Adenomyomatosis of the gallbladder resembling honeycomb in a child

Adenomyomatosis of the gallbladder is believed to be an uncommon pathologic condition of the gallbladder in childhood. Only three pediatric cases have been described in the literature up to now. Honeycomb gallbladder has been described in two adult patients; no patients have been reported in childhood until now. To the best of our knowledge, we report here the first case of adenomyomatosis of the gallbladder which resembled honeycomb, in a 9-year-old girl presented with recurrent abdominal pain. The diagnosis was made by ultrasound, and confirmed by magnetic resonance cholangiopancreatography and finally cholecystectomy. In conclusion, ultrasound scanning performed more generally in children presenting with recurrent abdominal pain might lead to accurate diagnosis of adenomyomotosis of the gallbladder during childhood.     

Inflammatory fibroid polyps in children:A new case report and a systematic review of the pediatric literature

AIM: To study that inflammatory fibroid polyps(IFPs) in children are extremely uncommon tumors that may occur throughout the gastrointestinal tract. METHODS: A systematic review of the pediatric literature and a report of a new case of IFP is also pres-ented. The Pub Med database was searched for original studies on pediatric IFPs since 1960, according to "Preferred reporting items for systematic reviews and meta-analyses" guidelines for systematic reviews. RESULTS: Five studies were finally enclosed, encompassing 6 children with IFPs(mean age 64 mo). Tumors were located in the stomach(2 patients), in the small bowel(2 patients), in the rectum(1 patient) and in the colon(1 patient). Open surgery was performed in all patients and complete excision of the mass was achieved in all cases. All patients are alive and free of symptom. Authors described a further case of a 3-year-old boy with a large duodenal IFP, in whom the tumor was removed by "en block resection". The presence of IFP throughout the gastrointestinal tract and its variable clinical appearances make it difficult to diagnose. An accurate pre-operative assessment is fundamental in order to differentiate IFP from other more aggressive gastrointestinal tumor, enabling unnecessary demolitive surgery. CONCLUSION: When complete resection of the IFP is achieved, the prognosis is excellent.     

Gallbladder disease,primary cholelithiasis,or gallbladder hydrops: review of 32 children

Gallbladder disease is generally considered uncommon in childhood, but in recent years the frequency seems to be increasing. Therefore, a review was made of children appearing with gallbladder disease during the past 10 years at the Department of Pediatric Surgery, St. Göran's Hospital, Stockholm. There were 32 patients, 15 boys and 17 girls. Two-thirds of the cases were diagnosed in 1988 or later. One-half were younger than 8 years; 7 were 2 years old or younger. Hemolytic disease was found in 2/32 patients. One patient had received total parenteral nutrition, but only for a period of 10 days. In 6/32 children the symptoms were interpreted to be caused by gallbladder dysfunction, either as intermittent hydrops or a defect of emptying after a fatty meal. One of these 6 also had a stone. Surgery was performed in 22 children: cholecystectomy in 15 and cholecystotomy with stone extraction in 7. Analysis of 14 stones showed that pigmentary stones were most common. We conclude that gallbladder disease in children seems to be increasing. The preoperative examination should include both ultrasound and cholecystography. We also find that stone extraction is an adequate procedure if there is good gallbladder function and no sign of cholecystitis. Correpondence: M.-L. Molander     

Adamantinoma in childhood: report of six cases and review of the literature

Background Adamantinoma is a rare slow-growing malignant bone tumour. Objective To describe the imaging appearances of six childhood cases and review the published literature. Materials and methods The database of the Working Group on Paediatric Oncology, Academic Medical Centre/Emma Children’s Hospital Amsterdam, was searched for cases of adamantinoma. Additionally a literature study was performed to identify cases of adamantinoma in childhood. Results We identified six local cases of adamantinoma of the long bones, two boys (age 3 and 8 years) and four girls (mean age 8.8 years, range 3.0–14.0 years). The location of the tumour was the tibia in five and the tibia and fibula in one patient. In two patients initially a different diagnosis was made, which led to a delay in appropriate treatment. None of the children showed pulmonary metastases and all underwent total gross resection. On follow-up (mean 6.1 years, range 1.6–12.0 years) all children remained disease-free. Besides a discussion of our six patients, imaging features, histopathology, surgical approach and a literature review of childhood adamantinomas is presented. Conclusion Although the incidence of adamantinoma is low, it is important to recognize this rare bone tumour, since in the early stages of the disease adequate treatment will result in an excellent prognosis. Part of this paper was presented at the Annual Meeting of the Radiological Society of North America, 2004, Chicago, USA.     

