Comparing Outcomes of Acute Versus Gradual Surgical Correction in Brachymetatarsia.

Brachymetatarsia consists of a shortened metatarsal resulting in a shorter toe. Pain with shoe wear and cosmetic concerns are the main reasons for surgical intervention. Surgical techniques to increase metatarsal length include acute lengthening with interpositional bone grafting or gradual lengthening with callus distraction. We performed a retrospective cohort study for 1 surgeon's patients at 1 institution over 10 years. Twenty-nine feet in 22 patients met inclusion criteria for acute correction; 16 feet in 11 patients were included for gradual correction. Mean ages were 26.3 ± 12.1 and 27 ± 10.8 in the acute and gradual groups, respectively (p = .79). Most patients were female: 95.4% of acute cases and 90.1% of gradual cases. Most involved lengthening the fourth metatarsal: 86.7% and 100% of acute and gradual groups, respectively (p = .54). Correction obtained amounted to 14.4 ± 2.97 mm (range, 10-22 mm) in acute cases and 14.8 ± 2.39 mm (range, 10-20 mm) in gradual cases (p = .81). The mean percent increase in metatarsal length was 21.1 ± 14% for acute and 22.6 ± 12.4% for gradual (p = .72). Mean consolidation was 8.9 ± 2.51 weeks for acute and 21.4 ± 10.8 weeks for gradual (p = <.001). Nonunions were most common in the gradual group (37.5%) with need for more revisional surgery (43.5%) compared with the acute group; both were statistically significant. We conclude that acute brachymetatarsia correction can obtain correction similar to the gradual technique with fewer postoperative complications and less osseous consolidation time.

Symptomatic Bilateral Talonavicular Coalition in a Seven-Year-Old Boy.

Congenital talonavicular coalition is less frequent than the talocalcaneal or the calcaneonavicular and accounts for around 1% of all tarsal coalitions. Commonly, patients are asymptomatic and rarely need surgical treatment. Herein, we present a case of bilateral symptomatic talonavicular coalition in a seven-year-old boy who underwent surgery with full relief of symptoms. We also review the literature and discuss this pathological condition in detail.

Results of treatment of congenital vertical talus by the Dobbs method.

Congenital vertical talus is a rare foot deformity. The hindfoot is valgus and equinus, the midfoot is dorsiflexed and forefoot is abducted due to a fixed dorsal dislocation of the navicular on the head of the talus and the cuboid on the anterior part of the calcaneus. The epidemiology and etiology of vertical talus is unknown. Dobbs et al. (J Bone Joint Surg Am 88(6):1192-200, 2006) described a minimally invasive alternative which allowed to avoid the need for extensive soft tissue release procedures in treatment of congenital vertical talus. Eleven congenital vertical talus feet (group 5 according to Hamanishi) in eight children (four boys and four girls) constituted the study material. Upon the diagnosis, the patients' age ranged from 5 to 26 months old (the mean - 14.6). The treatment involved serial manipulation and casting according to the reverse Ponseti method (from 4 to 7 casts) followed by a minimally invasive approach consisting in temporary stabilization of the talonavicular joint with the use of K-wire and Achilles tenotomy according to the Dobbs technique. Then patients continued the shoe and bar program for 2 years. The X-ray measurements on lateral radiographic included the talocalcaneal angle, tibiotalar angle and talar axis-first metatarsal base angle whereas AP radiographic images-the talocalcaneal angle and talar axis-first metatarsal angle. The Wilcoxon test was used to compare dependent variables. The final clinical assessment made during the last follow-up (the mean: 35.8 months, the range: 25-52) revealed that neutral position of the foot and normal range of motion were observed in ten cases and recurrence of foot deformity in one case. The last X-ray examination showed normalization all of radiological parameters, except for one case, and examined parameters were statistically significant. The minimally invasive technique described by Dobbs should be the first option in treatment of congenital vertical talus. It allows to reduce the talonavicular joint, brings good results and preserves foot mobility. The attention should be put on early diagnosis.

