2 and half year-old girl with refex sympathetic dystrophy (rsd)

7/28/2019 2 and Half Year-old Girl With Refex Sympathetic Dystrophy (RSD)

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Clinical Rehabilitation 2003; 1 7: 224227

Arnold 2003 10.1191/0269215503cr589

Address for correspondence: Fsun Gler-Uysal, ukurovaUniversitesi Tip Fakltesi, Fiziksel Tp ve RehabilitasyonAnabilim Day, 01330 Balcal, Adana, Turkey. e-mail:[email protected]

A 21/2-year-old girl with reex sympatheticdystrophy syndrome (CRPS type I): case reportF Gler-Uysal, S Bas, aran ukurova University, School of Medicine, Department of Physical Medicine andRehabilitation, Adana, Turkey, JHB Geertzen Groningen University Hospital, Department of Rehabilitation,The Netherlands and K Gnc ukurova University, School of Medicine, Department of Physical Medicine and

Rehabilitation, Adana, Turkey

Received 23rd August 2001; returned for revisions 28th August 2001; manuscript accepted 4th October 2001.

An unusual case of a 21/2-year-old girl with reex sympathetic dystrophy (RSD)

of the left arm is described. She is the youngest RSD case ever presented in

the literature. Upper extremity involvement is also rare in childhood RSD. Shehad both physical and psychological trauma in an earthquake preceding the

disease. The association of RSD with a psychological disorder is stressed and

awareness of the condition to the general paediatrician is recommended for

early diagnosis and successful treatment.

The occurrence of RSD in childhood has lonbeen thought to be rare,2,810 but it may be morcommon in children than previously thought.2,9

RSD could still be underestimated and misdiagnosed in the young paediatric age group. Delayin diagnosis averaged 12 months in a large seriesUnnecessary testing and referrals may create vicious circle, perpetuating signs and symptomin young children. It may also be confused witpsychiatric conditions such as conversion reactioand malingering.12

Children with RSD are usually of school agor in the teenage years.3,9 There is a preponderance of girls and also a marked tendency folower extremity involvement.4,9,13,14 We thereforpresent this rare case of a 21/2-year-old girl witRSD of the upper extremity as being thyoungest RSD case ever presented in the literature.

Introduction

Reex sympathetic dystrophy (RSD), also called

complex regional pain syndrome (CRPS) type I,

1

is a syndrome characterized by pain in one ormore extremities, usually associated with vaso-motor and sudomotor changes.2 The predomi-nant and most disabling feature of RSD ischronic and burning pain exacerbated by move-ment and emotional stress.3 Many diseases, pre-cipitating events or drugs have been associatedwith RSD, but trauma, especially fractures, hasbeen shown to be the most common initiatingfactor in adults.4 Opinions have varied widely onthe issue of a psychological aetiology.5,6 Stressfullife events besides trauma or surgery have alsobeen shown to be risk factors in RSD.7 There are,however, a number of cases for which no precip-itating factor can be identied.


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Reex sympathetic dystrophy syndrome: case report 22

Case

A 21/2-year-old girl was presented to the PhysicalMedicine and Rehabilitation outpatient clinic byher relatives, declaring that she could not use herleft hand and resisted even the touch of anyonewho attempted to examine it for the last three

months. The patient had experienced the Mar-mara earthquake in Turkey in August 1999 andhad been rescued after 12 hours of being trappedunder the ruins. Her parents had died during thesame disaster and she was being followed by thePaediatric Psychiatry Clinic with diagnoses ofnon-organic failure to thrive and acute stress dis-order. An orthopaedic consultation was assessedsoon after she had been rescued and no fracturenor soft tissue damage of the musculoskeletal sys-tem could be detected. The left hand pain was

thought to be due to a minor compression of theupper extremity exaggerated by her psychiatricsituation until she was referred to our clinic fora second opinion three months later. On admis-sion, her relatives declared that she could evenbe disturbed by the touch of the bed sheets toher hand during sleep. This resembled allodynia,which is a common symptom of RSD.9 Dys-trophic nail changes were observed on inspectionof the extremity. A slight atrophy of the hand andforearm with traces of hypertrichosis wasobserved. She had severe pain and slight limita-tion of passive exion of the ngers. Control radi-ographs of both extremities revealed prominentosteopenia of the carpal and metacarpal bones ofthe involved hand.

