Vol 6, No 3 (2018)

Original papers
Restoration of elbow active flexion via latissimus dorsii transfer in patients with arthrogryposis
Agranovich O.E., Kochenova E.A., Trofimova S.I., Petrova E.V., Buklaev D.S.

Abstract

Background. Severe hypoplasia (or aplasia) of the biceps brachii is a primary cause of restriction in activities of daily living in patients with arthrogryposis.

Aim. To estimate the possibility of restoring elbow active flexion via a latissimus dorsii transfer in patients with arthrogryposis.

Materials and methods. From 2011 to 2018, we restored active flexion of the elbow via a latissimus dorsi transfer to the biceps brachii in 30 patients with arthrogryposis (44 upper limbs). We used different regimes including clinical examinations, EMG donor and recipient sites, and CT of the chest wall and shoulder.

Results. The mean age of the patients was 4.0 ± 2.4 years, and the follow-up period was 3.2 ± 1.9 months. Follow-up results were available for 26 patients (30 upper limbs). The active postoperative elbow motion was 90.5 ± 14.9°. Elbow extension limitation occurred in 51% of cases (12.8 ± 4.3°) without any problems in activities of daily living. In total, 55.6% of patients had good results, 33.3% had satisfactory results, and 11.1% had poor results.

Discussion. Our latissimus dorsi transfer results were comparable with those of other authors. Transposition of the latissimus dorsi to the biceps brachii restores sufficient flexion of the elbow without severe elbow flexion contractures.

Conclusions. We suggest pedicle monopolar latissimus dorsi transfer as a reliable therapeutic option to restore active elbow flexion in patients with arthrogryposis having passive elbow flexion of 90° or higher before operation and donor muscle strain grade 4 or higher.

Pediatric Traumatology, Orthopaedics and Reconstructive Surgery. 2018;6(3):5-11
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Treatment of congenital long-bone deformities in children using the consequtive application of guided growth and external fixation: preliminary report
Vilensky V.A., Zakharjan E.A., Pozdeev A.A., Zubairov T.F., Pozdeev A.P.

Abstract

Aim. This study aimed to estimate the results of congenital long bone deformities using the consecutive application of guided growth and external fixation.

Materials and methods. We performed a retrospective analysis of the treatment results of 38 children with congenital deformities of long bones. Group 1 consisted of 17 children who underwent consecutive application of two methods: guided growth and external fixation. Group 2 (control group) consisted of 21 children who underwent isolated lengthening and deformity correction by external fixation.

Results. There were 14 complications in group 1 and 25 complications in group 2. Moreover, only seven cases in group 1 had complications requiring surgical treatment, whereas 17 cases in group 2 required operative treatment for complications. There was a relatively low level of refractures: zero cases in group 1 and three cases in group 2. The most common complication was a recurrence of deformity associated with the continuous growth of children: seven cases in group 1 and eight cases in group 2. However, no recurrence of the torsion component of deformity was observed in any group 1 cases, and repeated guided growth could be performed in the six cases of growing children.

Conclusion. The consecutive use of external fixation and guided growth to treat congenital deformities of the lower limbs is a promising direction for pediatric orthopedics because it reduces the incidence of complications. The repeated use of guided growth, because of its minimal invasiveness, is the most effective solution for the recurrence of deformity in a growing child.

Pediatric Traumatology, Orthopaedics and Reconstructive Surgery. 2018;6(3):12-24
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Fractures of lower limbs in children with spina bifida
Ivanov S.V., Kenis V.M., Prokopenko T.N., Fedoseyeva A.S., Ugurchieva M.A.

Abstract

Background. Spina bifida is a serious defect in the development of the spine and spinal cord. It is accompanied by several orthopedic disorders of the spine and lower limbs, including fractures of long tubular bones. In spina bifida, osteoporosis plays an important role in the pathogenesis of motor disorders.

Aim. The objective was to determine the patterns of occurrence and the clinical and radiological features of fractures of the long tubular bones of the lower limbs in children with sequelae of spina bifida.

