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Malaysian Orthopaedic Journal 2019 Vol 13 No 2 Sahdi H, et al

ABSTRACTCongenital humeroradial synostosis can occur as an isolatedclinical entity or as part of a syndrome. Bilateral elbow fixedflexion deformity is very incapacitating and challenging totreat. Here we present the case of a boy with fixed flexiondeformity of both elbows due bilateral humeroradialsynostosis. Other characteristic features of multiplesynostoses syndrome were also present in this child, his elderbrother and mother. We elected to improve the position of theright elbow by adapting the modified French osteotomydescribed by Bellemore et al.

Key Words: humerus, radius, synostosis, ankylosis, osteotomy

INTRODUCTIONMultiple synostoses syndrome is characterised by multiplejoint fusion including proximal symphalangism of fingersand toes, humeroradial synostosis, carpal and tarsalcoalition, alongside with unusual nasal features andconductive deafness1. Bilateral elbow ankylosis isfunctionally devastating. It poses a great challenge totreatment, as the ideal elbow position and method of elbowreconstruction is still debatable. Literature on themanagement of bilateral humeroradial synostosis in multiplesynostoses syndrome is limited.

CASE REPORTA six-year old boy presented with stiff elbows since infancy.He was born full term to non-consanguineous parents. Therewas no antenatal exposure to teratogens. The child has

difficulty in performing activities of daily living. Hecompletes schoolwork at kindergarten in a rather slow andawkward manner due to the rigid elbows. He has beenutilising modified cutlery to overcome his difficulties. Apartfrom hearing difficulty, he is an active child who loves toplay soccer.

The child’s 41-year old mother and 8-year old only brotherhad restricted elbow joint movements and hearingimpairment. There were no other family members withsimilar history of joint stiffness and hearing disorder.

The parents were concerned about his slowness inperforming academic tasks in school due to the elbowposition. We offered to fuse the right elbow in a morefunctional position. Taking into consideration the child’sconcerns, daily activities, hobbies and preferences, it wasdecided to realign the patient’s elbow to 90 degrees flexion.

On examination, the child had striking features of sharp noseand jaw. His elbows were fixed at 30 degrees of flexion (Fig.1), and midprone forearm position. The fingers and toesappeared to be short with lack of interphalangeal jointsbilaterally. Both mother and elder brother also had similarpresentations. Examination of the other organ systems wasunremarkable.

Plain radiographs of the elbow (Fig. 1) showed ankylosis ofthe humeroradial joint, with hypoplastic humeroulnar joint.The distal phalanges of ring and little fingers and fourth andfifth toes were absent. The middle phalanges of all fingersand toes were hypoplastic and fused with the proximalphalanges. There was carpal and tarsal coalition. Audiometryshowed conductive hearing deafness.

Modified French Osteotomy for Humeroradial Synostosisin a Child with Multiple Synostoses Syndrome: A Case

Report

Sahdi H, MS Ortho, Rasit AH, MS Ortho, Khoo CS*, MRCPCH UK, Bojeng A**, MMed Rad, Nur-Alyana BA***, MS Nursing

Department of Orthopaedics, Universiti Malaysia Sarawak, Kota Samarahan, Malaysia*Department of Paediatrics, Hospital Umum Sarawak, Kuching, Malaysia

**Department of Radiology, Universiti Malaysia Sarawak, Kota Samarahan, Malaysia***Department of Nursing, Universiti Malaysia Sarawak, Kota Samarahan, Malaysia

This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited

Date of submission: 25th February 2019Date of acceptance: 8th May 2019

Corresponding Author: Haniza Sahdi, Department of Orthopaedics, Faculty of Medicine and Health Sciences, Universiti Malaysia Sarawak(UNIMAS), Jalan Datuk Mohammad Musa, 94300 Kota Samarahan, Sarawak, MalaysiaEmail: hnizas@hotmail.com

doi: http://doi.org/10.5704/MOJ.1907.011

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Fig. 1: (a) Pre-operative photograph of the patient’s bilateral elbows in fixed flexion of 30 degrees. (b) The pre-operative right elbowradiograph demonstrating humeroradial synostosis with hypoplastic humeroulnar joint.

Fig. 3: (a) Post-operative photograph and (b) plain radiographs taken one year after surgery showing right elbow position of 90degrees and union of osteotomy site.

Fig. 2: (a and b) Intra-operative photographs and (c) illustration of the right elbow osteotomy. (a) Right elbow photographs showingthe humeroradial synostosis before osteotomy and (b) after completion of the French osteotomy and fixation. (c) Illustration ofthe right elbow showing plane of osteotomy and fixation in antero-posterior position and lateral position. * (A) Right arm; (B) right forearm; (C) humeroradial synostosis (D) right radius; (E) site of osteotomy; (F) right humerus.

(a) (b)

(a) (b) (c)

(a) (b)

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In a supine position, under general anaesthesia, the lateralaspect of the right elbow was approached using the extendedKocher’s incision. The radio-humeral synostosis wasaccessed through subperiosteal approach (Fig. 2). TwoKirschner wires were placed to mark the plane of osteotomy.Next, two 2.5 mm cortical screws were inserted, with eachscrew placed proximal and distal from the arthrodesis site,on the midline of the distal humerus lateral surface and theadjacent proximal radius (Fig. 2). Adapting the Frenchosteotomy modification by Bellemore et al, anterior wedgeosteotomy was performed, leaving the apex at the posteriorcortex intact (Fig. 2). Once the desired 90 degrees elbowflexion position was achieved, a figure-of-eight 1.0 mmcerclage wire was tightened around the screw heads (Fig. 2).The elbow was maintained in an above-elbow backslab forsix weeks until osseous bridging was achieved.

