OBJECTIVE: To explore the corrective strategies and effectiveness of osteotomy surgery for severe lower limb deformities in hypophosphatemic rickets. METHODS: A retrospective analysis was conducted on 29 patients with severe lower limb deformities of hypophosphatemic rickets who underwent surgical treatment between February 2012 and August 2024. There were 9 males and 20 females. The age ranged from 13 to 53 years, with an average of 24.6 years. All patients were deformities of both lower limbs, presenting as 24 cases of O-shaped legs, 2 cases of wind-blown deformities, and 3 cases of X-shaped legs. Based on the full-length films of both lower limbs in the standing position before operation, the osteotomy planes of the femur, tibia, and fibula were designed. Among them, if both the same-sided thigh and leg were deformed, staged surgeries of both lower limbs were selected. If only the thigh or leg were deformed, simultaneous surgeries of both lower limbs were selected. The femur deformity was corrected immediately after osteotomy at the deformed plane; the osteotomy fragment was temporarily controlled with an external fixator, which was removed after perform internal fixation with a steel plate. After fibular osteotomy, the Ilizarov frame or Taylor frame was installed on the tibia and fibula. The threaded rods were removed and then tibial osteotomy was performed on the deformed plane. Patients using the Taylor frame did not undergo deformity correction during operation. The external fixators were adjusted starting 7 days after operation to correct the varus, valgus, and rotational deformities of the lower limb. Patients using the Ilizarov frame corrected the rotational deformity of the tibia during operation. The external fixator was adjusted starting 7 days after operation to correct the varus and valgus deformities of the lower limb. During the treatment period, the patient could walk with partial weight-bearing on the operated limb with crutches. The external fixator was removed after the bone healed. Before operation and at last follow-up, the medial proximal tibial angle (MPTA), lateral distal tibial angle (LDTA), posterior proximal tibial angle (PPTA), anterior distal tibial angle (ADTA), anatomic lateral distal femoral angle (aLDFA), posterior distal femoral angle (PDFA), and mechanical axis deviation (MAD), lower limb rotation, limb length discrepancy (LLD) were measured. The self-made scoring criteria were adopted to evaluate the degree of lower limb deformity of the patients. RESULTS: All operations were successfully completed, and no complications such as nerve or vascular injury occurred. The adjustment time of the external fixator of the lower limb after operation was 28-46 days, with an average of 37.4 days. The wearing time of the external fixator ranged from 134 to 398 days, with an average of 181.5 days. Mild pin tract infections occurred in 2 limbs. The osteofascial compartment syndrome occurred in 1 limb after operation. No complications related to orthopedic adjustment of the external fixator occurred in other patients. All patients were followed up 6-56 months, with an average of 28.2 months. At last follow-up, full-length films of both lower limbs in the standing position showed that the coronal mechanical axes of the lower limbs of all patients returned to the normal. At last follow-up, MPTA, LDTA, PPTA, aLDFA, PDFA, MAD, lower limb rotation, LLD, and the score of lower limb deformity significantly improved when compared with those before operation ( P<0.05). There was no significant difference in ADTA between pre- and post-operation ( P>0.05). The degree of lower limb deformity were rated as moderate in 2 cases and poor in 27 cases before operation and as excellent in 7 cases, good in 18 cases, and moderate in 4 cases at last follow-up, with an excellent and good rate of 86.2%. CONCLUSION For severe lower limb deformities in hypophosphatemic rickets, immediate correction of deformities with femoral osteotomy and internal plate fixation, as well as gradually correction of deformities with tibiofibular osteotomy and circular external fixation (Ilizarov frame or Taylor frame), have satisfactory therapeutic effects.
Humans ; Male ; Osteotomy/instrumentation* ; Female ; Adult ; Retrospective Studies ; Tibia/abnormalities* ; Adolescent ; Femur/abnormalities* ; Middle Aged ; Fibula/surgery* ; Rickets, Hypophosphatemic/complications* ; Young Adult ; Treatment Outcome ; External Fixators ; Bone Plates ; Lower Extremity Deformities, Congenital/etiology*