Oral teratoma: a case report and literature review

Teratomas are true neoplasms composed of tissues from all three germinal layers. They have an unknown origin and eccentric microscopic appearance. Teratomas arising from the oral cavity are rare in the newborn; only 13 cases have been reported in the literature. We describe a male neonate with an oral teratoma originating from the anterior hard palate that was successfully treated with surgery.     

儿童创伤性胸主动脉假性动脉瘤一例报道及文献复习

目的 探讨创伤导致的儿童胸腹主动脉损伤的诊疗方法,以增加大家对这种临床罕见病的认识.方法 报道1例4岁女孩的创伤性胸主动脉瘤的诊断及治疗,并通过PubMed检索Medline数据库、检索Springer Link和Google Scholar等检索平台、及Embase、Ovid、中国生物医学文献数据库(CBM)、中国知网(CNKI)、万方和维普数据库,对儿童创伤性胸腹主动脉损伤的中英文文献进行系统性的综述.结果 一共43例年龄在14岁以下的创伤性胸腹主动脉损伤的患儿纳入本研究.交通伤26例是最常见的致伤机制.7例(16.3％)患儿伤后未及时诊断或初诊时漏诊.6例患儿随访中存在不同程度的后遗症.主动脉损伤以假性动脉瘤(20例)最常见,其次为内膜剥离(7例)及主动脉夹层(7例).27例患儿行开放主动脉修复术,7例行血管内治疗,7例患儿保守治疗,2例患儿行急诊手术,并在围手术期死亡.结论 血流动力学不稳或主动脉完全破裂的患儿需要急诊手术.而主动脉内膜环形剥离患儿发生并发症的可能性较高,需要限期手术.没有明显症状的内膜部分剥离或是迟发型假性动脉瘤的患儿可以保守治疗,但需要密切观察,如果假性动脉瘤进展,则需要手术.     

Transverse testicular ectopia: case report and review of the literature

A rare case of transverse testicular ectopia in a 2-year-old child presented as bilateral undescended, nonpalpable testes at the Children's Hospital, Banaras Hindu University, Varanasi. Because of its rarity and other interesting features, it is reported with a brief review of the literature. Offprint requests to: A. N. Gangopadhyay     

儿童过敏性肺炎1例并文献复习

目的 总结儿童过敏性肺炎的临床、影像学和病理学特点。方法 对病理确诊的1例9岁6个月的过敏性肺炎患儿的临床、影像学和病理学资料进行总结,并复习文献进行讨论。结果 以咳嗽4个月、气促为主要表现,无咳痰、无三凹征,肺内未闻及啰音,无杵状指趾。肺功能示混合性通气功能障碍。肺部高分辨CT表现为弥漫的细小结节影,以上肺为重,肺组织病理为细支气管炎、细支气管周围的间质淋巴细胞炎症和间质的多核巨细胞,无纤维化和结核结节,可见大量的泡沫细胞。脱离环境并予甲泼尼龙2 mg·kg-1·d-1治疗2周后,患儿咳嗽、气促症状有所减轻,肺功能有所改善,肺部CT的小结节影明显改善。糖皮质激素治疗2个月后呼吸道症状消失,肺部影像学和肺功能恢复正常。结论 过敏性肺炎为无特异的沉淀抗体的病例,主要依靠肺活检病理细支气管炎、细支气管周围间质炎症和找到非干酪的不典型肉芽肿或多核巨细胞确定诊断。亚急性过敏性肺炎用糖皮质激素治疗有效。     

