Objective To investigate the effectiveness of Salter osteotomy combined with subtrochanteric shortening and derotational osteotomy in treating Tönnis type Ⅲ and Ⅳ developmental dysplasia of the hip (DDH) in children and explore the urgical timing. Methods A retrospective collection was performed for 74 children with Tönnis type Ⅲ and Ⅳ DDH who were admitted between January 2018 and January 2020 and met the selection criteria, all of whom were treated with Salter osteotomy combined with subtrochanteric shortening and derotational osteotomy. Among them, there were 38 cases in the toddler group (age, 18-36 months) and 36 cases in the preschool group (age, 36-72 months). There was a significant difference in age between the two groups (P<0.05), and there was no significant difference in gender, side, Tönnis typing, and preoperative acetabular index (AI) (P>0.05). During follow-up, hip function was assessed according to the Mckay grade criteria; X-ray films were taken to observe the healing of osteotomy, measure the AI, evaluate the hip imaging morphology according to Severin classification, and assess the occurrence of osteonecrosis of the femoral head (ONFH) according to Kalamchi-MacEwen (K&M) classification criteria. Results All operations of both groups were successfully completed, and the incisions healed by first intention. All children were followed up 14-53 months, with an average of 27.9 months. There was no significant difference in the follow-up time between the two groups (P>0.05). At last follow-up, the excellent and good rates according to the Mckay grading were 94.73% (36/38) in the toddler group and 83.33% (30/36) in the preschool group, and the difference between the two groups was significant (P<0.05). The imaging reexamination showed that all osteotomies healed with no significant difference in the healing time between the two groups (P>0.05). There was no significant difference in AI between the two groups at each time point after operation (P>0.05), and the AI in the two groups showed a significant decreasing trend with time extension (P<0.05). The result of Severin classification in the toddler group was better than that in the preschool group at last follow-up (P<0.05). There was no significant difference in the incidence of ONFH between the two groups (P>0.05). In the toddler group, 2 cases were K&M type Ⅰ; in the preschool group, 3 were type Ⅰ, and 1 type Ⅱ. There was no dislocation after operation.ConclusionSalter osteotomy combined with subtrochanteric shortening and derotational osteotomy is an effective way to treat Tönnis type Ⅲ and Ⅳ DDH in children, and surgical interventions for children aged 18-36 months can achieve better results.
Objective To find a more perfect method of treating developmental dislocation of the hip(DDH). Methods From March 1994 toDecember 2002, on the basis of the ordinary operative method, Pemberton method was improved for 48 cases of DDH 49 articulatio coxae which had superficial and small acetabula but bigheads of femur. Osteotomy points were moved higher to enlarge area of bone flapturned over. A piece of full thickness ilium including periosteum was removed as a free bony graft. Capsula articularis growing thicker was made into 2 layers, of which the inner layer was used to tighten articularis and the outer layerwas used to stabilize bone flap. Results All patients were followed up for 18 months to 5 years, the function of articulatio coxae and degree of bony union, inclusion area to head of femur and ischemic necrosis of femoral head were observed through X-ray. According to Zhou Yongde’s criteria for the curative effect of congenital dislocation of the hip, there were 18 cases of 26-30 scores(3 hips), 27 cases of 21-25 scores, 2 cases of 1620 scores(3 hips), and 1 case under 15 scores, and the total superior ratewas 93.8%. Conclusion Improved Pemberton method can enlarge inclusion area tohead of femur, avoid decreasing acetabular volume, and stabilize articulatio well, which made operative indication bigger than original Pemberton method.
Objective To explore the efficacy and safety of the ketogenic diet (KD) in the treatment of genetic developmental and epileptic encephalopathy (DEE). Methods Clinical data from 42 children with genetically confirmed refractory epileptic encephalopathy treated in the Department of Neurology, Jinan Children’s Hospital, between January 2021 and October 2023 were retrospectively analyzed. A classic KD protocol was implemented, and outcomes including seizure frequency, electroencephalogram (EEG) improvement, and adverse reactions were observed at 3, 6, and 12 months post-treatment. Results Among the 42 children, the seizure-free rates at 3, 6, and 12 months of KD treatment were 16.7%, 16.7%, and 14.3%, respectively, while the effective seizure control rates were 69.0%, 52.4%, and 35.7%. At 3 months, comparison of baseline characteristics between the effective and ineffective groups showed no statistically significant differences in gender (P=0.095), age at onset (P=0.648), age at KD initiation(P=0.768), disease duration before KD (P=0.519), presence of abnormal brain MRI findings (P=0.226), epilepsy syndrome classification(P=0.344), or ion channel gene involvement (P=0.066). EEG improvement rates at 6 and 12 months were 54.2% (24 cases) and 42.8% (14 cases), respectively. Retention rates for KD at 3, 6, and 12 months were 100.0%, 71.4%, and 42.8%. Adverse reactions occurred in 7 patients (16.7%), primarily gastrointestinal symptoms (vomiting, constipation, diarrhea; 6 cases) and elevated uric acid (1 case), with no severe adverse events reported. Conclusion KD is an effective treatment for genetic DEE with favorable short-term safety, though long-term adherence requires attention.
