• Skip to main content
  • Skip to primary sidebar
  • Skip to footer
  • General Ortho
  • Procedures
  • Spine
  • Upper Limb
  • Lower Limb
  • Pain
  • Trauma
  • Tumors
  • Newsletter/Updates
  • About Us
  • Contact Us

Bone and Spine

Orthopedic health, conditions and treatment

Poland Syndrome – Presentation and Treatment

By Dr Arun Pal Singh

In this article
    • Relevant Anatomy
    • Features of Poland Syndrome
    • Causes of Poland Syndrome
      • Related Disorders or Sequences
    • Clinical Presentation of Poland Syndrome
    • Investigations
    • Treatment of Poland Syndrome
      • Male Patients
      • Female Patients

Poland Syndrome is a congenital condition characterized by the absence of chest wall muscles on one side of the body and short, webbed fingers of the limb of the same side.

The Poland syndrome is also known as Poland Anomaly, Poland Sequence, and Poland Syndactyly.

Relevant Anatomy

The pectoralis major is a large muscle of the upper chest wall which gives the chest its shape and forms the anterior edge of the axilla. It arises from sternum or breastbone, clavicle and cartilages of the second to the sixth ribs. It inserts on to shoulder and is particularly useful in pushing and rowing activities.

Pectoralis minor is underneath pectoralis major arises from the third to fifth ribs and functions to rotate the shoulder.

Poland syndrome, named after British surgeon Alfred Poland.

Males are more commonly affected than females. The right side is more affected than left.

The exact incidence of the disease is not known.

Most Poland syndrome cases arise sporadically.

However, familial involvement has been reported, suggesting some degree of genetic transmission.

Association with Mobius syndrome and Klippel-Feil syndrome is known. Malignancies like leukemia and non-Hodgkin lymphoma, may occur in patients with Poland syndrome.

chest muscles

Features of Poland Syndrome

There is the absence of the pectoralis minor on one side and the sternal part of the pectoralis major muscle.

The absence of the sternal head of the pectoralis major muscle is considered the minimal expression of this syndrome.

Other muscles which can be involved are the pectoralis minor, serratus, latissimus dorsi, and external oblique muscle.

Other variable associated features are

  • Underdevelopment or absent nipple and areola
  • Nipple  higher than the normal nipple
  • Absent of axillary hair
  • Hypoplastic of breast and underlying tissues in females
  • Skeletal abnormalities
    • Underdevelopment or absence of upper ribs
    • Elevation of the shoulder blade (Sprengel deformity)
    • Shortening of the arm
    • Underdevelopment of the forearm bones

 

Poland Syndrome
Image Credit

Causes of Poland Syndrome

Exact cause of Poland syndrome is not known.

Majority of cases appear to occur sporadically but rare familial patterns have been observed.

Early interruption of blood flow due to to the subclavian artery supply disruption has been suggested the initiating event. More proximal the occlusion, severer are the symptoms.

Depending upon the location and severity of involvement, the clinical presentation may of Poland Syndrome, Mobius Syndrome, Klippel-Feil Syndrome, and Sprengel deformity.

That is why Poland Syndrome is sometimes referred to as Poland sequence. [A sequence refers to a pattern of malformations derived from a single anomaly.]

Related Disorders or Sequences

Mobius Syndrome

Poland Syndrome has been reported to occur in association with Mobius Syndrome, known as Poland-Mobius Syndrome.

These patients are characterized by facial paralysis due to paralysis of the sixth and seventh cranial nerves (Mobius Syndrome) and features typically seen in Poland Syndrome.

Masklike expressionless face, drooped eyelids, tongue hypoplasia, poor suckling and swallowing, speech impairment and mental retardation are seen.

Charlie M Syndrome

It is a rare condition characterized by certain distinctive facial and limb malformations.

The syndrome is characterized by

  • Widely spaced eyes or ocular hypertelorism
  • Broad nose and small mouth
  • Cleft palate
  • Micrognathia – small jaw
  • Facial nerve paralysis in some cases
  • Absent or cone-shaped front incisors
  • Asymmetric abnormalities of the fingers and toes

Clinical Presentation of Poland Syndrome

The right side is more commonly involved. The patient would have obvious chest asymmetry and deformity.

The affected side would have absent nipple and areola with minimum subcutaneous fat. Tasymmetryery may be pronounced by rib cage abnormalities. The affected limb has short arm or forearm or both. There would the be the absence of the sternal head of the pectoralis major muscle.

The patient would have short joined fingers or brachysymphalangism.

Associated muscles which could be underdeveloped or absent are serratus, external oblique, pectoralis minor, latissimus dorsi, infraspinatus, and supraspinatus muscles. There could be a total absence of anterolateral ribs or lung herniation on the affected side.

Though Poland syndrome is present at birth, most of the patients do not seek consultation till late for aesthetic reasons.

The examination is carried out to know the status of following for purpose of treatment.

