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1.
J Hand Surg Eur Vol ; 49(3): 375-376, 2024 Mar.
Article in English | MEDLINE | ID: mdl-37882659

ABSTRACT

We herein report on the application of a novel motorized prosthetic hand in a child with upper extremity phocomelia.


Subject(s)
Artificial Limbs , Ectromelia , Upper Extremity Deformities, Congenital , Child , Humans , Ectromelia/surgery , Upper Extremity/surgery , Upper Extremity Deformities, Congenital/surgery , Hand , Prosthesis Design
2.
J Orthop Surg Res ; 18(1): 844, 2023 Nov 08.
Article in English | MEDLINE | ID: mdl-37936235

ABSTRACT

BACKGROUND: Fibular Hemimelia (FH) is the most common longitudinal limb deficiency. Significant limb length discrepancy (LLD) will necessitate long treatment times and multiple settings to compensate for LLD when associated with femoral shortening. This study evaluates the outcome of simultaneous femoral and tibial lengthening using the Ilizarov frame. METHODS: This retrospective study included the cases of 12 children with severe limb length discrepancy caused by combined FH and ipsilateral femoral shortening from May 2015 to August 2022. The total LLD ranged from 7 to 14.5 cm. All patients underwent single-session femoral and tibial lengthening using the Ilizarov ring external fixator technique. Additional procedures were performed in the same setting, including Achilles tendon lengthening, fibular anlage excision, peroneal tendons lengthening, and iliotibial band release. Follow-up ranged from 2 to 4 years. RESULTS: The planned limb lengthening was achieved in ten cases (83%). No cases of joint subluxation or dislocation were encountered. No neurovascular injury has occurred during the treatment course. In all cases, the bone healing index was better on the femoral side than on the tibia. Poor regeneration and deformity of the tibia occurred in two cases (16.6%). CONCLUSION: Simultaneous femoral and tibial lengthening using the Ilizarov fixator is a relatively safe procedure with the result of correction of total LLD in one session in a shorter time and less morbidity.


Subject(s)
Bone Lengthening , Ectromelia , Ilizarov Technique , Child , Humans , Tibia/diagnostic imaging , Tibia/surgery , Ectromelia/diagnostic imaging , Ectromelia/surgery , Ectromelia/complications , Retrospective Studies , Fibula/surgery , Bone Lengthening/methods , Leg Length Inequality/surgery , Leg Length Inequality/etiology , Leg , Treatment Outcome
3.
J Pediatr Orthop ; 43(7): e574-e582, 2023 Aug 01.
Article in English | MEDLINE | ID: mdl-37254033

ABSTRACT

BACKGROUND: Fibular hemimelia (FH) represents the most common deficiency of the long bones and is associated with multiple deformities. Reconstructive treatment with external fixators in FH restores normal lower extremity alignment and length with plantigrade feet for a balanced and effective gait. The aim of this study is to evaluate the outcomes of lower limb lengthening and simultaneous tri-plane deformity correction with a computer-assisted hexagonal external fixator in children with FH. METHODS: A retrospective review was performed for FH cases treated with a computer-assisted hexagonal external fixator in a tertiary referral center. Leg length discrepancy (LLD), interphyseal angles, tibiocalcaneal distances, healing index (HI), and callus shapes were analyzed for radiologic evaluation, and the Pediatric Quality of Life Inventory (PedsQL) was used for functional assessment. Limbs with HI <50 days/cm, PedsQL >75, and without regenerate fractures were considered successful lengthenings. RESULTS: Twenty-four limbs of 23 patients were included. The limbs were lengthened for a mean of 7.24 cm (range, 4.7 to 15.6). The initial LLD of 5.6 cm (range, 0.5 to 19 cm) increased to 1.7 cm (range, 0.1 to 6 cm), and the mean interphyseal angle was 12.7 degree (range, 1.5 to 54.2 degree), tibiocalcaneal distance was 0.85 cm (range, 0.1 to 1.7) at final follow-up. The most common regenerated bone morphology was cylindrical, as seen in 11 limbs (45.8%). The average PedsQL score was 83.5 (range, 69.5 to 96.7). Sixteen limbs (66.7%) had successful lengthening at their first, and 4 limbs (80%) had successful lengthening at their second surgeries. Seven limbs had complications requiring surgical intervention (29.1%), with 3 (12.5%) regenerate fractures after external fixators removal. CONCLUSIONS: Limb reconstruction with computer-assisted hexapod fixators is a successful and reliable option for the treatment of LLD in FH, and patients demonstrate good functional outcomes. Surgeons should be aware of potential complications and should utilize prophylactic measures when necessary. LEVELS OF EVIDENCE: Level III, retrospective comparative study.