Comparison of Different Surgical Techniques in Correction of Congenital Vertical Talus Deformity: A Systematic Review and Meta-Analysis of the Literature.

BACKGROUND: Congenital Vertical Talus (CVT) is a rare form of congenital rigid flatfoot. Numerous surgical techniques have been developed over the years in an attempt to definitively correct this deformity. We performed a systematic review and meta-analysis of the existing literature to compare the outcomes of children with CVT treated with different methods. METHODS: A detailed systematic search was conducted in accordance with PRISMA guidelines. Radiographic recurrence of the deformity, reoperation rate, ankle arc of motion, and clinical scoring was compared between the following 5 methods: Two-Stage Coleman-Stelling Technique, Direct Medial Approach, Single-Stage Dorsal (Seimon) Approach, Cincinnati Incision, and Dobbs Method. Meta-analyses of proportions were performed, and data were pooled through a random effects model using the DerSimonian and Laird approach. Heterogeneity was assessed using I^2 statistics. The authors used a modified version of the Adelaar scoring system to assess clinical outcomes. An alpha of 0.05 was used for all statistical analysis. RESULTS: Thirty-one studies (580 feet) met the inclusion criteria. The reported incidence of radiographic recurrence of talonavicular subluxation was 19.3%, with 7.8% requiring reoperation. Radiographic recurrence of the deformity was highest in the children treated with the direct medial approach (29.3%) and lowest in the Single-Stage Dorsal Approach cohort (11%) ( P <0.05). The reoperation rate was significantly lower in the Single-Stage Dorsal Approach cohort (2%) compared with all other methods ( P <0.05). There was no significant difference in the reoperation rates between the other methods. The highest clinical score was seen in the Dobbs Method cohort (8.36), followed by the group treated with the Single-Stage Dorsal Approach (7.81). The Dobbs Method resulted in the largest ankle arc of motion. CONCLUSION: We found the lowest radiographic recurrence and reoperation rates in the Single-Stage Dorsal Approach cohort, while the highest rate of radiographic recurrence was seen in those treated with the Direct Medial Approach. The Dobbs Method results in higher clinical scores and ankle arc of motion. Future long-term studies focusing on patient-reported outcomes are needed. LEVEL OF EVIDENCE: Level III.

The Prevalence of Bunions in the Setting of Brachymetatarsia.

Brachymetatarsia is caused by premature closure of the physis and is characterized by a short metatarsal. Additional foot conditions may exist in patients presenting with brachymetatarsia, such as hallux valgus (HV). A retrospective study was performed to evaluate the prevalence of HV and brachymetatarsia in the ipsilateral foot. Ninety-seven feet with congenital brachymetatarsia were reviewed in a multi-study cohort of 66 patients who underwent surgical correction between January 2005 and August 2020 at a single institution. The group was comprised of 61 females and 5 males, with a mean age of 27 years. HV deformities were verified with standardized anteroposterior radiographs. HV was present in 29 of 97 feet for a prevalence of 30% in the feet with brachymetatarsia. Our results demonstrate a 30% prevalence of HV associated with brachymetatarsia. This information is helpful for foot and ankle surgeons managing brachymetatarsia to determine appropriate conservative or surgical management of this condition.

Brachymetatarsia: A Classification for Surgical Treatment.

