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Orthopaedic Proceedings
Vol. 94-B, Issue SUPP_IV | Pages 152 - 152
1 Mar 2012
Ogonda L Laverick M Andrews C
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Introduction. Paediatric tibial fractures, unlike femoral fractures do not have much potential for overgrowth. In simple factures of the tibial shaft treated non-operatively the major problems are shortening and malunion. In complex injuries with extensive soft tissue disruption and bone loss, the long-term aim of reconstruction is to achieve union with a fully functional limb without limb-length inequality. Methods. Four children (Age range 6-12 years) who sustained high-energy grade III open fractures of the tibia were treated with acute shortening and bone transport. Any soft tissue reconstructive and secondary grafting procedures for delayed union were recorded. The children were prospectively followed up to fracture union. Distraction ostegenesis proceeded until limb length equality was achieved and the regenerate allowed to consolidate. Results. Three children had grade 3B injuries, 2 requiring flap reconstruction. One had a grade 3A injury. Mean acute shortening was 4.4cm (Range 2-9cm). Distraction osteogenesis was used to achieve limb-length equalisation. 2 children required secondary bone graft procedures to achieve union. At 3 years from injury, all children had overgrowth of the injured leg averaging 2cm. Discussion. Despite achieving equal limb lengths at the end of distraction osteogenesis the injured tibia overgrew by a mean of 2cm at three years post injury. This would suggest that even in the presence of extensive soft tissue trauma, as seen in these high energy injuries, the increased blood flow associated with metaphyseal corticotomy stimulates epiphyseal activity resulting in overgrowth. The value of stopping adjustments just short of achieving limb length equality to allow for expected overgrowth in the injured tibia merits further investigation


Orthopaedic Proceedings
Vol. 90-B, Issue SUPP_III | Pages 521 - 522
1 Aug 2008
Norrish AR Bates JHJ Harrison WJ
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Purpose of study: Long bone chronic osteomyelitis may result in limb length discrepancy by shortening of the affected bone when the physis is damaged. Little is known about the rates of overgrowth of infected long bones. This study documents the relative rates of overgrowth and undergrowth in a large series of chronic osteomyelitis patients. Methods: Forty-two consecutive patients presenting to our unit with chronic osteomyelitis of a long bone were included. There were no exclusion criteria. The mean age at presentation was 10.3 years. The mean duration of symptoms was 18.2 months prior to presentation. For 37 (88%) of patients the cause of osteomyelitis was haematogenous. Results: Three (7%) patients had shortening of the long bone compared to the unaffected side (average 2.5cm), whilst 13 (31%) patients had overgrowth of the affected bone (average overgrowth 2.2cm). The tibia was most commonly affected (20/42, 48%), followed by the femur (8/42, 19%) and the humerus (6/42, 14%). The average proportion of long bone involved on X-ray was 59%. At least one physis was affected in 8/42 (12%) patients (2 had undergrowth, 1 overgrowth). Conclusions: This large prospective series of patients shows the effect of osteomyelitis on the growth of long bones, in particular an overgrowth rate of 31%. The mechanism for this is probably related to the duration of symptoms. In areas of the world with poor access to health care, there is consequently a prolonged period of increased blood supply as a result of inflammation. This increased blood supply may make overgrowth limb length discrepancy more likely than undergrowth


Orthopaedic Proceedings
Vol. 90-B, Issue SUPP_III | Pages 570 - 570
1 Aug 2008
Ogonda L Laverick M Andrews C Madden M Cummings B
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Introduction: Paediatric tibial fractures, unlike femoral fractures do not have much potential for overgrowth. In simple factures of the tibial shaft treated non-operatively the major problems are shortening and malunion. In complex injuries with extensive soft tissue disruption and bone loss, the long-term aim of reconstruction is to achieve union with a fully functional limb without limb-length inequality. Methods: Four children who sustained high-energy grade III open fractures of the tibia were treated with acute shortening and bone transport. Any soft tissue reconstructive and secondary grafting procedures for delayed union were recorded. The children were prospectively followed up to fracture union. Distraction ostegenesis proceeded until limb length equality was achieved and the regenerate allowed to consolidate. Discussion: Despite achieving equal limb lengths at the end of distraction osteogenesis the injured tibia overgrew by 1–2cm at three years post injury. This would suggest that even in the presence of extensive soft tissue trauma, as seen in these high energy injuries, the increased blood flow associated with metaphyseal corticotomy stimulates epiphyseal activity resulting in overgrowth. The value of stopping adjustments just short of achieving limb length equality to allow for expected overgrowth in the injured tibia merits further investigation