嗜酸细胞性胃肠炎合并急性胰腺炎1例并文献复习

目的 提高对儿童嗜酸细胞性胃肠炎（EG）的认识。方法 报告1例EG合并急性胰腺炎患儿的诊断和治疗过程,系统检索文献并汇总复习类似病例相关报道,分析总结EG合并急性胰腺炎的临床特征。结果 男,14岁,以“反复腹痛、腹泻1周”主诉入院。初步诊断为急性胰腺炎,入院后查血嗜酸性粒细胞（EOS）0.62,血清IgE 393 U·mL-1,骨髓细胞学检查示EOS比例增加,内镜下显示胃肠道黏膜慢性炎症,病理活检显示十二指肠黏膜EOS浸润,诊断为EG。经泼尼松抗炎和抗过敏药物等治疗1周后病情缓解,血EOS降至正常,随访2年患儿病情稳定无反复。在万方、中国知网、EMBASE和PubMed等数据库系统检索相关文献11篇,汇总EG合并急性胰腺炎14例（儿童病例4例）,均以急性胰腺炎表现起病而误诊,以腹痛、恶心和呕吐等非特异性临床表现为主,外周血EOS均明显升高,腹腔积液EOS比例对诊断EG合并急性胰腺炎有重要提示意义,14例均有不同程度的胃、十二指肠黏膜异常改变。13例明确诊断后接受糖皮质激素治疗预后良好;1例手术切除胰腺后复发,诊断EG后予糖皮质激素治疗缓解。结论 EG合并急性胰腺炎罕见,对不明原因急性胰腺炎,且伴血EOS增多或常规抑酶抑酸治疗后临床转归差者,应警惕EG可能,完善内镜病理活检可以明确诊断。     

Varicella arthritis in childhood: a case report and review of the literature

Nonbacterial arthritis is a rare but well-recognized complication of acute varicella in children. Reported cases usually described monoarthritis of the knee that occurs at the onset of the rash or shortly after. Herein, we describe a case of arthritis of the hip that occurred in a 4-year-old girl 6 days before the onset of rash. The presence of varicella-zoster virus DNA in synovial fluid was confirmed by real-time polymerase chain reaction. A review of the literature reported 26 previous cases of aseptic arthritis due to varicella infection among children.     

DiGeorge综合征伴甲状腺功能亢进1例并文献复习

目的探讨DiGeorge综合征(DGS)合并甲状腺功能亢进(甲亢)患儿的临床特点,提高对该病的认识。方法报告1例DGS合并甲亢患儿的症状、辅助检查结果、外周血基因组DNA染色体芯片结果、治疗和随访情况;在中国期刊全文数据库(CNKI)和PubMed中检索DGS合并甲亢患儿的文献,检索时间为建库至2017年5月31日,总结DGS合并甲亢患儿的临床特征及其与遗传学异常的关联。结果患儿女,12岁,因"1年内抽搐发作2次,发现甲状腺功能异常4月"于2016年12月就诊于浙江大学医学院附属第一医院儿科。患儿有低钙抽搐、甲亢、身材矮小、智力异常、贫血和慢性中耳炎等多系统异常。染色体芯片检测结果显示22q11.21微缺失,缺失2 512 kb,基因组中位置18919095-21431174(hg19),诊断为DGS伴甲亢,予甲巯咪唑片(bid,起始为10 mg,2 d后改为5 mg)和补钙等对症治疗,血钙至正常范围后出院并继续口服甲巯咪唑片(5 mg,qd),出院3、6个月电话随访无抽搐发作。在PubMed中共检索到10篇英文文献报告了17例DGS合并甲亢患儿,病情不一,累及系统较多。结论 DGS临床表现多样,累及系统广泛,易误诊、漏诊和迟诊。对原发性甲状旁腺功能减退患儿,建议行染色体芯片分析并评估甲状腺功能。     

儿童胸腺淋巴上皮瘤样癌1例报道并文献复习

目的加强对儿童胸腺淋巴上皮瘤样癌(LELC)的认识。方法对1例诊断为儿重胸腺LELC患儿临床资料进行回顾性分析,并结合文献报道的10例儿童LELC进行分析。结果惠儿,12岁,男,表现为夜间睡眠不宁、胸闷;外周血白细胞升高,胸部CT示右中上纵膈可见类圆形软组织密度影;手术切除大部分肿物,病理报告为胸腺LELC;化疗后临床症状消失,6个月后原位复发。近20年来文献报道胸腺LELC患儿共10例,其中男6例,女4例。起病症状为胸痛、呼吸困难者6例,肥大性骨关节病(HOA)5例,证实EBV感染6例;8例在发病1年左右死亡。结论胸腺LELC是儿童时期罕见的恶性肿瘤,常合并EBV感染,存在免疫功能紊乱,临床特点为咳嗽、胸闷、胸腔巨大占位和HOA,手术难以完整切除,恶性程度高,预后极差。     

儿童肱动脉假性动脉瘤一例并文献复习

目的:报道1例儿童肱动脉假性动脉瘤的诊疗,并回顾相关文献总结此类疾病的病因、诊治及预后。方法:回顾性分析了2019年6月1例16天新生儿的左上肢肱动脉假性动脉瘤的临床资料。本例患儿出生后6d发现逐渐增大的左肘部包块。B型超声、左上肢MRI及CTA提示左肱动脉假性动脉瘤。经局部加压包扎治疗,包块持续增大并出现表面皮肤坏死...     