Materials and methods. From 2006 to 2017, 544 patients with spina bifida were examined and treated at the Turner Research Institute for Children’s Orthopedics. Clinical-neurological and radiographic methods were used. The neurosegmental level of spinal cord involvement was determined using the Sharrard classification, and the motor level was assessed according to the method proposed by Melbourne Medical University.

Results. The clinical picture of a fracture of a long tubular bone in a child with spina bifida has many characteristics. There was no abnormal mobility in the fracture site in 56% of cases, edema was absent in 88% of children, and pain in the fracture region was observed in only 19% of cases. The radiographic features of the atypical fracture of long tubular bones in children with sequelae of spina bifida included lack of a fracture line, presence of a hypertrophic periosteal reaction, and sclerosis areas at the fracture site.

Conclusion. The frequency and localization of fractures of the lower limbs in children with sequelae of spina bifida are determined according to the neurosegmental level. The clinical picture of fracture often differs from usual fractures by the absence of pain syndrome, edema in the fracture region, and displacement of bone fragments, which must be considered for diagnosis. The peculiarities of the clinical and radiological picture are associated with the presence of osteoporosis in this pathology due to a decrease in the motor activity level of the patients.

Pediatric Traumatology, Orthopaedics and Reconstructive Surgery. 2018;6(3):25-31
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Microsurgical toe-to-hand transfer in children with macrodactyly of the hand
Golyana S.I., Tikhonenko T.I., Govorov A.V., Zaytseva N.V.

Abstract

Background. At present, there are multiple approaches to the treatment of macrodactylia in children. Additionally, there are no comparative data on the cosmetic and functional aspects of corrective surgery versus microsurgical transplantations performed in children with a marked disproportion of the segment, making this study particularly relevant.

Aim. The aim of this study was to determine the role of microsurgical transplantation of the toes in the treatment of children with isolated macrodactylia, based on a comparative analysis of various surgical interventions.

Materials and methods. Twenty-five children with congenital macrodactylia of the hand were examined and surgically treated in the department of reconstructive microsurgery from 2013 to 2017. Of these, 13 (52%) had macrodactyly of the 2nd and/or 3rd fingers, and of the total number of hyperplastic hand segments (n = 45), 17 rays had hyperplasia greater than 25%.

Results and discussion. Stage-by-stage modeling resections of enlarged segments were performed in all children with ray hyperplasia that was about 25% of the size of intact fingers. In 4 cases, excision of soft tissues was combined with marginal resection of phalanges. When hyperplasia of the segment reached up to 10% of macrodactylia, single isolated modeling resection of soft tissues and bones was performed. Repeated modeling plasties in 16% (n = 4) of the children were accompanied by gross postoperative scars with the development of secondary angular deformities and loss of function of interphalangeal joints.

Nine children (n = 14) underwent amputation of hyperplastic fingers followed by microsurgical reconstruction of rays by autografting of toes. In 4 cases, the first finger was reconstructed, and in the other 5 cases, toes of both feet were transplanted to the positions 2 and 3 (n = 4) or 3 and 4 of fingers (n = 1). It was found that in cases of significant hyperplasia of the affected segments of the hand (hyperplastic segment is 1.5–2 times greater compared to normal ones), microsurgical autografting of toes is more effective and acceptable compared to bone and soft tissue resections.

Conclusions. In cases of segment hyperplasia less than 1.25-times the normal size, the optimal surgical interventions are stage-by-stage modeling resections. An alternative surgical treatment for children with hyperplasia of more than 1.25-times the normal size is microsurgical transplantation of the toes in the position of the involved rays of the hand, which results in good cosmetic and functional outcomes in these patients.

Pediatric Traumatology, Orthopaedics and Reconstructive Surgery. 2018;6(3):32-39
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Molecular genetic analysis of genes for detoxification and DNA repair in children with congenital deformities of the thoracic and lumbar spine
Sogoyan M.V., Khalchitsky S.E., Vissarionov S.V., Kokushin D.N., Filippova A.N.

