Research progress on the prognosis of patients with various types of Methylmalonic acidemia / 中华医学遗传学杂志

Methylmalonic acidemia (MMA) is a series of rare inherited organic acid metabolic disorders with variable and nonspecific clinical manifestations, in particular neurological symptoms such as vomiting, lethargy, etc. Even with timely treatment, patients may still have various degrees of neurological complications and can even die. The prognosis is mainly related to the type of genetic variants, level of metabolites, newborn screening, onset of disease and early initiation of treatment. This article has reviewed the prognosis of patients with various types of MMA and factors that may affect it.

Reoperation for recurrent pituitary apoplexy after the successful initial transnasal-sphenoidal approach surgery / 中华显微外科杂志

Objective To discuss the reoperation method for recurrent pituitary apoplexy after the successful initial transnasal-sphenoidal surgery.Methods Twenty-one patients with pituitary apoplexy were found recurrence after previous tumor resection via transsphenoidal surgery in 9 months to 5 years.In the reoperation,transsphenoidal approach would be still used.The anterior wall of sphenoid sinus and the sellar floor were located accurately,and then the remnants of them were removed as large as possible to expand the range of bone window.Next,the intrasellar hematoma was cleared away gently,and the residual tumor was removed progressively and thoroughly to make sure the sellar diaphragm subsiding fully and finally removed the whole tumor.Results Most of recurrent tumors were found to be of soft texture with rich blood supply,and 17 cases were still apoplexy.In 18 cases of the 21 patients,total resection was achieved.However,for the other 3 cases with tumor aggressive growth,only massive resection was achieved,and underwent stereotactic radiosurgery postoperatively.Postoperative clinical symptoms were alleviated in 15 cases,whose hormones were also decreased.Temporary diabetes insipidus and hypopituitarism appeared in 5 and 3 cases respectively,but all of these postoperative complications were relieved after symptomatic treatment.Two cases of intraoperative cerebrospinal fluid leakage were resolved by packing the cavity with muscle and fat flaps.All cases were followed up for 3 months- 5 years,residual tumor enlarged in only 1 case of massive resection,which umderwent transsphenoidal surgery for the third time,no other cases recurred.Conclusion Operation via the transsphenoidal approach of recurrent pituitary apoplexy after successful initial transsphenoidal surgery could achieve high total resection rate.While the accurate locating,full expansion of the saddle bone window,and repeatedly scraping the apoplexy tumor to get a full subsidence for the sellar diaphragm,are the key points to remove tumors totally as well as to improve the symptoms.

Application of neurophysiological monitoring and microsurgical technique in acoustic neurinoma resection / 中华显微外科杂志

Objective To investigate the application of neurophysiological monitoring and microsurgi-eal technique in acoustic neurinoma resection, exploring the significance of neurophysiological monitoring in facial and auditory nerve reservation of acoustic neuronma microsurgery. Methods Accompanied with EMG and BAEP nerve monitoring, 113 patients harboring acoustic neuroma were treated surgically by the subocipi-tal retrosigmoid approach for reserving facial and auditory nerve. The facial nerve was stimulated to evaluate its function during late-operation. Postoperative facial and auditory nerve function were valuated in all the postop-erative following up. Results All of them were treated microsurgically via the suboccipitai retrosigmoid ap-proach. Total tumors resection was achieved in 102 cases (90.3%), subtotal resection in 6 cases(5.3%) and partial resection in 5 case (4.4%). The facial nerve was preserved anatomically in 98 cases (86.7%), the functional valuation of facial nerve according to the House-Brakman (H-B) postoperatively: 86 cases (76.1%) in class Ⅰ -Ⅱ , 12 cases(lO.6%) in class Ⅲ-Ⅳ and 15 cases (13.3%) in class Ⅴ-Ⅵ. The acoustic nerve was preserved anatomically in 40 cases (35.4%). It denoted the good function of facial nerve responsing sensi-tively to electrostimulation ≤ 4mA at the end of operation. Conclusion Assisted with the intraoperative electrophysiological monitoring techniques, it would be greatly facilitate the preservation of facial and auditory nerve in acoustic neuroma resection. Simultaneously, it would valuate the functional convalescence by late-op-erative electrostimulation in the postoperation.

Microsurgery and strategies of patients with pituitary adenomas through single-nostril transsphenoidal approach / 中华显微外科杂志

Objective To analysis the therapeutic effect of microsurgery through single-nostril transsphenoidal approach on pituitary adenomas and to study the surgical skill and postoperative complications about microsurgery for pituitary adenomas.Methods The clinical manifestations,pathological type, removal percentage,postoperative complications and follow-up data of 241 cases with pituitary ademoma by microsurgery through single-nostril transsphenoidal approach were analysed retrospectively.Results 171(7 1%)cases were achieved total tumor removal,28(11.6%)cases were achieved subtotal tumor removal,26(10.7%)cases were achieved most tumor removal and 16(6.6%)cases were achieved partial tumor removal.Most of patients achieved better results,however there were 38(15.8%)eases with diabetes insipidus,12(4.9%)cases with transient worse sight,4(1.6%)cases with cerebrospinal fluid leakage,2(0.8%)cases with oculomotor paralysis,and no death.The postoperative complications were cured and outcome was considered as good.The follow-up period was 1-36 months in 241 patients and the clinical symptoms were improved by different degrees.Conclusion Pituitary adenomas can be treated by microsurgery through single-nostril transsphenoidal approach due to the time of operation shorten,the postoperative complications.