Subject(s)
Bone Lengthening , Ectromelia , Fractures, Bone , Child , Humans , Ectromelia/diagnostic imaging , Ectromelia/surgery , Ectromelia/complications , Retrospective Studies , Quality of Life , Bone Lengthening/adverse effects , External Fixators/adverse effects , Leg Length Inequality/etiology , Lower Extremity , Fractures, Bone/etiology , Computers , Treatment Outcome , Tibia/abnormalities
4.
Prosthet Orthot Int ; 47(1): 107-111, 2023 Feb 01.
Article in English | MEDLINE | ID: mdl-36791384

ABSTRACT

The likelihood of patellar instability and consequently, risk of patellar dislocations is higher in those with anatomical abnormalities. Fibular hemimelia is a congenital disorder resulting in partial or full absence of the fibula, often with absence of the lateral and cruciate ligaments, although this patient group rarely undergoes ligament reconstruction. There is potential for adverse outcomes, in the longer term, including, possible increased risk of patellar dislocation and pain in the knee and hip. We aim to investigate the potential risk of spontaneous, unprovoked patellar dislocation among patients with fibular hemimelia, through a review of medical records and radiological investigations. All patients with a diagnosis of fibular hemimelia were included (n = 25), regardless of ultimate approach to management. Tibiofemoral angle measurement and Caton-Deschamps indices were calculated where suitable radiology was available, to better establish extent of potential patellar instability. All the patients with normal Caton-Deschamps indices had only partial fibular absence, although this does not detract from absence or hypoplasia of the anterior cruciate ligament, as a risk factor for patellar dislocation by predisposing to anterior tibial translation. Notably, of the three patients with increased Caton-Deschamps indices, two had complete fibular absence and underwent definitive amputation surgery at age 18 months and 3 years, respectively. Ultimately, this was a young patient group and on-going follow-up might yield better understanding of knee stability. Maintaining a well-aligned lower limb throughout growth might be protective even in the presence of anatomical abnormalities. This article mainly aims to raise awareness among prosthetic and orthotic professionals regarding the increased risk of patella dislocations.


Subject(s)
Ectromelia , Joint Instability , Patellar Dislocation , Patellofemoral Joint , Humans , Infant , Anterior Cruciate Ligament , Ectromelia/diagnostic imaging , Ectromelia/surgery , Fibula/diagnostic imaging , Fibula/surgery , Joint Instability/diagnostic imaging , Joint Instability/surgery , Patella/surgery , Patellar Dislocation/diagnostic imaging , Patellar Dislocation/surgery , Patellofemoral Joint/surgery , Tibia/surgery
5.
Spine Deform ; 11(1): 243-246, 2023 01.
Article in English | MEDLINE | ID: mdl-35918628

ABSTRACT

PURPOSE: The purpose of this study is to present a case report of a patient with bilateral upper extremity phocomelia with progressive scoliosis, who underwent vertebral body tethering (VBT). METHODS: This is a case report on the use of VBT in a patient with scoliosis and bilateral congenital phocomelia, with 5 year follow-up. RESULTS: A male patient with bilateral phocomelia had early onset scoliosis that progressed to 45° at age 10. Surgical options were discussed, including traditional VBT, posterior spinal fusion, growing rods, magnetically controlled growing rods, and vertical expandible prosthetic titanium ribs. These options would limit the flexibility of the spine. Given these pitfalls, VBT was chosen, as it would address the scoliosis while maintaining trunk flexibility. Preoperatively, he had 45° right main thoracic curve, bending to 22°; he was Risser 0 with open triradiate cartilage. He underwent T6-T11 thoracoscopic VBT, with postoperative correction to 37°. Postoperatively, the patient was able to continue to use his lower extremities for writing, feeding, and personal grooming. He had no postoperative complications. At 3 years, his curve was 21°, and at 5 years was 19°. CONCLUSION: This case describes a novel technique for treating scoliosis in patients with bilateral phocomelia. Other forms of scoliosis surgical treatment limit motion of the spine. Due to this, we present VBT as an option for this unique set of patients for correcting scoliosis, while also preserving trunk flexibility for its role in feeding and self-care.