Orthopaedic Proceedings
Vol. 96-B, Issue SUPP_6 | Pages 3 - 3
1 Apr 2014
Burwell G Aujla R Grevitt M Randell T Dangerfield P Cole A Pratt R Kirby A Polak F Web J Moulton A
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Aim:. Right-Handed Girls With Rt-Ais Measured Using Holtain Equipment Have Upper Arm Length Asymmetry (Right-Minus-Left) Which Is: 1) Relatively Longer On Scoliosis Curve Convexity; 2) Significantly Associated With Scoliosis Curve Severity (Cobb Angle And Apical Vertebral Rotation); And 3) Transient, Decreasing With Age And Years After Menarche [1,2]. The Aim Is To Test Whether The Right Upper Arm Length Relative Overgrowth And Spinal Deformity Severity Were Associated With Right Or Left Upper Arm Length Size-For-Age. Method:. 94 Right-Handed Girls With Rt-Ais, Age 11–18 Years, (Mean Cobb Angle 46 Degrees, Range 10–102 Degrees), Were Evaluated Using A Harpenden Anthropometer For Upper Arm Length Asymmetry, Plotted Against Right And Left Upper Arm Length Standard Deviation Scores (Sds), Calculated From 378 Normal Girls, Age 11–18 Years. Results:. The Findings Show That Upper Arm Length Asymmetry Correlates Significantly With Right, But Not Left, Upper Arm Length Sds (Right Arm Spearman's Rho=0.330 P=0.001; Left Arm Rho=−0.013 P=0.902), And With No Other Limb Or Trunk Skeletal Region Sds. Cobb Angle And Avr Also Correlate Significantly With Right, But Not Left, Upper Arm Length Sds. Discussion:. The Abnormal Asymmetry Of Upper Arm Lengths Results From Relative Overgrowth Of Right Upper Arm. We Suggest This Asymmetry Results From An Abnormal Asymmetry Process (Eg Neurogenic) With Growth Velocity That Together Also Initiate The Trunk Deformity, Wherein Additional Factors Contribute To Scoliosis Progression Such As Upright Posture, Melatonin-Signalling Dysfunction And Osteopenia. The Right Upper Arm Length Overgrowth May Provide A Sentinel Of The Relative Anterior Spinal Overgrowth. Conflict Of Interest Statement: No conflict of interest


Orthopaedic Proceedings
Vol. 92-B, Issue SUPP_I | Pages 143 - 143
1 Mar 2010
Munakata Y Kan N Nagase K Kusaba A Kondo S Kato Y Kuroki Y
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A special surgical technique and consideration is necessary in the total hip arthroplasty for dysplastic osteoarthritis after Kalamchi and MacEwen Type III or IV deformity (so called “Perthes-like-deformity”). There have been few reports concerning the total hip arthroplasty for “Perthes-like-deformity”. We evaluated the clinical and radiological outcome of 52 uncemented hip arthroplasties for the lesion.

We have performed 106 hips of uncemented total hip arthroplasty for dysplastic osteoarthritis after Kalamchi and MacEwen Type III or IV deformity. Among them, 52 hips of 47 patients (11 males and 41 females) were evaluated with minimum of three years follow-up. The average age at the surgery was 52 (28 to 65). The average follow-up period was 4.8 (3 to 8.1) years. Against the developmental dysplasia or dislocation, 29 hips of 26 patients had been treated by casting or surgery in infancy. Thirteen hips of 11 patients had no previous treatment before the arthroplasty. Spongiosa metal cup (GHE: ESKA implants, Lübeck, Germany) was used for 33 hips of 28 patients and Zweymüller type cup (Allo-classic cup: Zimmer Inc., Warsaw, IN, Bicon cup: Smith & Nephew Orthopedics AG, Rotkreuz, Switzerland) for 19 hips of 19 patients. Spongiosa Metal stem (GHE: ESKA implants) was used for 23 hips of 19 patients and Zweymüller type stem (Alloclassic stem: Zimmer Inc., SL stem: Smith & Nephew Orthopedics AG) for 29 hips of 28 patients. The average operative time was 108 (53 to 233) minutes. The average blood loss during the surgery was 731(150 to 1749) milliliters. The adductor tendon release was added in 28 hips of 26 patients against the severe contracture. The patients were evaluated clinically (pre-surgical history, hip score, leg length discrepancy, Trendelenburg sign, and gait function) and radiologically (ATD before the surgery, alignment, and stability of implants). Average ATD before the surgery was −2.2 (−28 to 17) millimeters. The average leg length discrepancy was 1.9 (0 to 7) centimeters before the surgery and was improved to 0.1 (0 to 1) centimeters after the surgery. The average hip score was 54 (23 to 80) before the surgery and was improved to 90 (69 to 100) after the surgery. At the final follow-up, Trendelenburg sign was positive in 14 hips of 14 patients (26.9%) and the limping was not obvious in 38 hips of 33 patients (73.1%). All implants were stable at the final follow-up.

“Perthes-like-deformity” often has the severe deformity. It has a shortening or an absence of the neck and an excessive antetorsion of the femur. When it has the coxa magna, the acetabulum is shallow, has the narrow anteroposterior diameter, and has the thin wall like the osteophyte. It is frequently accompanied by shortening of leg and contracture, as the lesion arises from the development disorders. Thus, the total hip arthroplasty, especially uncemented one, is complicated. However, the satisfactory result can be obtained by careful consideration and surgical procedure such as a provision against the bleeding and the soft tissue release.


