显微手术治疗儿童下丘脑错构瘤六例及文献回顾

目的探讨显微手术治疗儿童下丘脑错构瘤的适应证、安全性及疗效。方法回顾性分析空军军医大学第二附属医院2016—2020年经显微手术治疗的6例儿童下丘脑错构瘤临床资料。男5例,女1例,平均年龄4.3岁;2例表现为性早熟,4例表现为痴笑性癫痫,其中1例为Pallister-Hall综合征。6例均经影像学检查确诊。罗氏分型:Ⅱ型5例,Ⅳ型1例。手术方式:经翼点入路5例,经纵裂-胼胝体-穹隆间入路1例。结果所有病例术后无尿崩、视力视野障碍等相关并发症。术后影像学复查见全切3例,次全切2例,大部分切除1例。随访2个月到4年,4例痴笑性癫痫术后未再次发作。2例性早熟病例身高增长速度较术前明显减缓,骨龄基本同术前,第二性征略有进展。结论儿童下丘脑错构瘤临床较为罕见,以痴笑性癫痫、中枢性性早熟为主要临床症状,前者手术治疗安全有效,后者宜首选药物治疗。     

小儿下丘脑错构瘤的诊断和治疗

目的 探讨小儿下丘脑错构瘤的诊断和显微外科手术治疗.方法 回顾分析1998年1月至2006年12月收治的5例下丘脑错构瘤,男2例,女3例,平均年龄44.4个月.2例主要表现性早熟,1例主要表现为痴笑样癫痫,2例表现为性早熟和痴笑样癫痫,MRI表现为脚问池和鞍上池等信号肿物,无强化,最大直径大小为13.9～21.0 mm.5例患儿均采用右翼点入路显微手术切除错构瘤.结果 5例下丘脑错构瘤均获全切除,病理证实为下丘脑错构瘤,术后患儿性早熟均停止,复查性激素均下降至儿童期水平;2例癫痫症状消失,1例癫痫症状明显减轻,无其他术后并发症.结论 小儿下丘脑错构瘤的症状明显,影像特殊,诊断明确;首选为显微手术切除错构瘤,全切后可治愈.     

儿童下丘脑错构瘤的临床及影像学表现

目的：为了提高对下丘脑错构瘤影像学和临床表现的认识,该文主要研究下丘脑错构瘤的临床特点、CT及MRI影像学表现。方法：回顾性分析了38例下丘脑错构瘤的CT及MRI影像资料。其中男25例、女13例,发病年龄1个月至15岁。38例均进行了MR扫描,10例又进行了CT扫描。38例全部经手术、病理证实。结果：主要临床表现为痴笑样癫癎、性早熟、其他类型癫癎（包括癫癎大发作及肢体抽搐等）和智力障碍。其中首发症状为痴笑样癫癎17例,性早熟13例,其他类型癫癎8例。影像学表现为脚间池占位,CT为等密度,MRI为等或稍长T1等或稍长T2信号影,增强扫描病变无强化。临床表现与病灶的大小以及是否有蒂无明显关系。结论：儿童出现性早熟和/或痴笑样癫癎,影像表现为脚间池占位,CT等密度,MRI等或稍长T1等或稍长T2信号影,增强扫描病变无强化,此时应考虑到下丘脑错构瘤。［中国当代儿科杂志,2009,11（5）：364-366］     

伽玛刀治疗儿童下丘脑错构瘤24例

目的 探讨伽玛刀治疗儿童下丘脑错构瘤(HH)的疗效.方法 回顾性分析本院伽玛刀治疗中心1997-2006年使用OUR-XGD型旋转式伽玛刀治疗24例HH患儿的临床资料.男9例,女15例;首发症状为性早熟19例(79.17%),痴笑性癫癎5例(20.83%);有手术史6例(25%);诊断依据性早熟、痴笑性癫癎和MRI表现;症状随访24～91个月;影像学随访24～82个月;以肿瘤体积缩小和不变为肿瘤局部控制;治疗边缘剂量9～18 Gy,中心剂量26.7～36.0 Gy,等剂量曲线45%～55%.结果 24例中症状改善6例(25%),痴笑性癫癎改善优于性早熟;19例肿瘤体积(79.17%)缩小,5例(20.83%)不变,肿瘤控制率达100%.无1例出现视神经损伤.结论 伽玛刀可作为HH患儿主要治疗手段之一,对于术后残余肿瘤更具意义.     