Brachymetatarsia is a congenital osseous and soft tissue deformity of a ray(s) of the foot. Because there is no particular consensus of methodology of lengthening for brachymetatarsia, the authors introduce a comprehensive anatomic classification and a surgical guide to treatment of each classification type. This classification combines the number of the metatarsal(s) affected and the letter(s) indicating the type of brachymetatarsia deformity (A = axial deficiency of the metatarsal, B = bowing of the metatarsal, C = congruency of metatarsal phalangeal joint). This study reviewed of 300 brachymetatarsals in 166 patients. Fifty of the 166 (30%) patients had bilateral brachymetatarsia. Of the 300 metatarsals with brachymetatarsia, 64 (21%) were first metatarsals, 22 (7%) were second metatarsals, 28 (9%) were third metatarsals, 12 (4%) were fifth metatarsals, and 174 (58%) were fourth metatarsals. Classification types that were found was a total of 165 (55%) type A, a total of 6 (2%) type B, a total of 72 (24%) type AB, a total of 39 (13%) type AC, and a total of 18 (6%) type ABC. A total of 16 (10%) male and 150 (90%) female patients were evaluated. The mean preoperative amount of shortening of the metatarsal was 15 mm (range, 4-20 mm), as determined by the preoperative metatarsal parabola deficiency, equating to 30% of the preoperative metatarsal length. Brachymetatarsia is a complex congenital deformity which until now has not been critically analyzed. This study outlines a comprehensive brachymetatarsia classification system which provides an accurate diagnosis of the deformity and offers a surgical treatment algorithm.

The Role of the Shortened Proximal Phalanx in the Setting of Brachymetatarsia.

Brachymetatarsia is a condition in which a metatarsal bone does not grow out to full length. This is caused by premature physeal closure. The proximal phalanx associated with the shortened metatarsal helps achieve the natural parabola of the foot. A hypoplastic proximal phalanx is a common finding in patients with brachymetatarsia. The goal of this study was to determine the length of the proximal phalanx in the setting of brachymetatarsia, and how much the shortening is attributed to the clinically smaller toe. We performed a retrospective study to evaluate the length of the proximal phalanx in the shortened ray. After the metatarsal was brought out to the desired length of correction, the proximal phalanx was measured on radiographs. Ninety-seven feet with congenital brachymetatarsia were reviewed in a cohort of 66 patients who underwent surgical correction between January 2005 and February 2020 at a single institution. The group was comprised of 61 females and 5 males, with a mean age of 27.5 years. The average length of the proximal phalanx associated with the affected metatarsal was noted to be 18.9 ± 3.83 mm for males and 15.6 ± 4.02 mm for females. Our results indicate the shortened proximal phalanx is 5 mm shorter when compared to normal population and is a contributing factor to the shortened clinical appearance of the digit in brachymetatarsia. Treating surgeons should be aware of this to better educate patients on the influence of the digit on the overall shortening seen in cases of brachymetatarsia.

Subacute two stage metatarsal lengthening with gradual distraction for brachymetatarsia: A consecutive case series and literature review.

BACKGROUND: This study examined the functional and clinical outcomes of subacute two stage metatarsal lengthening with gradual distraction for brachymetatarsia. This technique was developed to overcome the disadvantages of one-stage metatarsal lengthening and gradual distraction. METHODS: Four feet of three patients with congenital brachymetatarsia underwent subacute two stage metatarsal lengthening with gradual distraction. Pain, function, and alignment were assessed preoperatively and at follow-ups using the American Orthopaedic Foot and Ankle Society (AOFAS) lesser metatarsophalangeal-interphalangeal scale, and any complications were recorded. RESULTS: The patients were followed up for a mean of 18.1 ± 6.9 (range, 12.6-28.1) months. The mean metatarsal length gain was 15.2 ± 3.2 (range, 12.1-18.5) mm, and the corresponding percent increase was 32.5 % ± 7.0 % (range, 25.7-41.1 %). The mean AOFAS score (0-100) was 97.5 ± 5.0 at the final follow-up. The external fixator index was 10.2 ± 1.5 (range, 8.1-11.6) days/cm. None of the patients experienced metatarsophalangeal stiffness, subluxation or dislocation of the metatarsophalangeal joint, loss of correction, pin tract infection, delayed union, nonunion, or angular deformities. CONCLUSION: Subacute two stage metatarsal lengthening with gradual distraction is a reliable alternative treatment for brachymetatarsia.