Orthopaedic Proceedings
Vol. 104-B, Issue SUPP_13 | Pages 75 - 75
1 Dec 2022
Rousseau-Saine A Kerslake S Hiemstra LA
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Recurrent patellar instability is a common problem and there are multiple demographic and pathoanatomic risk factors that predispose patients to dislocating their patella. The most common of these is trochlear dysplasia. In cases of severe trochlear dysplasia associated with patellar instability, a sulcus deepening trochleoplasty combined with a medial patellofemoral ligament reconstruction (MPFLR) may be indicated. Unaddressed trochlear pathology has been associated with failure and poor post-operative outcomes after stabilization. The purpose of this study is to report the clinical outcome of patients having undergone a trochleoplasty and MPFLR for recurrent lateral patellofemoral instability in the setting of high-grade trochlear dysplasia at a mean of 2 years follow-up. A prospectively collected database was used to identify 46 patients (14 bilateral) who underwent a combined primary MPFLR and trochleoplasty for recurrent patellar instability with high-grade trochlear dysplasia between August 2013 and July 2021. A single surgeon performed a thin flap trochleoplasty using a lateral para-patellar approach with lateral retinaculum lengthening in all 60 cases. A tibial tubercle osteotomy (TTO) was performed concomitantly in seven knees (11.7%) and the MPFLR was performed with a gracilis tendon autograft in 22%, an allograft tendon in 27% and a quadriceps tendon autograft in 57% of cases. Patients were assessed post-operatively at three weeks and three, six, 12 and 24 months. The primary outcome was the Banff Patellar Instability Instrument 2.0 (BPII 2.0) and secondary outcomes were incidence of recurrent instability, complications and reoperations. The mean age was 22.2 years (range, 13 to 45), 76.7% of patients were female, the mean BMI was 25.03 and the prevalence of a positive Beighton score (>4/9) was 40%. The mean follow-up was 24.3 (range, 6 to 67.7) months and only one patient was lost to follow-up before one year post-operatively. The BPII 2.0 improved significantly from a mean of 27.3 pre-operatively to 61.1 at six months (p < 0 .01) and further slight improvement to a mean of 62.1 at 12 months and 65.6 at 24 months post-operatively. Only one patient (1.6%) experienced a single event of subluxation without frank dislocation at nine months. There were three reoperations (5%): one for removal of the TTO screws and prominent chondral nail, one for second-look arthroscopy for persistent J-sign and one for mechanical symptoms associated with overgrowth of a lateral condyle cartilage repair with a bioscaffold. There were no other complications. In this patient cohort, combined MPFLR and trochleoplasty for recurrent patellar instability with severe trochlear dysplasia led to significant improvement of patient reported outcome scores and no recurrence of patellar dislocation at a mean of 2 years. Furthermore, in this series the procedure demonstrated a low rate (5%) of complications and reoperations


Orthopaedic Proceedings
Vol. 99-B, Issue SUPP_22 | Pages 10 - 10
1 Dec 2017
Hotchen AJ Garcia-Pulido P Gojanur A Stohr K
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Aims. This case series aims to describe the clinical consequences of juxta-physeal sub-acute osteomyelitis in children, specifically growth and limb deformity. Methods. All children diagnosed with osteomyelitis between 2014 and 2016 at a single University Teaching Hospital in the UK were included. Juxta-physeal sub-acute osteomyelitis was identified using magnetic resonance imaging obtained within 48-hours of presentation. These cases were followed up prospectively on a regular basis in the outpatient clinic. Any clinical evidence of limb or growth deformity was evaluated using long-leg standing radiographs. Results. During the study period, 63 paediatric osteomyelitis cases were identified and four of these (6%) had juxta-physeal sub-acute osteomyelitis. All bone infections were located either in the distal femur or proximal tibia. All cases were treated with six weeks of intravenous ceftriaxone and three children underwent surgical procedures. All four cases developed a growth deformity in the affected limb. Conclusions. A variety of growth disturbances can occur following sub-acute osteomyelitis which could be secondary to physeal stimulation and overgrowth. In this series, overgrowth occurred in the physis immediately adjacent to the Brodie's abscess. Subsequently, the presence of a medial abscess caused a valgus deformity and a lateral abscess caused a varus deformity. This phenomenon has not been well-described in the literature. The tibial and femoral physes are amongst the most active in the body, which may explain the reason for the observed overgrowth deformity in these cases. The age of the patient and the method of treatment did not appear to influence the emergence of the growth deformity. None of our patients had recurrence or development of chronic osteomyelitis within the measured time period. In view of these findings, we recommend regular follow-up including assessment for limb deformity for a minimum of 3-years following the treatment of sub-acute osteomyelitis