Subject(s)
Ectromelia , Scoliosis , Humans , Male , Child , Scoliosis/complications , Scoliosis/diagnostic imaging , Scoliosis/surgery , Thoracic Vertebrae/surgery , Vertebral Body , Ectromelia/complications , Ectromelia/diagnostic imaging , Ectromelia/surgery , Treatment Outcome
6.
Plast Reconstr Surg ; 150(6): 1321-1331, 2022 12 01.
Article in English | MEDLINE | ID: mdl-36126198

ABSTRACT

BACKGROUND: Sirenomelia is a rare syndrome in which the infant is born with the legs fused from the pelvis to the feet. Sirenomelia is often fatal in the neonatal period because of multiple other anomalies. The feet may be absent; if present, they are often splayed outward or face backward. There are no case reports of any patient with this syndrome who has been able to walk after separation of the legs. METHODS: The authors report on their patient with sirenomelia who was born with the feet facing backward but otherwise normal-appearing hips and thighs and no other anomalies that would lead to fatality in the near future. After preoperative tissue expansion, the authors performed separation of the legs with through-knee amputations, utilizing a vascularized flap from the lower part of the legs based on the sciatic vessels for coverage of the perineum. There was no need for skin grafts or dermal matrices and the patient was referred to physical therapy after recovery from surgery in an attempt to allow her to ambulate. RESULTS: The patient began to ambulate on her stumps early after surgical repair and is now walking with stubby prostheses. Her other medical issues have remained stable and nonproblematic. CONCLUSIONS: Selected patients with sirenomelia may be able to walk after separation of the legs, depending on the status of other congenital differences as well as the status of the legs when separated. Careful workup with multidisciplinary planning of overall care as well as surgical care is essential.


Subject(s)
Abnormalities, Multiple , Ectromelia , Female , Humans , Infant, Newborn , Abnormalities, Multiple/surgery , Ectromelia/diagnosis , Ectromelia/surgery , Hip , Pelvis , Syndrome
7.
Rev. cuba. ortop. traumatol ; 36(3)sept. 2022. ilus
Article in Spanish | LILACS, CUMED | ID: biblio-1441773

ABSTRACT

Introducción: La hemimelia tibial o hemimelia paraxial longitudinal tibial, es una deficiencia congénita de la tibia. Esta deficiencia de los miembros inferiores longitudinal tibial, es muy rara y su frecuencia está en el orden de 1: 1 000 000 de niños nacidos vivos. Objetivo: Presentar un caso de hemimelia tibial diagnosticado por medio del cuadro clínico y radiografías y tratado quirúrgicamente. Presentación del caso: Paciente masculino de dos horas de nacido, atendido por presentar malformación congénita a nivel de la pierna derecha que se presentaba acortada con una prominencia dura a nivel proximal y el pie con deformidad marcada en supinación, aducción y rotación interna. Se realizó examen físico exhaustivo de la extremidad afecta y se constató el acortamiento evidente de la misma. Se indicó radiografía anteroposterior y lateral de la pierna y se observó que el segmento proximal de la tibia y el peroné estaban bien, pero con implantación alta, por lo que se diagnosticó una hemimelia tibial tipo II de Jones. Luego del alta el niño recibió seguimiento por consulta de Genética y el servicio de Ortopedia donde se decidió someterlo a un primer tiempo quirúrgico a los 6 meses de edad, mediante tibialización del peroné. A los 10 meses se realizó un segundo tiempo quirúrgico para centrar el astrágalo al peroné. Conclusiones: La hemimelia tibial se considera un diagnóstico poco frecuente en nuestro medio. No existe prevención conocida. El tratamiento es complejo y altamente especializado, y en algunos casos requiere la amputación temprana del miembro afectado para adaptar al paciente al uso de prótesis(AU)