Abstract

Introduction. Spine congenital curvatures, which form from anomalies in the development of vertebral bodies, comprise 3.2% of the general structure of vertebral column deformities. Several such anomalies present during adolescence lead to severe and rigid curvature of the spinal column and are often accompanied by irreversible neurological disorders. The timely detection of the progressive forms of curvature and early surgical treatment are measures that prevent against neurological deficit development and gross congenital deformities of the spine in children. However, it is extremely difficult to predict the course of congenital spinal column deformation in infants based on clinical and radiological investigations alone. Therefore, the study of congenital malformation genetic markers is an essential and immediate task.

Materials and methods. Two hundred 1.2–16-year-old children with congenital deformities of the thoracic and lumbar spine were examined using clinical and radiation diagnostic methods. Molecular genetic studies were performed by analyzing several polymorphic regions in the genes for the first and second stages of detoxification and DNA repair, which are of clinical importance as predisposing factors in several congenital malformations. Polymorphisms were determined using the polymerase chain reaction (PCR) method. The results were determined using gel electrophoresis of DNA in a polyacrylamide gel.

Results and discussion. The polymorphisms of the genes CYP1A2, NAT2, GSTM1, GSTT1, GSTP1, XRCC1, XRCC3 and their frequency distributions among patients with congenital spine deformities (CSD) were studied. The results for each gene are presented in the digital diagrams, and their indicators are compared with the values of the control group.

Conclusion. In most patients (83%) with spinal congenital deformations, there were mutations of candidate genes in the homozygous state; however, the simultaneous carriage of several mutant alleles in patients with CSD was more than twice that in the control group. Children with multiple and combined defects in spine development noted the presence of more mutations in the genes for detoxification and DNA repair. The obtained results already assume to a certain extent the course of the spine congenital deformity in patients at an early age. However, the final evaluation and identification of molecular genetic criteria for the progressive course of spine congenital deformities in children requires further study.

Pediatric Traumatology, Orthopaedics and Reconstructive Surgery. 2018;6(3):40-46
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Comparative evaluation of desflurane and sevoflurane anesthesia during surgical correction of vertebral and spinal cord injury in children
Kozyrev A.S., Aleksandrovich Y.S., Zaletina A.V., Ivanov M.D., Pavlova M.S., Strelnikova A.S.

Abstract

Introduction. Currently, inhaled third-generation anesthetic agents, such as sevoflurane and desflurane, are commonly used in pediatric practice. Their properties and efficiencies are studied in detail. Information about the effectiveness and safety of these drugs as emergency anesthesia in children is very limited; there are no comparative studies.

The aim of this study was to conduct a comparative evaluation of desflurane and sevoflurane to maintain anesthesia during the surgical correction of vertebral and spinal cord injury in children.

Material and methods. This study included seventy-four 12–18-year-old patients (mean age, 14 years) who underwent immediate surgical correction of unstable fractures of the thoracolumbar and lumbar spine at the Turner Scientific Research Institute for Children’s Orthopedics between 2015 and 2017. The patients were categorized into two groups: group D, in which anesthesia was maintained with desflurane (35 patients), and group C, in which anesthesia was maintained with sevoflurane (39 patients).

The following parameters were studied: systolic, diastolic, and average blood pressure (BP); heart rate (HR); respiratory recovery time; time to extubation; time to instruction completion; and presence of complications intraoperatively and within 24 h after surgery, including pronounced intraoperative hypotension, bradypnea, and desaturation (SpO2 < 95%) in the postextubation period, agitation, nausea, vomiting, and measured blood loss.

Results. A comparative evaluation of the investigated parameters revealed that the systolic, diastolic, and average BP and HR in both groups did not exceed the limits of acceptable values. The results of the intraoperative monitoring of capillary blood parameters in all patients were within the reference range and did not differ significantly between groups. An analysis of the indicators reflecting the rate of awakening revealed that all stages of the termination of anesthesia were performed more quickly in group D. There was a comparable number of postoperative nausea and vomiting episodes in both groups. Group C displayed a high incidence of postoperative agitation. There were no related adverse respiratory effects in group D, whereas three patients reported such effects in group C.