Orthopaedic Proceedings
Vol. 104-B, Issue SUPP_5 | Pages 18 - 18
1 Apr 2022
Varasteh A Gangadharan S James L
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Introduction. Amputation or disarticulation is a reliable option for management of severe foot deformities and limb-length discrepancies, the surgical restoration of which are unpredictable or unfavourable. Of the various surgeries involving foot ablation, Syme's amputation is preferred for congenital deformities as it provides a growing, weight bearing stump with proprioception and cushioning. Materials and Methods. We reviewed data of all children who underwent Syme's amputation over the past 13 years at our institution. Surgical technique followed the same principles for Syme's but varied with surgeons. Results. Ten boys and ten girls, with an average age of 18 months and average follow up of 70 months were included in the study. The most common indication was fibular hemimelia. Wound complications were reported in three children, phantom pain in one, heel pad migration in two. None had wound dehiscence, flap necrosis, stump overgrowth, or calcaneal regrowth. None of this required surgical intervention. One child required an amputation at a higher-level secondary to a congenital malformation of nervous tissue in the affected leg. Prosthetic compatibility was 94.7 % and none used mobility aids. Six children participated in sports. Conclusions. Syme amputation is a safe and potentially advantageous procedure in children, with a low incidence of complications to offer patients with non-salvageable foot conditions. It offers good prosthetic use with minimal risk of complications and can offer patients a functional solution with only one surgical intervention throughout their childhood


Orthopaedic Proceedings
Vol. 95-B, Issue SUPP_29 | Pages 31 - 31
1 Aug 2013
Firth G Kontio K Mosquijo J
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Purpose:. Despite advances in limb reconstruction, there are still a number of young patients who require trans-tibial amputation. Amputation osteoplasty is a technique described by Ertl to enhance rehabilitation after trans-tibial amputation. The purpose of the present study was to evaluate the results of the original Ertl procedure in skeletally immature patients, and to assess whether use of this procedure would result in a diminished incidence of bony overgrowth. Methods:. Four consecutive patients (five amputations) treated between January 2005 and June 2008 were reviewed. Clinical evaluation consisted of completion of the prosthesis evaluation questionnaire (PEQ) and physical examination. Radiographic analysis was performed to evaluate bone-bridge healing, bone overgrowth and the medial proximal tibial angle (MPTA). Results:. The best mean PEQ result in the Question section was 91.8 (Range 74–100) for ‘Well being’ and the worst mean score was 66.6 (Range 50–78) for the sub-section ‘Residual limb health’. Examination of the residual limbs revealed no bursae were present and all knees were stable with full range of movement. All bony bridges united at an average age of 1.7 months (Range 1–2). One case required stump revision for bony overgrowth, and one case developed asymptomatic mild genu varum. Conclusions:. In this series, the original Ertl osteomyoplasty shows good functional and prosthetic use with only one bony overgrowth requiring revision surgery


Orthopaedic Proceedings
Vol. 101-B, Issue SUPP_14 | Pages 36 - 36
1 Dec 2019
Depypere M Moonen C Alaerts R Hoekstra H Sermon A Nijs S Metsemakers W
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Aim. Negative-pressure wound therapy (NPWT) is often propagated as treatment option for fracture-related infection (FRI). After surgical debridement and repeated NPWT dressing changes, the wounds are often closed by free flaps. Sometimes even healing by secondary intention seems an alternative. Recently, concerns have been raised on the long-term use of NPWT as it could be related to bacterial overgrowth and possible re-infection. The purpose of this study was to conduct a retrospective evaluation of the influence of long-term NPWT on tissue culture results and outcome in FRI patients. Method. Between January 1. st. , 2015 and December 31st, 2018, a total of 852 patients were treated with NPWT for different indications on the Department of Trauma Surgery. Inclusion criteria for this study were patients with a closed fracture, stabilized with osteosynthetic fixation and complicated with a confirmed FRI according to the FRI consensus definition. Patients were included when they received at least three NPWT dressing changes in the operating room. Exclusion criteria were patients younger than 18 years, or the absence of cultures results from dressing changes. Results. During the study period 23 patients met the inclusion criteria. According to the tripartite classification of Willenegger and Roth, one patient had an early, 14 a delayed and 8 patients a late onset FRI. Overall, 139 NPWT dressing applications were performed, with an average amount of six per patient. In 14 patients (61%) and 57 dressing changes (41%), at least 2 tissue cultures showed the same pathogen or at least one, in case of highly virulent organisms (e.g. S. aureus) during a single dressing exchange. Coagulase-negative staphylococci were present in 33% of the cases, followed by Enterococcus spp. (21%), S. aureus (16%), non-fermentative gram negative bacilli (14%) and Enterobacteriaceae (7%). Furthermore, 17 exchanges showed polymicrobial growth. Five patients had repeatedly significant growth of the same pathogen despite adequate antimicrobial therapy, within this group one patient was immunocompromised. Conclusions. In a large amount of patients (61%), a significant number of positive culture results could be acquired, even in the presence of adequate local and systemic antimicrobial therapy. The clinical relevance of these results remains unclear. This said, it seems important to limit the duration of NPWT as prolonged treatment could increase bacterial overgrowth and possible (re-)infection. Therefore, a rapid definitive soft tissue coverage should be encouraged. Future larger prospective clinical trials are required