Braquimetatarsia: propuesta de tratamiento en base a técnicas de alargamiento: a propósito de dos casos / Brachymetatarsia: treatment proposal based on lengthening techniques: about two cases / Braquimetatarsia: proposta de tratamento baseada em técnicas de alongamento: sobre dois casos

Braquimetatarsia es el acortamiento anormal de uno o más metatarsianos. Tiene una incidencia de 0.02%-0.05% predominando en mujeres de 25 a 1. En un 72% puede ser bilateral. La etiología no es clara pero se plantea el cierre prematuro de la fisis dando un retardo en el crecimiento. La mayoría de las veces presenta síntomas causados por alteración en la mecánica de la parábola metatarsal como dolor e hiperqueratosis. Puede existir metatarsalgia y agregar en la evolución desviaciones digitales adicionales. El principal motivo de consulta es estético. El objetivo del reporte es mostrar dos técnicas quirúrgicas y los resultados clínicos correspondientes usando el score AOFAS, además de los resultados imágenológicos con hasta 5 años de seguimiento. Se describen dos pacientes y se analizan dos técnicas quirúrgicas diferentes para alargar los metatarsianos. Se trata de alargamientos agudos con interposición de injerto intercalar, en un caso mediante el procedimiento de Sandro Gianini con injerto de cresta ilíaca y en el otro con injerto de metatarsiano adyacente con modificación de la longitud del segundo y tercer metatarsiano restituyendo en ambos casos la parábola metatarsal. Obtuvimos buenos resultados al igual que series numerosas pudiendo recomendarse en casos similares.

Brachymetarsal is the abnormal shortening of one or more metatarsals. It has an incidence of 0.02%-0.05%, predominantly in women from 25 to 1. In 72% it can be bilateral. The etiology is not clear, but premature closure of the physis is suggested, giving growth retardation. Most of the time it presents symptoms caused by an alteration in the mechanics of the metatarsal parabola, such as pain and hyperkeratosis. There may be metatarsalgia and add additional digital deviations in the evolution. The main reason for consultation is aesthetic. The objective of the work is to show the surgical technique and the clinical results using the AOFAS score, in addition to the imaging results with up to 5 years of follow-up. Two patients are described and two different surgical techniques to lengthen the metatarsals are analyzed. These are acute lengthenings with intercalary graft interposition, in one case using the S.Gianini procedure with an iliac crest graft and in the other with an adjacent metatarsal graft with modification of the length of the second and third metatarsals, restoring in both cases the metatarsal parabola. We obtained good results. results as well as numerous series and can be recommended in similar cases.

Braquimetatarsal é o encurtamento anormal de um ou mais metatarsos. Tem incidência de 0,02%-0,05%, predominantemente em mulheres de 25 a 1 ano. Em 72% pode ser bilateral. A etiologia não é clara, mas sugere-se o fechamento prematuro da fise, causando retardo de crescimento. Na maioria das vezes apresenta sintomas decorrentes de uma alteração na mecânica da parábola metatarsal, como dor e hiperqueratose. Pode haver metatarsalgia e adicionar desvios digitais adicionais na evolução. O principal motivo da consulta é a estética. O objetivo do trabalho é mostrar a técnica cirúrgica e os resultados clínicos utilizando o escore AOFAS, além dos resultados de imagem com até 5 anos de seguimento. Materiais e métodos: São incluídos dois pacientes e analisadas duas técnicas cirúrgicas diferentes para alongar os metatarsos. São alongamentos agudos com interposição de enxerto intercalar, em um caso utilizando a técnica de S. Gianini com enxerto de crista ilíaca e no outro com enxerto de metatarso adjacente com modificação do comprimento do segundo e terceiro metatarsos, restaurando em ambos os casos o metatarso parábola. Obtivemos bons resultados, bem como inúmeras séries, podendo ser recomendados em casos semelhantes.

Longitudinal Epiphyseal Bracket of the First Metatarsal: A Case Series on Treatment of Deformities Associated.

