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Vol 4, No 1
Vol 4, No 1 February 2008 ISSN 1708-8569
Review articles
Original articles
Clinical summary
Case reports
Fetal cardiac surgerya big challenge in the 21st century
  Zhao-Kang Su, En Chen

Shanghai, China

Author Affiliations: Heart Center, Shanghai Children's Medical Center, Medical School, Shanghai Jiaotong University, Shanghai 200127, China (Su ZK, Chen E)

Corresponding Author: Zhao-Kang Su, MD, Heart Center, Shanghai Children's Medical Center, Medical School, Shanghai Jiaotong University, Shanghai 210027, China (Tel: 86-21-38626161 ext 6888; Email: chien.1944@yahoo.com.cn)

  [Abstract] [Full Text] [PDF]  
Review articles:
Glucagon-like peptide 1 based therapy for type 2 diabetes
  Bao-Sheng Yu, An-Ru Wang

Nanjing, China

Author Affiliations: Department of Pediatric Endocrinology, the Second Affiliated Hospital of Nanjing Medical University, Nanjing 210011, China (Yu BS, Wang AR)

Corresponding Author: Bao-Sheng Yu, Department of Pediatric Endocrinology, the Second Affiliated Hospital of Nanjing Medical University, Nanjing 210011, China  (Tel: 86-25-83062672; Fax: 86-25-58509994; Email: drbsyu@yahoo.com.cn)

Background: Incidence of type 2 diabetes mellitus (T2DM) has increased in young people in recent years and new therapies are required for its effective treatment. Glucagon-like peptide 1 (GLP-1) is a potent blood glucose-lowering hormone produced in the L cells of the intestine. It may be potentially effective in the treatment of hyperglycemia in patients with T2DM. 

Data sources: PubMed database were searched with the terms "GLP-1", "incretins" and "diabetes".

Results: GLP-1 is a product of the glucagon gene, and its secretion is controlled by both neural and endocrine signals. GLP-1 lowers plasma glucose by stimulating insulin and suppressing secretion of glucagons, thus inhibiting gastric emptying and reducing appetite. GLP-1 exerts these actions by the engagement of structurally distinct G-protein-coupled receptors (GPCRs). In patients with T2DM, GLP-1 increases insulin secretion and normalizes both fasting and postprandial blood glucose when given as a continuous intravenous infusion. However, the native hormone is unsuitable as a drug because it is broken down rapidly by dipeptidyl peptidase IV (DPP-4) and cleared by the kidneys. Fortunately, many GLP-1 agonists or analogues and DPP-4 inhibitors have been found or developed, such as exendin-4, exenatide, liraglutide, CJC1131, vidaliptin and P32/98. Clinical trials have shown their therapeutic functions in T2DM with little adverse reaction.

Conclusion: A GLP-1 based therapy will be safe and effective for the treatment of T2DM.

Key words: glucagon-like peptide 1; incretins; type 2 diabetes mellitus

                   World J Pediatr 2008;4(1):8-13

  [Abstract] [Full Text] [PDF]  
Current concept about postoperative cholangitis in biliary atresia
  Yi Luo, Shan Zheng

Shanghai, China

Author Affiliations: Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai 200032, China (Luo Y, Zheng S)

Corresponding Author: Shan Zheng, MD, Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai 200032, China (Tel: 86-21-54524666 ext 2042; Fax: 86-21-64038992; Email: szheng@shmu.edu.cn)

Background: Postoperative cholangitis characterized by fever and acholic stool and positive blood culture is a common and serious complication following Kasai's operation for biliary atresia. The aim of this review was to describe the pathogenesis, clinical manifestations, medical treatment and outcome of postoperative cholangitis. 

Data sources: Articles on biliary atresia retrieved from Pubmed and MEDLINE in the recent 10 years were reviewed.

Results: The pathogenesis of postoperative cholangitis is still controversial. Recent methods for the diagnosis of postoperative cholangitis include urinary sulfated bile acids (USBA) and magnetic resonance cholangio-pancreaticography (MRCP). High-dose steroids and oral antibiotics have been used to reduce the incidence of postoperative cholangitis, and recurrent cholangitis leads to a lower survival rate.

Conclusions: Cholangitis is one of the most important determinants of long-term survival after the Kasai's procedure. The knowledge on postoperative cholangitis has been increasing in the past 10 years, showing a lower incidence of the disease and better therapeutic results.