Orthopaedic Proceedings
Vol. 91-B, Issue SUPP_I | Pages 130 - 130
1 Mar 2009
Norrish A Bates J Harrison W
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A number of series report limb length discrepancy in long bone chronic osteomyelitis, however in most cases, it is shortening of the affected bone. This is thought to be due to damage in the affected growth plate leading to early growth arrest. However, it is known that the inflammatory state of chronic osteomyelitis results in an increased blood supply and, as in other conditions such as rheumatoid arthritis, the increased blood supply results in overgrowth of the affected bone. In order to study the effect of long bone chronic osteomyelitis on limb length, we designed a prospective trial of 42 consecutive patients presenting to our unit with chronic osteomyelitis of a long bone. The inclusion criteria were all patients presenting with a long bone osteomyelitis. There were no exclusion criteria. The mean age at presentation was 10.3 years. The mean duration of symptoms of 18.2 months prior to presentation. For 37 (88%) of patients the cause of osteomyelitis was haematogenous. On examination, 3 (7%) patients had shortening of the long bone compared to the unaffected side (of an average of 2.5cm), whilst 13 (31%) patients had overgrowth of the affected bone (average overgrowth 2.2cm). The most common bone affected was the tibia (20/42, 48%), followed by the femur (8/42, 19%) and the humerus (6/42, 14%). All patients underwent radiographic analysis, and the average percentage of long bone affected was 59%. 8/42 (12%) of patients had at least one physis affected (2 of these patients had undergrowth and 1 had overgrowth). This large prospective series of patients is the first in the world literature to show the effect of osteomyelitis on the growth of long bones, in particular an overgrowth rate of 31%. We suggest that the mechanism for this is related to the duration of symptoms. In areas of the world where there is poor access to health care, there is consequently a prolonged period of increased blood supply as a result of inflammation. This increased blood supply may make limb length discrepancy is more likely to be due to overgrowth rather than undergrowth


Orthopaedic Proceedings
Vol. 91-B, Issue SUPP_I | Pages 62 - 62
1 Mar 2009
Christodoulou G Tagaris G Sdougkos G Vlachos A Vris A
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Introduction: This study examines the proximal tibial metaphyseal fractures in children and specifically the valgus deformity and leg overgrowth of the tibia. Methods: We examined 27 children with proximal tibial metaphyseal fractures. Among them, 11 presented with greenstick fractures, 6 with complete, 5 with hairline, 3 with torus and 2 with stress fractures. The mean age was 7 years old (1–14). The average follow up period was 9.5 years. Fifteen children were below the age of 7 while 12 were between 8–14 years old. Twenty five patients were treated conservatively and 2 surgically. Results: Valgus deformity occurred during the follow up period in 73% of the children aged below 7 y.o. and 17% of the older children. The higher values of valgus deformity ranged between 8–18 degrees and were observed at 10–18 months post-traumatically, especially in younger ages and after inadequate reductions. At the time of the final follow up examination, satisfactory spontaneous correction of the deformity, inversely proportional to age was observed in all cases. The degree of final valgus deformity ranged between 1–9 degrees. Overgrowth of the affected extremity was observed in 74% of the cases and ranged between 0, 4 and 1, 5 cm. Tibial overgrowth is not dependent to skeletal age. None of torus and stress fractures developed valgus deformity or longitudinal overgrowth. Compartment syndrome occurred in one case. Conclusion: A high tendency to valgus deformity, especially in younger ages, was observed in proximal tibial metaphyseal fractures, even among undisplaced or surgically treated ones. Approximately two years post-traumatically, the beginning of a progressive spontaneous satisfactory correction of the deformity – inversely proportional to age – was noticed. It is worth mentioning that valgus deformity up to 10 degrees and tibial overgrowth up to 1, 5 cm cause no functional or cosmetic problems. The above observations prevent us from unnecessary surgical correction. Conservative treatment is preferred for the proximal tibial metaphyseal fractures. Operative treatment is indicated after inadequate reduction, especially in older children and after open fractures


Orthopaedic Proceedings
Vol. 96-B, Issue SUPP_10 | Pages 29 - 29
1 Jul 2014
Pinto R Harrison W Huson S Graham K Nayagam S
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The purpose of this study is to report a unique overgrowth syndrome and discuss the insights into the complex orthopaedic management. Written consent to report this case was granted. The patient's condition, wrongly diagnosed as Proteus syndrome, is characterised by a genetic mutation in PIK3CA, a critical regulator of cell growth. This lead to unregulated cellular division of fibroblasts isolated to the lower limbs. The legs weighed 117 kg, with a circumference of >110 cm. In addition to lower limb overgrowth, numerous musculoskeletal and organ pathologies have been encountered since birth requiring treatment from a wide variety of healthcare specialists and basic scientists. At 32 years, the patient developed septicaemia secondary to an infected foot ulcer. Amputation had been discussed in the elective setting, however the presence of sepsis expedited surgery. The above knee amputation took 9 hours and four assistants including a plastic surgeon. A difficult dissection revealed a deep subcutaneous fatty layer that integrated with deep muscle, massive hypertrophy of cutaneous nerves and the sciatic nerve and ossification within the distal quarter of the quadriceps muscles requiring osteotomy. The lower limb osteology was grossly aberrant. The size of the amputated limb did not permit use of a tourniquet and cell salvage reintroduced 10.5 litres of blood with a further 6 units of red cells intra-operatively. The leg stump successfully took to a split-skin graft. A unique phenomenon was witnessed post-operatively whereby the stump continued to grow due to upregulation of fibroblasts secondary to trauma. Targeted genetic therapies have been successfully developed to suppress this stump growth. This unique and unclassified overgrowth syndrome was caused by a mutation in the PIK3CA gene. Orthopaedic management of the oversized limb was complex requiring multiple surgeons and prolonged general anesthetic. A multi-disciplinary approach to this condition is required for optimizing outcomes in these patients


