HJAR Sep/Oct 2022

HEALTHCARE JOURNAL OF ARKANSAS  I  SEP / OCT 2022 45 nificant limb malformations may be best treated with amputation rather than ex- treme attempts at limb salvage. Recently, a meta-analysis of fibular hemimelia pa- tients showed better patient satisfaction and fewer operations and complications with amputation compared to reconstruc- tion. 12 This is an extremely difficult deci- sion for families. Shared decision-making and ongoing, open discussions can help families work through these decisions in such a way as to support the child and, ideally, minimize time away from school and peers to avoid significant disruption to childhood. Conclusions Management of pediatric limb defor- mity can be highly rewarding for patients and families, referring physicians and subspecialists alike. Substantial improve- ments in surgical techniques have allowed for better outcomes, enhanced mobility, and improved quality of life for patients. However, primary care physicians and subspecialists should recognize that pedi- atric limb deformities do not always exist in a vacuum and should consider further workup and treatment of systemic illness in this group of patients. Ultimately, the patient’s life may depend on it. n REFERENCES 1 Bedard, T.; Lowry, R.B.; Sibbald, B.; et al. “Con- genital limb deficiencies in Alberta-a review of 33 years (1980-2012) from the Alberta Congenital Anomalies Surveillance System (ACASS).” Amer- ican Journal of Medical Genetics 167A, no. 11. (Nov. 2015): 2599-609. doi:10.1002/ajmg.a.37240. 2 Syvanen, J; Nietosvaara, Y.; Ritvanen, A.; et al. “High risk for major nonlimb anomalies asso- ciated with lower-limb deficiency: a popula- tion-based study.” Journal of Bone and Joint Surgery. American 96, no. 22 (Nov. 19, 2014): 1898-904. doi:10.2106/JBJS.N.00155. 3 Ghanem, I. “Epidemiology, etiology, and genet- ic aspects of reduction deficiencies of the lower limb.” Journal of Children’s Orthopaedics 2, no. 5 (Oct. 1, 2008): 329-32. doi:10.1007/s11832-008- Scott J. Schoenleber, MD Pediatric Orthopedic Surgeon Arkansas Children’s 0098-9. 4 Liao, Y.M.; Li, S.L.; Luo, G.Y.; et al. “Routine screening for fetal limb abnormalities in the first trimester.” Prenatal Diagnosis 36, no. 2 (Feb. 2016): 117-26. doi:10.1002/pd.4724. 5 Radler, C.; Myers, A.K.; Hunter, R.J.; et al. “Pre- natal diagnosis of congenital femoral deficiency and fibular hemimelia.” Prenatal Diagnosis 34, no. 10 (Oct. 2014): 940-5. doi:10.1002/pd.4396. 6 Centers for Disease Control and Prevention. “Nutrition, Physical Activity, and Obesity: Data, Trends and Maps.” https://www.cdc.gov/nccd- php/dnpao/data-trends-maps/index.html. Ac- cessed July 19, 2022. 7 Jardaly, A.; McGwin, G., Jr.; Gilbert, S.R. “Blount Disease and Obstructive Sleep Apnea: An Un- der-recognized Association?” Journal of Pedi- atric Orthopedics 40, no. 10 (Nov./Dec. 2020): 604-607. doi:10.1097/BPO.0000000000001591. 8 Griggs, C.L.; Perez, N.P.; Chan, M.C.; Pratt, J.S. “Slipped capital femoral epiphysis and Blount disease as indicators for early metabolic surgi- cal intervention.” Surgery for Obesity and Re- lated Diseases 15, no. 10 (Oct. 2019): 1836-1841. doi:10.1016/j.soard.2019.06.024. 9 Nichols, L.R.B. “Has the Threshold for Epiph- ysiodesis Versus Lengthening Changed in the Era of Magnetically Controlled Nails?” Journal of Pediatric Orthopedics 41, suppl 1 (July 1, 2021): S24-S32. doi:10.1097/BPO.0000000000001801. 10 Nasto, L.A.; Coppa, V.; Riganti, S.; et al. “Clini- cal results and complication rates of lower limb lengthening in paediatric patients using the PRECICE 2 intramedullary magnetic nail: a mul- ticentre study.” Journal of Pediatric Orthope- dics 29, no. 6 (Nov. 2020): 611-617. doi:10.1097/ BPB.0000000000000651. 11 Laubscher, M.; Mitchell, C.; Timms, A.; et al. “Outcomes following femoral lengthening: An initial comparison of the Precice intramedullary lengthening nail and the LRS external fixator monorail system.” The Bone & Joint Journal 98- B, no. 10 (Oct. 2016): 1382-1388. doi:10.1302/0301- 620X.98B10.36643. 12 Elmherig, A.; Ahmed, A.F.; Hegazy, A.; et al. “Amputation Versus Limb Reconstruction for Fibula Hemimelia: A Meta-analysis.” Journal of Pediatric Orthopedics 40, no. 8 (Sep. 2020): 425- 430. doi:10.1097/bpo.0000000000001510 Scott J. Schoenleber, MD, is an assistant professor of orthopedic surgery at the University of Arkansas for Medical Sciences and practices atArkansas Chil- dren’s Hospital andArkansas Children’s Northwest. Schoenleber attended medical school at the Mayo Clinic in Rochester, Minnesota. He then completed a residency in orthopedic surgery at the University of Miami/Jackson Memorial Hospital in Miami fol- lowed by a fellowship in pediatric orthopedic sur- gery at Nemours/AI duPont Hospital for Children in Wilmington,Delaware.His primary interests include limb lengthening and deformity correction,the use of multidisciplinary clinics to improve patient care,and counseling for families withmusculoskeletal abnor- malities detected on prenatal testing. especially strong association of obesi- ty and adolescent Blount’s disease in the pediatric population. Without orthope- dic and metabolic treatment, these chil- dren are at risk of developing metabolic syndrome and lifelong problems. Recent literature has pointed towards possible associations of Blount’s disease with hy- pertension and obstructive sleep apnea, 7 which are often likely underappreciated and can impact surgical risk stratification. In part because of the perils of pediatric obesity, Blount’s disease has recently been suggested as an indication in itself for metabolic and bariatric surgery. 8 Surgical options: evolving techniques and indications Our techniques for managing lower ex- tremity deformities follow similar princi- ples regardless of age or etiology. Surgery seeks to improve function, relieve pain, and should ultimately improve quali- ty of life. Historically, limb lengthening has been reserved only for patients with substantial discrepancies (greater than 5 centimeters). However, there is evidence that this threshold is changing in the era of internal lengthening nails. 9 Internal lengthening nails allow for one to perform an osteotomy, place a telescoping rod into the bone, and use a mechanism to gradu- ally distract the two ends of the bone. This all-internal technique reduces the reliance on bulky external fixator systems, miti- gates infection risks, and improves patient tolerance and cosmesis. 10, 11 However, ex- ternal fixators certainly still have a major role in management, especially for very young patients or those with multiplanar deformities. However, even with better surgical techniques, some young children with sig-