

Pathophysiology:

• Ribs originate from the mesoderm.
• Bifid ribs may therefore be associated with other mesodermal abnormalities.
• Look specifically for malformations in other organs of mesodermal origin, i.e. heart & kidneys. [1]

Prognosis:

• That of underlying cause.
• There is little information in the literature about the clinical significance of bifid ribs.

References:

• [1] - Charles I, Scott J. Pectoral girdle, spine, ribs, and pelvic girdle. In: Stevenson RE, Hall JG, Goodmann RM, eds. Human Malformations and Related Anomalies, vol 2. Oxford University Press: New York, 1993:655–697.
• [2] - Rai S, Gauba K. Jaw cyst-basal cell nevus-bifid rib syndrome: a case report. J Indian Soc Pedod Prev Dent 2007; 25:137–139.
• [3] - Freeman AF, Holland SM. The hyper-IgE syndromes. Immunol Allergy Clin North Am 2008; 28:277–291.
• [4] - Sharma RC, Mahajan V, Sharma NL, Sharma AK. Kindler syndrome. Int J Dermatol 2003; 42:727–732.
• [5] - Schumacher R, Mai A, Gutjahr P. Association of rib anomalies and malignancy in childhood. Eur J Pediatr. 1992;151:432–434. [PubMed]
• [6] - K. Cağli. Thoracic outlet syndrome in an adolescent with bilateral bifid ribs. Clin Anat. 2006 Sep;19(6):558-60.
• [7] - Wu-Chul Song, Sang-Hyun Kim, Dae-Kyoon Park, and Ki-Seok Koh. Bifid Rib: Anatomical Considerations in Three Cases. Yonsei Med J. 2009 April 30; 50(2): 300–303
• [8] - Martin EJ. Incidence of bifidity and related rib abnormalities in Samoans. Am J Phys Anthropol. 1960;18:179–187.
• [9] - Lim CK, Lee KW, Bin JC, Rhee BC. Congenital anomalies of the ribs. J Korean Soc Plast Reconstr Surg. 1982;18:487–495.
• [10] - Bitar GJ, Herman CK, Dahman MI, Hoard MA. Basal Cell Nevus Syndrome: Guidelines for Early Detection. Am Fam Physician. 2002 Jun 15;65(12):2501-2505.

Image: Bifid Rib (CT Chest)

Image Credit: Dr Frank Gaillard (radpod.org) (cc)

Tags: Bifid Rib - Gorlin-Goltz Syndrome - Job Syndrome - Kindler Syndrome - Mesoderm - Neuroblastoma - Rib - Samoan
Posted by Medicalchemy
Medicalchemy Group: History of Medicine - Images - Mnemonics - Syndromes - Acute Medicine - Anaesthesiology - Anatomy - Anthropology - Biochemistry - Cardiology - Dentistry - Dermatology - Drugs - Emergency Medicine - Endocrinology - Family Medicine - Forensic Medicine - Gastroenterology - Genes - Genetics - Geriatrics - Gynecology - Haematology - Health Informatics - Hepatology - Immunology - Infection - Intensive Care - Medical Education - Metabolic Medicine - Microbiology - Nephrology - Neuroanatomy - Neuroscience - Nuclear Medicine - Nutrition - Obstetrics - Occupational Health - Oncology - Ophthalmology - Orthopaedics - Otolaryngology - Paediatrics - Palliative Care - Parasitology - Pathology - Pharmacology - Physiology - Proteomics - Psychiatry - Public Health - Radiology - Respiratory - Rehabilitation - Rheumatology - Sports Medicine - Surgery - Toxicology - Tropical Medicine - Urology - Vascular - Virology.