Objective To investigate the anatomical features of congenital chordee without hypospadias in children and to discuss the diagnosis and treatment.Methods From August 1984 to December 2004, 94 children with chordee withouthypospadias treated in the West China Hospital of Sichuan University were classified and analyzed for anatomical alterations. Their ages ranged from 18 months to 13 years (mean 6.9 years). Ninety-four patients were divided into four groups. With intraoperation artificial erection, the patients with penis straightened after degloving were classified as type Ⅰ patients (skin-tethering), those with peins straightened after fibrotic tissue in Buck’s fascia released as type Ⅱ patients (dysgenetic fascia), those with normal urethra and orthoplasty failed after degloving and removing fibrotic tissue as type Ⅲ patients (corporal disproportion), and those with dysgenetic urethra tethering the corpora cavernosa as type Ⅳ patients (short urethra).Results In type Ⅰ (n=31, 32.9%) patients, the ventral skin and dartos fascia were contracted while Buck’s fascia and the urethra was normal, in some (7 cases) scrotal skin extended to the ventral portion of penis (webbed penis). In type Ⅱ (n=45, 47.9%), contracture of Buck’s fascia was evident and the thickening fibrotic tissue constituted the chief obstacle to orthoplasty, though in some skin was shortened. In type Ⅲ (n=6), the dorsal and ventral sides of the corpora cavernosa were disproportionated, and the morphologically normal urethra tightly adhered to the ventral aspect of corpora cavernosa. In some cases ventral skin and fascia were contracted, but orthoplasty could notbe achieved through releasing these layers. In type Ⅳ (n=12, 12.8%), the distal urethra was paper-thin and lacking corpus spongiosum, or dense fibrotic bandswere found to be deep to the urethra. The urethra tethered the corpora cavernosaand formed a bow-to-string relation. The overlying skin and fascia were contracted in varying degrees while none had significance in straightening the penis. After operation, the length of penis increased to 6.9 cm from 5.2 cm on average and the chordee was corrected to 1.6° from 42.6° before operation on average. The patients were followed up 1 months to 15 years. The results were satisfactory.Chordee remained in 2 cases, fistula and urethral stricture occurred in 2 casesrespectively, fistula in association with urethral stricture and diverticulum in 1 case; the operation was given again and the results was satisfactory. Conclusion Patients with chordee without hypospadias may be divided into four types depending on which layer of the ventral penis constitutes thechief contribution to chordee. A systematic approach with repeated artificial erection tests is needed in determining the classification and surgical correction.
ObjectiveTo analyze the outcomes of complicated congenital heart diseases (CCHD) patients accepting multiple (>2) re-sternotomy operations.MethodsWe retrospectively analyzed the clinical data of 146 patients undergoing multiple cardiac re-sternotomy operations between 2015 and 2019 in our center. There were 95 males and 51 females with an age of 4.3 (3.1-6.8) years and a weight of 15.3 (13.4-19.0) kg at last operation.ResultsThe top three cardiac malformations were pulmonary atresia (n=51, 34.9%), double outflow of right ventricle (n=36, 24.7%) and functional single ventricle (n=36, 24.7%). A total of 457 sternotomy procedures were performed, with 129 (88.3%) patients undergoing three times of operations and 17 (11.7%) patients undergoing more than three times. Fifty-two (35.6%) patients received bi-ventricular repair, 63 (43.1%) patients received Fontan-type procedures, and 31 (21.2%) patients underwent palliative procedures. Ten (6.8%) patients experienced major accidents during sternotomy, including 7 (4.8%) patients of urgent femoral artery and venous bypass. Eleven (7.5%) patients died with 10 (6.8%) deaths before discharge. The follow-up time was 20.0 (5.8-40.1) months, and 1 patient died during the follow-up. The number of operations was an independent risk factor for the death after operation.ConclusionSeries operations of Fontan in functional single ventricle, repeated stenosis of pulmonary artery or conduit of right ventricular outflow tract post bi-ventricular repair are the major causes for the reoperation. Multiple operations are a huge challenge for CCHD treatment, which should be avoided.
ObjectiveTo explore the experience of surgical repair for congenital aortic stenosis (AS) in our center.MethodsWe retrospectively reviewed the clinical data of 145 children diagnosed with AS, who underwent aortic repair from 2008 to 2019, with or without aortic insufficiency (AI), including 104 males and 41 females with a median age of 2.9 (0.6, 7.8) years. The preoperative and intraoperative data, early and long-term valvular function, long-term survival rate and freedom from reoperation and aortic valve replacment (AVR) were analyzed.ResultsThere were 120 patients receiving commissurotomy, 15 valvuloplasty with extra pericardium patch, and 25 AVR. The mean follow-up time was 0.25-11.20 (4.2±2.8) years. Survival rate at 10 years was 92.3%. Freedom from reoperation and AVR was 63.2% and 60.4% respectively. Multivariate analysis revealed that using a patch during surgery (P=0.036) was an independent risk factor for reoperation. A history of preoperative balloon dilation (P=0.029) and significant preoperative AI (P=0.001) contributed to AVR.ConclusionSurgical treatment of congenital aortic valve stenosis in children is a safe and effective method that provides enough time to achieve a more definitive solution. Using a patch during surgery increases reoperation hazard. A history of previous balloon dilation and significant preoperative AI may result in AVR during the follow-up.