At 12 months post-surgery (Fig. 3), the child was able to eatand drink with the right upper limb without adaptive devices.His writing speed had improved and he managed to completeacademic tasks within the stipulated time.

DISCUSSIONHumeroradial synostosis can occur either as an isolateddeformity or as part of a syndrome. Multiple synostosessyndrome is characterised by brachy-symphalangism of thefingers and toes and multiple joint fusions of wrists, feet andelbows. Characteristic facial appearance includes a broad,tubular-shaped or hemicylindrical nose without flarednostrils and thin upper lip. Conductive deafness, hypoplasticor absent middle phalanges are the other features of thissyndrome1.

Multiple synostoses syndrome can be familial or sporadic.The mode of inheritance in the familial type can beautosomal recessive or dominant. Autosomal recessivemultiple synostoses syndrome has no ulnar hypoplasia ordistal ulnar malformation. Symmetrical involvement of thelimbs is a feature of autosomal dominant subset1.

There is no ideal angle or position to fuse the elbow. Thepatient will face significant functional limitations regardlessof selected position. Literatures suggests elbow fusion angleof between 45 to 110 degrees although historically, 90degrees has been accepted as the best position2. The finalposition of elbow fusion should be determined on anindividual basis, considering age, gender, hand dexterity,occupation, functional ability of the ipsilateral shoulder andwrist as well as the contralateral upper limb, functionalrequirements and patient preference.

Literature on improving elbow position due to humeroradialsynostosis in multiple synostoses syndrome is scarce.Kakarala in 20063, utilised the distraction osteogenesis

technique with Ilizarov ring fixator system to correct fixedflexion deformity of elbow in a child with bilateralcongenital humeroradioulnar synostosis. However, themethod requires the patient to accept the inconvenience fromthe bulky construct, strict compliance with distractionprocedure and meticulous pin site dressing.

French4 described a method of lateral closing wedgeosteotomy using two parallel screws and figure-of-eightwires to correct cubitus varus deformity from malunitedsupracondylar humerus fractures in 1959. He originally usedthe posterior approach to the distal humerus and left theperiosteum intact medially. Bellemore and associates5

modified French’s technique by making a posterolateralincision and kept the medial cortex of the supracondylarhumerus intact during the osteotomy. The intact periosteumacted as hinge that provided better control for the reductionof the osteotomy fragments and aided union of the humerusosteotomy. Considering the benefits, we adapted themodified French method by Bellemore by performing ananterior closing wedge osteotomy with intact posteriorcortex apex for easier reduction, and fixed the compositeusing the French method (Fig. 2).

Dome osteotomy, step-cut osteotomy and three-dimensionalosteotomy are technically demanding compared to theFrench method. Fixation solely by K-wires can becomplicated with pin tract infection, pin loosening, unsightlyscar and loss of fixation5. Arthrodesis with plate and screwsin this case was not chosen as it required more extensive softtissue stripping. Furthermore, the atrophic nature of thechild’s muscles would cause prominent implant if thismethod of fixation was utilised. Elbow arthroplasty toreconstruct a functional joint is unsuitable in view of theatrophic bone base and insufficient muscle bulk. Distractionosteogenesis technique requires compliance from patientswith regards to long treatment period and pin care. Thismethod has other complications such as pin site problems,nerve palsies, malunion, re-fracture and infection.

In conclusion, fixed flexion of bilateral elbows is verydisabling to the patient. Therefore, fusing the elbow in amore functional position is indicated as in this case. We haveyet to come across any literature reports of the application ofthe French osteotomy technique to congenital elbow fusioncases. We have successfully modified the French techniqueinto an anterior closing wedge osteotomy to achieve thedesired elbow position in a child with multiple synostosessyndrome.

CONFLICT OF INTERESTSThe authors declare no conflict of interest with respect to theauthorship and/or publication of this article. No financialsupport was received.

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REFERENCES

1. Hunter AG, Cox DW, Rudd NL. The genetics of and associated clinical findings in humero-radial synostosis. Clin Genet. 1976;9: 470-8.

2. Kovack TJ, Jacob PB, Mighell MA. Elbow arthrodesis: a novel technique and review of the literature. Orthopedics. 2014; 37(5):313-9.

3. Kakarala G, Kavarthapu V, Lahoti O. Distraction osteogenesis to improve limb function in congenital bilateral humeroradialsynostosis. Acta Ortho Belg. 2006; 72(6): 765-8.

4. French PR. Varus deformity of the elbow following supracondylar fractures of the humerus in children. Lancet. 1959; 2(7100):439-41.

5. Bellemore MC, Barrett IR, Middleton RW, Scougall JS, Whiteway DW. Supracondylar osteotomy of the humerus for correctionof cubitus varus. J Bone Joint Surg Br. 1984; 66(4): 566-72. 

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