The diagnosis of RSD was conrmed andanti-inammatory medication (nimesulide50 mg b.i.d.) along with intranasal calcitonin(50 IU q.o.d.) and vitamin D supplementation(400 IU g.d.) were started. She was also takinganxiolytic medication (tizanidine) for her psychi-atric condition. The patient started a rehabilita-tion programme of passive range of motionexercises in the beginning, followed by oc-cupational therapy to the left hand which dimin-ished the pain and resulted in a dramaticrecovery of left hand function within threemonths. At six months after trauma, she wasable to use her left hand for eating and playingagain. She had left the guarding posture andcooperated well with the examination of left

hand function, which revealed slight limitation opassive exion of interphalangeal joints as well adystrophic nail and skin changes. One year aftethe initiating event, she only had mild coldnesand minimal hypertrichosis of the left hand buno pain or limitation in joint mobility and hanfunction.

Discussion

Children with RSD are usually of school age oin their adolescence and a marked tendency folower extremities has been described.10,11 Thvery young age of our case as well as the involvement of the upper extremity is remarkablunusual. She presented severe pyschologicaproblems due to loss of both parents and experi

encing hours under the ruins after the earthquake. She cried continuously during her rsvisits to our clinic. The diagnosis was reacheafter a detailed interview with her aunt, a thorough physical examination and radiographiinvestigation excluding fractures. The diagnosiof RSD is mainly based on clinical grounds. Radography can be supplementary, showing osteopenia of the affected extremity, but bone scaresults can be controversial.2,13,15

Half of the children with RSD have mino

trauma or illness identied as an initiating factor.6,11 Signicant emotional dysfunction is alsfound in the majority.11 The analysis of 21 famlies of children with RSD has revealed that RSDis frequently related to stress.6 Our case is a goodemonstration of both factors playing a role ithe aetiology of RSD.

The prognosis of RSD in children has bee

Clinical messages

Reex sympathetic dystrophy could easilybe misdiagnosed in the very young paedi-atric age group.

Unnecessary testing and referrals maycreate a vicious circle in children and shouldbe avoided.

Early and correct diagnosis depends on theawareness of the condition by clinicians.


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226 F Gler-Uysalet al.

2 Cimaz R, Matucci-Cerinic M, Zulian F, Falcini F.Reex sympathetic dystrophy in children. J ChildNeurol1999; 14: 36367.

3 Cassidy JT, Petty RE. Musculoskeletal painsyndromes of nonrheumatic origin. In: CassidyJT, Petty RE eds. Textbook of paediatricrheumatology. New York: WB Saunders, 1995:10832.

4 Kozin F. Painful shoulder and the reex sympathetdystrophy syndrome. In: Koopman WJ ed.Arthritisand allied conditions. Baltimore: Williams & Wilkin1997: 1887922.

5 Lynch ME. Psychological aspects of reexsympathetic dystrophy: a review of the adult andpaediatric literature. Pain 1992; 49: 33747.

6 Sherry DD, Weisman R. Psychological aspects ofchildhood reex neurovascular dystrophy. Pediatrics1998; 81: 57278.

7 Geertzen JH, de Bruijn-Kofman AT, de Bruijn H,van de Wiel HB, Dijkstra PU. Stressful life eventsand psychological dysfunction in Complex RegionalPain Syndrome type I. Clin J Pain 1998; 14:14347.

8 Fermaglich DR. Reex sympathetic dystrophy inchildren. Pediatrics 1977; 60: 88183.

9 Wilder RT, Berde CB, Wolohan M, Vieyra MA,Masek BJ, Micheli LJ. Reex sympatheticdystrophy in children. J Bone Joint Surg 1992; 74 A91019.