Asymptomatic gastric duplication in a child: report of a new case and review of the literature

Antral duplication cyst with an antral mucosal lining accounts for 2.2% of all gastric duplications, which represent 4–8% of all alimentary tract duplications. They usually become symptomatic before 2 years of age. We report a 6-year-old child with no previous history of symptoms who was admitted to our hospital because of frequent vomiting and acute abdominal pains for the previous 2 days. Abdominal examination revealed a mass in the epigastric area.     

Transient erythroblastopenia of childhood in siblings: case report and review of the literature

Transient erythroblastopenia of childhood is characterized by anemia due to decreased production of red blood cell precursors. It is almost always self-resolving and requires clinical intervention only in severe cases. This article describes 2 cases in half-siblings diagnosed approximately 10 years apart. A review of the literature identifies 11 other sibling pairs. Our case suggests an autosomal dominant pattern of inheritance. To date, the gene involved in the development of transient erythroblastopenia of childhood has not been identified.     

Odontogenic tumors in childhood - case report and review of literature

BACKGROUND: Odontogenic tumors are a heterogenic group of neoplasias of the oral cavity, whose biological behaviour reaches from true hamartomas to highly malignant tumors. PATIENT: We describe the case of a 4 year old caucasian girl presenting with eruption disturbance of a primary tooth and corresponding maxillary tumor. After surgical removal of the mass, histological examination classified the tumor as ameloblastic fibroodontoma. This very rare neoplasm is of odontogenic origin. DISCUSSION: Following our case report, the variety of odontogenic tumors regarding tumor biology, clinical symptoms and therapeutic options in childhood are discussed. CONCLUSION: In case of complaints or clinical symptoms paediatricians should take the possibility of such neoplasias into consideration.     

Torsion of an indirect hernia sac within a hydrocele causing acute scrotum: case report and review of the literature

We report a case of acute scrotum in a 2-year-old child caused by torsion of an indirect hernia sac within a hydrocele. This is an extremely rare cause of acute scrotal swelling and pain in the pediatric population, with this case representing only the eighth instance reported in the literature. Despite the scarcity with which this diagnosis has been encountered, clinicians should include torsion of an indirect hernia sac on the differential diagnosis for acute scrotum in a child.     

儿童Wernicke's脑病1例并35例文献复习

目的 探讨儿童Wernicke's脑病临床特点以及诊断与治疗,提高对该病的认识.方法 总结1例以严重脓毒症为首发表现的儿童Wernicke's脑病的临床表现、诊断及治疗特点,并对国内外近10年报道的35例儿童病例进行文献复习.结果 包括本例患儿共36例,男22/36例,年龄2个月～16岁.除本例外均有基础疾病:喂养不当25/35例、长期呕吐5/35例、免疫抑制治疗4/35例、长期完全胃肠外营养(未添加维生素)3/35例以及神经性厌食1/35例;有典型临床"三联征(精神状态改变、眼部体征以及共济失调)"6/36例,其他临床表现:意识障碍24/36例、感染22/36例、神经病理征及肌张力改变18/36例、惊厥17/36例,发育迟缓4/36例、生长停滞2/36例.行脑脊液常规检测31/36例,异常7/31例(蛋白略增高);检测脑脊液乳酸水平4/36例,均增高;血清乳酸水平检测7/36例、6/7例增高;血清丙酮酸检测4/36例,均增高;硫胺索焦磷酸盐活力(TPPE)检测9/36例,均增高;血清硫胺素水平检测2/36例、1/2例降低;行颅脑CT检查20/36例,16/20例双基底节区低密度、1/20例弥漫性脑皮质萎缩;行颅脑MRI检查13/36例,均表现为双侧对称性乳头体、基底节区异常信号、7/13例还表现中脑被盖、中央导水管及Ⅲ、Ⅳ脑室周围白质区异常信号.诊断方法:在行颅脑MRI的13例中,根据MRI确立诊断12/13例、"三联征"+MRI确立3/13例、根据尸检1/13例;行TPPE和(或)乳酸测定11例中,确立诊断9/11例.初始治疗:33/36例经胃肠外补充硫胺素、1/36例给药方式不详、1/36例经口服给药、1/36例未补充;硫胺素剂量:29/35例每日100 mg、3/35例剂量不详、2/35例每日50 mg、1/35例每日600 mg.初始治疗后34/35例临床症状在24 h～1周内缓解,1/36例无反应死亡.存活34例中,19例接受随访,2～2.5个月内17/19例完全康复.结论 儿童Wernicke's脑病常无特异性临床表现,误诊率较高,颅脑MRI特征性表现为双侧乳头体、基底节区、中央导水管周围区等对称性异常信号.特征性颅脑MRI异常信号结合临床硫胺素治疗后快速反应有助于临床诊断.早期、及时补充大剂量硫胺素后,多数病例神经学异常可快速改善,预后良好.