Introduction: Tibial hemimelia or tibial longitudinal paraxial hemimelia is a congenital deficiency of the tibia. This tibial longitudinal lower limb deficiency is very rare and its frequency is in the order of 1: 1,000,000 live births. Objective: To report a case of tibial hemimelia diagnosed through the clinical condition and radiographs and treated surgically. Case report: We report the case of a two-hour-old male patient, treated for a congenital malformation at the level of the right leg that was shortened with a hard prominence at the proximal level and the foot with marked deformity in supination, adduction and internal rotation. An exhaustive physical examination of the affected limb was carried out and its evident shortening was confirmed. Anteroposterior and lateral X-rays of the leg were indicated and it revealed that the proximal segment of the tibia and fibula were fine, but with high implantation, for which a Jones type II tibial hemimelia was diagnosed. After discharge, the child was followed up by the Genetics consultation and the Orthopedics service, where it was decided to undergo a first stage surgery at 6 months of age, by means of tibialization of the fibula. At 10 months, a second surgical time was performed to center the talus to the fibula. Conclusions: Tibial hemimelia is considered a rare diagnosis in our setting. There is no known prevention. Treatment is complex and highly specialized, and in some cases requires early amputation of the affected limb to adapt the patient to the use of a prosthesis(AU)


Subject(s)
Humans , Male , Infant, Newborn , Tibia/abnormalities , Foot Deformities, Congenital/genetics , Ectromelia/surgery , Ectromelia/diagnostic imaging , Leg/abnormalities , Natural Childbirth/methods
8.
Paediatr Anaesth ; 32(8): 967-969, 2022 08.
Article in English | MEDLINE | ID: mdl-35531655

ABSTRACT

A 10-month-old girl who had tetra-amelia syndrome and congenital maxillomandibular fusion (syngnathia) was scheduled for the surgical fusion separation. Anesthetic management for this case was considerably challenging. Standard monitoring was still applied to the patient's extremities. IV access was suspected to be difficult but firmly needed before intubation to provide resuscitation during an emergency. Connecting anesthetic circuit with nasopharyngeal airway was the preferred technique due to its benefits such as maintaining spontaneous ventilation, providing inhaled anesthetic, as well as monitoring oxygenation and ventilation. Importantly, the cornerstones for handling such complicated cases are multidisciplinary approach and teamwork.


Subject(s)
Anesthetics , Ectromelia , Jaw Abnormalities , Mouth Abnormalities , Ectromelia/complications , Ectromelia/surgery , Female , Humans , Infant , Jaw Abnormalities/complications , Jaw Abnormalities/surgery , Mouth Abnormalities/complications
9.
J Am Acad Orthop Surg ; 30(13): e899-e910, 2022 07 01.
Article in English | MEDLINE | ID: mdl-35486897

ABSTRACT

Proximal focal femoral deficiency is a congenital transverse deficiency in which the femur is globally smaller with a typical proximal deformity at the hip that may include distal involvement of knees, leg, and feet. Congenital femoral deficiency (CFD) describes a broader spectrum of longitudinal deficiency inclusive of proximal focal femoral deficiency. CFD may also include lateral distal femoral hypoplasia, knee cruciate ligament deficiency, rotational instability, patellar dislocation, fibular hemimelia, ray absence, and contralateral limb involvement. Treatment intends to maximize function by limb equalization and deformity correction ranging from nonsurgical management using prosthetics to amputation and may include lengthening, shortening, and complex limb reconstruction. Management decisions depend on overall severity and the patient and family's preferences and priorities. Owing to its complexity, CFD is best treated by clinicians with considerable deformity treatment experience who can help guide decision making and embark on a treatment course that will maximize the functional outcome.


Subject(s)
Ectromelia , Ectromelia/surgery , Femur/surgery , Fibula , Humans , Leg , Leg Length Inequality/surgery , Leg Length Inequality/therapy
10.
Disabil Rehabil ; 44(12): 2763-2773, 2022 06.
Article in English | MEDLINE | ID: mdl-33331793