Key words: biliary atresia; cholangitis; prophylactic antibiotics

                   World J Pediatr 2008;4(1):14-19

  [Abstract] [Full Text] [PDF]  
Original articles:
Vitamin A deficiency and child feeding in Beijing and Guizhou, China
  Jing-Xiong Jiang, Liang-Ming Lin, Guang-Li Lian, Ted Greiner

Beijing, China and Washington, USA

Author Affiliations: National Center for Women's and Children's Health, Beijing, China (Jiang JX, Lian GL); Capital Institute of Pediatrics, Beijing, China (Lin LM); PATH, Washington DC, USA (Greiner T)

Corresponding Author: Jing-Xiong Jiang, National Center for Women's and Children's Health, Beijing 100013, China (Tel: 86-10-64484396; Fax: 86-10-64296782; Email: jiangjingxiong@chinawch.org.cn)

Background: Vitamin A deficiency (VAD) is one of the three major micronutrient deficiencies in the world. In order to investigate the status of VAD and child feeding in China, we conducted the survey in Beijing city and Guizhou province.

Methods: We included a high socioeconomic area (Beijing) and a low socioeconomic area (Guizhou province) in China in our study. Participants included 1236 randomly selected children aged 0-71 months from stratified clusters (628 in Beijing and 608 in Guizhou), 409 from urban and 827 from rural areas. A food intake frequency questionnaire was used for dietary assessment. Fluorescence microanalysis was carried out to measure serum retinol concentrations. Serum retinol cut-off values of less than 20 g/dl and 30 g/dl were defined as sub-clinical VAD and suspected sub-clinical VAD, respectively.

Results: No xerophthalmia or night blindness was found. The mean concentration of serum retinol was 31.5 g/dl in the high socioeconomic group, and 26.5 g/dl in the low socioeconomic group. Rural infants had lower concentrations of serum retinol compared with the urban ones (26.98.1 g/dl vs 31.87.3 g/dl). The prevalence of sub-clinical VAD among all the children was 7.8%, and increased to 15.7% in children from the low socioeconomic group. In infants from the high socioeconomic area, the prevalence of suspected sub-clinical VAD was 38.0%, increasing to 59.5% in infants from the low socioeconomic area. The children from the low socioeconomic area had significantly lower frequency of intake of meat than the children in other groups. The prevalence of suspected sub-clinical VAD was higher in the children with lower consumption frequency of vitamin A rich foods than the children with higher consumption frequency of vitamin A rich foods.

Conclusions: VAD appears to be a moderate public health problem in certain areas of China. In areas with low socioeconomic status, VAD in childrean is more severe, and infants may be the group at the highest risk for VAD. Inadequate intake of vitamin A rich foods may result in VAD. A comprehensive long-term national strategy needs to be fostered in China for the treatment and prevention of the deficiency.

Key words: infants; feeding and eating disorders; socioeconomic status; vitamin A deficiency

World J Pediatr 2008;4(1):20-25

  [Abstract] [Full Text] [PDF]  
Glucose metabolism disorder in obese children assessed by continuous glucose monitoring system
  Chao-Chun Zou, Li Liang, Fang Hong, Zheng-Yan Zhao

Author Affiliations: Department of Endocrinology, Children's Hospital, Zhejiang University School of Medicine, Hangzhou 310003, China (Zou CC, Liang L, Hong F, Zhao ZY)

Corresponding Author: Li Liang, MD, Children's Hospital, Zhejiang University School of Medicine, 57 Zhugan Xiang, Hangzhou 310003, China (Tel: +86-571-88318645 or 13396585352; Fax: +86-571-87078641; Email: zou108cc@yahoo.com)

Background: Continuous glucose monitoring system (CGMS) can measure glucose levels at 5-minute intervals over a few days, and may be used to detect hypoglycemia, guide insulin therapy, and control glucose levels. This study was undertaken to assess the glucose metabolism disorder by CGMS in obese children.

Methods: Eighty-four obese children were studied. Interstitial fluid (ISF) glucose levels were measured by CGMS for 24 hours covering the time for oral glucose tolerance test (OGTT). Impaired glucose tolerance (IGT), impaired fasting glucose (IFG), type 2 diabetic mellitus (T2DM) and hypoglycemia were assessed by CGMS.

