Outcomes of Operative Treatment of Forearm Deformity in Children with Osteogenesis Imperfecta: 18 Cases
Round 1
Reviewer 1 Report
Comments and Suggestions for AuthorsThis paper reported the results of correctional osteotomy in 18 patients with OI.
- There was no conclusion in the abstract and the manuscipt. what is the conclusion?
- Please describe the surgical indication of forearm deformity in OI patients.
- There were no radiological and functional results. How did the authors conclude the better outcomes after surgeries? If there are no functional results, at least radiological improvement must be provided.
- Please change the title. Delete the "clinical" because there were no clinical outcomes.
- If the authors can not provide any objective outcomes, this paper is not origical paper, change to Case reports.
Author Response
Please see the attachment
Author Response File: 
Author Response.pdf
Reviewer 2 Report
Comments and Suggestions for AuthorsGeneral comments:
I)The abstract did not report exactly the findings.
II) Several statements were not supported by references.
III) The overall manuscript was mostly descriptivr with insufficient analysis of the findings.
IV) The discussion was mixed with the findings. Comparison with the literature, either confirmation of the findings or differences were not discussed.
V) As pointed by the authors in the discussion: “This study has several significant limitations. First, this is a retrospective series of forearm deformities in all patientswith OI who have been treated at our institution and therefore has all the limitations of a retrospective study. Second, we do not have consistent functional data, as we have only been collecting regular functional data from our patients on a consistent basis for the past 12-18 months. We are therefore unable to reliably report on function before and after surgery. Anecdotally all patients and their families have been queried in follow up if they are glad that they have had their forearms fixed and they all universally are glad that they did with the most common reasons given being improvement in function, especially with wheelchair mobility and decreased number of forearm fractures.”
Minor comments:
1) Abstract, Lines 10-12, p1: Specify IRB in “A retrospective IRB approved study of patients with OI who underwent forearm osteotomy and fixation of one or bothforearm bones between 12/2011 and 08/2018 was performed.”
2) Abstract, lines 14-16: There was insufficient data to support “All patients who were old enough to report stated thatthey were satisfied with their decision to have the surgery and that they perceive that their fracture rate was decreased, and their function was improved. ”
3) Abstract, lines 14-16: There was insufficient data to support “No patient expressed that they had lost function. There were multiple complications,the most common being wire migration which required either replacement or advancement of the wire. Despite this children with OI were pleased with their outcomes.”
4) Introduction, lines 23-24, p1: Add references to support “Osteogenesis imperfecta (OI) is a heterogeneous genetic disorder most commonly causing type I collagen abnormalities.”
5) Introduction, lines 24-26, p1: Add references to support “Abnormal type I collagen results in frequent fractures often from minimal trauma, bowing deformities of long bones, short stature and scoliosis.”
6) Introduction, lines 26-29, p1: Add references to support “Disease severity varies significantly with some patients experiencing few fractures and little extra-skeletal manifestations (typically Type I) to patient with severe OI who present with frequent fractures, bowing of long bones, short stature and often scoliosis (Types III and IV).”
7) Introduction, lines 33-36, p1: Rephrase “Upper extremity deformity correction has been gaining traction in recent years with several studies showing humeral deformity correction and rodding led to improved function and decreased fracture burden.[2-4]”
8) Materials and Methods, Table 1: line 51, p2: Enlarge columns so that text could be placed correctly in one line.
9) Results, Unilateral forearm surgery patients, lines 87-90, p4: Indicate average age and its range to support “Nine patients (6 females and 3 males) had surgery performed on one forearm, with a total of 13 surgeries. The OI typedistribution is shown in Table 2.”
10) Results, Bilateral forearm surgery patients, Line 108, p5: Indicate average age and its age range in “Nine patients (4 females and 5 males) had surgery on both forearms, for a total of 35 surgeries.”