Longitudinal epiphyseal bracket of the first metatarsal, also known as first enclosed metatarsal, is a rare congenital disorder characterized by an abnormal development in the length of the first metatarsal ray because of the asymmetric presence of a longitudinal epiphyseal bracket. This causes interruption in the lengthways development of the affected bone, which becomes squat and short, with a trapezoidal or triangular shape, leading to a hallux varus deformity. First enclosed metatarsal occurs in 2% to 14% of all congenital defects in the hands and feet; with bilateralism in 75% of cases and a greater incidence in male patients. The deformity is classified as a differentiation defect; it is frequently associated with abnormalities such as syndactyly or polydactyly. There are different surgical treatments reported in the literature. Most of them are aimed at the excision of the epiphyseal bracket before complete skeletal maturity and frequently in the first year of life to promote a normal lengthways growth of the bone. In this study, the authors present three cases of bilateral first enclosed metatarsal in which the surgical treatment, aimed at lengthening the first metatarsal ray by using the Penning Minifixator, was instead carried out at the end of growth. This different surgical approach allowed the planning of a surgical operation involving both the skeletal structures and the surrounding soft tissue.

Callus distraction for brachymetatarsia - A comparison between an internal device and the external fixator.

BACKGROUND: Symptomatic patients with severe brachymetatarsia are commonly treated with callus distraction using external mini-fixator (EF) or internal device (ID). This study points out advantages and limitations of both methods comparing clinical and radiographical parameters. METHODS: Retrospective analysis of 21 metatarsal bones in twelve patients. Twelve metatarsals were treated with ID (Genos Mini), nine with EF (MiniRail). RESULTS: Mean lengthening distance was 17.3 mm using EF and 11.7 mm using ID (p = 0.016). Adverse results were observed in 89% of metatarsals treated with EF and in 33% treated with ID (p = 0.011). Postoperative surgical intervention was required in 33% using EF compared to 0% using ID (p = 0.031). Mean total German Foot Function Index (FFI-T) improved from 49 to 33 using EF and from 47 to 22 using ID (p < 0.001). CONCLUSION: CD is a reliable surgical treatment for BMT. Surgeons should be aware of implant-related advantages and complications when counseling patients about treatment options.

8 out of 10 patients do well after surgery for tarsal coalitions: A systematic review on 1284 coalitions.

BACKGROUND: The primary aim was to determine the clinical success rate after treatment for talocalcaneal (TCC) and calcaneonavicular coalitions (CNC). The secondary aim was to evaluate the complication, recurrence and revision rate. METHODS: A search was carried out in MEDLINE, EMBASE and Cochrane Library. Methodological quality was assessed using the Methodological Index for Non-Randomised Studies (MINORS) criteria. The primary outcome was the clinical success rate and was pooled per type of coalition and treatment modality. 95% Confidence Intervals (CI) of the success rates were calculated. Secondary outcomes included complication rates, coalition recurrence rates, revision rates and pain improvement using the Visual Analogue Scale (VAS). A sub-analysis on interposition material was performed. RESULTS: 43 articles comprising of 1284 coalitions were included, with a pooled mean follow-up of 51 months. Methodological quality was fair. The overall pooled success rate for TCCs was 79% (95% CI, 75%-83%). Conservative treatment, open resection and arthroscopic resection of TCCs resulted in success rates of 58% (95% CI, 42%-73%), 80% (95% CI, 76%-84%) and 86% (95% CI, 71%-94%), respectively. CNCs have an overall success rate of 81% (95% CI, 75%-85%), with 100% (95% CI, 34%-100%), 80% (95% CI, 74%-85%) and 100% (95% CI, 65%-100%) for conservative treatment, open resection and arthroscopic resection, respectively. Pooled complication rates of 4% (95% CI, 3%-7%) for TCCs and 6% (95% CI, 4%-11%) for CNCs were found. The success rates of resection with and without interposition material for TCCs were 83% (95% CI, 78%-87%) and 79% (95% CI, 65%-88%), and for CNCs 81% (95% CI, 76%-86%) and 69% (95% CI, 44%-85%), respectively. CONCLUSION: Treatment of tarsal coalitions can be considered good to excellent as well as safe, with an overall clinical success rate of 79% for TCCs and 81% for CNCs. Arthroscopic resection of the coalition appears to be non-inferior to open resection of TCCs and CNCs. LEVEL OF EVIDENCE: Level IV, Systematic Review.