Results: Five children failed to complete CGMS test. The glucose levels in ISF measured by CGMS were highly correlated with those in capillary samples (r=0.775, P<0.001). However, the correlation between ISF and capillary glucose levels was lower during the first hour than that in the later time period (r=0.722 vs r=0.830), and the ISF glucose levels in 69.62% of children were higher than baseline levels in the initial 1-3 hours. In 79 obese children who finished the CGMS, 2 children had IFG, 2 had IGT, 3 had IFG + IGT, and 2 had T2DM. Nocturnal hypoglycemia was noted during the overnight fasting in 11 children (13.92%).

Conclusions: Our data suggest that glucose metabolism disorder including hyperglycemia and hypoglycemia is very common in obese children. Further studies are required to improve the precision of the CGMS in children.

Key words: glucose metabolism disorders; hyperglycemia; hypoglycemia; impaired glucose tolerance; obesity; type 2 diabetic mellitus

World J Pediatr 2008;4(1):26-30

  [Abstract] [Full Text] [PDF]  
Effect of phototherapy on blood endothelin and nitric oxide levels: clinical significance in preterm infants
  Guo-Sheng Liu, Hui Wu, Ben-Qing Wu, Run-Zhong Huang, Li-Hua Zhao, Yan Wen
  Guangzhou, China

Author Affiliations: Department of Pediatrics, First Affiliated Hospital, Jinan University, Guangzhou, China (Liu GS, Wu H, Wu BQ, Huang RZ, Zhao LH, Wen Y)

Corresponding Author: Guo-Sheng Liu, MD, Department of Pediatrics, First Affiliated Hospital, Jinan University, Guangzhou, China (Tel: 86-20-38688642; Email: tlgs@jnu.edu.cn)

Background: Phototherapy may have an adverse effect on the hemodynamics of preterm infants, and endothelin (ET) and nitric oxide (NO) are both the powerful vasoactive substances. This study was designed to observe the effect of phototherapy on blood levels of ET and NO in preterm infants.

Methods: Sixty-four preterm infants with hyper-bilirubinemia requiring phototherapy were studied. Among them, 31 patients were born at 32-36 weeks' gestational age (GA), and 33 patients were 32 weeks GA. Control group included 26 full-term infants with hyperbilirubinemia requiring phototherapy. All patients were treated with continuous phototherapy for 24 hours. Blood samples were collected before and after phototherapy. The amount of ET in the blood samples was determined by radioimmunoassay, and NO levels were determined using nitrate reductase. Heart rate, respiratory rate, apnea, and mean arterial blood pressure (MABP) were monitored regularly (defined interval: hourly, 4 hours, etc) during phototherapy.

Results: Blood ET levels measured after 24 hours of phototherapy were higher than the pretreatment values, as were blood NO levels measured after 12 hours and 24 hours of phototherapy. Both increases were statistically significant (P<0.05) in the GA32 weeks group. In the GA>32 weeks group, blood NO levels measured after 24 hours of phototherapy were higher than the pretreatment values; these changes were also statistically significant (P<0.05). In the GA32 weeks group, heart rate increased and the MABP decreased during phototherapy. The changes after 24 hours of phototherapy compared to the pretreatment values were statistically significant. A few episodes of apnea occurred during phototherapy in the GA32 weeks group. This was significantly higher than that in the other two groups.

Conclusions: Under phototherapy, blood levels of ET and NO were significantly higher in preterm infants, especially in preterm infants of 32 weeks GA.

Key words:  endothelins; hyperbilirubinemia; nitric oxide; phototherapy

                                                                                                                        World J Pediatr 2008;4(1):31-35

  [Abstract] [Full Text] [PDF]  
Hemogram and bone marrow morphology in children with chronic aplastic anemia and myelodysplastic syndrome
  Jin-Quan Wen, Hai-Lin Feng, Xu-Qing Wang, Ju-Ping Pang

Xi'an, China

Author Affiliations: Department of Hematology, Xi'an Children's Hospital, Xi'an 710003, China (Wen JQ, Feng HL, Wang XQ, Pang JP)

Corresponding Author: Jin-Quan Wen, MD, Department of Hematology, Xi'an Children's Hospital, Xi'an 710003, China (Tel: 86-29-87692105; Fax: 86-29-87692009; Email: wenjinquan@163.com)

 Background: Aplastic anemia (AA) and myelodysplastic syndrome (MDS) are both acquired disorders in which bone marrow fails to produce or release sufficient blood cells. Anemia, infections and thrombocytopenia are common signs of such diseases. Clinically, it is difficult to distinguish chronic aplastic anemia (CAA) from MDS, especially from MDS without splenomegaly. As prognosis and treatment of AA and MDS are different, it is extremely important to make a differential diagnosis for  the two diseases. 

