JBJS Editor-in-Chief Dr. Marc Swiontkowski brought to OrthoBuzz’s attention a recent “Family Partnerships” essay published in Pediatrics. The 4 “speakers” in the essay chronicle the suffering and pain of Lindsay Ellingworth, who was born with a congenital lower-limb deficiency. Lindsay’s young parents opted for limb lengthening over amputation plus a prosthesis after several orthopaedic consultations and an agonizing, confusing decision-making process.
Orthopaedic surgeon Dr. David Hootnick entered the picture about 10 years ago, when he first saw Lindsay, who was by then a young nursing student with ongoing problems associated with the index procedure—including scoliosis and chronic neck and back pain. The extent of the original deformity (30% femur shortening at birth) made Lindsay a “nonideal candidate for lengthening,” says Dr. Hootnick, but he adds that “Lindsay had a normal-appearing foot, making it all the more understandable that her parents balked at removing an apparently healthy part of their beloved child.”
Lindsay describes her limb-lengthening and years of treatments for complications as “a living nightmare.” Now an adult, she has concluded that “the doctors put a pretty bow on limb lengthening.” Pediatrician and bioethicist Dr. Amy Caruso Brown acknowledges in the essay the untenable bind Lindsay’s parents found themselves in when having to make a decision before their child was old enough to express preferences. “It is difficult to accept that a procedure that sounds as drastic and anachronistic as amputation might have fewer complications than the seemingly more sophisticated alternative,” Dr. Brown said.
According to Lindsay’s mother Rene Mauchin, “Although she has endured so much, Lindsay still laughs and celebrates life.” When asked for a takeaway that might help other families in similar situations, Ms. Mauchin said, “You have a right to know everything the doctors know…Don’t hesitate to see several doctors, and ask for evidence to back up their recommendations.”
Every month, JBJS publishes a review of the most pertinent and impactful studies published in the orthopaedic literature during the previous year in 13 subspecialties. Click here for a collection of all OrthoBuzz Specialty Update summaries.
This month, Mark T. Dahl, MD, co-author of the August 21, 2019 “What’s New in Limb Lengthening and Deformity Correction,” selected the five most clinically compelling findings from among the 40 noteworthy studies summarized in the article.
–Authors of a retrospective study of 119 patients with Crawford type-II congenital pseudarthrosis of the tibia found a 69% union rate at maturity. They did not identify specific factors influencing rates of union or refracture, however.1
–The models created with this technology can help surgeons preoperatively assess specific anatomical geometries. Corona et al.2 used 3-D-printed titanium truss cages, along with the Masquelet technique, to treat massive infected posttraumatic defects.
Growth Prediction in Limb Lengthening
–A comparative evaluation of the predictive accuracy of 4 methods to correctly time epiphysiodesis in 77 patients found the multiplier method to be the least accurate. In a separate study of 863 epiphysiodeses, authors reported a 7% complication rate.3 The most common complication was incomplete arrest that resulted in angular deformities; half of those cases required reoperation.
Congenital Limb Deficiencies
–Over 16 years, Finnish children born with lower-limb deficiencies had 6 times the number of hospital admissions and 10 times the number of days in hospital per child, compared with children born without a limb deficiency.4
- Shah H, Joseph B, Nair BVS, Kotian DB, Choi IH, Richards BS, Johnston C, Madhuri V, Dobbs MB, Dahl M. What factors influence union and refracture of congenital pseudarthrosis of the tibia? A multicenter long-term study. J Pediatr Orthop. 2018 Jul;38(6):e332-7.
- Corona PS, Vicente M, Tetsworth K, Glatt V. Preliminary results using patient-specific 3D printed models to improve preoperative planning for correction of post-traumatic tibial deformities with circular frames. Injury. 2018 Sep;49(Suppl 2):S51-9.
- Makarov MR, Dunn SH, Singer DE, Rathjen KE, Ramo BA, Chukwunyerenwa CK, Birch JG. Complications associated with epiphysiodesis for management of leg length discrepancy. J Pediatr Orthop. 2018 Aug;38(7):370-4.