10 Lemahieu RA, Van Laere C, Verbruggen LA.Reex sympathetic dystrophy: an underreportedsyndrome in children? Eur J Pediatr1988; 147:4750.

11 Barbier O, Allington N, Romboutts JJ. Reexsympathetic dystrophy in children: review ofa clinical series and description of theparticularities in children. Acta Orthop Belg 1999;65: 9197.

12 Silber TJ, Majd M. Reex sympathetic dystrophysyndrome in children and adolescents. Report of 18cases and review of the literature. Am J Dis Child1988; 142: 132530.

13 Stanton RP, Malcolm JR, Wesdock KA, SingsenBH. Reex sympathetic dystrophy in children: anorthopedic perspective. Orthopedics 1993; 16:77379.

14 Dietz FR, Mathews KD, Montgomery WJ. Reexsympathetic dystrophy in children. Clin Orthop 1990258: 22531.

15 Ashwal S, Tomasi L, Neumann M, Schneider S.Reex sympathetic dystrophy syndrome in childrenPediatr Neurol1988; 4: 3842.

16 Ruggeri SB, Athreya BH, Doughty R, Gregg JR,Das MM. Reex sympathetic dystrophy in children.Clin Orthop 1982; 163: 22530.

17 Herregods P, Willems J, Chappel R.Pseudodystrophy at the lower limb in children. ClinRheumatol1997; 16: 42528.

reported to be more benign than in adults,2,15,16

as in our case who demonstrated an excellentoutcome. Yet cases with severe residual dysfunc-tion have also been described.9 Optimal treat-ment is still under debate. Literature data suggestthat once early diagnosis is established, conserv-ative therapy (mainly mobilization and physical

therapy along with psychotherapy when needed)can be sufcient.2,9,14 RSD in children can be aself-limiting condition which responds well tomild analgesics and physical therapy.16 Lessconventional pharmacologic treatments, such astricyclic antidepressants, calcitonin, bisphospho-nates, alpha- and beta-adrenergic antagonists,anticonvulsants, calcium channel blockers andsteroids as well as sympathetic blocks have alsobeen used.9,11 Nonresponders are usually patientswith a delayed diagnosis or those with severe psy-

chological problems.2It has recently been suggested that childhood

RSD is completely different from true RSD andshould be referred to as pseudodystrophy or dis-use-related dystrophy.17,18 This rather mild formof RSD may also be seen in adults. According toHerregods et al.17 and Driessens,18 bone scan is ofmajor importance in differential diagnosis for itis always negative in pseudodystrophy. Yet thereare reports which support the lack of utility ofa bone scan in the diagnostic work-up of

RSD.15,19In conclusion, we describe this rare case of a

very young child with RSD of her left arm inorder to remind clinicians of this entity even intoddler years. The diagnosis of RSD in this veryyoung age group undoubtedly depends on theawareness of the condition by clinicians. It is sug-gested that the syndrome should be suspectedwhenever the severity of the pain does not t thepattern of injury and the pain is associated withextreme sensitivity to light touch (allodynia),

episodic mottling, cyanotic discoloration of theskin, or swelling, or a difference in the skintemperature compared with the contralateralextremity.

References

1 Merksey H, Bogduk N. Classication of chronicpain: descriptions of chronic pain syndromes anddenitions of terms. Seattle: IASP Press, 1994.


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Reex sympathetic dystrophy syndrome: care report 22

18 Driessens M, Dijs H, Verheyen G, Blockx P. Whatis reex sympathetic dystrophy? Acta Orthop Belg1999; 65: 20217.

19 Allen G, Galer BS, Schwartz L. Epidemiology of

complex regional pain syndrome: a retrospectivechart review of 134 patients. Pain 1999; 80:53944.


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2 and half year-old girl with refex sympathetic dystrophy (rsd)

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