Congenital Synostosis of the Knee: Long-term Outcomes of Limb Reconstruction Surgery.

BACKGROUND: Congenital synostosis of the knee is a rare condition with limited data on treatment options and outcomes. This study reports clinical findings, treatment approach, and surgical/clinical outcomes for congenital synostosis of the knee. METHODS: An institutional review board-approved retrospective review of patients with congenital synostosis of the knee presenting to 2 institutions between 1997 and 2021 was performed. RESULTS: Eight patients (13 knees) with a median follow-up of 11.3 years (3.3 to 17 y) were included. Seven patients had associated syndromes. Patients presented with an average knee flexion deformity of 100° (range 60 to 130°) and delayed walking ability. Seven patients had associated upper extremity hypoplasia/phocomelia. The average age at the index surgery was 4.3 years (range 1.2 to 9.2 y). Synostosis resection with gradual deformity correction was performed in most patients. An attempt was made at a mobile knee in some patients, but all went on to knee fusion. Mean flexion deformity at final follow-up was 11.6° (range: 0 to 40°) and 5 limbs were fused in full extension. Mean limb length discrepancy at final follow-up was 6.8 cm (range: 0 to 8 cm). All patients maintained their improved ambulation status at final follow-up. Twenty-two complications were identified. CONCLUSIONS: Reliable correction of the deformity associated with congenital knee synostosis was achieved at a median follow-up of 11 years. Importantly, all patients maintained their improved ambulation at final follow-up. This is the largest study on patients with congenital knee synostosis and outlines a reconstructive approach to improve ambulatory status. LEVEL OF EVIDENCE: Level IV.

Growth modulation in idiopathic angular knee deformities: is it predictable?

PURPOSE: To evaluate the temporal and spatial sequence of events following temporal hemiepiphysiodesis in idiopathic knee varus/valgus. METHODS: This is a retrospective multicentre study on 372 physes in 206 patients. The average rate of correction (ROC) was calculated; univariate and multivariate analysis were performed. RESULTS: In all, 92% of the femoral physes were followed for more than one year/reached skeletal maturity. Of those, 93% were corrected to a mechanical lateral distal femoral angle (mLDFA) of 85° to 89°; 2% did not, while 5% were over-corrected. A total of 92% of the tibial physes were followed for more than one year/reached skeletal maturity. Of those, 92% were corrected to a mechanical medial proximal tibial angle (mMPTA) of 85° to 89°; 2% did not, while 6% were over-corrected. Factors significantly influencing success and ROC were age, direction and magnitude of deformity. Femoral ROC was significantly faster than tibial ROC: 0.85° versus 0.78°/month, respectively (p = 0.05). Femoral valgus ROC was significantly faster than varus ROC: 0.90° versus 0.77°/month, respectively (p = 0.04). A constant was derived to calculate the amount of correction. Significant correlation was found between calculated and actual mLDFA in valgus deformity during the first year (r = 0.58 to 0.87, p < 0.01). Calculated mLDFA of varus deformity did not correlate with actual mLDFA. Significant correlation was found when calculating mMPTA correction in all deformities. CONCLUSIONS: Femur corrects faster than tibia; valgus femoral deformities are corrected faster than varus. Valgus correction in the distal femur/proximal tibia as well as varus correction in the tibia in idiopathic patients is highly predictable. The constant derived is the first tool which enables predicting and monitoring amount of correction in hemiepiphysiodesis when correcting angular deformities around the knee. LEVEL OF EVIDENCE: IV.

Guided growth: preliminary results of a multinational study of 967 physes in 537 patients.