OBJECTIVE: To evaluate the functional and aesthetic evaluation of external fixator lengthening through plantar approach for fourth brachymetatarsia. METHODS: A retrospective analysis was conducted on 20 patients (23 feet) with fourth brachymetatarsia who met the selection criteria between January 2016 and January 2024, including 3 males and 17 females, with 8 left, 9 right, and 3 bilateral cases. The mean age was 24.7 years (range, 14-51 years). The preoperative metatarsal shortening length was (13.8±3.2) mm. The preoperative American Orthopaedic Foot and Ankle Society (AOFAS) forefoot score was 79.5±3.9, the visual analogue scale (VAS) score of appearance satisfaction was 1.7±0.8, and the appearance index (AI) score was 13.6±0.9. All patients underwent external fixator lengthening through plantar approach. The lengthening length of metatarsal bone, lengthening ratio, healing time, and healing index were recorded. Functional outcomes were assessed using the AOFAS forefoot score, VAS score of appearance satisfaction, and quality-of-life impact with AI questionnaire. RESULTS: All 20 patients were followed up 14-55 months with an average of 36.3 months. During the follow-up, complications occurred in 4 cases (17.4%), including 2 cases of metatarsophalangeal joint stiffness, which had no significant effect on the function and appearance. Delayed union of osteotomy occurred in 1 case (healed at 12 weeks after operation). Pin loosening occurred in 1 case and recovered after outpatient reinforcement. No complications related to plantar scar occurred. At last follow-up, the lengthening length of metatarsal bone was (13.9±3.1) mm, and the lengthening ratio was 25.8%±5.6%. All cases achieved bony union, with a mean healing time of (64.3±12.5) days and a healing index of (46.9±4.8) d/cm. At last follow-up, AOFAS score was 98.9±2.1, the VAS score of appearance satisfaction was 9.3±0.7, and the AI score was 0.6±0.8, which significantly improved when compared with those before operation ( t=27.398, P<0.001; t=32.994, P<0.001; t=56.135, P<0.001). CONCLUSION External fixator lengthening through plantar approach is a safe and effective technique for fourth brachymetatarsia, achieving satisfactory functional and aesthetic outcomes.
Humans ; Male ; Female ; Adult ; External Fixators ; Retrospective Studies ; Bone Lengthening/instrumentation* ; Middle Aged ; Metatarsal Bones/abnormalities* ; Adolescent ; Young Adult ; Treatment Outcome ; Patient Satisfaction ; Esthetics ; Osteotomy/methods* ; Foot Deformities, Congenital/surgery*

Objective:To discuss the clinical efficacy of low-temperature radiofrequency ablation assisted by endoscopy combined with resection and drainage of cervical abscess for the treatment of congenital pyriform sinus fistula （CPSF） in the acute inflammatory period of children. Methods:Clinical data of 30 patients with CPSF in the acute inflammatory period who received low-temperature radiofrequency ablation assisted by endoscopy under laryngoscope, combined with resection and drainage of cervical abscess, from January 2018 to December 2023 were reviewed. After the operation, patients were followed up closely at different stages. All patients underwent color Doppler ultrasound and electronic laryngoscopy, and the results were analyzed. Results:All 30 children successfully completed the surgery without pharyngeal fistula, dysphagia, perifistula, or distal fistula infection, and the incision in the neck healed well. The follow-up survey ranged from 6 months to 2 years, and no recurrences were observed. Conclusion:Low-temperature radiofrequency ablation assisted by endoscopy combined with resection and drainage of cervical abscess is a promising method for treating CPSF in the acute inflammatory period. It is less traumatic, simple, safe, has a significant curative effect, and a low recurrence rate. This approach can be used as a supplementary operation for CPSF in children and provides a new way for clinical treatment.
Humans ; Pyriform Sinus/abnormalities* ; Abscess/surgery* ; Drainage ; Fistula/congenital* ; Female ; Male ; Child ; Radiofrequency Ablation ; Treatment Outcome ; Postoperative Period ; Endoscopy ; Laryngoscopy ; Inflammation ; Child, Preschool