- Syvänen J, Helenius I, Koskimies-Virta E, Ritvanen A, Hurme S, Nietosvaara Y. Hospital admissions and surgical treatment of children with lower-limb deficiency in Finland. Scand J Surg. 2018 Nov 19:1457496918812233. [Epub ahead of print]
Every month, JBJS publishes a Specialty Update—a review of the most pertinent and impactful studies published in the orthopaedic literature during the previous year in 13 subspecialties. Here is a summary of selected findings from Level I and II studies cited in the August 19, 2015 Specialty Update on limb lengthening and deformity correction:
Pediatric Disorders and Trauma
–A modified guided-growth technique for insertion of tension-band plates decreased operative time, radiation exposure, and incision size.1
–Two meta-analyses concluded that, although oral or intravenous bisphosphonates in children with osteogenesis imperfecta increased bone mineral density, evidence of reduction in fracture rates was inconclusive.2, 3
–A systematic review of 40 studies on surgical management of posttraumatic cubitus varus in children noted an overall complication rate of 14.5%, with no single technique being substantially safer or more effective.4
Lower-Limb Trauma/Reconstruction in Adults
–A prospective randomized study on the surgical treatment of complex knee dislocations with ligament reconstruction found a significantly lower risk of delayed ligament failure with adjunctive hinged external fixation compared with a hinged knee brace.
–A prospective randomized study comparing biplanar external fixation with reamed interlocking intramedullary nailing for treating open tibial shaft fractures found similar healing rates and functional outcomes one year postoperatively.5
–Patients with extra-articular distal tibial fractures treated with circular external fixators had earlier weight-bearing and superior function compared with those managed with plate fixation.6
–A randomized controlled trial of patients with medial compartment knee osteoarthritis reported similar radiographic outcomes six years postoperatively among those who had opening-wedge high tibial osteotomy compared with those who had undergone closing-wedge high tibial osteotomy. The closing-wedge group had fewer complications but greater prevalence of conversion to total knee arthroplasty.
Foot and Ankle Reconstruction
–A multicenter prospective study comparing ankle arthroplasty with ankle arthrodesis noted similar patient-reported outcomes, although revision rates and major complications were higher following ankle replacement.
Managing Postoperative Complications
–A comparative study noted a lower prevalence of pin-site infections with the use of chlorhexidine (9.2%) compared with povidone-iodine (27.9%) following external fixation.7
–A randomized study revealed a 27% reduction in external fixation time with the use of low-intensity pulsed ultrasound for tibial osteoplasty.8
–A randomized trial in patients undergoing bilateral tibial lengthening showed no improvement in postoperative pain or ankle-joint mobility following botulinum toxin A injection in the calf muscle.9
New Tools and Techniques
–In a matched-pair study, patients undergoing femoral lengthening using a motorized intramedullary nail showed better consolidation indices, better knee mobility, and decreased complication rates compared with conventional external fixation.10
- MasquijoJJ, Lanfranchi L, Torres-Gomez A, Allende V. Guided growth with the tension band plate construct: a prospective comparison of 2 methods of implant placement. J Pediatr Orthop. 2015 Apr-May;35(3):e20
- Dwan K, Phillipi CA, Steiner RD, Basel D. Bisphosphonate therapy for osteogenesis imperfecta. Cochrane Database Syst Rev. 2014;7:CD005088. Epub 2014 Jul 23
- Hald JD, Evangelou E, Langdahl BL, Ralston SH. Bisphosphonates for the prevention of fractures in osteogenesis imperfecta: meta-analysis of placebo-controlled trials. J Bone Miner Res.2014 Nov 18
- Solfelt DA, Hill BW, Anderson CP, Cole PA. Supracondylar osteotomy for the treatment of cubitus varus in children: a systematic review. Bone Joint J. 2014May;96-B(5):691-700
- Rodrigues FL, de Abreu LC, Valenti VE, Valente AL, da Costa Pereira Cestari R,Pohl PH, Rodrigues LM. Bone tissue repair in patients with open diaphyseal tibial fracture treated with biplanar external fixation or reamed locked intramedullary nailing. Injury. 2014 Nov;45(Suppl 5):S32-5
- Fadel M, Ahmed MA, Al-Dars AM, Maabed MA, Shawki H. Ilizarov external fixation versus plate osteosynthesis in the management of extra-articular fractures of the distal tibia. Int Orthop. 2015 Mar;39(3):513-9. Epub 2014 Dec 5