11) Results, Single versus multiple forearm surgery, line 119, p5: Indicate average age and its age range in“Fifteen forearms in eleven patients required only one surgery, see table 3 for OI types ”
12) Discussion,lines 148-152, p6: Separate findings and analysis. Merge into the Result section“The most common cause for revision surgery was prominent wires that required revision or shortening. We have addressed this by developing special tamps to bury the tip of the wires. Since adopting this burying technique we have observed fewer migrated wires post-surgery. Also, as k-wires cannot elongate with patients as they grow, 5 patients required procedures for revision of a wire that no longer protected the entire length of the bone or revision for bowing at the tip of a wire.”
13) Discussion, lines 159-162, p7: Merge into the Result section “One of our questions in reviewing our cases was if procedures that involved fixation of the ulna only increased the odds of requiring repeat surgical intervention to the radius. Among our 8 patients with forearms whose index procedure onlytreated the ulna, only 3 have developed deformity of the radius that required intervention. Two additional patients required a repeat trip to the operating room to correct the ulna again but still did not require surgical intervention of the radius.”
14) Discussion, lines 170-180, p7: indicate if it was confirmed by the present work or if there was any difference to support “Prior to 1980 there were only 5 case reports of operative treatment of forearm deformities in OI patients. An orthopaedic review article on OI published in 1971 discussed operative treatment in patients with OI and had one forearm procedure listed.[7] One of the case series on OI bony deformity surgical treatment was published in 1973 by Tiley and Albright. They reviewed all of their patients during a 12 year period. They identified four patients who had forearm deformity correction. They found that these patients had the most severe disease in their group of patients. These patients had severely limited forearm rotation and tended to hold their hands in a semi-pronated position. They found that the proximal ulna angulated posterolaterally and the radius tended to angulate medially and the distal forearm tended to bow in the opposite compensatory direction to the proximal bow. They report decent deformity correction at the time of surgery butdidn't feel that it helped to improve the patients forearm rotation and that ultimately as the children grew they had recurrent deformity or hardware complications.[8] These procedures were all performed prior to modern medical treatment of OI”
15) Discussion, lines 181-189, p7: Indicate if it was confirmed by the present work or if there was any difference to support “One of the first studies to discuss operative interventions for children with OI and upper extremity deformities was published in 1980 by Root [9]. He reported that the indication for operative deformity correction of the upper extremity was when the upper extremity deformities interfered with upper limb function; including crutch use, inability to reach particular parts of the body or deformities associated with recurrent fractures. He reported on a total of 24 procedures in the upper extremity; 12 humerus corrections, 5 radius correction and 7 ulna corrections, with a total of 6 patients withforearm deformity corrections. In the paper he discussed that rodding of the radius and ulna is a very technically challenging procedure due to the often poor bone quality and the fact that the bones are usually severely deformed and are short. The authors concluded that these procedures are so extremely difficult that they felt that the operation is tobe rarely indicated.”
16) Discussion, lines 191-196: , p7: Indicate if it was confirmed by the present work or if there was any difference to support “The majority of their patients were type III similar to our series. The indications were forearm deformity resulting in functional limitations or recurrent fractures. They found that after forearmsurgery the patient's self-care and mobility scores increased significantly. They utilized K-wires for stabilization of boththe radius and ulna in 16 cases, radius only in 5 cases and ulna only in 1 case. They also found several complications, most commonly prominent wires which required revision. They also had 2 cases of non-union which required repeat operation.[6] ”
17) Discussion, lines 197-202: , p8: Indicate if it was confirmed by the present work or if there was any difference to support “Franzone et al [11] published their series of upper extremity surgeries in 2017 which included 19 forearms. The average age of their humeri and forearm surgeries was 8.7 years with most patients having type III OI. The most commonindication for surgery was progressive deformity with recurrent fractures resulting in functional difficulties. They utilized fixation in the radius and ulna in 10 patients, radius only in 3 patients and ulna only in 6 patients. Implants included K- wires and flexible nails. This series had 7 forearm revisions with 4 patients requiring treatment for rod prominence or migration. Three patients required revision surgery due to re-fracture with rod bending.”