ABSTRACT

PURPOSE: Longitudinal fibular deficiency (LFD) is the most common congenital long bone deficiency. This study aimed to objectively assess the physical performance of children and adolescents with LFD compared with unaffected peers, and to examine trends over age for subgroups of the LFD population. METHODS: Differences between children with LFD and unaffected peers were examined with hand-held dynamometry for lower-limb muscle strength, Six-Minute Walk Test, Timed up and down stairs test, Star Excursion Balance Test, and Standing long jump. RESULTS: Thirty-nine children with LFD and 284 unaffected peers participated. Children with LFD performed at a lower level than their unaffected peers, on all measures of physical performance (mean 2.1 z-scores lower, all p < 0.01), except in long jump (p = 0.27). When comparing the performance of children with LFD to their unaffected peers across four age groups, there was a significant between-groups difference on all strength measures, and on the Six-Minute Walk distance, between children with and without LFD. These differences were smallest in young children (3-6 years) and largest in the older children (15-18 years) (all p < 0.01). Children with no lengthening surgery performed better on the Six-Minute Walk Test, covering a greater distance during the test, than those who had surgery (mean difference 83 metres, p < 0.01). There were no significant differences between children who had or had not undergone an amputation. CONCLUSIONS: Children with LFD performed at a significantly lower level than unaffected peers on all measures of physical performance other than jumping. The largest differences were in older children. This paper provides baseline functional data for future interventions in LFD. LEVEL OF EVIDENCE: Cross-sectional study.Implications for RehabilitationThis paper provides the first baseline functional data using validated objective measures on a consecutive cohort of children and adolescents with longitudinal fibular deficiency.Children with LFD performed significantly worse than their unaffected peers on all measures of physical performance other than jumping, with children falling further behind their peers as they age.Children who undergo an amputation typically have the most severe anatomical presentation and yet perform at an equivalent functional level.This paper identifies multiple modifiable impairments that represent potential opportunities for rehabilitation professionals to target with conservative treatment options to improve functional performance.


Subject(s)
Ectromelia , Adolescent , Amputation, Surgical , Child , Child, Preschool , Cross-Sectional Studies , Ectromelia/surgery , Fibula/abnormalities , Fibula/surgery , Humans , Physical Functional Performance
11.
Anaesthesiol Intensive Ther ; 52(4): 352-353, 2020.
Article in English | MEDLINE | ID: mdl-33165889

ABSTRACT

Choosing the right anaesthetic technique and postoperative analgesia after major surgery can be a great challenge for paediatric anaesthetists, especially when younger children are concerned. The simultaneous use of systemic analgesics with adjuncts in combination with single-shot blocks performed at the right time may facilitate the patient's recovery and result in a comfortable postoperative period.


Subject(s)
Anesthesia, Epidural/methods , Nerve Block/methods , Pain, Postoperative/prevention & control , Ectromelia/surgery , Female , Humans , Infant , Pain Management/methods
12.
J Craniofac Surg ; 31(6): e574-e577, 2020 Sep.
Article in English | MEDLINE | ID: mdl-32569048

ABSTRACT

Roberts Syndrome is an extremely rare syndrome reporting about 150 cases in the literature, with a very low survival rate. The authors present a case of a female patient with Roberts Syndrome who also had a coronal craniosynostosis. The aim of this case report is to present a case of a patient with Roberts Syndrome with a brachycephaly that required management of fronto-orbital advancement. In conclusion Roberts Syndrome is a rare disease, which can have different skeletal variations. This syndrome can manifest itself with craniosynostosis, with the requirement of a comprehensive management to correct it and avoid compression of the brain with endocranial hypertension.


Subject(s)
Craniofacial Abnormalities/surgery , Ectromelia/surgery , Frontal Bone/surgery , Hypertelorism/surgery , Orbit/surgery , Craniosynostoses/surgery , Female , Humans , Infant , Plastic Surgery Procedures
13.
JBJS Case Connect ; 10(1): e0267, 2020.
Article in English | MEDLINE | ID: mdl-32224648

ABSTRACT

CASE: A 20-year-old man with a history of right lower extremity fibular hemimelia previously treated with PRECICE femoral nail lengthening presented with a broken magnetic nail and a displaced fracture through an ununited distraction osteogenesis site. Using a combination of techniques, we removed the broken implant while maintaining the achieved limb length and preserving the native biology without bone grafting. CONCLUSION: The unique challenges associated with the removal of a broken PRECICE femoral nail are described, with a technique for implant removal that preserves the achieved length, the innate biology of the distraction osteogenesis site, and promoting union without bone grafting.