Conclusions. The use of desflurane and sevoflurane provides a favorable hemodynamic profile intraoperatively and is not accompanied with the development of clinically significant side effects. Desflurane reduces the probability of certain adverse effects in the immediate postoperative period, provides a faster awakening, and has the possibility of reliable assessment of neurological status after surgery.

Pediatric Traumatology, Orthopaedics and Reconstructive Surgery. 2018;6(3):47-55
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The valuation of the residual and secondary deformation of the medium face zone in patients with unilateral cleft lip
Semyonov M.G., Mushkovskaya S.S., Kovalenko K.U.

Abstract

Backround. The treatment of patients with cleft lip and palate is a complicated medical-social problem. The residual and secondary deformations of the nose, upper lip, and dentoalveolar require surgical correction in these patients after undergoing complete reconstruction as a child.

Aim. To systematize the degree of severity of the residual secondary deformation of the medium face zone in adult patients after previous surgery.

Materials and methods. This scientific work was based on the results of 42 adult patients with unilateral cleft lip after undergoing complete reconstructive surgery as a child. The evaluation of residual and secondary deformations of the nose, upper lip, and vestibule was performed in adults aged 25–40 years. The typical residual deformation at these locations was determined and separated into three groups and given a value.

Results. We observed that 91% of the patients with unilateral cleft lip had a deformation in the medium face; of these patients, 15% had severe deformation with an absolute indication for surgery.

Conclusion. The residual and secondary deformations of the medium face zone were diagnosed in 91% (80.6%–96.7%) of the patients operated using the Miro-Limberg-Obuyhovoi method in the remote period. Moreover, 15% of these deformations had an absolute indication for surgery. The three numbers used in the evaluation system of the residual and secondary deformations of the medium face zone in patients with a unilateral cleft lip enable impartial and quick determination of the intensity of one or more deformations of the nose, lip, or vestibule of the mouth.

Pediatric Traumatology, Orthopaedics and Reconstructive Surgery. 2018;6(3):56-62
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Clinical cases
Bilateral coxa vara and tibia vara associated with severe short stature in a girl manifesting a constellation of bone lesions with exclusive involvement of the lower limbs
Al Kaissi A., Grill F., Ganger R., Kircher S.

Abstract

In most instances, a toddler is seen with unilateral varus of the tibia, usually the deformity appearing slightly more distal than the knee joint. Radiographs of the focal fibrocartilaginous dysplasia show a characteristic abrupt varus at the metaphyseal — diaphyseal junction of the tibia. Cortical sclerosis is in and around the area of the abrupt varus on the medial cortex. A radiolucency may appear just proximal to the area of cortical sclerosis. The aetiology of such defects and the pathogenesis of the deformity are mostly unknown. Many of the associated factors suggest that the condition at least partly results from a mechanical overload of the medioproximal tibial physis.

The evaluation of a child with suspected pathologic tibia vara begins with a thorough history. A complete birth and developmental history should include the age at which the child begun walking. The medical history should identify any renal disease, endocrinopathies, or known skeletal dysplasia. The physical examination also should include the child’s overall lower extremity alignment and symmetry, hip and knee motion, ligamentous hyperlaxity, and tibial torsion.

We describe on a 17 year-old-girl who manifests severe short stature associated with multiple orthopaedic abnormalities, namely, bilateral coxa vara and tibia vara. Radiographic documentation showed bilateral and symmetrical involvement of the lower limbs with the extensive form of fibrocartilaginous dysplasia, osteoporosis, and osteolytic lesions. The constellation of the malformation complex of osteolytic lesions, fibrocartilaginous changes and the polycystic like fibromas are not consistent to any previously published reports of fibrocartilaginous dysplasia. To the best of our knowledge, it seems that fibrocartilaginous changes are part of a novel type of skeletal dysplasia.