Orthopaedic Proceedings
Vol. 103-B, Issue SUPP_13 | Pages 123 - 123
1 Nov 2021
Heydar A Şirazi S
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Introduction and Objective. Pectus carinatum is a common congenital anterior chest wall deformity, characterized by outward protrusion of sternum and ribcage resulted from rib cartilage overgrowth. The protrusion may be symmetrical or asymmetrical. Pectus carinatum association with mitral valve diseases, Marfan's syndrome, and scoliosis enforces that poor connective tissue development as possible etiological factor. Despite the coexistence of pectus carinatum and scoliosis has attracted the attention of some researchers, the association between pectus carinatum and the other spinal deformities has not been studied comprehensively. The frequency of spinal deformity in patients with pectus carinatum and the mutual relationships of their subtypes are needed to be studied to determine the epidemiological character of the combined deformity and to plan patient evaluation and management. Our study aimed to investigate the association, define the incidence and evaluate the characteristics between different types of spinal deformities and Pectus carinatum. Materials and Methods. Radiological and physical examinations were performed for 117 pectus carinatum patients in Marmara university hospital/Turkey in the years between 2006 and 2013. The incidence of spinal deformity was calculated. Spinal deformities were classified as scoliosis, kyphosis, kyphoscoliosis, and spinal asymmetry, whereas pectus carinatum were subdivided into symmetric and asymmetric subgroups. The relationship between spinal deformities and the symmetrical-asymmetric subtype of pectus excavatum was statistically analyzed, Pearson chi-square test was used to compare the association of qualitative data. The significance level was accepted as p <0.05. Lastly, the angular values of the deformities of scoliosis and kyphosis patients were measured using the Cobb method. In this way, the magnitude of the deformity was given as a numerical value. Results. Spinal deformity was detected in 23 (17 symmetrical PE and 6 asymmetrical PE) of 117 pectus excavatum patients. Scoliosis and kyphosis were seen equally in symmetrical pectus carinatum, whereas scoliosis was seen in 33.3% and kyphosis in 50% in asymmetric pectus carinatum patients, respectively. However, there were no statistically significant differences in the distribution of scoliosis and kyphosis in patients with symmetrical and asymmetrical PE. Idiopathic scoliosis constituted the most common scoliosis group. Congenital kyphosis was not found in any kyphosis patient. The average Cobb angle of scoliosis patients was 32°, and the mean T2-T12 kyphosis angle of these patients was 55.5°, while the average kyphosis angle of those with kyphosis deformity was 71°. Conclusions. Patients with Pectus carinatum have a higher incidence of spinal deformities than the normal population. Such high concomitant incidence should be taken under consideration in evaluating and treating patients presenting with either deformity


Orthopaedic Proceedings
Vol. 103-B, Issue SUPP_6 | Pages 6 - 6
1 May 2021
Chatterton BD Kuiper J Williams DP
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Introduction. Circumferential periosteal release is a rarely reported procedure for paediatric limb lengthening. The technique involves circumferential excision of a strip of periosteum from the metaphysis of the distal femur, tibia and fibula. This study aims to determine the mid to long-term effectiveness of this technique. Materials and Methods. A retrospective case series was performed of all patients undergoing circumferential periosteal release of the distal femur and/or tibia between 2006 and 2017. Data collected included demographics, surgical indication, post-operative limb-lengths and complications. Data collection was stopped if a further procedure was performed that may affect limb-length (except a further release). Leg-length discrepancies were calculated as absolute values and as percentages of the longer limb-length. Final absolute and percentage discrepancies were compared to initial discrepancies using a paired t-test. Results. Eighteen patients (11 males) were identified, who underwent 25 procedures. The mean age at first surgery was 5.83 (SD 3.49). The commonest indication was congenital limb deficiency (13 patients). In 23 procedures the periosteum was released in two limb segments (distal femur and distal tibia), whereas in two patients it was released in a single limb segment. Five patients underwent repeat periosteal release, and one patient had three periosteal releases. Mean follow-up was 63.1 months (SD 33.9). Fifteen patients had sufficient data for statistical analysis. The mean initial absolute discrepancy was 2.01cm (SD 1.13), and the mean initial percentage discrepancy was 4.09% (SD 2.76). The mean final absolute discrepancy was 1.00cm (SD 1.62), and the mean percentage final discrepancy was 1.37% (SD 2.42). The mean reduction in absolute discrepancy was 0.52 cm (95%CI −0.04–1.08; p=0.068, paired t-test), and the mean reduction in percentage discrepancy was 2.00% (95% CI 1.02–2.98, p=<0.001 paired t-test). In five patients the operated limb overgrew the shorter limb. Conclusions. Circumferential periosteal release produces a modest decrease in both absolute and percentage limb-length discrepancy, although the outcome is variable and some patients may experience overgrowth of the operated limb