Methods: The medical records of 31 patients with CAA and 17 patients with MDS were retrospectively reviewed. Hemogram, bone marrow smear and biopsy for those patients were analyzed.

Results: The mean counts of monocytes and platelets in the peripheral blood of the CAA patients were significantly lower than those of the MDS patients. Bone marrow smear showed a reduction of cellularity in CAA patients. The mean counts of myeloblasts+promyelocytes, myeloblasts+proerythroblasts, and megakaryocytes in the bone marrow of CAA patients were markedly lower than those in MDS patients. But the mean lymphocyte count was reversed. Bone marrow cells showed morphological abnormalities in MDS. Hematopoietic tissue in the bone marrow biopsy decreased obviously in more than 96% of the patients with CAA. Adipose tissue in the bone marrow of CAA patients increased obviously. A reduction or deficiency (<2 cell/piece) of megakaryocytes was noted in 28 patients with CAA. Fibrous tissue in the bone marrow was detected in 5 patients with CAA. Bone marrow biopsy results showed hypercellular changes in 12 MDS patients. Ten patients showed aggregated erythroblasts which were in the same stage of development, and 15 patients had abnormal localization of immature precursors (ALIP).

Conclusions: Blood cell counts can be decreased in addition to the reduction of cellularity in the bone marrow without dyshematopoiesis in CAA patients. Peripheral blood monocytes, fibrous tissue and cellularity in bone marrow are increased in MDS. Dyshematopoiesis and ALIP may appear characteristically in the children with MDS. Histology of bone marrow is important in the differential diagnosis of MDS and CAA.

Key words: aplastic anemia; bone marrow; children; diagnosis; myelodysplastic syndrome

                  World J Pediatr 2008;4(1):36-40

  [Abstract] [Full Text] [PDF]  
Is pneumoperitoneum an absolute indication for surgery in necrotizing enterocolitis?
  Vijai D. Upadhyaya, A. N. Gangopadhyay, Anand Pandey, Ashish Upadhyaya,

Varanasi, India

Author Affiliations: Department of Pediatric Surgery, IMS, BHU, Varanasi, India (Upadhyaya VD, Gangopadhyay AN, Pandey A, Upadhyaya A, Moahan TV, Gopal SC, Gupta DK)

Corresponding Author: Vijai D. Upadhyaya, Department of Pediatric Surgery, IMS, BHU, Varanasi, 221005, India (Tel: +91-0542-2309275; Email: upadhyayavj@rediffmail.com)


Background: Necrotizing enterocolitis (NEC) is the most common gastrointestinal medical/surgical emergency in neonates. Non-operative support is needed in 70% of NEC cases, and surgical intervention in the rest 30%. Historically, pneumoperitoneum has been considered as an absolute indication for laparotomy. In the present study we emphasize that pneumoperitoneum is not an absolute indication for exploratory laparotomy in NEC cases. 

Methods: We prospectively studied 58 patients with severe NEC having pneumoperitoneum on abdominal X-ray in the last 5 years. At the time of admission, the patients were given intravenous fluid, total parental nutrition, blood transfusion and broad spectrum antibiotics followed by abdominal tapping (paracentesis). All the patients with pneumoperitoneum were closely monitored for 48 hours if abdominal tapping was repeated. When the disease seemed to worsen clinically, radiologically and laboratorially, the patient was subjected to exploratory laparotomy.

Results: Of the 58 patients, 40 were treated conservatively whereas 18 underwent surgical intervention. The overall mortality in the present study was 12.1%, including 5% of the patients managed conservatively and 27% of the patients undergoing surgery.

Conclusions: Pneumoperitoneum is not an absolute indication for surgery in cases of neonatal NEC. Most of the patients can be treated conservatively.

