April 201624 THE ANTISEPTIC

CLINICAL HISTORY

• A 9year old boy comes topediatric outdoor with H\Oswelling over back,whichhave been present for last3-4years,and are graduallyincreasing in size.

• O/E-Bonyprominenceswerenoted on the back

Prominent 2nd digit of bothfeet

Fig. A - The photograph shows multiple prominences over the

back.

Fibrodysplasia Ossificans Progressiva - A Case ReportPRADIPTA KUMAR MISHRA, SUCHISMITA PANDA, RISHAV RAJ

Dr. Pradipta Kumar Mishra, MD Final Year,Dept of Radiology, VSS Medical College, Burla, Sambalpur, Odisha 768 017.Dr. Suchismita Panda, MD 2nd Year,Dr Rishav Raj, MD Final Year, Dept. of Pediatrics,Hi Tech Medical College, Pandra, Rasulgarh,Bhubaneshwar, Odisha - 751 025.

Specially Contributed to "The Antiseptic" Vol. 113 No. 4 & P : 24 - 25

X Ray B\L Feet AP View

X-RAY shows–

muscles,asmarked by the arrowsin blue.

X-Ray LS Spine & Pelvis

Fig. B - The photograph shows short first toe and long second

toe of both feet.

There ismicrodactyly of firsttoe in both feet.

The 2nd toes of bilateralfeet show elongated proximalphalange.

There is Hallux valgusdeformity of both feet.X Ray Chest PA View

XraychestandupperabdomenPA view shows colums of bonetraversingtherightaxillaryregionand left lower chest(probablyinvolving the intercostal

X-ray LS spine with pelvisshows bony columns over leftlower chest wall with broadfemoral neck bilaterallyX-Ray Skull & Hands

A)Xrayskullshowsnoapparentbony abnormality.

B)Xray BlL hands showmicrodactyly of BlL thumbsdue to phalangeal shortening

25 THE ANTISEPTIC April 2016

Discussion:

• Fibrodysplasia ossificansprogressiva (FOP) is a rareanddisablingsyndrome,whichischaracterizedbyheterotopicossifications and skeletaldeformities.

• So far, around 200 patientswith FOP have been reportedin theworld literature.

• The disorder tends to inheritasanautosomaldominanttrait,but the majority of cases aresporadicmutations.

• The age of onset ismostly inthe first two decades of life,andnogenderpreferenceshavebeen described.

• C\F-The first clinical featureincludesalocalizedandrapidlyprogressiveswelling,mainlyinmuscle bundles of the neck,shoulder,andupperarms.Thelesions may become warm,erythematous, and tender

Radiographic features

After months and even years,other regions, such as pelvis,face, jaws, back, extremities,abdominal wall, and chest willbe involved and torticollis andkyphosis may occur. Gradually,muscles are replaced by ossifiedorcalcifiedtissues,whichmaybelocated across joints. This leadsto restricted rangeofmotionandeven ankylosis in joints, so thatthe patient would be called a“stoneman.”

AlthoughthediagnosisofFOPis clinical, it is confirmed byimaging.Theimagingfeaturesare-• hallux valgus• monophalangic first toe• shortenedmetacarpals• pseudoexostoses (ossification

of ligamentous insertions)• Microdactyly of the first

metacarpal/metatarsal

Differential diagnosis:

• Scleroderma & CRESTsyndrome

• juvenile fibromatosis• dermatomyositisREFERENCES:

1. Essentials of skeletal radiology-Yochum & Rowe,vol-2,chapter-8 skeletal dysplasias,P-737

2. Orthopedic Imaging-A practical approach,by Adam Greenspan part VII-congenital and developmental anomalies

3. 1. Kocyigit H, Hizli N, Memis A.etal, A severely Disabling disorder: fibrodysplasia ossificans progressiva: Clinical Rheumatology 2001; 20:273-275 2.

4. Magryta CJ, KligorPhatak SV, Kolwadkar P K, Phatak M S. Images: Fibrodysplasia ossificans progressiva. Indian J Radiol Imaging 2003;13: 389-91

Nonsurgical Management of Gallbladder Carcinoma

• Gallbladder carcinoma (GBC) is a devastating diseasewith dismal results in terms of cure.

• Surgical resection (Ro) at an early stage is the gold standard treatment.

• Majority of patients present at advanced unresectable stage.

• Adjuvantchemoradiation is indicated inpatientswith resectedpT2andhigherornodepositiveGBC.

Hepatitis C Treatment:

• Hepatitis C is a major cause of chronic liver disease, cirrhosis, end-stage liver disease andHCC.

• Treatment is recommended forallpatientswithchronichepatitisC infection,except thosewithshort life expectancies that cannot be remediated by treating HCV, by transplantation or byother directed therapy. Patients with short-life expectancies owing to liver disease should bemanaged in consultationwith an expert (AASLD)

• Safe and effective interferon-free therapy is available to cure chronic hepatitis C across allHCV genotypes.