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Objective To summarize the experience of the ventriculoperitoneal shunt in treating children with methylmalonic aciduria combined with hydrocephalus,and to assess the clinical value.Methods From September 2012 to May 2016,a total of 12 patients with methylmalonic aciduria combined with hydrocephalus in Peking University First Hospital were enrolled,including 7 boys and 5 girls.All the 12 patients underwent ventriculoperitoneal shunt.Drug therapy was performed after surgery.The clinical manifestations and imaging findings were used as the basis for adjusting the pressure of the diverter valve appropriately.The clinical condition of patients were evaluated retrospectively.The patients' clinical symptoms,signs,imaging materials,surgical complications and postoperative prognosis were analyzed.Results All the cases were followed up for 3 to 36 months,no death case and no serious postoperative complications of hydrocephalus occurred.Clinical symptoms of intracranial hypertension were relieved or disappeared.The head circumference progressive enlargement stopped.The anterior fontanelle tension decreased significantly.Setting-sun sign of eyes disappeared.One out of 4 cases with convulsion and epilepsy was relieved after the operation.Seven cases of poor vision or vision loss,postoperative visual acuity were improved though not recover to normal eyesight.One case of the children with hearing loss,postoperative hearing recovered.During the follow-up period,the head CT showed that the ventricle was narrowed significantly,interstitial brain edema improved obviously.Conclusion Ventriculoperitoneal shunt is a effective method for treating children with methylmalonic aciduria combined with hydrocephalus,which is beneficial for patients with these diseases.
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SUMMARY Hemophilia A is aninherited bleeding disorder, lack of coagulation factorⅧ( FⅧ) , and if combined with intracranial malignant tumor, the operation risk is very high. Department of Pediatric Sur-gery in Peking University First Hospital used coagulation factor replacement therapy, succeeded in the operation of 2 cases of intracranial malignant tumor with hemophilia A in children, with no abnormal bleeding events. The establishment of the multi subject cooperation group before operation, good preoper-ative preparation, enough alternative factors, and close postoperative monitoring, are the key to the suc-cessful treatment.
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Objective To analyze the categories of co-existed nervous system anomalies in children with meningomyelocele in order to consummate the treating strategy for meningomyelocele.Methods Sixty-nine children who had complete clinical data diagnosed as meningomyelocele in Department of Pediatric Surgery,Peking University First Hospital from Jan.2005 to Jun.2013,were retrospectively analyzed.Preoperative spinal MRI was routinely performed.Ultrasonagraphy,CT,MRI or combination of the 2 measures were adopted to evaluate the brain.Microscopically excision of the sac,exploration of the spinal canal,brisement of the tethered cord and meningomyelocele repair were routine operation procedures.Other lesions detected during operation were treated accordingly.MRI findings and intraoperative findings were also compared.Results Preoperative spinal MRI found 59 cases of cone bit,24 cases of syringomyelia,20 cases of mild syringomyelus,14 cases of diastematomyelia,6 cases of intraspinal cyst,6 cases of intraspinal lipoma.Nineteen cases of hydrocephalus were diagnosed by iconography,among which 5 cases complicated with caudal herniation of cerebellar tonsils.Seventeen cases of diastematomyelia,8 cases of intraspinal lipoma,6 cases of intraspinal cyst were detected and treated during operation.Three cases of intraspinal lipoma and 3 cases of osseous diastematomyelia were ignored by preoperative MRI reports.Two cases of intraspinal epidermoid cyst were misdiagnosed as general cyst.One case of teratoma who was not found on MRI was detected by operation and resected.Syringomyelus or syringomyelia was found in all the children complicated with hydrocephalus.Sixty-one cases(88.4％) among the 69 cases complicated with other nervous system anomalies.All of patients were followed up for 4-71 months,averaged 23.8 months.All of patients had no new symptom.Conclusions Children with meningomyelocele often complicate with multiple nervous system anomalies.Preoperative brain and entire spinal iconography,together with thorough intraoperative exploration,are very important so as to avoid omission the co-existed lesions during operation.