18) Discussions, lines 205-207,, p8: Add reference to support “Many patients with type III OI cannot take an implant larger than a 0.062" k-wire, and therefore the concerns of wire bending and subsequent re-bending of the forearm are present. ”
19) Discussions, Figure 3 and Figure 4, lines 208-214, p8: Move into the result section “We have attempted to decrease the frequency ofwire migration by bending the tip of the ulnar wire into a shepherd's crook and/or keeping the wire straight and fullytamping it into the cartilage of the olecranon. (Figure 3) For the radial wire we have had a special tamp fashioned (Figure 4) that fits the end of the 0.062" k-wire and confirm both radiographically and under direct vision that the wire is completely buried in the cartilage of the radial styloid. Our current cohort of patients is not large enough and hasn't been followed long enough to determine if these changes have decreased our wire prominence problems, longer term follow up will help to determine this.”
20) Institutional Review Board Statement, lines 241-243, p9: Add more information to support “Institutional Review Board Statement: The study was conducted according to the guidelines of the Declaration of Helsinki, and approved by the Institutional Review Board (or Ethics Committee) of The University of Nebraska Medical Center (protocol code # 243028-16-EP, date of approval).”
Author Response
Please see the attachment.
Author Response File: Author Response.pdf
Round 2
Reviewer 1 Report
Comments and Suggestions for AuthorsThis paper is not suitable for original paper.
This paper can be accepted for case report
So, the title should be changed followings: Outcomes of Operative Treatment of Forearm Deformity in Children with Osteogenesis Imperfecta : 18 cases
Author Response
The title was changed to your recommendation and to a case report.
Thank you for your feedback.
Reviewer 2 Report
Comments and Suggestions for AuthorsOverall comments:
Several concerns were adequately addressed, while one major and one minor concerns were insufficiently addressed:
Major comments:
V) As pointed by the authors in the discussion: “This study has several significant limitations. First, this is a retrospective series of forearm deformities in all patients with OI who have been treated at our institution and therefore has all the limitations of a retrospective study. Second, we do not have consistent functional data, as we have only been collecting regular functional data from our patients on a consistent basis for the past 12-18 months. We are therefore unable to reliably report on function before and after surgery. Anecdotally all patients and their families have been queried in follow up if they are glad that they have had their forearms fixed and they all universally are glad that they did with the most common reasons given being improvement in function, especially with wheelchair mobility and decreased number of forearm fractures.”
Author's answers:
Noted
Reviewer's answer: It was not addressed
Minor comments:
1) Abstract, Lines 10-12, p1: Specify IRB in “A retrospective IRB approved study of patients with OI who underwent forearm osteotomy and fixation of one or bothforearm bones between 12/2011 and 08/2018 was performed.”
Changed to:
A retrospective study, approved by The Unviersity of Nebraska Medical Center IRB was conducted with OI patients who underwent forearm osteotomy and fixation of one or both forearm bones between 12/2011 and 08/2018.
Reviewer’s answer:
Specify abbreviation IRB
Author Response
The other reviewer recommended changing the title and format of the paper to a case review. The title was changed to: Outcomes of Operative Treatment of Forearm Deformity in Children with Osteogenesis Imperfecta : 18 cases. Please consider this as well.
Major comments: Based on the data that was available and the retrospective nature of this review the conclusion was changed to:
In conclusion, forearm deformity correction and treatment of fractures with intramedullary wire fixation is a potential option in children with OI who have recurrent fracture or deformity of the forearm. Both fractures and osteotomies have the potential of healing well. Complications are common, with the majority of these related to backing out of the smooth implants or due to fracture or deformity at the end of the wires associated with growth.
Minor: IRB was changed to Institutional Review Board in the abstract.
Round 3
Reviewer 1 Report
Comments and Suggestions for AuthorsPlease accept this paper as a case report.
Reviewer 2 Report
Comments and Suggestions for AuthorsMy main comment was insufficiently addressed.
