OBJECTIVE: To introduce a surgical approach for reconstruction of nail folds in congenital complete syndactyly release. METHODS: A narrow flap and a broad flap were raised on the common distal phalanx to cover the denuded nail-edge in 30 fingers of 15 cases whose webs were separated. RESULTS: All of the flaps were successfully transferred and survived. The reconstructed nail folds had satisfied figure in 21 out of 30 fingers. The nail folds in the other 9 fingers, covered by a broad flap in 2 fingers and by a narrow flap in 7 fingers, were a little smaller than normal. All of the 30 fingers had normal fullness of pulp and no twisty nails. CONCLUSION: The reconstruction of nail folds by double pulp flap can be performed with a one-stage technique, and the outcome is satisfactory, which make it as a good surgical approach to reconstruct nail folds in congenital complete syndactyly release.
Twenty five children with congenital biliary dilatation were treated with hepatico-jejuno-duodenostomy following excision of choledochal cysts between 1983 and 1985. The age ranged from two months to eleven years. The last follow-up ranged from 6-9 years (mean 7.5 years). All patients were free of jaundice with normal growth and development and none had peptic ulcer. The results from the last follow-up was better than that of the first one. This procedure was safe, effective and physiologically appealing.
Objective To report the clinical characteristics and treatment analysis of 3 cases of congenital ulnar collateral flexor contracture of the forearm and take a reference for clinic. Methods A total of 3 patients with congenital ulnar collateral flexor contracture of the forearm were admitted between February 2019 and August 2021. Two patients were male and 1 was female, and their ages were 16, 20, and 16 years, respectively. The disease durations were 8, 20, and 15 years, respectively. They all presented with flexion deformity of the proximal and distal interphalangeal joints of the middle, ring, and little fingers in the neutral or extended wrist position, and the deformity worsened in the extended wrist position. The total action motion (TAM) scores of 3 patients were 1 and the gradings were poor. The Carroll’s hand function evaluation scores were 48, 55, and 57, and the grip strength indexes were 72.8, 78.4, and 30.5. Preoperative CT of case 2 showed a bony protrusion of the flexor digitorum profundus tendon at the proximal end of the ulna; and MRI of case 3 showed that the ulnar flexor digitorum profundus presented as a uniform cord. After diagnosis, all patients were treated with operation to release the denatured tendon, and functional exercise was started early after operation. Results The incisions of 3 patients healed by first intention. Three patients were followed up for 12, 35, and 12 months, respectively. The hand function and the movement range of the joints significantly improved, but the grip strength did not significantly improve. At last follow-up, TAM scores were 3, 4, and 4, respectively, among which 2 cases were excellent and 1 case was good. Carroll’s hand function evaluation scores were 95, 90, and 94, and the grip strength indexes were 73.5, 81.3, and 34.2, respectively. ConclusionCongenital ulnar collateral flexor contracture is a rare clinical disease that should be distinguished from ischemic muscle contracture. The location of the contracture should be identified and appropriate surgical timing should be selected for surgical release. Active postoperative rehabilitation and functional exercise can achieve good hand function.
Objective To present the surgical treatment experiences of congenital choledochal cyst (CCC). MethodsOne hundred and fortyfive patients in 152 CCC were analyzed retrospectively and followed in west China hospital of Sichuan university from 1964 to 1999. ResultsOne hundred and fortyfive cases underwent operation and 6 of them died after operation. The incidence of hepatocirrhosis within first year after birth is higher than those over one year old （P＜0.05）. Thirtynine cases underwent cystoduodenostomy or cystojejunostomy. One hundred and six children underwent cyst resection and biliary tract reconstruction (with single RouxY hepaticojejunostomy 48 cases, intussusceptive valve and rectangular valve to the line of RouxY hepaticojejunostomy 37 and 21 cases respectively). Seventyseven patients were followedup (means 4.68 years). Two of 3 cases with ascending cholangitis after single RouxY hepaticojejunostomy underwent reoperations with an intussusceptive valve added to the line of RouxY hepaticojejunostomy and the symptoms disappeared. All of them have a good outcome. Conclusion The younger the patients, the less severe the liver damaged, and its prognosis are better. The procedure that cyst resection totally and an intussusceptive valve added to the line of RouxY hepaticojejunostomy should be carried out early as soon as possible.
Objective To investigate the results of the surgical treatment of adult congenital heart disease (ACHD). Methods We retrospectively analyzed the clinical data of 502 patients with ACHD underwent operation treatment in Fu Wai Hospital between January 2012 and June 2015. There were 205 males and 297 females at age of 18-65(34.01±11.97) years and weight of 19-96 (58.60±12.60) kg. Results Atrial septal defect had the highest incidence of ACHD, the proportion accounted for 43.8%.The followed was ventricular sepal defect, accounting for 26.6%. And the third is tetralogy of Fallot, accounting for 5.4%. The age ranged from 18 to 65 years. A percentage of 65.13% patients received operation when they are younger than 40 years, with the most patients at age of 20-29 years, the least patients at age of 30-39 years. We completed a total of 471 patients of early orthodontic treatment with operation success rate at 99.57%. We completed 29 patients palliative operation with early operation success rate at 96.55%. And there were 2 patients with re-operation at success rate of 100.00%. The postoperative patients with ventilator assisted time was 19.03 h. The mechanical ventilation time was 19.03 h. ICU treatment time was 2.22 d. Postoperative complications occurred in 51 patients with incidence rate at 10.15%. Conclusion It is very important to impose proper surgical method on different patients. The operation doctor should be trained skillfully. Perioperative management is also a key stage to the success of the operation.