- Cam R, Demir Korkmaz F, Oner Şavk S. Effects of two different solutions used in pin site care on the development of infection. Acta Orthop Traumatol Turc.2014;48(1):80-5
- Salem KH, Schmelz A. Low-intensity pulsed ultrasound shortens the treatment time in tibial distraction osteogenesis. Int Orthop. 2014 Jul;38(7):1477-82. Epub 2014 Jan 7
- Lee DH, Ryu KJ, Shin DE, Kim HW. Botulinum toxin A does not decrease calf pain or improve ROM during limb lengthening: a randomized trial. Clin Orthop Relat Res.2014 Dec;472(12):3835-41
- Horn J, Grimsrud Ø, Dagsgard AH, Huhnstock S, Steen H. Femoral lengthening with a motorized intramedullary nail. Acta Orthop. 2015 Apr;86(2):248-56. Epub 2014 Sep 5
Bone is one of the most biologically unique tissues in the human body. What distinguishes bone from most other tissues is that, when injured, it heals by regeneration of its original tissue as opposed to the formation of scar. Recognizing this phenomenon, surgeons as early as the turn of the nineteenth century initiated efforts to lengthen limbs by performing osteotomies and initiating distraction immediately. They had various degrees of success. By the middle of the twentieth century, this technique had been refined thanks to the pioneering work of Dr. Gavril Ilizarov, who recognized the importance of a latency period and the rate and rhythm of the distraction. Ilizarov devoted his life to the research and advancement of a minimally invasive procedure that could be used to treat limb-length deformities and injuries by stimulating the formation of new bone. While Ilizarov and others continued to refine these techniques for clinical use, clinician-scientists recognized that the ability to regenerate tissue through slow, steady, and rhythmic distraction at the site of an osteotomy could yield substantial new knowledge in the field of bone repair and regeneration. Following this recognition, experimental studies demonstrated that the process of distraction osteogenesis is driven by the formation of new blood vessels; thus, angiogenesis precedes osteogenesis.
In the August 2015 issue of JBJS Reviews, Compton et al. discuss the potential biological basis for the phenomenon of distraction osteogenesis and recognize the importance of angiogenesis. Vascular endothelial growth factor (VEGF) has been shown to be one of the most essential and important growth factors in the development of bone regeneration and is regulated by its upstream promoter, hypoxia-inducible factor-1 alpha (HIF-1-α). Further research is needed to fully understand the role of specific molecules and genetic mechanisms in the development of regenerate bone.
In another article in the August 2015 JBJS Reviews, Christopher Iobst notes that limb lengthening as a technique has many advantages, yet the surgeon needs to be vigilant about potential lengthening complications such as joint contractures, joint subluxation, and fractures. While an exciting field, the primary goal of limb lengthening is to produce healthy regenerate bone of the desired length without these complications. In addition, the experience should be as easy and comfortable as possible for the patient. A comprehensive and accurate assessment of limb deformities is essential for successful treatment. In order to accomplish this goal, the concept of preparatory operative treatment has been introduced. Such treatment involves preparing the limb for lengthening by first stabilizing the adjacent joints and removing known soft-tissue constraints. Soft-tissue constraints such as the iliotibial band also may need to be addressed at the time of lengthening. Similar preparation has been outlined prior to lengthening in patients with fibular hemimelia. This stepwise and comprehensive approach is vital to the success of the lengthening.
Iobst also explains how patient selection for limb lengthening is extremely important. Lengthening can be a long and stressful process, and a preoperative assessment of the patient’s psychosocial situation is recommended before the lengthening is started. Advances in limb lengthening techniques involving combinations of external and internal fixation and internal fixation alone with intramedullary rods are presented. Completely internal lengthening nails may have potential advantages over combinations with external fixation, including elimination of pin-track infections and a lower risk of neurovascular injury.
These two articles addressing the scientific and clinical aspects of limb lengthening offer a comprehensive review of this fascinating and important topic. I hope you enjoy reading them!
Thomas A. Einhorn, MD
Editor, JBJS Reviews