  • Stage of breast development
  • Status of the latissimus dorsi muscle – Present or absent or hypoplastic
  • Extraocular movements – to look for associated Mobius syndrome.
  • Abnormal elevation and/or underdevelopment of the scapula
  • Sprengel Shoulder

Investigations

CT scan is done to determine the extent to which the muscles and bones of the chest wall are affected.

CBC and blood film examination are done when leukemia and non-Hodgkin lymphoma is suspected.

Treatment of Poland Syndrome

Many patients may not seek he treatment. The treatment of Poland Syndrome would depend on the specific symptoms.

Generally, the patients present for treatment of the chest deformity and breast asymmetry. Some patient may not want surgical treatment and counseling would suffice.

Following are important determinants that influence the timing and options for reconstruction:

  • Gender
  • Breast development
  • Presence of latissimus dorsi muscle,
  • The degree of chest wall deformity.

Male Patients

Chest deformity can be reconstructed with the latissimus dorsi muscle as early as age 13 years. Rib abnormalities if severe, need to be addressed.

Female Patients

Breast reconstruction provides the best results once the breast has fully developed.

Before that, one may consider early treatment with a temporary expander especially if the patient is psychologically being affected.

[Breast implants and expander should not be placed in patients who are too young.]

Upon completion of breast development (18-19 years of age), the tissue expander can be removed and the breast can be reconstructed with the latissimus muscle transposed over a permanent implant.

If the latissimus is absent, opposite latissimus dorsi or other adjoining muscles may be tried.

Sometimes, therapeutic tattooing is used to compensate for a missing nipple.

Spread the Knowledge
  • 1
    Share
  •  
    1
    Share
  • 1
  •  
  •  
  •  
  •  

Filed Under: Pediatric

About Dr Arun Pal Singh

Arun Pal Singh is an orthopedic and trauma surgeon, founder and chief editor of this website. He works in Kanwar Bone and Spine Clinic, Dasuya, Hoshiarpur, Punjab.

This website is an effort to educate and support people and medical personnel on orthopedic issues and musculoskeletal health.

You can follow him on Facebook, Linkedin and Twitter

Reader Interactions

Leave a Reply Cancel reply

Your email address will not be published. Required fields are marked *

This site uses Akismet to reduce spam. Learn how your comment data is processed.

Primary Sidebar

Congenital Trigger Thumb, Notta nodule is marked

Pediatric or Congenital Trigger Thumb

Congenital trigger thumb or pediatric trigger thumb as it is called now is a condition that results in abnormal flexion at the interphalangeal joint. That is why it also called flexion contracture of the thumb. It is a separate entity than the adult trigger thumb. This occurs due to thickening and constriction of fibrous tendon […]

Classification of Acetabular Fractures

Acetabular Fractures Presentation and Treatment

Acetabular fractures are uncommon but important because they involve a major weight-bearing joint in the lower extremity.  Being intraarticular fractures, these fractures require anatomical reduction for good long-term function of the hip joint. A failure to achieve anatomical reduction could lead to the breakdown of the cartilage surface due to articular incongruity present leading to degenerative […]

charite disc and xray. One of the motion preservation surgeries

Motion Preservation Surgery of Spine

Motion preservation surgery in the spine refers to a group of surgeries which aim at preservation of the spine motion by replicating normal or near normal biomechanics. The fundamental to all these surgeries is the use of devices, that replicate these functions. Decompression and fusion are classic procedures of spine surgery. Motion preservation surgeries are […]

Leg Length Measurement with Tape

True and Apparent Leg Length Measurement

True and apparent leg length refer to limb lengths in absolute terms and in compensated forms. The limb length measurement is used to find leg length discrepancy if any. When there is a shortening of the limb, the body compensates by tilting the pelvis down, equinus position of the foot and flexion of the opposite […]

hip bone diagram

Hip Bone Anatomy or Pelvic Bone[Ilium-Pubis-Ischium]

Hip bone is also known as innominate bone or pelvic bone and is formed by the fusion of three bones namely ilium, ischium and pubis bones. Hip bone forms part of the pelvis and takes part in hip joint articulation. The term hip bone can create confusion in the mind of the reader. To understand […]

Normal supination of Foot Movement

Supination of Foot and Oversupination

Supination of the foot is the natural movement of the foot as it rolls out during the gait cycle. Supination of foot is the movement of the subtalar joint (between the talus and calcaneus)  and consists of three movements – inversion, plantar flexion, and adduction. Inversion – Sole of the foot is turned inwards Plantar […]

complications-of-bone-grafting

Bone Grafts – Types, Mechanism, Techniques and Complications

Bone grafts are used to repair and rebuild diseased bones in your hips, knees, spine, and sometimes other bones and joints. A bone graft transplants bone tissue. Grafts can also repair bone loss caused by some types of fractures or cancers. Once your body accepts the bone graft, it provides a framework for the growth […]

Browse Articles

Footer

Pages

  • About
    • Policies
    • Contact Us

Featured Article

Principles of Treatment of Fractures

It is a dictum in orthopedics that no two fractures are alike. Every fracture behaves differently from other. In the same part fractured, there could … [Read More...] about Principles of Treatment of Fractures

Search Articles

© Copyright: BoneAndSpine.com