儿童颞叶症状性癫痫的手术治疗

目的：探讨儿童颞叶症状性癫痫的原因和临床特征并总结手术治疗经验。方法：对我院自1994年1月至2002年8月手术治疗的17例儿童颞叶症状性癫痫，男11例，女6例，年龄3～14岁，平均9岁，均以癫痫为首发症状，病程13d到7年，平均为4．3年。手术方式为：单纯病灶切除6例，病灶加前颞叶切除11例，其中13例再行皮层脑电极检查，12例仍发现致病波，则加大病灶切除范围和／或病灶周围皮质区多处软膜下横纤维切断。结果：17例患儿无手术死亡及并发症。随访16例，时间为6个月～7年，术后症状完全消失者12例(占70．6％)，术后癫痫发作显著减少者(发作频率不到原来的25％)3例(17．6％)，术后癫痫发作减少者(发作频率不到原来的50％)2例(11．8％)。结论：在皮层脑电极监测下，多种手术方法结合治疗颞叶症状性癫痫患儿安全有效。     

儿童胚胎发育不良性神经上皮肿瘤

目的 探讨儿童胚胎发育不良性神经上皮肿瘤(dysembryo plastic neroepithelial tumor,DNT)的临床特点、诊断及手术治疗.方法 回顾性分析了2003年1月至2008年5月手术治疗的13例儿童胚胎发育不良性神经上皮肿瘤的临床和病理资料.结果 患儿以难治性癫痫为主要临床表现.本组患儿均接受了显微外科手术治疗,其中全切10例,近全切3例.磁共振成像示皮质或皮质下低T1、高T2信号,病灶边界清晰,无水肿及占位效应.肿瘤病理学检查可见特异性胶质神经元成分.随访13例患儿中2例于术后1年内出现癫痫发作,近全切的1例表现为强直性发作,全切的1例为失神性发作,均与术前发作形式相同,但发作频率明显减少(频率减少≥75%),术后随访癫痫发作控制满意.结论 DNT为良性肿瘤,手术治疗效果好.准确诊断对本病的治疗有重要意义.     

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目的总结儿童下丘脑、垂体区肿瘤临床特点。方法对四川大学华西第二医院1998～2004年15例下丘脑、垂体区肿瘤病例进行分析。结果15例患者中,年龄4～15岁,男5例,女10例,以先后或同时出现的多饮多尿、生长迟缓、软弱、缺乏食欲、性早熟等为主要表现,头部MRI检查15例下丘脑、垂体区肿瘤,其中12例颅咽管瘤,2例下丘脑错构瘤,1例松果体瘤。结论下丘脑、垂体区肿瘤以颅咽管瘤最常见,临床以多种内分泌功能紊乱为主要表现;儿童下丘脑、垂体区肿瘤就诊时可能没有颅内高压症状;头部MRI是确诊下丘脑、垂体区肿瘤最重要的检查手段。     

儿童偏头痛与癫痫相关性的研究

目的 探讨儿童偏头痛与癫痫的相关性及鉴别诊断。方法 对32例偏头痛患儿（其中9例并临床癫痫发作）进行脑电图及临床分析。结果 32例中脑电图异常18例，其中14例为偏头痛，异常率为43.75%,4例枕叶癫痫，3例普通型头痛伴癫痫发作，脑电图3次复查正常，2例复杂型偏头痛伴临床癫痫发作，脑电图均表现异常。结论 儿童偏头痛常常表现脑电图非特异性异常，甚至痫样放电，但以脑电图异常不能代替临床诊断，头痛性癫痫的诊断必须结合临床表现和脑电图检查结果综合判定，并与偏头痛进行鉴别。某些偏头痛可以伴癫痫发作，两者交叉重叠。极易混淆，对头痛性癫痫的诊断应慎重。     

