Congenital Forearm Pseudarthrosis, a Systematic Review for a Treatment Algorithm on a Rare Condition

Abstract

Background: A congenital forearm pseudarthrosis is a rare condition and is strongly associated with neurofibromatosis type 1. Several surgical techniques are described in the literature, but the most optimal treatment strategy remains unclear. This systematic review aims to develop a treatment algorithm that may aid in clinical decision making. Methods: The PROSPERO registration number for this study was CRD42018099602 and adheres to the PRISMA guidelines for systematic reviews. Embase, MEDLINE, Cochrane Central, Web of Science, and Google Scholar databases were searched for published studies reporting on congenital forearm pseudarthrosis not related to other underlying pathologies like bacterial infection or fibrous dysplasia. Results were not restricted by date or study type, only English literature was allowed. Studies were assessed for quality using the critical appraisal checklist for case reports from the Joanna Briggs Institute. Patient characteristics, underlying disease, type of surgery, union rate, and functional outcome were extracted from included studies. Results: Of 829 studies identified, 47 were included in this review (17 case series and 30 case reports, a total of 84 cases). A onebone forearm procedure showed highest union rates (92%), however, it results in loss of forearm rotation. Free vascularized fibula grafting showed high union rates (87%) and was related to good functional outcome of elbow flexion and forearm rotations. Other procedures showed disappointing outcomes. Conclusions: Congenital forearm pseudarthrosis is best treated with a free vascularized fibula grafting, a one-bone forearm procedure should be used as a salvage procedure. Evidence extracted from the case reports was sufficient to generate a treatment algorithm to be used in clinical pediatric practice. Level of Evidence: Level IV-therapeutic.

Does the PROSPERO registration prevent double review on the same topic?

“…However, we have recently found a similar review article on the same topic by Siebelt et al ,5 which was registered on PROPERO on July 2018 (before our review registration) and published in June 2019. We find it somewhat disappointing to “discover” the registration procedure of PROSPERO of our review did not indicate that another review on the same topic was underway while our project of review was approved.…”

A case of congenital pseudoarthrosis of forearm

“…We retained the intramedullary nail till last follow up, so as to provide additional internal support to the bone [10] . A review of literature, reveals no high level of evidence [7] . There are multiple case reports and some case series', reflecting the rare occurence.…”

“…The most common orthopedic manifestation is Pseudoarthrosis of tibia [5,10] . Occurrence of similar pathology in forearm is a rare occurence and as such treatment principles are not well defined [2,7] . Our case had no progressive union of radius with complete absence of distal ulna.…”

Subcutaneous Calcification and Fixed Flexion Deformity of the Right Elbow Joint in a Child with a GNAS Mutation: A Case Report

“…Congenital forearm pseudoarthrosis is also an extremely rare condition, most likely associated with neurofibromatosis type1 (NF1) or fibrous dysplasia (4). Moreover, Cafe au lait spots in NF1 would be present in about 69% of cases with pseudoarthrosis (5). Post-traumatic injuries can lead to joint dislocation or pseudoarthrosis ( 6), but neither the patient's history nor clinical and biochemical examinations were suggestive of the mentioned aetiologies for this patient's joint deformity.…”