OBJECTIVE: To explore clinical efficacy of congenital brachymetatarsia with immediate metatarsal lengthening. METHODS: From March 2015 to December 2020, 7 patients with brachymetatarsia were treated, including 6 females and 1 male;aged range from 18 to 30 years old;there were 5 patients with metatarsal microsomia on one foot, 2 patients with metatarsal microsomia on the first and fourth right foot, and immediate extension of metatarsal microsomia on the first and fourth right foot;two patients were short metatarsal bones of both feet. The length of short metatarsal bone, length of normal metatarsal bone, distance of short metatarsal bone and healing of bone graft were observed before and 12 months after operation. American Orthopaedic Foot and Ankle Society (AOFAS) scores were used to evaluate clinical efficacy and observe complications. RESULTS: Seven patients were followed up for 12 to 24 months. All metatarsal bones were extended to satisfactory length and bone graft were healed completely. Metatarsal length and shortening distance were improved from 3.55 to 5.90 cm and 0.77 to 1.46 cm before operation to 4.31 to 6.87 cm and 0.04 to 0.57 cm at 12 months after operation. Postoperative X-ray of the affected foot at 12 months showed bone healing was achieved between metatarsal bone and bone graft in 7 patients, and the parabolic shape of the distal metatarsal bone recovered after operation. AOFAS scores improved from 40 to 70 before operation to 88 to 95 points at 12 months after operation, and 6 patients were excellent and 1 good. CONCLUSION Immediate extension of metatarsal bone for congenital brachymetatarsia, the transplanted bone grew well during the process of bone grafting healing, the occurrence of bone nonunion was reduced, the short metatarsal bone was restored to a satisfactory length, and the toe function restored well.
Humans ; Female ; Male ; Metatarsal Bones/abnormalities* ; Adult ; Adolescent ; Young Adult ; Bone Lengthening/methods* ; Foot Deformities, Congenital/surgery*

This paper focuses on the diagnosis and treatment of disorders of laryngeal airway in children, including congenital anomalies, infection, and tumor of the larynx to provide a new technology for managing these diseases. Based on the characteristic of them, the pediatric upper airway is dedicated to the clinical evaluation of airway obstruction and the assessment of the compromised pediatric airway, including clinical evaluation of symptoms, diagnostic endoscopy, and imaging examination. Information on endoscopic techniques used for dealing with different degrees of pediatric airway comprised is provided, also this techniques could diagnose what kinds of airway disorder. For example, determining the a particular laryngeal cleft at the initial otolaryngology encounter, flexible laryngoscopy should be performed. In order to define the extent of any identified cleft, the rigid bronchoscopy should be completed to evaluate for classification of laryngeal cleft including typeⅠ, Ⅱ, Ⅲa, Ⅲb, Ⅳa, Ⅳb under general anesthesia. The decision to pursue any therapy for the disorders of laryngeal airway in children should be based on the severity of the patient's symptoms, endoscopic and imaging examination. There are two medical treatments including nonsurgical therapy and surgical therapy. For example the laryngeal cleft, approximately half of type 1 and select type 2 patients can be managed entirely with nonsurgical therapy. Medical management is multifaceted. Under recommendations from the feeding team, a modified diet with thickened feeds and possibly altered feeding position should be initiated. Endoscopic surgical repair is the current gold standard for definitive repair of type 1, the majority of type 2, and selected type 3 clefts. Finally, information on new techniques used into the future for dealing with the diagnosis and treatment of disorders of laryngeal airway in children in this paper.
Child ; Humans ; Infant ; Larynx/surgery* ; Laryngoscopy ; Endoscopy ; Bronchoscopy ; Congenital Abnormalities/surgery*