Since October 1975, 35 patients with congenital constriction bands were treated. Follow-up for 10 months to 14 years showed that the results of correction were satisfactory except 1 patient died from other disease. The etiolology, the concurrent deformities and the method of treatment were discussed.
Objective To introduce a method of the surgicalcorrectionof Poland’s syndrome in children. Methods From May 1990 to May 2002, 3 female children with Poland’s syndrome were treated. One child of 12 years old with defects of the right second and third costal cartilages, pectoralis major and pectoralis minor underwent repair of chest wall with graft of autologous costal cartilage from left sixth costal cartilage and transfer of the latissimus dorsi flap. The other 2 children, 3 and 16 years old, with defects of theleft second, third and fourth costal cartilages, pectoralis major and pectoralis minor underwent graft of autogenous costal cartilage from the right sixth costal cartilage and implant of Dexon mesh. Results Three patients were followed up 1, 7 and 10 years postoperatively, respectively.The contour of chest wall in 3 patients were improved. The functions of the chest, back and upper limbs in the deformitis side were good and the growth and development of thechildren were normal during follow-up postoperatively.Conclusion The congenital deformities of multiple systems and parts in the body are involved in Poland’s syndrome so that it is necessary to make a proper surgical plan according to the extent of lesions for a satisfactory result.
ObjectiveTo discuss the effectiveness of using dorsal two wing-shaped advancement flap to reconstruct finger web for treatment of congenital syndactyly.MethodsBetween August 2014 and August 2017, 30 cases of congenital syndactyly were treated, including 18 males and 12 females with an average age of 2.5 years (range, 1.5-5 years). Eight cases were of bilateral hands syndactyly and 22 cases of single hand syndactyly. There were 39 webs of syndactyly (including 1 case of syndactyly of middle finger, ring finger, and little finger). Among them, 11 webs were complete and 28 webs were incomplete. At the dorsum, a flap with V-shaped tip and two wing-shaped pedicle was designed and was just sewed up with an anchor-shaped incision at the palm. Distal end of fingers were separated by serrated flap and were sutured after removal of fatty tissue. In 11 cases with tight skin connection, the defect area at lateral and distal end of fingers was repaired by small pieces of full-thickness skin graft.ResultsAll the flaps survived completely after operation, and no flap necrosis occurred. The skin grafts on the distal side of the finger survived and the wound healed by first intension. All 30 cases were followed up 6-12 months, with an average of 9 months. Postoperative flexion and extension function of fingers were good, and the web depth and width were normal. At last follow-up, according to the Swanson et al. standard, 20 fingers were graded as excellent, 8 as good, and 2 as fair, with an excellent and good rate of 93.3%.ConclusionThe effectiveness of using dorsal two wing-shaped advancement flap to reconstruction finger web for treatment of congenital syndactyly is satisfactory.
Abstract: Eisenmenger’s syndrome is a kind of pathophysiologic syndrome that occurs in the end stage of congenital heart disease. The diagnosis of Eisenmenger’s syndrome is clear, but its early clinical presentations are nonspecific and its prognosis is poor. Recently, the therapeutic regimen is steadily improved by the endless efforts that lots of scientists have made. Treatments including new drugs, modified surgery, extracorporeal membrane oxygenation, transplantation and gene therapy can not only relieve symptoms, correct abnormal pulmonary hemodynamics, but also can improve prognosis to some extent. We will review and introduce the advance of research on Eisenmenger’s syndrome, including its pathogenesis, diagnosis and treatment.
ObjectiveTo summarize the minimally invasive experiences and medium-long-term results of perventricular device closure of ventricular septal defects (VSD) under transesophageal echocardiography (TEE) guidance.MethodsWe retrospectively analyzed the clinical data and medium-long-term follow-up results of 783 patients who undertook perventricular device closure under TEE guidance in Dalian Children’s Hospital from July 2011 to January 2020, in which perimembrane VSD were found in 598 patients, VSD with aortic valve prolapse in 135 patients and muscular VSD in 2 patients. There were 463 males and 320 females at age of 5 months to 13 years with average age of 3.3±1.2 years, and body weight of 5.9-51.0 (15.9±8.3) kg. The left ventricular defect diameter of the VSD ranged from 5.0 to 11.0 mm, with an average of 6.3±1.2 mm. The right ventricular defect diameter of the VSD ranged from 2.3 to 8.0 mm, with an average of 4.3±0.9 mm.ResultsThe procedures were completed successfully in 753 patients. The device of 1 patient (0.1%) fell off and embedded in the right pulmonary artery after the operation, and the occluder was taken out and the VSD was closed with cardiopulmonary bypass (CPB) in the secondary operation. One patient (0.1%) appeared Ⅲ degree atrioventricular block in 2 years after operation. The device was taken out and VSD was closed with CPB in the secondary operation, and the patient gradually reached to sinus rhythm in post-operation. Eight patients (1.1%) presented delayed pericardial effusion in 1 week after operation, and were cured by pericardiocentesis with ultrasound-guided. Symmetric occluders were used in 580 patients, eccentric occleders were used in 171 patients and muscular occluders were used in 2 patients. The follow-up time was 9 months to 9 years. The rate of loss to follow-up was 96.7% (704/728). No residual shunt, occlude-loss or arrhythmia was found during follow-up. Conclusion The minimally invasive penventricular device closure of VSD guided by TEE is safe and availabe. Medium-long-term follow-up results are satisfactory, it is worthy of clinical promotion, and longer term follow-up is still needed.