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Vol 5, No 3
Vol 5, No 3 August 2009 ISSN 1708-8569
 
Review articles
Original articles
Case reports
Clinical experience
Correspondence
   
Review articles:
New surgery for better outcomes: shaping the field of congenital heart disease
  Jacques G Leblanc
  Background: The field of congenital heart disease is constantly evolving through better understanding of the disease itself, albeit its history, prevalence, genetics, and follow-up. Concurrently surgical techniques and approaches have been developed, modified, and refined.

Data sources: The impact of interventional pediatric cardiology has been dramatic. The field of catheter-based therapies has exploded with the first pulmonary valve balloon angioplasty in 1982. With evolving stent technology, they are now used in multiple areas, including pulmonary arteries, vena cavae, aortic and arch and descending aorta for coarctation. The hybrid surgery concept involves a multidisciplinary team of interventional cardiologist and surgeon combining catheter-intervention and surgery in the surgical theater such as pulmonary artery stent implantation associated with pulmonary valve replacement. Furthermore, in selected cases, pulmonary valve device implantation is becoming an accepted approach to a surgical problem.

Results: Balloon angioplasties, stent implantations, hybrid surgeries and pulmonary valve device implantation are performed with a very low mortality and morbidity. The risks and benefits outweigh the ones associated to surgical procedures.

Conclusion: With fast developing interventional therapies, the work of pediatric cardiologists and cardiac surgeons is more intertwined than ever in search of better outcomes for the children with congenital heart disease.

Key words: catheter intervention;hybrid surgery; valve implantation

World J Pediatr 2009;5(3):165-168

  [Abstract] [Full Text] [PDF]  
Syndromic autism: causes and pathogenetic pathways
  Arianna Benvenuto, Romina Moavero, Riccardo Alessandrelli, Barbara Manzi,
  Author Affiliations: Department of Neurosciences, Pediatric Neurology Unit, Tor Vergata University, via Montpellier, 1, 00133 Rome, RM, Italy (Benvenuto A, Moavero R, Alessandrelli R, Manzi B, Curatolo P)

Corresponding Author: Paolo Curatolo, MD, Department of Neuroscience, Pediatric Neurology Unit, Tor Vergata University, via Montpellier 1, 00133, Rome, Italy (Tel: +390620900249; Fax: +390620900018; Email: curatolo@uniroma2.it)

doi:10.1007/s12519-009-0033-2

Background: Autism is a severe neurodevelopmental disorder known to have many different etiologies. In the last few years, significant progresses have been made in comprehending the causes of autism and their multiple impacts on the developing brain. This article aims to review the current understanding of the etiologies and the multiple pathogenetic pathways that are likely to lead to the autistic phenotype.

Data sources: The PubMed database was searched with the keywords "autism" and "chromosomal abnormalities", "metabolic diseases", "susceptibility loci".

Results: Genetic syndromes, defined mutations, and metabolic diseases account for less than 20% of autistic patients. Alterations of the neocortical excitatory/inhibitory balance and perturbations of interneurons' development represent the most probable pathogenetic mechanisms underlying the autistic phenotype in fragile X syndrome and tuberous sclerosis complex. Chromosomal abnormalities and potential candidate genes are strongly implicated in the disruption of neural connections, brain growth and synaptic/dendritic morphology. Metabolic and mitochondrial defects may have toxic effects on the brain cells, causing neuronal loss and altered modulation of neurotransmission systems.

Conclusions: A wide variety of cytogenetic abnormalities have been recently described, particularly in the low functioning individuals with dysmorphic features. Routine metabolic screening studies should be performed in the presence of autistic regression or suggestive clinical findings. As etiologies of autism are progressively discovered, the number of individuals with idiopathic autism will progressively shrink. Studies of genetic and environmentally modulated epigenetic factors are beginning to provide some clues to clarify the complexities of autism pathogenesis. The role of the neuropediatrician will be to understand the neurological basis of autism, and to identify more homogenous subgroups with specific biologic markers.