Key words: necrotizing enterocolitis; neonates; pneumoperitoneum; surgery

                   World J Pediatr 2008;4(1):41-44

  [Abstract] [Full Text] [PDF]  
Functional constipation in children: investigation and management of anorectal motility
  Zheng-Hong Li, Mei Dong, Zhi-Feng Wang

Author Affiliations: Department of Pediatrics (Li ZH, Dong M), Department of Gastroenterology (Wang ZF), PUMC Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing 100730, China

Corresponding Author: Mei Dong, MD, Department of Pediatrics, PUMC Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing 100730, China (Tel: 86-10-65296276; Fax: 86-10-65296271; Email: worldlizhengh@yahoo.com.cn)

Background: Constipation is a common disease in children. Despite many causes, constipation is most often functional. This study was undertaken to investigate the anorectal motility in children with functional constipation as compared with healthy children and to determine the efficacy of management based on the results of anorectal manometry.

Methods: A multi-functional manometry was used to detect the anorectal manometry indexes of 8 patients with functional constipation (11.44.8 years) as well as those of 10 healthy children (10.53.5 years) from May 2004 to June 2005. The patients received a combined treatment regimen including probiotics (bifid triple viable bacterial tablet), prebiotics (lactulose) and regular defecation according to the results of anorectal manometry. The efficacy of these conservative measures was estimated during the course of treatment.

Results: No statistical difference was found in the indexes of effective length of the anal sphincter, anal tract maximal systolic pressure and the duration of more than 50% maximal systolic pressure between the two groups. But minimal sensitivity and maximal tolerated volume between the two groups were different significantly. Seven of the eight patients got better with the conservative treatment.

Conclusions: Abnormalities exist in the anorectal motility of the children with functional constipation. Conservative treatment regimen based on the results of anorectal manometry is significantly effective.

Key words: constipation; manometry; motility; rectum

  [Abstract] [Full Text] [PDF]  
Enflurane requirement for blocking adrenergic responses to incision in infants and children
  Xuan Wang, Xue-Feng Zhang

Background: Enflurane is one of the most commonly used inhaled anesthetics in China, but its requirement to block adrenergic responses after skin incision in pediatric patients is still unknown. This study was to determine the minimum alveolar anesthetic concentration (MAC) of potent inhaled anesthetics required to blunt the adrenergic response to skin incision of enflurane (MACBAR) in infants and children. 

Methods: Twenty-eight patients, 10 infants (6-12 months) and 18 young children (1-6 years), were studied. The 18 children were randomly assigned into two groups, with or without fentanyl. Anesthesia was induced with 3 mg/kg propofol and 0.15 mg/kg vecuronium, and maintained with enflurane in 100% oxygen. Fentanyl (3 g/kg) was given intravenously 5 minutes before incision for the patients of fentanyl group. The "up and down" method (with 0.3 MAC as a step size and 1 MAC as the start dose) was applied to determine MACBAR. The response was considered positive if the mean arterial pressure (MAP) or heart rate (HR) increased 15% after incision. The MACBAR was calculated as the mean of four independent cross-over responses in each group.

Results: MACBAR of enflurane in children of 1-6 years old was 3.2% (95% CI, 2.8%-3.6%) and was reduced to 2.2% (95% CI, 1.8%-2.5%) by 3 g/kg fentanyl. In infants of 6-12 months old, the MACBAR of enflurane was 3.4% (95% CI, 3.0%-3.8%).

Conclusions: MACBAR of enflurane in infants older than 6 months is similar to that in young children. The MACBAR of enflurane decreases with co-administration of fentanyl in the pediatric population.

Key words: anesthesia; children; enflurane; infants

                    World J Pediatr 2008;4(1):49-52

  [Abstract] [Full Text] [PDF]  
Considerations before repair of acquired rectourethral and urethrovaginal fistulas in children
  Guo-Chang Liu, Hui-Min Xia, Ying-Quan Wen, Li-Yu Zhang, Zhong-Min Li

Background: Acquired rectourethral or urethrovaginal fistula between the rectum or vaginal and lower urinary tract is an uncommon entity, which occurs as a consequence of pelvic disorder, including trauma, iatrogenic injury, inflammatory bowel disease, pelvic neoplasm or infection. But when is it appropriate to repair the fistula and what methods to be chosen? There has been no consensus on them. This study was undertaken to determine the timing of the procedure and the repair of rectourethral and urethrovaginal fistula. 