BACKGROUND: Guided growth by tension band plating is commonly used to correct coronal plane deformity. The purpose of this study was to measure the effect and further define parameters that influence results in coronal plane deformity around the knee. METHODS: The retrospective multicentre study included data on 967 physes in 537 patients, with an average follow-up of 16 months after plate insertion. Alignment analysis was compared preoperatively and in at least two measurements postoperatively, as well as with parameters that influence the rate and amount of correction. RESULTS: Average age at plate implantation was 11.35 years (SD 3.29).Of those with femoral deformities, 85% of the patients finished the treatment and of those, 70% were corrected to standard alignment, while 14% have not yet achieved correction, and are still growing.Of those with tibial deformities, 75% of the patients finished the treatment and of those 80% were corrected to standard alignment, while 25% have not yet achieved correction and are still growing.The calculated rate of correction was 0.77°/month for the femur and 0.79°/month for the tibia.In terms of complications, the overall rate of infection was 1.48%. In three patients (0.55%) screw breakage was recorded.Factors found to significantly influence the amount of correction were age at plate implantation and direction of deformity. CONCLUSION: Temporary hemiepiphysiodesis takes the advantage of physiological physeal growth to effectively treat angular deformities. Success of treatment is influenced by the age of the patient at plate implantation and direction of deformity. LEVEL OF EVIDENCE: IV.

Prolonged use of foot abduction brace reduces the rate of surgery in Ponseti-treated idiopathic club feet.

PURPOSE: There is conflicting evidence related to factors affecting the rates of recurrence of idiopathic club feet using the Ponseti method. We attempt to evaluate the predictors of success and failure in our physiotherapy-led Ponseti club foot clinic. METHODS: We evaluated 189 children with 279 club feet with a mean follow-up of 6.3 years for the following: Pirani score at presentation, number of casts for correction, indication for Achilles tenotomy, and the duration of foot abduction brace (FAB) use, in relation to outcome. Outcome measures were the need for additional surgery and functional scores. Based on the pattern and rate of ossification of the tarsal bones in idiopathic club foot, a much longer FAB weaning protocol was designed and practiced since 2000. The objective of this study was to answer the question of whether a prolonged period of FAB use reduces the need for surgery in Ponseti-treated idiopathic club foot. RESULTS: Thirty-six feet (12.9 %) underwent additional surgery. The Pirani score and the number of cast changes had no influence on the rate of surgery. The duration of FAB use had a significant effect on the outcome, i.e., the rate of surgery and functional scoring. Operated children used the FAB for 28 months versus 33 months in the non-operated group (p < 0.05). Only a minor delay in the attainment of walking age was noted (average 15 months). CONCLUSIONS: The duration of FAB treatment was found to be the most influential on the functional results and on rate of surgery. Close follow-up and longer FAB weaning program reduced the rates of recurrence.

V osteotomy and Ilizarov technique for residual idiopathic or neurogenic clubfeet.

PURPOSE: To report the treatment outcomes of V osteotomy and Ilizarov technique for residual idiopathic or neurogenic clubfeet. METHODS: 13 patients (14 feet) aged 8 to 18 years underwent V osteotomy via the calcaneus and talus, followed by gradual distraction of soft tissue and bone for foot reconstruction. Eight of the clubfeet were idiopathic and had undergone previous surgeries. The remaining 6 were neurogenic and their pathologies were: Charcot-Marie-Tooth disease (n=2), myelomeningocele (n=2), neurofibromatosis (n=1), and distal arthrogryposis (n=1). Three of them had undergone previous surgeries. The Ilizarov frames were retained for 3 to 6 months and the patients were followed up for 1.8 to 8.9 years. Range of movement of the ankle and foot, appearance and position, gait, pain, function, and patient satisfaction were assessed according to the modified clubfoot grading system. The talo-1st metatarsal angle was measured on anteroposterior radiographs. RESULTS: Scores associated with the appearance and position of the foot, and thus patient satisfaction were significantly improved, but not for range of movement, pain, and function. The mean preoperative and final talo-1st metatarsal angles were 39.7 and 8.7 degrees, respectively (p<0.01). Ten feet achieved the plantigrade position, one had residual equinus, and 3 had residual adduction and supination. CONCLUSION: Patient satisfaction improved significantly despite no major improvement in pain, function, and range of movement of the ankle and foot. This reflects the importance of the appearance and position of the foot, and justifies the decision to undergo this long and demanding procedure.

