Case of the Month 15 September 2016 What is your diagnosis History 19yrold male patient referred to our department for preoperative thoracic imaging Physical Examination healthy ID: 911376
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Slide1
History: 19-yr-old male patient referred to our department for preoperative thoracic imaging
Case of the Month 15September 2016
What is your diagnosis ?
Slide2Slide3History: 19-yr-old male patient referred to our department for preoperative thoracic imaging.
Physical Examination: healthy, assymetrical chest wall, soft tissue-hypoplasia left thorax, cutaneous syndactyly left handQuestion: What are the key findings on the CXR?Case of the Month 15
September 2016
Author:
T Frauenfelder
Zurich
Slide4I
ncreased lucency of the left hemithoraxCase of the Month 15
Slide5Case of the Month
15Question: What is shown on the CT images?
Slide6Case of the Month
15Absence of the left pectoralis musclesAbsence of mammilla
Slide7Case of the Month
15Additional findings:Hypoplastic sternumRudimental bone nucleus of the manubriumSterno-clavicular articulation with the sternal body
Slide8Case of the Month
15DiagnosisPOLAND SYNDROME
Differential Diagnosis
-
Amazia
-
Pectus
carinatum
with
aplasia of
pectoralis
-
Jeune
Syndrome
- Postoperative deformation
Slide9Case of the Month
15DiscussionPoland syndrome is a congenital unilateral absence of the pectoralis major and minor muscle and is a recognised cause of unilateral hyperlucent hemithorax Absence of the serratus anterior muscle, deformities of ribs, soft tissue, ipsilateral arm and hand may also be observed
The syndrome is usually sporadic, although rare familial cases have been described
Poland Syndrome affects
males:females
= 3:1
right
side:left
side = 2:1
Slide10Case of the Month
15DiscussionCHEST RADIOGRAPHY Unilateral hypertranslucent hemithorax
HIGH-RESOLUTION CT
Absence of
pectoralis
major and minor muscle
Absence
of the serratus anterior
muscle
Deformities
of ribs, soft
tissue
Slide11Case of the Month
15Further ReadingPOLAND SYNDROME1. Dillman JR, Sanchez R, Ladino-Torres MF et al, Expanding upon the unilateral hyperlucent hemithorax in children, Radiographics, 2011 May-Jun;31(3):723-41. doi:10.1148/rg.3131051322. Moir CR, Johnson CH. Poland's syndrome.
Semin Pediatr Surg. 2008;17:161–6.3. Urschel HC., Jr Poland's syndrome. Chest Surg Clin N Am. 2000;10:393–403.
4
.
Legbo
JN. Poland's syndrome: Report of a variant. J Natl Med Assoc. 2006;98:97–9.
5
.
Poullin P,
Toussirot
E,
Schiano
A et al., Complete and dissociated forms of Poland’s Syndrome (5 cases), Rev
Rhum
Mal
Osteoartic
, 1992 Feb;59(2):114-20.
6
.
Fokin
A,
Robisec
F, Poland’s syndrome revisited, Ann
Thorac
Surg
74 (6), 2002, 2218-2215.
Doi
: 10.1016/S0003-4975(02)04161-9.
7
.
Legbo
JN. Poland's syndrome: Report of a variant. J Natl Med Assoc. 2006;98:97–9.
8
.
Gashegu
J,
Byiringiro
JC,
Nyundo
M. Poland syndrome: A case report. East Cent Afr J Surg. 2009;14:112–4.9. Seyfer AE, Fox JP, Hamilton CG. Poland syndrome: Evaluation and treatment of the chest wall in 63 patients. Plast Reconstr Surg. 2010;126:902–11.