Orthopaedic Proceedings
Vol. 91-B, Issue SUPP_I | Pages 57 - 57
1 Mar 2009
Monsell F Eastwood D Hoey S Kangesu L Harper J Sebire N
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Background: Proteus Syndrome is a rare, sporadic overgrowth disorder for which the underlying genetic defect remains unknown. Although the clinical course is well-described, there is no systematic histopathological description of the lesional pathology. Objective: To describe the histopathological features encountered in a series of patients with Proteus syndrome from a single centre. Patients/Methods: Patients with Proteus syndrome who had undergone therapeutic surgical resection or biopsy were identified from a database and the histopathological findings were reviewed, with particular reference to descriptive features of the underlying tissue abnormality. Results: There were 18 surgical specimens from nine patients, median age 4 (range 1–9) years, including four main categories; soft tissue swellings (lipomatous lesions), vascular anomalies (vascular malformation and haeman-gioma), macrodactyly (hamartomatous overgrowth) and others (sebaceous naevus and non-specific features). In all cases the clinical features of overgrowth were due to increased amounts of disorganised tissue, indicating a hamartomatous-type defect in which normal tissue constituents were present but with an abnormal distribution and architecture. Vascular malformations represented a prominent category of lesions, accounting for 50% of the specimens, predominantly comprising lymphatic and lymphovascular malformations. No malignancy or cytological atypia was identified in any case. Conclusions: The histopathological features of lesions resected from children with Proteus syndrome predominantly include hamartomatous mixed connective tissue lesions, benign neoplasms such as lipomata and lymphatic-rich vascular malformations


Orthopaedic Proceedings
Vol. 85-B, Issue SUPP_III | Pages 261 - 261
1 Mar 2003
Monsell F Hosalker H Harper P
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The Proteus syndrome involves asymmetrical gigantism, verrucous epidermal naevi, vascular malformations, hamartomas and hyperostosis. The clinical features have frequently been described, but the radiological features have not been studied in detail. This paper describes the radiological features of a group of 18 patients (12 male, 6 female) that presented to the Department of Dermatology and Orthopaedic Surgery of this institution. Plain radiographs of each affected area were obtained to assist in the diagnosis and subsequent management of each patient. These radiographs were evaluated in an attempt to define the radiological anatomy of the osseous lesions. The abnormalities were classified as involving abnormal ossification, hyperostotic overgrowth or ectopic calcification. The individual features of each group will be presented. We attempted to define radiological parameters that were specific to this condition and therefore useful in diagnosis. We considered a number of radiological measurements and found a consistent alteration in bony architecture of the upper and lower limbs of affected individuals. This association was not detected in other overgrowth symptoms. This is the first objective radiological parameter that assists in the diagnosis of this rare condition


Orthopaedic Proceedings
Vol. 94-B, Issue SUPP_XXVII | Pages 30 - 30
1 Jun 2012
Burwell R Aujla R Grevitt M Randell T Dangerfield P Cole A Kirby A Polak F Pratt R Webb J Moulton A
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Introduction. In patients with adolescent idiopathic scoliosis (AIS), anomalous extra-spinal left-right skeletal length asymmetries in upper limbs, periapical ribs, and ilia beg the question as to whether these bilateral asymmetries are connected in some way with pathogenesis. The upper arm and iliac length asymmetries correlate significantly with adjacent spinal curve severity respectively in thoracic and lower (thoracolumbar and lumbar) spine. In lower limbs, skeletal length asymmetries and proximo-distal disproportion are unrelated to spinal curve severity. Overall, these observations raise questions about mechanisms that determine skeletal bilateral symmetry of vertebrates in health and disorder, and whether such mechanisms are involved in the cause of this disease. We investigated upper arm length (UAL) asymmetries in two groups of right-handed girls aged 11–18 years, with right thoracic adolescent idiopathic scoliosis (RT-AIS, n=98) from preoperative and screening referrals (mean Cobb angle 45°) and healthy controls (n=240). Methods. Right and left UAL were measured with a Harpenden anthropometer of the Holtain equipment, by one of four observers (RGB, AAC, RKP, FJP). UAL asymmetry was calculated as UAL difference, right minus left, in mm. Repeatability of the measurements was assessed by technical error of the measurement (TEM) and coefficient of reliability (R). Results. In girls with RT-AIS, UAL asymmetry was greater than it was in healthy girls (mean 5·9 mm vs 2·5 mm, ANOVA p<0·001, correcting for age), regressed negatively with age (p<0·001, r= –0·374), and correlated significantly with Cobb angle (r=0·342, p=0·001) and apical vertebral rotation (Perdriolle, r=0·291, p=0·004). In healthy girls, UAL asymmetry was unrelated to age. Plotted against years after estimated menarcheal age, right UAL overgrowth reduced significantly for girls with RT-AIS (r= –0·312, p=0·006, n=76) but not for healthy girls (r=0·000, p=0·985, n=121), which was a significant finding (p=0·052, ANOVA). Conclusions. The abnormal overgrowth of right upper arm length may be secondary, or pathogenetically associated with the RT-AIS trunk deformity. The negative regression of UAL asymmetry may result from (1) older girls having less residual growth and/or (2) a transient, or resolving, asymmetry process common to arm and trunk. We hypothesise that the pathogenetic process of RT-AIS may include two components: a transient bilateral asymmetry process and growth velocity, both of which affect trunk and arm growth. In the spine, these rarely lead to scoliosis resolution because biomechanical, postural, melatonin-signalling, and other factors sustain and aggravate the curve. Four pathomechanisms may induce the asymmetry process in trunk and arms involving (1) neuromuscular function, (2) motor cortex, (3) sympathetic nervous system, and (4) intrinsic time-tallies in growth plates, some of which suggest therapeutic possibilities