Methods: From 1998 to 2006, we treated 19 children with rectourethral or urethrovaginal fistula, including rectourethral fistula in 15 boys and urethrovaginal fistula in 4 girls. The mean age of the patients was 6.2 years (range, 8 months to 11.5 years). The fistula occurred after pelvic fracture in 10 patients, and after iatrogenic injury in 9 including 4 after radical operation for Hirschsprung's disease and 5 due to anorectal malformation. Preoperatively, the general and local infections were controlled thoroughly, and complications such as urethral stricture and secondary megacolon were treated at first. At least 6 months after the last procedure, all patients underwent the 1-stage York-Mason procedure (via parasacrococcygeal incision) without colostomy and suprapubic cystostomy. Intraoperatively, the entire fistulous tract was excised completely.

Results: Infection and partial dehiscence of the wound occurred in 2 patients respectively. All fistulae were closed successfully without fecal incontinence or postoperative anal stricture. No patient suffered from urinary incontinence after fistula repair. The scars around the fistula were removed because they would shrink and lead to subsequent urethral occlusion or stricture.

Conclusions: The timing of operation for acquired rectourethral or urethrovaginal fistula is appropriate at least 6 months after the last procedure. The 1-stage York-Mason procedure for the repair of the fistula is feasible and effective.

Key words: fistula; rectourethral fistula; urethrovaginal fistula; York-Mason approach

                    World J Pediatr 2008;4(1):53-57

  [Abstract] [Full Text] [PDF]  
Expression and clinical significance of stem cell marker CD133 in human neuroblastoma
  Qiang-Song Tong, Li-Duan Zheng, Shao-Tao Tang, Qing-Lan Ruan, Yuan Liu,

Background: Recent evidences indicate that CD133, a kind of transmembrane protein, can be used as a marker to isolate stem cells from tumors originating from neural crest. This study was undertaken to explore the expression and clinical significance of stem cell marker CD133 in neuroblastoma (NB). 

Methods: Immunohistochemical staining was used to detect the expression of CD133 in 32 patients with NB and 8 patients with ganglioneuroblastoma (GNB). The relationships were analyzed among CD133 expression, international neuroblastoma staging system (INSS) stages, pathological classification, and postoperative survival time of NB patients.

Results: The expression rates of CD133 in NB and GNB were 46.9% (15/32) and 37.5% (3/8) respectively, mainly in cytoplasm of neuroblastoma cells. The expression rates of stage 1-2, stage 3-4 and stage 4S were 30.7%, 57.9% and 37.5%, respectively. The differences in various stages were significant (P<0.05). The positive rate of CD133 in patients with unfavorable histology (52.4%) was significantly higher than that in patients with favorable histology (36.8%) (P=0.007). The survival time of CD133 negative patients was significantly longer than that of CD133 positive patients (P=0.026).

Conclusions: CD133 which might be correlated with the development and progression of NB can serve as one of the important indicators for prognosis of NB .

Key words: CD133 protein; gene expression; neuroblastoma
  [Abstract] [Full Text] [PDF]  
Clinical summary:
Esophageal achalasia of unknown etiology in infants
  Vijay D Upadhyaya, AN Gangopadhyaya, DK Gupta, SP Sharma, Vijayendra Kumar, SC Gopal
  Background: Achalasia cardia is an uncommon disease in children particularly in infants. We present 8 cases of achalasia who were encountered over a 12-year period. In infantile achalasia, respiratory symptoms predominate and vomiting may easily be mistaken for gastroesophageal reflux (GER). Vomiting of uncurdled milk is characteristic of achalasia.

Methods: In this retrospective study, the data were obtained from records of the Department of Pediatric Surgery IMS, BHU Varanasi, India. The patients were diagnosed by clinical examination and barium study. Any other associated anomalies were noted in these patients. The patients underwent esophagocardiomyotomy with antireflux procedure via the abdominal route.

Results: In the present series, 7 patients survived. The follow-up study after operation showed remarkable relief of symptoms with satisfactory weight gain. No post-operative death occurred in the patients. Achalasia associated with alacrimia was noted in one infant who was lost to follow up. One infant was initially diagnosed as having gastric volvulus, but exploration revealed achalasia cardia.