儿童颈椎外伤的诊断及治疗

目的 探讨儿童颈椎外伤的临床特点、诊断、治疗方法及难点和要点.方法 回顾性分析我院1995年1月至2005年12月期间治疗的儿童颈椎外伤病例的临床资料,同时检索分析国内外关于儿童颈椎外伤的文献.结果 11例患儿纳入本研究,其中男8例,女3例;平均年龄(12.1±4.0)岁;受伤机制包括坠落伤(3例),运动伤(3例),车祸伤(2例),直接暴力伤(2例),因娱乐致伤(1例).受伤部位分布为C1-34例,C4-77例.4例合并神经系统症状,其中1例为无骨折脱位型脊髓损伤.4例接受手术治疗,7例行保守治疗.结论 儿童颈椎外伤相对少见,其在临床表现、影像学检查及治疗方法上均存在与年龄相关的特点.熟悉这些特点对儿童颈椎外伤患儿的诊断及治疗至关重要.     

婴儿阑尾盲肠多重套叠并急性阑尾炎一例并文献复习

目的 探讨婴儿期阑尾盲肠多重套叠并急性阑尾炎的临床病理和诊治方法.方法 回顾性分析1例阑尾盲肠多重套叠并急性阑尾炎8月龄婴儿的临床资料,并通过PubMed、ProQuest、中国知网(CNKI)、万方和维普数据库中检索2016年5月前的报道并复习相关文献资料,对各类儿童阑尾套叠进行系统性综述.结果 本例阑尾盲肠多重套叠并急性阑尾炎经手术诊断,整复套叠后切除阑尾并经病理证实,术后顺利康复.共检索到215例阑尾套叠中儿童52例,阑尾盲肠套叠136例,仅3例阑尾自身套叠中有2例为儿童;检索到肠套叠并阑尾炎66例,儿童59例,其中小于1岁的患儿27例,回结型肠套叠43例,此外,检索到回肠盲肠型套叠7例和盲肠盲肠型套叠并阑尾炎1例均为成人患者,所有病例均行手术治疗而痊愈.儿童阑尾盲肠多重套叠并急性阑尾炎者未见报道.结论 婴儿阑尾盲肠多重套叠并急性阑尾炎罕见,如套叠未导致回盲瓣排空完全梗阻则无典型症状体征,诊断困难,易误、漏诊.因此,对经保守治疗不能解除肠梗阻且不能解释其原因时,只要B型超声提示“同心圆征”或“套筒征”就应果断行手术探查以明确诊断并及时治疗.     

Perforation of Meckel's Diverticulum by a Peanut Presenting as a Mesentery Abscess

Background

Perforation of Meckel''s diverticulum by a foreign body in children is rarely reported and is usually associated with localized or generalized fibropurulent peritonitis.

Case Presentation

The authors encountered such a case in a 4-year-old boy with perforation of Meckel''s diverticulum by a piece of peanut presenting as a mesentery abscess. The diverticulum was wide-based and histology showed a transmural perforation at the tip of the diverticulum.

Conclusion

Local inflammation due to irritation of the foreign body and progressive pressure necrosis at the tip of the diverticulum may be the pathogenesis of mesentery abscess. A search of the English literature did not reveal any similar case.     

Performance of QuantiFERON-TB testing in a tuberculosis outbreak at a primary school

This study compared the effectiveness of the QuantiFERON-TB Gold (QFT) assay with the Mantoux tuberculin skin test to detect Mycobacterium tuberculosis infection in 29 children during a school outbreak of tuberculosis. Of the 21 children with M. tuberculosis infection, 11 had a radiograph suggestive of the infection. The QFT assay was positive in all 21 of the children, and the Mantoux test was negative at first testing in 2 children (1 of whom was the sentinel case). The findings demonstrate that the QFT test is extremely useful in accurately identifying infected and uninfected children, permitting rapid intervention.     