Abstract：

Objective Wernicke's encephalopathy (WE) is an acute neuropsychiatric syndrome resulting from thiamine deficiency, which is associated with significant morbidity and mortality. The disorder is still greatly underdiagnosed in children because of either a relatively non-specific clinical presentation in some cases or unrecognized clinical setting The aim of this literature review was to provide knowledge of pediatric WE in an effort to assist in early diagnosis, thereby reducing the morbidity and mortality. Methods The clinical manifestations, characteristic magnetic resonance imaging ( MRI), diagnosis and treatment of one case and the other 35 cases reported in the last decade in children were summarized. Results Thirty-six cases (22 boys and 14 girls, 2-month to 16-year-old) were analyzed. All the other 35 cases except for our case had underlying diseases: improper feeding in 25/35 cases, long-time vomiting in 5/35 cases,immunosuppressive therapy in 4/35 cases, long-time total parenteral nutrition without multivitamin preparations supplementation in 3/35 cases and anorexia nervosa in 1/35 case. The classic triad ( mentalstatus changes, nystagmus and ophthalmoplegia, and ataxia) was seen in 6/36 cases. The other clinical manifestations included consciousness disturbance in 24/36 cases, infection in 22/36 cases, pathological reflex and muscular tension changes in 18/36 cases, convulsion in 17/36 cases, developmental delay in 4/36 cases and failure to thrive in 2/36 cases. Cerebrospinal fluid examination was performed in 31/36 cases,and a slightly raised protein concentration was seen in 7/31 cases. The cerebrospinal fluid lactate levels were detected in 4/36 cases ( all increased ), serum lactic acid levels in 7/36 cases ( 6/7 cases increased ),serum pyruvate in 4/36 cases ( all increased), thiamine pyrophosphate effect ( TPPE ) in 9/36 cases ( all increased), and serum thiamine in 2/36 cases (increased in 1/2 cases). The brain computed tomography (CT) scan was conducted in 20/36 cases and 16/20 cases showed abnormal hypodensity in bilateral basal ganglia, one case revealed diffuse cortical atrophy. The brain MR scan was conducted in 13/36 cases and all the 13 cases revealed symmetrical abnormal signal in bilateral mamillary body and basal ganglia, and 7/13 cases showed abnormal signals in the tegmentum of midbrain, cerebral aqueduct and white matter around the third and fourth ventricles. The diagnosis of WE was confirmed by MR in 12 cases, triad combined with MR in 3 cases, autopsy in 1 case among the 13 cases who underwent MR scan. The diagnosis of WE was confirmed by the TPPE and/or lactate levels in 9/11 cases. The initial thiamine was given by intravenous or intramuscular infusion in 33/36 cases, unknown method in 1 case, orally in 1 case and no thiamine was used in 1 case. The dosage of thiamine was 100 mg daily in 29/35 cases, unknown in 3/35 cases, 50 mg daily in 2/35 cases, 600 mg daily in 1/35 case. 34/35 patients' clinical symptoms improved during 24 hours to 1 week after initial treatment, and 1 case died due to no response to thiamine. Nineteen patients were followed up for 2-2. 5 months and 17 cases recovered completely. Conclusion Wernicke' s encephalopathy can be difficult to diagnose because of a relatively non-specific clinical presentation. The characteristic MRI findings and the dramatic response of neurological signs to parenteral thiamine will assist early clinical diagnosis.Early and timely thiamine supplementation could reverse the clinical features and improve the prognosis in most cases.