A combined use of a free vascularised flap and an external fixator for reconstruction of lower extremity defects in children.

PURPOSE: To describe a combined use of a free vascularised flap and an external fixator for reconstruction of lower extremity defects in children, and correction of equinus contracture developed after removal of the external fixator using a circular dynamic frame. METHODS: Seven children (4 males) aged 4 to 12 (mean, 8) years were treated with 9 free vascularised flaps for 8 limbs (bilaterally in one patient and for a failed flap in another). Patient pathologies included: 3 soft tissue degloving injuries, one soft tissue and bone avulsion, one severe burn contracture, one resurfacing of soft tissue and bone necrosis, and one osteosarcoma resection defect. Free flap reconstruction was delayed in 6 patients (range, 3 weeks to 4 years). Static external fixators were used to stabilise the free vascularised flaps at the time of reconstruction, with the ankle in a neutral position. RESULTS: The mean follow-up was 5 (1-10) years. All flaps but one survived; the failed one was immediately reconstructed with a contralateral, latissimus dorsi flap. One anastomosis following a Kirschner-wire injury was successfully revised. Six patients had pin tract infections and were treated with oral antibiotics. Two patients developed equinus contracture 6 and 3 years later, after removal of the external fixator, and were corrected by distraction, using a dynamic Ilizarov frame. CONCLUSION: The combined use of a free flap and an external fixator for salvage of lower extremities is useful in children. Late development of equinus contracture can be safely corrected by distraction, without compromising flap viability.

Congenital absence (ankylosis) of the knee.

A congenital, unilateral, fixed flexion deformity in a neonate was diagnosed as a congenital absence of the knee. A single cartilage mass, with fusion of the lower femoral and upper tibial ossification centres, was demonstrated by imaging studies. This condition has been reported in the literature only once before. Surgery on our patient, which was performed at the age of two years, consisted of separation of the fused cartilaginous anlage and gradual correction of the deformity using an Ilizarov frame.

Effects of long-term administration of N-3 polyunsaturated fatty acids (PUFA) and selective estrogen receptor modulator (SERM) derivatives in ovariectomized (OVX) mice.

We studied the beneficial effects of dietary consumption of n-3 polyunsaturated fatty acids (PUFA) and two selective estrogen receptor modulator (SERM) derivatives (SERM-I and SERM-II) and their combined effect on serum lipids, skin dermis and adipose layers, bone marrow adipogenesis, and cytokine secretion in mice. Two different ovariectomized (OVX) models were studied: treatment began immediately post-OVX in one and 3 months post-OVX in the other. Our results showed that n-3 PUFA and both SERMs decreased triglyceride levels in the serum, and that SERMs also decreased serum cholesterol levels while n-3 PUFA had no similar effect. SERMs had no effect on IL-6, IL-1 beta, or IL-10 levels, but they decreased ex vivo tumor necrosis factor (TNF-alpha). N-3 PUFA decreased secretion of non-induced IL-6 and TNF-alpha from cultured BMC and IL-1 beta levels in vivo (i.e., in bone marrow plasma), but its main effect was a significant elevation in the secretion of IL-10, a known anti-inflammatory cytokine. OVX-induced B-lymphopoiesis was not affected by LY-139481 (SERM-I) while LY-353381 (SERM-II) exhibited an estrogen-antagonistic effect in sham and OVX mice and elevated the amount of B-cells in bone marrow. Fish oil consumption prevented the elevation in B-lymphopoiesis caused by OVX, but had no curative effect on established augmented B-lymphopoiesis. This activity could be mediated via the elevation of IL-10 which was shown to suppress B-lymphopoiesis. Both SERMs and n-3 PUFA inhibited the increase in adipose tissue thickness caused by OVX in mice. Our results showed that n-3 PUFA, could prevent some of the deleterious outcomes of estrogen deficiency that were not affected by SERMs. We observed no significant beneficial effects of the combined administration of SERM-I, SERM-II, and PUFA on the studied parameters.The exact mechanism by which polyunsaturated fatty acids exert their activities is still not clear, but peroxisome proliferator-activated receptors (PPARs) might be involved in processes which are modulated by n-3 PUFA.