Severe psychomotor developmental delay resulting from early postnatal (within 3 months) seizures can be diagnosed as Early-Infantile Developmental and Epileptic encephalopathies (EIDEE). Its primary etiologies include structural, hereditary, metabolic and etc. The main pathogenesis may be related to the inhibition of normal physiological activity of the brain by abnormal electrical activity and the damage of the brain neural network. Ohtahara syndrome and Early Myoclonic Encephalopathy (EME) are typical types of EIDEE. The principle of treatment is to improve the cognitive and developmental function by controlling frequent seizures. When the seizure is difficult to control with drugs, surgical evaluation should be performed as soon as possible, and surgical treatment is the first choice for patients suitable for surgery. The types of surgery can be divided into excision surgery, dissociation surgery, neuromodulation surgery and etc. The current status of surgical treatment of EIDEE was described, and the curative effect of surgical treatment was explored, so as to help clinicians choose appropriate treatment methods.
Developmental epileptic encephalopathies (DEEs) are a group of disorders characterized by early-onset seizures, abnormal electroencephalogram (EEG) patterns, and developmental delay or regression. They are characterized by complex etiology and are often refractory to treatment, severely impacting affected children, particularly infants and toddlers, and pose a challenge in pediatric neurology. In recent years, with the rise of precision medicine, an increasing number of pathogenic genes associated with DEEs have been discovered. However, the specific pathogenic mechanisms and signaling pathways of these genes in the body still require further investigation. This article primarily discusses the genetic patterns of DEEs and the selection of genetic testing, emphasizing the timing of genetic testing assisted by the epilepsy phenotype, especially in DEEs associated with single-gene mutations and new therapeutic drugs, to aid in clinical decision-making for DEEs. It also introduces the use of neurobiological models for DEE research to effectively advance epilepsy research, thereby enabling targeted gene therapy.
ObjectiveTo measure and analyze the radiographic characteristics of the leg length discrepancy in adult patients with unilateral developmental dysplasia of the hip (DDH).MethodsThe clinical data of 112 patients with unilateral DDH who met the selection criteria between January 2016 and June 2018 were retrospectively analyzed. There were 16 males and 96 females with an age of 20-76 years (mean, 42.9 years). According to the Crowe classification, there were 25 hips of type Ⅰ, 26 hips of type Ⅱ, 15 hips of type Ⅲ, and 46 hips of type Ⅳ (26 hips of type ⅣA without secondary acetabular formation, and 20 hips of type ⅣB with secondary acetabular formation). Full-length X-ray films of the lower limbs in the standing position were used to measure the following parameters: greater trochanter leg length (GTLL), greater trochanter femoral length (GTFL), lesser trochanter leg length (LTLL), lesser trochanter femoral length (LTFL), tibial length (TL), and intertrochanteric distance (ITD). The above parameters on the healthy and affected sides were compared and the difference of each parameter between the healthy and affected sides was calculated. Taking the difference of 5 mm between the healthy side and the affected side as the threshold value, the number of cases with the healthy side was greater than 5 mm and the affected side was greater than 5 mm were counted respectively. The difference of the imaging parameters between the healthy side and the affected side were compared between different Crowe types and between type ⅣA and type ⅣB.ResultsThere was no significant difference in GTLL and LTFL between healthy and affected sides (P>0.05); LTLL and TL of affected side were longer than healthy side, GTFL and ITD were shorter than healthy side, and the differences were significant (P<0.05). The constituent ratio of long cases on the affected side of TL and LTLL was greater than the constituent ratio of long cases on the healthy side, while the constituent ratio of long cases on the healthy side of GTFL and ITD was greater than the constituent ratio of long cases on the affected side; there was no obvious difference in the constituent ratio of long cases on the healthy side or the affected side of GTLL and LTFL. The comparison between different Crowe types showed that only the difference in TL between type Ⅰ and type Ⅳ was significant (P<0.05), the difference of each imaging parameter among the other types showing no significant difference (P>0.05). Compared with type ⅣB, the differences of GTLL, TL, and ITD of type ⅣA were bigger, and the differences were significant (P<0.05); the differences of other parameters between type ⅣA and type ⅣB were not significant (P>0.05).ConclusionIn adult unilateral DDH patients, the leg length on the healthy side and the affected side is different, and the difference mainly comes from the TL and ITD, which should be paid attention to in preoperative planning.
Objective To compare the biomechanical effects between rotational acetabular osteotomy and Chiari osteotomy for developmental dysplasia of the hip (DDH) by biomechanical test. Methods Sixteen DDH models of 8 human cadaver specimens were prepared by resecting the upper edge and posterior edge of acetabulum. And the Wiberg central-edge angle (CE) of the DDH model was less than 20°. Then the rotational acetabular osteotomy was performed on the left hip and Chiari osteotomy on the right hip. When 600 N loading was loaded at 5 mm/minute by a material testing machine, the strain values of normal specimens, DDH specimens, and 2 models after osteotomies were measured. Results In normal specimens, the strain values of the left and right hips were 845.63 ± 533.91 and 955.94 ± 837.42 respectively, while the strain values were 1 439.03 ± 625.23 and 1 558.75 ± 1 009.46 respectively in DDH specimens, which was about 2 times that of normal hips. The morphology and X-ray examinations indicated that the DDH model was successfully established. The strain value was 574.94 ± 430.88 after rotational acetabular osteotomy, and was significantly lower than that of DDH specimens (t=4.176, P=0.004); the strain value was 1 614.81 ± 932.67 after Chiari osteotomy, showing no significant difference when compared with that of DDH specimens (t=0.208, P=0.841). The strain value relieved by rotational acetabular osteotomy was significantly higher than that by Chiari osteotomy (t= — 2.548, P=0.023). Conclusion Rotational acetabular osteotomy is better than Chiari osteotomy in relieving hip joint stress of DDH.