Conclusions: Esophageal achalasia is a rare disease in children and its origin is generally indeterminable. Achalasia cardia should be emphasized in the differential diagnosis of an infant presenting with signs and symptoms of esophageal obstruction.

Key words: esophageal achalasia; esophagomyotomy; etiology; gastroesophageal reflux; infants

  [Abstract] [Full Text] [PDF]  
Case reports:
Dermoid cyst of the posterior fossa associated with congenital dermal sinus in a child
  Chun-Quan Cai, Qing-Jiang Zhang, Xiao-Li Hu, Chun-Xiang Wang
  Background: Intracranial dermoid cysts are congenital benign neoplasms. Hydrocephalus and abscess as the principal manifestations of the posterior fossa dermoid cyst are rare. We present a case of obstructive hydrocephalus and abscess induced by an adjacent dermoid cyst with occipital dermal sinus. 

Methods: A 2-year-old girl presented with headache and vomiting. Physical examination showed nothing abnormal except for a small subcutaneous nodule above the occipital protuberance with a small skin opening. She had no neurological deficits. Neuroradiological studies including CT and MRI showed a cyst located in the posterior fossa. The cyst in the posterior fossa with occipital dermal sinus was diagnosed. She was treated by radical excision of the occipital cyst through a suboccipital approach, and was followed up.

Results: Histopathologic examination suggested a dermoid cyst with an abscess. Bacterial investigation revealed Staphylococcus epidermidis, and appropriate systemic antibiotic therapy was given. The child recovered and a 2-year follow-up was uneventful.

Conclusions: Posterior fossa dermoid cyst should be considered in all children with occipital skin lesions, especially dermal sinus. CT and MRI scan are helpful in the diagnosis of the lesion. Neurosurgical treatment of the lesion should be planned early to prevent infections such as abscess and meningitis.

Key words: abscess; cranial dermal sinus; dermoid cyst; hydrocephalus; posterior fossa

                    World J Pediatr 2008;4(1):66-69

  [Abstract] [Full Text] [PDF]  
Diagnosis and treatment of blue rubber bleb nevus syndrome in children
  Zhao-Hui Deng, Chun-Di Xu, Shun-Nian Chen

Shanghai, China

Background: Blue rubber bleb nevus syndrome (BRBNS) is characterized by distinctive vascular malformations of skin and the gastrointestinal tract, often leading to chronic anemia and intestinal bleeding. It usually presents right after birth or during early infancy. Though the disease is inherent, its occurrence is sporadic. Thus it is usually not timely diagnosed. We analyzed the clinical characteristics and treatment of this disorder in order to improve the diagnosis and treatment. 

Methods: Three patients with BRBNS treated at our hospital during 2002-2003 and 39 patients from the literature reported during 1965-2003 were reviewed in terms of the diagnosis and treatment. BRBNS may be diagnosed as cutaneous cavernous hemangioma associated with the same lesion of the gastrointestinal tract and other organs.

Results: Our 3 patients suffered from cutaneous angioma and gastrointestinal hemangioma. In 39 patients reported in the literature, cutaneous angioma was observed in all of them, and gastrointestinal hemangioma in 31. Additionally, the lesions were also found in other organs such as the brain (7 patients), joint (2), liver (2), eye (1), kidney (1) and spleen (1). Cutaneous angioma was located on the surface of the skin, including body (93%), limbs (86%), hip (36%) and face (26%). Gastrointestinal hemangioma was more common in the small intestine (100%) than in the colon (74%) and stomach (26%). When the joint was involved by hemangioma, pathologic fracture or overgrowth of bone needed traction and amputation (1 patient respectively). For significant gastrointestinal bleeding, endoscopic techniques (8 patients), surgical excision (5), or both (1) were performed. Recurrent bleeding was successfully treated by endoscopic laser combined with steroid or interferon in one patient.

Conclusions: BRBNS in children presents atypical symptom and systemic complications. It should be dealt with seriously if gastrointestinal bleeding or orthopedic complication occurs. Treatment includes conservative, endoscopic and surgical options. Its recurrence with new angioma in the gastrointestinal tract needs laser-steroid therapy.

Key words: blue rubber bleb nevus syndrome; diagnosis; hemangioma; therapy

  [Abstract] [Full Text] [PDF]  
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