Efficacy of prenatal ultrasonography in confirmed club foot.

Club foot can be diagnosed by ultrasound of the fetus in more than 60% of cases. We have correlated the accuracy of the prenatal findings in 281 ultrasound surveys with the physical findings after birth and the subsequent treatment in 147 children who were born with club foot. The earliest week of gestation in which the condition was diagnosed with a high degree of confidence was the 12th and the latest was the 32nd. Not all patients were diagnosed at an early stage. In 29% of fetuses the first ultrasound examination failed to detect the deformity which subsequently became obvious at a later examination. Club foot was diagnosed between 12 and 23 weeks of gestation in 86% of children and between 24 and 32 weeks of gestation in the remaining 14%. Therefore it can be considered to be an early event in gestation (45% identified by the 17th week), a late event (45% detected between 18th and 24th weeks) or a very late event (10% recognised between 25th and 32nd weeks). We cannot exclude, however, the possibility that the late-onset groups may have been diagnosed late because earlier scans were false-negative results. The prenatal ultrasonographic findings were correlated with the physical findings after birth and showed that bilateral involvement was more common than unilateral. There was no significant relationship between the prenatal diagnosis and the postnatal therapeutic approach (i.e., conservative or surgical), or the degree of rigidity of the affected foot.

Primary chronic sclerosing (Garré's) osteomyelitis in children.

Three children with unifocal nonpyogenic inflammatory bony lesions with a prolonged, fluctuating course are reported. The lesions were located at the metaphyseal region of long bones. Three was progressive sclerosis and hyperostosis in the tibia or femur, such as the changes described in Garré's osteomyelitis. No pus was released by exploration of the lesions. Tissue and blood cultures were negative. The histology was typical of chronic osteomyelitis: the symptoms returned intermittently over several years, together with the development of sclerosis but without disturbance of bone growth. It is not clear whether Garré's chronic sclerosing osteomyelitis is a different entity from chronic recurrent multifocal osteomyelitis.

Visual evoked cortical potential can be used to differentiate between uncorrected refractive error and macular disorders.

The visual evoked cortical potential (VECP) is widely used to verify complaints of reduced visual performance and to identify the site of the disorder. In this study, we investigated the correlation between reduced visual acuity and VECP in volunteers with normal corrected visual acuity and in patients suffering from inherited macular degeneration or from age related macular degeneration (ARMD). Flash evoked VECP was not affected by the visual acuity in the cases of refractive error and in ARMD patients but was reduced in amplitude and delayed in implicit time in the patients suffering from inherited macular degeneration. The VECP elicited by pattern reversal checkerboard (PVECP) was not affected by the quality of the visual image in volunteers with uncorrected refractive error when checks of 60' or larger were used but were considerably reduced in size and prolonged in implicit time for checks smaller than 15'. In both groups of patients suffering from macular dysfunction, pattern reversal VECP was very subnormal and was characterized by prolonged implicit time compared to values expected from their visual acuity. These findings indicate that the PVECP does not directly correlate with visual acuity but rather with foveal function. Therefore, we suggest that recordings of PVECP can be used to differentiate between refractive error and macular disorders as causing reduction in visual acuity when other clinical signs are missing or not available.

Nato3 is an evolutionarily conserved bHLH transcription factor expressed in the CNS of Drosophila and mouse.

The evolutionarily conserved basic helix-loop-helix (bHLH) transcription factors play important roles during development. Here we report the identification of Nato3 (nephew of atonal fer3) orthologs in Drosophila, C. elegans, mouse, and man, all of which share a high degree of similarity within the bHLH domain. Expression analysis revealed Nato3 transcripts in the central nervous system of both fly and mouse embryos. In the fly, Dnato3 is highly expressed in 9-15h embryos in a few ventral nerve cord cells and a subset of neurons in the brain. In mouse, the MNato3 transcripts were detected from embryonic day 7 until 5 weeks postnatally, with highest levels in the midbrain, thalamus, hypothalamus, pons, and medulla oblongata. In contrast to the brain, expression in the spinal cord was limited to the embryonic stages.

