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Servelle-Martorell syndrome with extensive upper limb involvement: a case report.

Karuppal R, Raman RV, Valsalan BP, Gopakumar Ts, Kumaran CM, Vasu CK - J Med Case Rep (2008)

Bottom Line: The arm muscles and muscles of the limb girdle were atrophic.The forearm and hand bones were hypoplastic and tender.We report a case of Servelle-Martorell syndrome with extensive involvement of the entire upper limb and periscapular region.

View Article: PubMed Central - HTML - PubMed

Affiliation: Medical College, Calicut, Kerala, India. drrajuortho@rediffmail.com

ABSTRACT

Introduction: Angio-osteohypotrophic syndrome is also known as Servelle-Martorell angiodysplasia. It is characterized by venous or, rarely, arterial malformations, which may result in limb hypertrophy and bony hypoplasia. Extensive involvement of the upper limb is a rare feature of Servelle-Martorell syndrome. Cases with minimal upper limb involvement have been described in the literature.

Case presentation: A young man presented with multiple separate swollen areas over the right upper limb and functional difficulty since birth. The arm muscles and muscles of the limb girdle were atrophic. The forearm and hand bones were hypoplastic and tender.

Conclusion: We report a case of Servelle-Martorell syndrome with extensive involvement of the entire upper limb and periscapular region. Servelle-Martorell syndrome is highlighted as one of the causes of angiodysplastic limb hypertrophy.

No MeSH data available.


Related in: MedlinePlus

Magnetic resonance imaging scan multiple dilated veins in the superficial aspect of the right upper limb with bone hypoplasia.
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Figure 3: Magnetic resonance imaging scan multiple dilated veins in the superficial aspect of the right upper limb with bone hypoplasia.

Mentions: An MRI study showed multiple dilated veins in the superficial aspect of the right upper limb (Figure 3). The muscles of the limb were replaced by an abnormal signal. The triceps, biceps and deltoid were partially involved. No intra-osseous venous malformation was seen. The arteries were normal.


Servelle-Martorell syndrome with extensive upper limb involvement: a case report.

Karuppal R, Raman RV, Valsalan BP, Gopakumar Ts, Kumaran CM, Vasu CK - J Med Case Rep (2008)

Magnetic resonance imaging scan multiple dilated veins in the superficial aspect of the right upper limb with bone hypoplasia.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2394530&req=5

Figure 3: Magnetic resonance imaging scan multiple dilated veins in the superficial aspect of the right upper limb with bone hypoplasia.
Mentions: An MRI study showed multiple dilated veins in the superficial aspect of the right upper limb (Figure 3). The muscles of the limb were replaced by an abnormal signal. The triceps, biceps and deltoid were partially involved. No intra-osseous venous malformation was seen. The arteries were normal.

Bottom Line: The arm muscles and muscles of the limb girdle were atrophic.The forearm and hand bones were hypoplastic and tender.We report a case of Servelle-Martorell syndrome with extensive involvement of the entire upper limb and periscapular region.

View Article: PubMed Central - HTML - PubMed

Affiliation: Medical College, Calicut, Kerala, India. drrajuortho@rediffmail.com

ABSTRACT

Introduction: Angio-osteohypotrophic syndrome is also known as Servelle-Martorell angiodysplasia. It is characterized by venous or, rarely, arterial malformations, which may result in limb hypertrophy and bony hypoplasia. Extensive involvement of the upper limb is a rare feature of Servelle-Martorell syndrome. Cases with minimal upper limb involvement have been described in the literature.

Case presentation: A young man presented with multiple separate swollen areas over the right upper limb and functional difficulty since birth. The arm muscles and muscles of the limb girdle were atrophic. The forearm and hand bones were hypoplastic and tender.

Conclusion: We report a case of Servelle-Martorell syndrome with extensive involvement of the entire upper limb and periscapular region. Servelle-Martorell syndrome is highlighted as one of the causes of angiodysplastic limb hypertrophy.

No MeSH data available.


Related in: MedlinePlus