Optimal management of phenylketonuria: a centralized expert team is more successful than a decentralized model of care

OBJECTIVE: To compare phenylketonuria (PKU) management by a centralized, expert team in the Province of Nova Scotia (NS) with the decentralized approach in New Brunswick (NB). STUDY DESIGN: Retrospective chart review documented frequency of outpatient visits, phenylalanine (Phe) concentration, and medical formula use. Structured telephone interviews with the 8 regional NB dietitians (NB-D) documented their knowledge and support in PKU management. Patients with PKU (n=108; age, birth to 42 years) reside in NB (n=69) and NS (n=39). More were lost to contact in NB than in NS (9/69 vs 1/39) and more were completely off diet in NB than in NS (24/60 vs 1/38, P=.05). All 15 children <2 years old followed by a PKU team in either NS or Saint John, NB had optimal Phe levels. Children 2 to 12 years of age in NS had better Phe control and more medical visits than in NB (P <.01). Older patients had more episodes of elevated Phe levels (P=.01). Formula was dispensed in appropriate yearly amounts to 52% in NB and >95% in NS. Mental handicap or borderline intelligence was common in both NB (44%) and NS (42%). All NB-D wished additional specialized medical, nursing, or social work assistance. CONCLUSIONS: PKU management appears to be more effective with an expert, coordinated team approach.     

Adrenal hemorrhage: a rare cause of severe neonatal jaundice

Adrenal hemorrhage in newborns is not uncommon. The clinical presentation may be very protean and the diagnosis difficult. Ultrasonography is useful in making the diagnosis. A patient presenting with severe neonatal jaundice due to adrenal hemorrhage is described.     

The theory of community based health and safety programs: a critical examination

This paper examines the theoretical underpinning of the community based approach to health and safety programs. Drawing upon the literature, a theory is constructed by elucidating assumptions of community based programs. The theory is then put to test by analyzing the extent to which the assumptions are supported by empirical evidence and the extent to which the assumptions have been applied in community based injury prevention practice. Seven principles representing key assumptions of the community based approach to health and safety programs are identified. The analysis suggests that some of the principles may have important shortcomings. Programs overwhelmingly define geographical or geopolitical units as communities, which is problematic considering that these entities can be heterogeneous and characterized by a weak sense of community. This may yield insufficient community mobilization and inadequate program reach. At the same time, none of the principles identified as most plausible appears to be widely or fully applied in program practice. The implication is that many community based health and safety programs do not function at an optimum level, which could explain some of the difficulties in demonstrating effectiveness seen with many of these programs.     

Traumatic aneurysm of the ascending aorta in a child

A case of traumatic aneurysm of the ascending aorta in a child is reported. This complication must be suspected in a child who sustains severe injury from closed thoracic trauma and develops an abnormally wide anterior mediastinum. A mechanism for ascending aortic rupture and aneurysm is proposed. Aortography is essential when aortic injury and its complications are suspected.     

Application of a Live Varicella Vaccine to the Immunosuppressive Children and the Incibence of a Case of Herpes Zoster after Vaccination

A live varicella vaccine was applied to 13 susceptible children to varicella virus. Three were receiving steroid therapy, and one of them was treated with both steroid and anticancer drugs. Immunosuppressive therapy and anticancer medication were not suspended before and after vaccination. Serological responses were observed in 11 of 13 vaccinated children by fluorescent antibody to membrane antigen (FAMA) test 6 to 7 weeks after Vaccination. Mild rash appeared as only a clinical reaction in 3 of the vaccinated children. However, 3 and a half months later, a vaccinee with acute myeloblastic leukemia, developed herpes zoster. Our observation suggested a possibility of contracting zoster in immune compromised hosts after vaccination with a live varicella vaccine.