Bilateral symmetric stress fractures in a toddler.

Stress fractures are a common injury among adolescent athletes and military recruits. The increase in child participation in organized sport activities has contributed to the inclusion of the skeletally immature age group among those who may suffer from this problem. Bilateral simultaneous symmetric tibial stress fractures that are infrequent in older children are even more rare in toddlers. This entity may cause a diagnostic problem as it must be differentiated from infectious disease, acute trauma or even from the result of a battered child.

Serrated W/M osteotomy. Results using a new technique for the correction of infantile tibia vara.

The conventional osteotomies used to treat infantile tibia vara (Blount's disease) may require internal fixation and its subsequent removal. These techniques, which carry the risk of traction injury, and potential problems of stability and consolidation, do not always succeed in correcting the rotational deformity which accompanies the angular deformity. We have used a new surgical approach, the serrated W/M osteotomy of the proximal tibia, to correct infantile tibia vara in 15 knees of 11 patients. We present the results in 13 knees of nine patients who have been followed up for a mean of eight years. The mean angular correction achieved after operation was 18 +/- 5.8 degrees. The mean femorotibial shaft angle was corrected from 14.2 +/- 3.7 degrees of varus to 4.6 +/- 4.4 degrees of valgus. At the last follow-up, the mean angular correction had reduced to 1.3 +/- 4.9 degrees of valgus without compromising the rotational correction and the overall good clinical results. All the patients and parents were satisfied, rating the result as excellent or good. There were no major postoperative complications and no reoperations. Eight patients were free from pain and able to perform physical activities suitable for their age. One complained of occasional pain. This procedure has the advantage of allowing both angular and rotational correction with a high degree of success without the need for internal fixation.

Obstetric outcome in women with congenital uterine malformations.

Ninety-eight cases of congenital uterine malformations were diagnosed with hysterosalpingography (HSG). Symmetric uterine malformations, composed of bicornuate uterus, uterus didelphys and septate uterus, constituted 80% of the cases. Of them, 75% were of the bicornuate type. Infertility (55%) and a suggestion of cervical incompetence (24%) were the main indication for HSG. Premature deliveries (29%), spontaneous first-trimester abortions (24%), ectopic pregnancies (3%), abnormal fetal presentations (23%) and a high cesarean section rate (27.5%) occurred in those patients. The highest rate of first-trimester abortions (47%) and the lowest rate of term deliveries (21%) occurred in the group with T-shaped uteri as compared to a 7% rate of early abortions (P less than .05) and 61% rate of term deliveries (P less than .03) in the unicornuate uterus group. In 30% of the patients with uterine malformations, cervical incompetence was diagnosed. Improved obstetric outcomes occurred in patients treated with cervical cerclage. There was a statistically significant difference between the number of premature deliveries and spontaneous late abortions in the group without cerclage (50%) and that with cerclage (21%) (P less than .001).

Cervical cerclage--its role in the pregnant anomalous uterus.

Twenty-nine cases of cervical incompetence were found among 98 women diagnosed as having a congenital uterine anomaly on hysterosalpingography, a high incidence of 30%. The highest incidence was found in the bicornuate uterus group--38%. The incidence of premature delivery and late abortion was higher in this group than in the rest of the patients with uterine anomalies (55% and 68%, vs. 45% and 32%). An obvious improvement in obstetrical performance was noted after cerclage. In the cervical incompetence group, term deliveries increased from 26% to 63%. Premature deliveries and late abortions dropped from 74% to 37%. Even in the patients with anomalous uterus without proven diagnosis of cervical incompetence, term deliveries increased from 64% to 96%, and pregnancies terminating prematurely dropped from 35.6% to 4%, following cerclage. No doubt exists as to the need for cerclage in cases of cervical incompetence. We also believe it should be performed prophylactically in cases of bicornuate uterus. The concept of routine prophylactic cerclage in all cases of uterine anomalies should be considered.