Subject(s)
Device Removal/methods , Leg Length Inequality/surgery , Osteogenesis, Distraction/instrumentation , Bone Nails/adverse effects , Ectromelia/surgery , Humans , Male , Young Adult
14.
J Pediatr Orthop ; 40(8): 425-430, 2020 Sep.
Article in English | MEDLINE | ID: mdl-31972725

ABSTRACT

PURPOSE: Fibula hemimelia is the most common congenital deficiency of long bones. Primary treatment options include amputation with prosthetic fitting or limb reconstruction. The aim of our study was to conduct a systematic review comparing amputation with limb reconstruction for fibula hemimelia. METHODS: MEDLINE, EMBASE, Web of Science, Elsevier Scopus, and the Cochrane Registry of Clinical Trials were searched from 1951 to 2019 for studies that evaluated amputation versus limb reconstruction for fibula hemimelia. Random effect models were utilized for the meta-analytic comparisons of amputation versus limb reconstruction for patient satisfaction and surgical complications. Descriptive, quantitative, and qualitative data were extracted. RESULTS: Seven retrospective cohort studies were eligible for the meta-analysis, with a total of 169 fibula hemimelia cases. Amputation resulted in an odds ratio of 6.8 (95% confidence interval: 2.4, 19.2) when compared with limb reconstruction in terms of patient satisfaction. Furthermore, limb reconstruction was found to have an odds ratio of 28 (95% confidence interval: 7.8, 100.3) for complications. The total surgical complication rates in the amputation and limb reconstruction groups were 0.2 and 1.2 complications per limb. The rate of surgical procedures per patient was 1.5 and 4.2 for amputation and limb reconstruction, respectively. CONCLUSIONS: The cumulative evidence at present indicates better patient satisfaction with less surgical complications and less number of procedures with amputation for fibula hemimelia when compared with limb reconstruction. Absence of uniform protocols make it difficult to compare results accurately. LEVEL OF EVIDENCE: Level III-therapeutic.


Subject(s)
Amputation, Surgical , Ectromelia/surgery , Fibula , Patient Satisfaction , Plastic Surgery Procedures , Postoperative Complications , Amputation, Surgical/adverse effects , Amputation, Surgical/methods , Fibula/abnormalities , Fibula/surgery , Humans , Outcome Assessment, Health Care , Plastic Surgery Procedures/adverse effects , Plastic Surgery Procedures/methods
15.
Acta Orthop Belg ; 86(3): 383-390, 2020 Sep.
Article in English | MEDLINE | ID: mdl-33581021

ABSTRACT

The purpose of this survey in Belgium and the Netherlands was to assess treatment variation in glenohumeral osteoarthritis between experienced and less experienced orthopedic surgeons, and to investigate perioperative treatment after shoulder arthroplasty in a large group of orthopedic surgeons. Orthopedic surgeons specialized in shoulder surgery were invited to complete a survey between November 2013 and February 2015. Seventy-one percent of the approached surgeons com-pleted the survey. Less experienced surgeons (< 6 years) and surgeons from the Netherlands find patient characteristics (e.g. smoking p=0.01) more relevant than more experienced surgeons (≥ 6 years) and surgeons from Belgium. Less experienced surgeons will less likely (p=0.001) perform resurfacing arthroplasty compare to experienced surgeons. The less and the experienced surgeons use similar indications for a reverse shoulder arthroplasty regarding age limit and cuff arthropathy without osteoarthritis. Less experienced surgeon will more likely (p=0.003) prescribe a low molecular weight heparin during the hospital stay after a shoulder arthroplasty. In this survey, we found a decrease in the use of resurfacing arthroplasty and a strong increase in the use of reverse shoulder arthroplasty. Besides, there is little consensus concerning pre-operative planning, patient characteristics, surgical technique, and patient reported outcome measures. Level of evidence: IV.


Subject(s)
Ectromelia/diagnostic imaging , Ectromelia/surgery , Fibula/diagnostic imaging , Fibula/surgery , Imaging, Three-Dimensional , Plastic Surgery Procedures/methods , Surgery, Computer-Assisted/methods , Child , Female , Humans , Tomography, X-Ray Computed
16.
Ann Plast Surg ; 83(4): 436-438, 2019 10.
Article in English | MEDLINE | ID: mdl-31524738

ABSTRACT

Ulnar hemimelia is a rare congenital deformity. The severity of the deformity increases with the number of fingers that are absent. Clearly, grip function is impossible with a mono-finger hand. Here, we present a case report of a patient with bilateral ulnar hemimelia that included the absence of radial fingers and also deformity in the toes of his left foot. A toe-to-hand transfer was performed successfully to help the patient gain grip function. Both the patient and the parents were very pleased with the result. To our knowledge, this is the first case report of a patient with ulnar hemimelia and absent radial fingers who was treated with a toe-to-hand transfer.The most important factors in a successful surgery are careful preoperative planning and microsurgical expertise. Angiography is the key to careful planning, and physiotherapy should also be done to increase the patient's functions. The psychological well-being of patients and parents will be positively affected after a successful surgery.