Objective:To analyze the efficacy of endoscopic surgical repair in the treatment of type Ⅲ laryngeal clefts and to explore the feasibility of the treatment for type Ⅲ laryngeal clefts. Methods:The clinical data of 6 children with type Ⅲ laryngeal clefts who underwent endoscopic surgical repair in our department from June 2018 to January 2023 were analyzed retrospectively. The operation was performed under combined intravenous and general anesthesia,preserving the spontaneous breathing of the children. With the assistance of 4 mm/0° endoscope, radiofrequency knife or laryngeal scissors were used to make fresh wounds along the edge of laryngeal clefts,and cotton pads infiltrated with adrenaline （1:10 000） were used to compress the wound. 6-0 PDP suture was used to suture 3-6 stitches according to the extent of laryngeal clefts. Modified barium swallowing test （MBS） was performed 3 months after operation. Results:All the children were successfully treated with endoscopic surgical repair. After surgery, 2 cases were transferred to pediatric intensive care unit （ICU） for 7 days of monitoring, and the rest were transferred back to the general ward. There were no postoperative complications. The symptoms of dysphagia, laryngitis and recurrent pneumonia were improved in all children. According to the follow-up results of postoperative MBS,no aspiration was found in all children, and 2 children had intermittent cough when drinking large amounts of water. During the follow-up, there were 2 cases of recurrence, and the cracks were completely repaired after the second endoscopic surgical repair treatment,and no recurrence has been observed so far. Conclusion:Endoscopic surgical repair can be applied to some children with type Ⅲ laryngeal clefts with less intraoperative bleeding and fewer operative complications. It can significantly improve the symptoms such as swallowing dysfunction and recurrent pneumonia. It is a safe and effective surgical treatment.
Child ; Humans ; Laryngoscopy/methods* ; Laryngoscopes ; Retrospective Studies ; Larynx/surgery* ; Pneumonia ; Congenital Abnormalities/surgery*

At present, there is no unified gold standard for the treatment of laryngeal clefts. Type Ⅰ laryngeal clefts with mild symptoms can be treated conservatively, such as thick diet feeding and using proton pump inhibitor to control reflux, while Ⅱ-Ⅳ laryngeal clefts mostly requires surgical intervention.There are many different surgical methods for the treatment of laryngeal clefts, including injection laryngoplasty, endoscopic surgical repair of laryngeal clefts and open laryngoplasty through anterior cervical approach. How to choose a more suitable surgical plan for children is a problem worth discussing. This article will review the literature on the surgical treatment of laryngeal clefts both domestically and internationally, and summarize the current situation and challenges of surgical treatment of laryngeal clefts.
Child ; Humans ; Congenital Abnormalities/surgery* ; Endoscopy ; Laryngoplasty/methods* ; Laryngoscopy/methods* ; Larynx/surgery*

Objective: Our aim of this study is to describe the outcomes of a series of patients who underwent cleft repair and posterior cartilage grafts laryngotracheoplasty (LTP) from anterior midline cervical approach for type Ⅲ laryngotracheoesophageal clefts (LETC). Methods: A review of patients with type Ⅲ LETC between May 2017 and December 2021 was performed. Demographic features including gender, age at surgery, weight, airway support, feeding status, and airway and other comorbidities were collected preoperatively. Patients were evaluated in breathing, swallowing and phonation postoperatively. The developmental status and morbidities were recorded. Results: Five patients who underwent cleft repair and posterior cartilage grafts LTP from anterior midline cervical approach were included. All patients survived and thrived postoperatively. At last follow-up, 3 patients were able to successfully extubate with acceptable voice, and 2 patients were tracheostomied. Four patients were able to be fed orally without aspiration, and one patient needed to be fed by thick food. Conclusion: The combination of cleft repair and posterior cartilage grafts LTP from anterior midline cervical approach is an effective and safe treatment for type Ⅲ LETC.
Cartilage/transplantation* ; Congenital Abnormalities/surgery* ; Humans ; Larynx/surgery* ; Retrospective Studies

Congenital Abnormalities/surgery* ; Congenital Microtia/surgery* ; Ear/surgery* ; Hearing ; Hearing Aids ; Hearing Tests ; Humans ; Reconstructive Surgical Procedures

Osteogenesis imperfecta is a heterogeneous group of connective tissue diseases that is predominantly characterized by bone fragility and skeletal deformity. Two siblings with undiagnosed type I osteogenesis imperfecta underwent orthognathic surgery for the treatment of facial asymmetry and mandibular prognathism. The authors report two cases of combined orthodontics and orthognathic surgery in patients with type I osteogenesis imperfecta, mandibular prognathism, and facial asymmetry.
Congenital Abnormalities ; Connective Tissue Diseases ; Facial Asymmetry ; Humans ; Orthodontics ; Orthognathic Surgery ; Osteogenesis Imperfecta ; Osteogenesis ; Prognathism ; Siblings