Key words: autism; candidate genes; etiologies; pathogenetic pathways

                  World J Pediatr 2009;5(3):169-176

  [Abstract] [Full Text] [PDF]  
Original articles:
Messages from the Aalst Allergy Study
  Dirk Van Gysel, Elke Govaere, Katia M.C. Verhamme, Erenik Doli, Frans De Baets
   

Background: The prevalence of sensitization and allergic disease has increased significantly worldwide. The aim of the "Aalst Allergy Study" was to document prevalences of sensitization and allergic symptoms, and to evaluate the effect of personal and environmental influences on these prevalences in an unbiased Belgian pediatric population.

Methods: A cross-sectional study was performed in an unbiased population of 2021 Belgian schoolchildren (3.4-14.8 years). Skin prick testing with the most common aeroallergens was performed. Allergic symptoms as well as potential risk factors for sensitization and allergic disease were documented by a parental questionnaire.

Results: The prevalence of sensitization to the most common aeroallergens and the prevalence of allergic diseases (eczema, asthma and rhinoconjunctivitis) were in line with the data in the literature. The association of current allergic symptoms with sensitization was only significant in the children aged ≥6 years. Age, gender, body mass index, bedroom environment and exposure to pets were the factors significantly associated with sensitization and allergic symptoms.

Conclusions: Our study corroborates the reported prevalences of sensitization and allergic diseases. Moreover the study illustrates the complexity of the search for factors involved in the process of sensitization and allergic disease. The impact of different potential causative factors is not only influenced by mutual interactions of these factors, but also by the existence of distinct subtypes of disease.

Key words: Aalst Allergy Study; allergic symptoms; risk factors; sensitization; skin prick test

World J Pediatr 2009;5(3):182-190

  [Abstract] [Full Text] [PDF]  
Airway foreign body removal by flexible bronchoscopy: experience with 1027 children during 2000-2008
  Lan-Fang Tang, Ying-Chun Xu, Ying-Shuo Wang, Cai-Fu Wang, Guo-Hong Zhu, Xing-Er Bao, Mei-Ping Lu, Lian-Xiang Chen, Zhi-Min Chen
   

Background: Foreign body aspiration (FBA) into the tracheobronchial tree is a common problem in children necessitating prompt recognition and management. This study aimed to report our experience in airway foreign body removal by flexible bronchoscopy in children.

Methods: A total of 1027 patients with FBA were reviewed retrospectively. They were 626 boys and 401 girls aged from 5 months to 14 years with a median age of 17 months. The clinical manifestations, radiological findings, bronchoscopic findings and complications of the procedure were analyzed.

Results: Among the patients, only 53.4% had a definite history of FBA. The most frequent symptom was paroxysmal cough (84.3%), followed by stridor or wheezing, fever and dyspnea. Chest X-ray showed emphysema in 68.8% of the patients, atelectasis in 13.3% and bronchopneumonia in 56.3%. A bronchoscope was inserted intranasally in most children, but through mouth and endotracheal tube in 17 and 3 children, respectively. Foreign bodies were removed successfully by flexible bronchoscopy with disposable grasping forceps or biopsy forceps in 938 (91.3%) of the patients. The other 89 patients turned to rigid bronchoscopy. During the procedures, 132 (12.9%) of the patients showed transient hypoxia, which was alleviated by oxygen supplement and/or temporary cessation of the procedure. A small amount of bleeding was found in 17 patients and bradycardia in 3. Air leak and laryngeal edema were noted in 2 patients and relieved within 24 hours.

Conclusions: Flexible bronchoscopy is useful and safe in retrieving airway foreign bodies in children. With skilled personnel and perfect equipments, flexible bronchoscopy could be considered as the first choice for the removal of airway foreign body.

Key words: child; flexible bronchoscopy; foreign body; safety

                   World J Pediatr 2009;5(3):191-195

  [Abstract] [Full Text] [PDF]  
EEG and MRI findings and their relation with intellectual disability in pervasivedevelopmental disorders
  Özlem Ünal, Özlem Özcan, Özgür Öner, Melda Akcakin, Ayla Aysev, Gülhis Deda
   

Background: The diagnostic category pervasive developmental disorders (PDDs) refer to a group of five disorders: autism, Rett syndrome, childhood disintegrative disorder, Asperger syndrome, and pervasive developmental disorder not otherwise specified (PDD-NOS). EEG abnormalities and seizures are considered much frequent in autistic subjects with comorbid intellectual disability (ID). In this study, we aimed to evaluate the EEG and MRI findings and their relation with ID in pervasive developmental disorder.