Subject(s)
Abnormalities, Multiple/surgery , Ectromelia/surgery , Foot Deformities, Congenital/surgery , Hand Deformities, Congenital/surgery , Plastic Surgery Procedures/methods , Toes/transplantation , Abnormalities, Multiple/diagnostic imaging , Child , Ectromelia/diagnostic imaging , Follow-Up Studies , Foot Deformities, Congenital/diagnostic imaging , Hand Deformities, Congenital/diagnostic imaging , Hand Strength/physiology , Humans , Male , Microsurgery/methods , Microsurgery/rehabilitation , Plastic Surgery Procedures/rehabilitation , Toes/surgery , Treatment Outcome
17.
J Pediatr Orthop ; 39(9): e708-e717, 2019 Oct.
Article in English | MEDLINE | ID: mdl-31503232

ABSTRACT

BACKGROUND: Fibular hemimelia is the most common deficiency involving the long bones. Paley classification is based on the ankle joint morphology, identifies the basic pathology, and helps in planning the surgical management. Reconstruction surgery encompasses foot deformity correction and limb length equalization. The SUPERankle procedure is a combination of bone and soft tissue procedures that stabilizes the foot and addresses all deformities. METHODS: We retrospectively reviewed 29 consecutive patients (29 limb segments), surgically treated between December 2000 and December 2014. Among the 29 patients, 27 were treated with reconstructive procedures. Type 1 (8 patients) cases were treated with only limb lengthening, and correction of tibial deformities. Type 2 (7 patients) cases were treated by distal tibial medial hemiepiphysiodesis or supramalleolar varus osteotomy. In type 3 (10 patients) cases, the foot deformity was corrected using the SUPERankle procedure. Type 4 (2 patients) cases were treated with supramalleolar osteotomy along with posteromedial release and lateral column shortening. In a second stage, limb lengthening was performed, using the Ilizarov technique. In the remaining 2 patients (type 3A and type 3C), amputation was performed using Syme technique as a first choice of treatment. RESULTS: The results were evaluated using Association for the Study and Application of Methods of Ilizarov scoring. Excellent results were obtained in 15 of 27 (55%) patients. Six (22%) patients had good results, 4 (14.8%) had fair results, and 2 (7%) had poor results. Mean limb length discrepancy at initial presentation was 3.55 cm (range: 2 to 5.5 cm) which significantly improved to 1.01 cm (range: 0 to 3 cm) after treatment (P=0.015). CONCLUSIONS: Our results and a review of the literature clearly suggest that limb reconstruction according to Paley classification, is an excellent option in the management of fibular hemimelia. Our 2-staged procedure (SUPERankle procedure followed by limb lengthening) helps in reducing the complications of limb lengthening and incidence of ankle stiffness. Performing the first surgery at an earlier age (below 5 y) plays a significant role in preventing recurrent foot deformities. LEVEL OF EVIDENCE: Level IV.


Subject(s)
Ankle Joint/surgery , Ectromelia/classification , Ectromelia/surgery , Fibula/surgery , Leg Length Inequality/surgery , Plastic Surgery Procedures/methods , Amputation, Surgical , Bone Lengthening , Child , Child, Preschool , Ectromelia/complications , Female , Humans , Ilizarov Technique , Infant , Leg Length Inequality/etiology , Male , Osteotomy , Retrospective Studies , Tibia/surgery
18.
JBJS Case Connect ; 9(2): e0254, 2019.
Article in English | MEDLINE | ID: mdl-31211748

ABSTRACT

CASE: Gollop-Wolfgang Complex (GWC) includes congenital absence of the tibia with ipsilateral distal femur bifurcation associated with hand/foot ectrodactyly. A 20-month-old male presented with GWC, including left bifid distal femur with ipsilateral tibial hemimelia and absent extensor mechanism, hypoplastic bilateral thumbs, and right foot tarsal-type preaxial polydactyly. Left through-knee amputation preserving growth and contralateral polydactyly reconstruction were performed. Complications of wound infection and dehiscence were successfully treated. Patient is 44 months old and ambulating with left knee disarticulation prosthesis and right supramalleolar orthosis. CONCLUSIONS: Through-knee amputation and prosthetic fitting provides limb function for GWC patients with tibial hemimelia lacking an extensor mechanism.