Plantar approach for treatment of brachymetatarsia: A novel approach with an aesthetic consideration.

In the past, open osteotomy was always performed through a dorsal approach in the surgical treatment of brachymetatarsia, which created scar formation on the dorsal skin, subsequently resulting in dissatisfaction with cosmetic results. In this study, we provided a plantar approach to avoid forming scars on the dorsal side. A retrospective review was conducted in nine patients (13 feet) with brachymetatarsia treated with an open osteotomy and gradual bone lengthening through a plantar approach. Visual analogue scale (VAS) was used to evaluate the satisfaction of foot appearance, and we designed a questionnaire called appearance index (AI) to assess the influence of foot appearance on quality of life after surgery. The complications were also recorded during the follow-up. The patients were followed up for 34.8 ± 23.7 months. All cases were healed with a time of 64.4 ± 7.1 days and a healing index of 44.1 ± 7.8 d/cm. Satisfaction VAS for foot appearance improved from a preoperative score of 1.7 ± 1.3 points to a postoperative score of 9.3 ± 0.5 points. The AI improved from a preoperative score of 9.2 ± 0.8 points to a postoperative score of 0.6 ± 0.7 points. Complications were observed in three feet (23.1%), but none was related to the plantar approach. In conclusion, the plantar approach for metatarsal osteotomy and pins fixation was a safe and efficient technique with a satisfactory cosmetic result for the patients. No complications related to the novel approach, such as neurovascular injury, were reported.

Aetiology, diagnosis, and treatment of brachymetatarsia: a narrative review.

Brachymetatarsia (BM), or hypoplastic metatarsal, is an abnormal shortening of one or more metatarsal bones with a female-to-male ratio of 10.53:1. Different causes are described in the literature, such as congenital, acquired, or iatrogenic, associated with different conditions and syndromes. Its presence may develop deformity and pain; however, often feet are pain free and the major worries of patients are cosmetics. Non-operative treatments aim to improve the comfort of metatarsal heads and the possible dorsal conflict through comfortable shoes or the use of specific orthotics. The surgical treatment is anything but straightforward, with "one-stage" or "two stage" techniques, the latter better called "by gradual distraction". One-stage procedures are more rapid techniques but have limited ability to restore the desired length due to neurovascular compromise caused by acute lengthening. Insufficient correction is also possible. On the contrary, by gradual distraction procedures allow gradual distraction lengthening of more than 1.5 cm, but require the use of an external fixator, with a higher risk of complications in more than about 50% of surgeries. The adjacent metatarsal shortening should be considered in combination with other techniques, to diminish the excessive lengthening. In each case, surgeries should be always decided on each patient's concerns, deformities, and clinical needs.

Surgical management of an isolated symptomatic talonavicular coalition: a paediatric perspective.

Talonavicular (TN) coalition is a rare pathological union of the talus and navicular bones. We report the case of a 7-year-old girl with a symptomatic TN coalition, who underwent operative management with a lateral column lengthening procedure using autologous iliac crest bone grafting. There are no complications to report and the graft was incorporated at an early stage. At 3 year follow-up the patient has remained pain-free since the operation and maintained alignment. To our knowledge, this is the first reported case of TN coalition treated with reconstructive surgery in a paediatric patient.

Congenital Vertical Talus.

Congenital vertical talus represents a congenital structural foot deformity characterized by the classical rocker bottom deformity. The main feature is dislocation of the talonavicular joint along with contractures of the dorsolateral tendons of the foot and tendo Achilles. In the past treatment consisted of 2- or single-stage more or less extensive soft tissue releases including reduction of the talonavicular joint following casting in the first phase. Nowadays all feet are treated by serial casting, closed or miniopen talonavicular joint reduction, and percutaneous achillotenotomy. Functional results of the miniinvasive method are superior to those of the former more extensive surgical releases.