Objective To compare the clinical effect between high flow rate modified ultrafiltration （HMUF） and conventional modified ultrafiltration（CMUF）, and the effect on hemodynamic data and inflammatory mediators. Methods Forty children were divided into two groups with random number table, HMUF group and CMUF group, 20 cases each group. Hematocrit （HCT） and hemodynamic changes were recorded and the concentration of tumor necrosis factor （TNF） and interleukin 6 （IL-6） were measured. Results The operations were done uneventfully with moderate hypothermia cardiopulmonary bypass in 40 patients. Duration of ultrafiltration of HMUF group （7.83±0. 75 min） was less than that of CMUF group （13.86±1.95 min, P〈0.01）. The volumes ultrafiltrated of HMUF group （440.00±91.86ml） was more than that of CMUF group （372.22±56.52ml, P〈0.01）. There are no significant differences about the hemodynamic data, HCT, TNF and IL-6 between two groups. Conclusion The HMUF is safe and efficient,when it was used after pediatric cardiopulmonary bypass, the duration of ultrafiltration can be shortened significantly.
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.
Abstract： Objective To evaluate clinical outcomes of painless flexible fiberoptic bronchoscopy in the treatment for pulmonary complications in postoperative pediatric patients with congenital heart diseases. Methods We retrospectively analyzed clinical data of 58 patients who received fiberoptic bronchoscopy for pulmonary complications after surgical repair for atrial septal defect， ventricular septal defect， tetralogy of Fallot， double outlet right ventricle or transposition of the great arteries in First Affiliated Hospital of Harbin Medical University From August 2009 to February 2012. There were 26 male patients and 32 female patients with their age ranging from 20 days to 2 years. Olympus xp-60 fiberoptic bronchoscopy was used for removal of airway secretions and bronchial alveolar lavage under anesthesia with propofol， fentanyl and lidocaine. Pulse oximetry， respiratory sound and chest X-ray changes were observed. Results The examination time of painless fiberoptic bronchoscopy was 10-25 minutes in the 58 patients. After removal of airway secretions by fiberoptic bronchoscopy， their respiratory status improved significantly， pulse oxygen saturation increased by 5%-12%， and their pulmonary crackles were significantly reduced. In 29 patients with atelectasis， their pulmonary lobes demonstrated significant reexpansion in chest X-ray reviews on the next day after fiberoptic bronchoscopy， and 8 patients received another fiberoptic bronchoscopy treatment on the next day to achieve complete pulmonary reexpansion. One patient with severe pulmonary hypertension （PH） stayed in intensive care unit （ICU） for 6 days， 3 patients with transposition of the great arteries stayed in ICU for 5 days， 3 patients with double outlet of right ventricle and moderately high PH stayed in ICU for 4 days， and all the other patients were discharged from ICU within 48 hours after admission. There was no severe complication related to fiberoptic bronchoscopy， except 2 patients with minor nasal mucosa bleeding who were cured with hemostatic drugs and local compression. Conclusion Painless flexible fiberoptic bronchoscopy can significantly enhance clinical outcomes and shorten ICU stay for postoperative pediatric patients with congenital heart diseases.
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.
Congenital tracheal stenosis (CTS) is a rare but potentially life-threatening disease which results in congnital airway lesion. CTS is often associated with cardiovascular anomalies and presented with a wide spectrum of symptoms. CTS has challenged pediatric surgeons for decades. Various classic approaches and new techniques, including computational fluid dynamics, tissue-engineering trachea, and 3D printing have been proposed for diagnosis and treatment of CTS. This review provides a snapshot of the main progress of diagnosis and treatment of CTS.
OBJECTIVE: To evaluate the operative methods of biliary tract reconstruction after cystectomy of congenital choledochal cyst(CCC). METHODS: One hundred and six cases with CCC underwent cystectomy and biliary tract reconstruction in our hospital from July 1984 to December 1999 were followed up. Among them, there were three kinds of procedures in biliary tract reconstruction: with single Roux-Y hepaticojejunostomy in 48 cases, with intussusceptive valve to the line of Roux-Y hepaticojejunostomy in 37 cases, with rectangular valve to the line of Roux-Y hepaticojejunostomy in 21 cases. RESULTS: Fifty nine cases were followed up for 4.68 years in average. There were 3 cases with ascending cholangitis after single Roux-Y hepaticojejunostomy. And the symptom disappeared in 2 cases underwent reoperation with an intussueceptive valve plasty to the line of Roux-Y hepaticojejunostomy. No patients suffered from ascending cholangitis in the precautionary valve plasty group. CONCLUSION: It suggests that the postoperative ascending cholangitis can be prevented effectively if standard cystectomy and prophylactic intussusceptive valve added to the line of Roux-Y hepaticojejunostomy are carried out. The procedure should be performed as soon as possible providing the child is tolerable.
Objective To summarize the experience of the superior vena cava and pulmonary connection surgery for functional single ventricle (SV) with total anomalous pulmonary venous (TAPVC). Methods We retrospectively analyzed the clinical data of 10 patients with SV and TAPVC in our hospital from January 2012 through June 2014. There were 7 males and 3 females at average age of 90.33±86.53 months. The 10 patients were with right atrial isomerism, 9 with heterotary and asplenia syndrome. Five patients were anatomic single ventricle and others were with functional uni-ventricle. Nine patients were with supracardiac pattern TAPVC and one was with intracardiac TAPVC. All patients were operated unilateral or bilateral bidirectional Glenn procedure with TAPVC correction. Results The arterial oxygen saturation (SaO2) increased prominently after operation (86%±6% vs. 79%±6%, P<0.01). There were 3 patients with low cardiac output syndrome, one patient with severe arrhythmia, 4 patients with serious pleural effusion, 4 patients with hospital-acquired infection, and 3 patients with central nervous system complications (epilepsy or hemiplegia). One died because of hemorrhage and pulmonary thrombosis, and the other died of hypoxemia and mutiple organ dysfunction syndrome (MODS). Conclusion Glenn is one of palliated procedure choice for SV/TAPVC patients. The indication for surgery and perioperative management individually is crucial.