Subject(s)
Abnormalities, Multiple/surgery , Disarticulation/methods , Ectromelia/surgery , Femur/abnormalities , Hand Deformities, Congenital/surgery , Lower Extremity/surgery , Tibia/abnormalities , Abnormalities, Multiple/diagnostic imaging , Black or African American , Aftercare , Ectromelia/diagnostic imaging , Femur/diagnostic imaging , Femur/surgery , Foot Orthoses/standards , Hand Deformities, Congenital/diagnostic imaging , Humans , Infant , Lower Extremity/diagnostic imaging , Male , Prosthesis Fitting/standards , Radiography , Tibia/diagnostic imaging , Tibia/surgery , Treatment Outcome
19.
Medicine (Baltimore) ; 98(3): e14031, 2019 Jan.
Article in English | MEDLINE | ID: mdl-30653110

ABSTRACT

RATIONALE: Tibial hemimelia is known as a rare congenital lower limb deficiency. It has been classified into different types based on Jones classification, and the traditional treatment of tibial hemimelia is amputation. Here we present a variant and unclassified case of tibial hemimelia, which was caused by osteomyelitis. And the lower limb with tibial hemimelia was salvaged by asymmetric limb lengthening. PATIENT CONCERNS: 19-year-old girl had the shortened and curved left lower extremity with walking abnormalities. DIAGNOSIS: The patient's deformity was caused by osteomyelitis of tibia occurred when she was 18 month old. The tibial shaft was absent, while the proximal and distal tibia was present but was hypoplastic with radiographic analysis. The fibula was hypertrophied and curved like the capital letter C. The leg length discrepancy (LLD), mostly coming from the left lower leg, was 22 cm. INTERVENTIONS: We were able to salvage the limb successfully by 5 operations, including releasing soft tissue, fusing the proximal tibiofibular joint, fibular osteotomy, femur lengthening, and fibular lengthening. OUTCOMES: The whole treatment time for the patient was 3 years and 2 months, and she was followed up for 5 years afterward. The length of femur lengthening and fibula lengthening during the reconstruction were 7.8 cm and 11 cm, respectively. Most of the deformities were corrected, except that the left lower limb was still 2 cm shorter than the contralateral limb, and the 34 mm of mechanical axis deviation (MAD) of left lower limb remained. The reason why the patient's lower limbs were asymmetric was that the femur and fibular lengthening were performed within the affected limb only. Overall, the patient was very satisfied with her asymmetric limbs and its function after surgeries. LESSONS: The LLD in this case mainly came from tibial hemimelia. However, the fibula was unable to be lengthened to 22 cm during the lower leg distraction process because of blood flow disturbance. We could only lengthen the femur to salvage the limb in this situation. Even though the patient still had a few residual deformities and a pair of asymmetric lower limbs, she was satisfied with the function and appearance of the reconstructed limb. Therefore, the lower limb with tibial hemimelia can be salvaged by asymmetric limb lengthening in special cases.


Subject(s)
Bone Lengthening/methods , Ectromelia/surgery , Leg Length Inequality/surgery , Osteomyelitis/complications , Tibia/abnormalities , Tibia/surgery , Ectromelia/etiology , Female , Humans , Leg Length Inequality/etiology , Young Adult
20.
J Pediatr Orthop B ; 28(2): 144-152, 2019 Mar.
Article in English | MEDLINE | ID: mdl-30234726

ABSTRACT

We describe a technique for optimal use of fibula in reconstruction of type II tibial hemimelia. Six affected children with mean age of 1.4 years and treated over a 5-year period were reviewed. All underwent staged reconstruction by lowering the fibula to below knee level using Ilizarov soft tissue distraction, transfer of distal fibula under proximal tibia, and foot centralization. Mean follow-up period was 3.6 years (range: 1.6-6.05 years). Mean age at follow-up was 4.4 years, and increase in length was 4.08 cm. Tibiofibular union and foot centralization were universally achieved. Mild residual equinovarus deformity was present in three children and braced. Our technique allowed significant length gain and foot centralization in toddlers without distraction osteogenesis.


Subject(s)
Ectromelia/surgery , Fibula/surgery , Plastic Surgery Procedures/methods , Tibia/abnormalities , Child , Child, Preschool , Ectromelia/diagnosis , Female , Follow-Up Studies , Humans , Infant , Male , Retrospective Studies , Tibia/surgery , Treatment Outcome
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