Pediatric Traumatology, Orthopaedics and Reconstructive Surgery. 2018;6(3):63-69
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Fibrous hip ankylosis in adolescents: non-standard approach to treatment (clinical observation)
Kozhevnikov O.V., Kralina S.E., Ivanov A.V.

Abstract

The development of secondary deforming coxarthrosis in childhood and adolescence, as a rule, is accompanied by the formation of a pronounced deformity of the hip joint, up to ankylosis, which significantly limits the function of the lower limb and leads to early disability. In most patients, hip ankylosis develops in a vicious position with the resulting flexion-intracavity installation of the lower limb. If such a condition is encountered in childhood and adolescence, treatment currently remains debatable. Various methods are used, ranging from arthroplastic organ-preserving interventions and corrective osteotomies to joint replacement. In this report, we present a clinical case of children treated with post-infectious secondary deforming coxarthrosis with fibrous ankylosis of the hip joint in a vicious position. We applied a coherent combination of modern treatment methods: distraction in the apparatus, arthroscopy, intra-articular injections, physiotherapy, and other rehabilitation. The treatment results were evaluated over a two-year period. In this case, the rational use of a consistent set of remedial measures helped to improve joint function, socialize the patient, and postpone surgery for joint replacement for at least 2 years.

Pediatric Traumatology, Orthopaedics and Reconstructive Surgery. 2018;6(3):70-77
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Review
Degenerative hip disorders in children
Rubashkin S.A., Sertakova A.V., Dohov M.M., Timaev M.C.

Abstract

A group of degenerative hip disorders in children is discussed in the current review. The key pathogenic focus of these disturbances is an initial hyaline cartilage alteration or subchondral bone, which provokes damage of the epiphyseal hip zone. Eventually, such events lead to a local inflammatory reaction in the hip joint, cytokine cascade with hypoxia and ischemia, and apoptosis and necrosis in the hip. Developmental hip dysplasia, Legg-Calvé-Perthes disease, and slipped capital femoral epiphysis are analyzed in this review as the spreading forms of degenerative hip disorders in children. The key points of etiology, pathogenesis, diagnostics, and treatment of each disease are characterized. A group of degenerative hip joint diseases remains under the close supervision of pediatric orthopedists and traumatologists because of their high prevalence, severity of clinical manifestations, damage of life quality, and development of complications in the form of arthritis. In addition, the lack of unified approaches to the application of treatment methods for degenerative hip joint diseases is the subject of discussion among surgeons and often causes a decrease in the quality of care in terms of time and volume.

Pediatric Traumatology, Orthopaedics and Reconstructive Surgery. 2018;6(3):78-86
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Discussion
Medical care during the first months of life for children with a facial nerve birth injury
Kriukova I.A., Ikoeva G.A., Saidasheva E.I., Baindurashvili A.G., Stepanova Y.V.

Abstract

Background. Among all cranial nerves, the facial nerve is the most exposed to birth injuries. Difficulties with medical assistance during the first months of life for children with a facial nerve birth injury is known and debated. According to the literature data, the scope of diagnostic and treatment activities varies, and these treatments have not always demonstrated effectiveness.

Aim. We discuss the protocol of medical assistance for newborns and children during the first months of life with a facial nerve birth trauma.

Materials and methods. We analyzed domestic and foreign literature dedicated to facial nerve birth trauma.

Results. The results showed the necessity of a multidisciplinary approach for patients with facial nerve birth trauma involving neurologists, ophthalmologists, otolaryngologists, audiologists, maxillofacial surgeons, geneticists, doctors for rehabilitation medicine, and microsurgeons. Key directions of medical assistance during the first months of life include the prevention of the development of ophthalmologic complications; topical and etiological differential diagnosis; and dynamic observation to timely resolve whether surgical treatment is necessary.

Conclusion. The integration of developed protocols in clinical practice is essential for understanding the etiology, pathogenesis, natural history, differential diagnostics, and prior treatment by medical doctors of different specialties to improve the quantity of medical assistance.

Pediatric Traumatology, Orthopaedics and Reconstructive Surgery. 2018;6(3):87-95
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