Orthopaedic Proceedings
Vol. 92-B, Issue SUPP_I | Pages 40 - 40
1 Mar 2010
Jones CB Tressel WD Endres TJ Ringler JR Bielema DJ
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Purpose: Pediatric femoral fracture treatment is varied. Each treatment has advantages and disadvantages. The goal of treatment is to avoid complications, reduce costs, and return function. Percutaneous bridge plating has many advantages and little disadvantages. The purpose of this study was to examine the results of percutaneous bridge plating for pediatric femoral diaphyseal fractures. Method: Over a 4-year time span (2002–2005), all pediatric femoral fractures were diagnosed. A retrospective chart review was completed and only percutaneous bridge plating treatment was analyzed. Results: 78 fractures were noted in 73 patients. Average age was 9 (range 3–16). Gender was 56 males and 22 females. Most common mechanisms were falls 15 (19%), MVA 12 (15%), and pedestrians 9 (12%). Four fractures (5%) were open. Forty-three fractures (61%) were associated with polytrauma. Time to operation averaged 1 day (0–11 days, 89% 0–1 day). Most plates were 3.5 combi locked plates with a lesser but equal number of 3.5 DCP and 4.5 DCP. Length of stay averaged 5 days (range 0–45 days, 18% 0–1 day, 58% 2–4 days). The majority of patients (58%) began weight bearing at 2–6 weeks. Callus formation began at 2–6 wks (84%). Fracture healing occurred by 6 weeks in 91%. Limp was resolved by 3 months in 54%. Pain was resolved by 3 months in 90%. Patients were back to active daily living without restrictions by 3 months in 96% of the fractures. Complications were noted with 4 superficial wounds, 4 problematic scars, 3 leg overgrowth < 12 mm, and 3 distal prominent plates. No nonunions or refractures were noted. Hardware (HW) was removed on average by 6 months (range 3 mo to never). Outpatient percutaneous HW removal was performed in 100% of the cases. Conclusion: Percutaneous bridge plating for pediatric femoral fractures is predictable and effective with minimal complications. Asymptomatic femoral overgrowth was minimal


Orthopaedic Proceedings
Vol. 86-B, Issue SUPP_II | Pages 162 - 162
1 Feb 2004
Flieger I Leonidou O Pettas N Mourafetis T Pertsemlides D
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Fracture of the lateral humeral condyle accounts for approximately 15% of all elbow fractures in children. We studied 68 fractures of the lateral humeral condyle, which were treated surgically over a period of six years during 1994 to 2000. There were 49 boys and 19 girls. The average age was 6,5 years (2,5 – 13 years). All cases were treated with open reduction and internal fixation with two divergent K-wires for safer stabilisation of the fracture. Subsequently a cast was applied for 4–6 weeks to the radiological union of the fracture, where the K-wires were removed. The patients were evaluated with clinical and radiological criteria. Sixty-five cases were classified as Milch type II fractures and three were Milch type I fractures. According to the Jacob classification, which records the degree of displacement, 23 cases were type II fractures and 45 cases were type III fractures. The mean follow up was 4 years (2–8 years). Analysis of the postoperative radiographs showed that radiological union was achieved in all cases. The mean time to radiological union of the fracture was 4,5 weeks (3–8 weeks). At latest follow up we observed abnormalities of the shape of the distal humerus due to overgrowth of the lateral humeral condyle in 40% of the cases. In 4 patients we observed pseudocubitus varus due to overgrowth of the lateral humeral condyle and in 3 patients we observed mild cubitus varus (< 5°). Clinically in all cases there was painless free movement of the elbow. We conclude that satisfactory anatomical reduction of the fracture gives good clinical results. The radiological abnormalities observed seem not to play an important role in the final result