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@#ObjectiveTo assess the effect of Duckett and Duplay technique on repairing severe hypospadias.Methods70 cases with severe hypospadias accepted urethraplasty with a penile and scrotal skin tube by Duckett's and Duplay's technique. ResultsThe hypospadias of 65 cases were successfully repaired in one stage, 3 cases complicated urethral fistula,2 caese complicated urethral stenosis with urethral diverticulum, which was significantly improved compared with the scrotal cutaneous island flap techniques(P<0.05). ConclusionDuckett's and Duplay's techniques is a good method in the treatment of severe hypospadias with little complication.
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Objective To explore the feasibility of simple transanal colon pull-through operation in the management of Hirschsprung's disease(HD) in children. Methods 57 case of HD were performed by transanal macrocolon pull-through operation from July 2001 to May 2002. Results 53 cases underwent simple transanal colon pull-through operation successfully. The mean operative time was 1.5h (1h～2h) and blood loss was 5ml～10ml. Dejection and food taking recovered at the first day after operation in 51 patients; dejection recovered at the third day after operation in 2 patients. 4 patients with long segmental macrocolon, implicated in transverse colon, were operated on by laparoscope-assited surgery, and they began to defecate and take food in 2d～3d postoperatively. The mean operative time was 3h(2h～3.5h). 57 cases had been followed up for (2～9)months, and their dejection function recovered well. Conclusions It is feasible to treat HD in children through simple transanal colon pull-through operation. The method has the advantages of minimal invasion, less bleeding, quicker recovery, shorter hospital stay and good recovery of dejection function.
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Objective To explore the diagnosis and treatment of intestinal duplication by means of laparoscope. Methods 9 cases suspected intestinal duplication before operation underwent intraperitoneal exploration through two-trocar laparoscope. Patients diagnosed as intestinal duplication were performed by enterectomy and end-to-end intestinal anastomosis outside abdominal cavity through a 10mm trocar. Results All cases were operated on under lapascope with a mean operative time of 80min(30min～110min).The postoperative hospital stay was 4d～5d . No complications occurred. Conclusions Two-trocar laparoscope is an effective method to diagnose and treat intestinal duplication.
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@#ObjectiveTo investigate the relationship between the prognosis and both of treatment way and curative age of congenital muscular torticollis.Methods138 children accepted conservative and/or operative treatment according to different ages.A prospective study had been made. ResultsConservative treatment was given to 82 children,71 of them were <1-year-old. 49 of them were follwed up and had satisfactory outcome.7 patients who was >1-year-old had operations later because of asymmetry of head.63 children received operations.56 of them were followed up over one year,47 cases had good-looking,the outcome of other 9 was not satisfied.Conclusions Operative intervention was necessary for >1-year-old patients. Manipulation was suitable way to cure <1-year-old patients.It was the best operating time at the age of 1-5 years old.
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@#ObjectiveTo explore methods and prevent complication with transection and intra fixation of breastbone and costa to treat pectus excavatum in children. Methods35 cases cured with transection and intra fixation of breastbone and costa to treat pectus excavatum were analyzed. Results35 cases have good contours in one operation. 1 case's pleurisy was wounded slightly. 2 case complicated with lung infection. Conclusionstransection and intra fixation of breastbone and costa is a simple methods to treat pectus excavatum in children and it has little complication..It's orthopaedic is obvious. It is worth to spread.
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Objective To explore a new therapy for Hirschsprung's disease ( HD). Methods From Jul 2001 to Aug 2003,72 cases underwent transanal pull-through plus partial myoctomy of internal sphincter. Rectoanal manometry was done for 27 patients perioperatively. Forty-eight patients were followed-up and evaluated according Reding standard. Results Pre-and post-operative rest pressure was (38 ?8) mm Hg and (22?6 ) mm Hg respectively (P