Reconstruction of Foot and Ankle Defects Using Free Lateral Arm Flap: A Retrospective Review of Its Versatile Application.

BACKGROUND: Successful reconstruction of the feet and ankles remains challenging due to limited quantities of soft tissue and laxity. The free lateral arm flap (LAF) is an alternative to conventional flaps and has been widely used due to advancements in its flap characteristics. This study is aimed at utilizing the advantages of this flap to validate its increased applications for foot and ankle defects. METHODS: Twenty patients with various LAF types between May 2011 and May 2020 were enrolled. Clinical data was retrospectively collected, and defect sites were classified according to the subunit principle. We utilized various LAF types, such as LAFs with sensate, extended, osteomyocutaneous, or myocutaneous flaps, as necessary. A two-point discrimination test was performed, and results were statistically compared between flaps. RESULTS: Among the diverse etiologies of skin defects, chronic inflammation was the most common cause of defects. Various LAF types, including LAFs with fasciocutaneous, extended fasciocutaneous, musculocutaneous, and osteomyocutaneous flaps, were used. The versatility of free LAF helped successfully cover various defects in all cases. Results of the two-point discrimination test were statistically significant between groups. CONCLUSIONS: Free LAF is a unique soft tissue free flap that is more versatile than other flaps, allowing flaps to be continuously modified and applied to various foot and ankle defects under different clinical conditions.

Nonincisional Osteotomy for Gradual Lengthening by Callus Distraction for Congenital Brachymetatarsia.

Gradual lengthening by distraction osteogenesis is widely used for congenital brachymetatarsia. The usual presenting complaint is the patient's cosmetic appearance. Osteotomy is an integral element. A nonincisional surgical approach for osteotomy can reduce dorsal longitudinal scarring and help preserve the periosteal blood supply to the bone during surgical dissection. Between June 2003 and January 2019, we performed gradual lengthening by callus distraction with nonincisional osteotomy for congenital brachymetatarsia on 13 digits in 5 patients. All 5 patients were female, and their average age was 18 years old. The lengthened bones involved 3 first metatarsals, 2 third metatarsal, and 8 fourth metatarsal bones. The mean gain in length was 15.2 mm (10-21 mm). The mean duration of distraction was 36.2 days (30-48 days). The mean duration of consolidation was 62.8 days (28-103 days). The lengthened segment consolidated in all cases. One patient had early consolidation and did not wish to undergo further surgery. There were no cases of trouble due to drilling for osteotomy. All patients had a normal gait and were satisfied with the cosmetic results.

Anaesthesia and orphan disease: management of a case of Nicolaides-Baraitser syndrome undergoing cleft palate surgery.

BACKGROUND: Nicolaides-Baraitser syndrome (NCBRS) is a rare disease caused by mutations in the SMRCA2 gene, which affects chromatin remodelling and leads to a wide range of symptoms including microcephaly, distinct facial features, recurrent seizures, and severe mental retardation. Until now, less than 100 cases have been reported. CASE PRESENTATION: A 22-month old male infant with NCBRS underwent elective cleft palate surgery. The anaesthetists were challenged by the physiological condition of the patient: narrow face, very small mouth, mild tachypnea, slight sternal retractions, physical signs of partial monosomy 9p, and plagiocephalus, midface hypoplasia, V-shaped cleft palate, enhanced muscular hypotension, dysplastic kidneys (bilateral, estimated GFR: approx. 40 ml/m2), nocturnal oxygen demand, and combined apnea. In addition, little information was available about interaction of the NCBRS displayed by the patient and anaesthesia medications. CONCLUSIONS: The cleft palate was successfully closed using the bridge flap technique. Overall, we recommend to perform a trial video assisted laryngoscopy in the setting of spontaneous breathing with deep inhalative anaesthesia before administration of muscle relaxation to detect any airway difficulties while remaining spontaneoues breathing and protective reflexes.