Methods: A retrospective, cross-sectional and non-experimental study was performed. Subjects included 81 patients diagnosed with autism or PDD-NOS according to the DSM-IV criteria. The age range of the patients was 2-15 years (mean 6.6 years, SD 3.0). Among them, 21 (25.9%) were girls and 60 boys (74.1%).

Results: Patients with severe ID had a higher rate of EEG abnormalities (P=0.03) than patients without ID as well as patients with mild or moderate ID. The association remained significant after the structural MRI abnormalities were controlled (P=0.04). The severity of ID was not associated with abnormal MRI. The most frequent EEG and MRI abnormalities were active epileptic anomaly/paroxysmal abnormality and cerebral atrophy/periventricular leukomalacia, respectively. Almost a third of the EEG abnormalities were associated with temporal cortex and adjacent cortical structures.

Conclusions: Consistent with previous studies, almost a fourth of the patients in this relatively large sample of patients with pervasive developmental disorders had EEG and/or MRI abnormalities. EEG results indicate that temporal cortex may play a significant role in pervasive developmental disorders.

Key words: autism; electroencephalography; magnetic resonance imaging;  pervasive developmental disorders

                 World J Pediatr 2009;5(3):196-200

  [Abstract] [Full Text] [PDF]  
Detection of intestinal bifidobacteria and lactobacilli in patients with Hirschsprung's disease associated enterocolitis
  Di-Hua Shen, Cheng-Ren Shi, Jing-Jing Chen, Shi-Yao Yu, Yan Wu, Wen-Bo Yan
   

Author Affiliations: Department of Pediatric Surgery, Xin Hua Hospital, School of Medicine, Shanghai JiaoTong University, 1665 Kongjiang Road, Shanghai 200092, China (Shen DH, Shi CR, Chen JJ, Yu SY, Wu Y, Yan WB)

Corresponding Author: Cheng-Ren Shi, Department of Pediatric Surgery, Xin Hua Hospital, School of Medicine, Shanghai JiaoTong University, 1665 Kongjiang Road, Shanghai 200092, China (Tel: 86-21-65790000 ext 8285; Fax: 86-21-65795173; Email: shicr629@163.com)

doi:10.1007/s12519-009-0038-x

Background: The etiology of Hirschsprung's disease associated enterocolitis (HAEC) is unknown. Previous investigations have suggested that several factors such as dilation of proximal bowel, changes in colonic mucosal defence, and overgrowth of toxigenic bacteria may be related with it. This study was to quantify bifidobacteria and lactobacilli in the feces of Hirschsprung's disease (HD) patients with or without enterocolitis and those of normal children.

Methods: Fresh stool specimens were collected at the first three days of the admission from 30 HD patients (aged 2 weeks to 2 years) and 15 healthy age- matched non-HD patients in the morning once a day for at least three days. All of them have not been given probiotics or antibiotics at least 7 days before stool collection. Hematoxylin-eosin and acetylcholinesterase histochemical staining on rectal biopsies of patients with HD confirmed the diagnosis of HD in all 30 patients. The 30 HD patients were divided into two groups based on the clinical history of enterocolitis: the HAEC group (n=10) and HD group (n=20). Fecal bifidobacteria and lactobacilli were consecutively quantified by SYBR Green I-based real-time PCR assay. Data were analyzed using SAS v. 12.6 for Windows. All tests were two-tailed, and P values <0.05 were considered statistically significant.

Results: The mean levels of bifidobacteria were 7.35±0.59, 8.16±1.17, and 8.35±0.74 in the HAEC, HD and control groups, respectively. The bifidobacteria colonization levels were lower in the HAEC group than in the HD and control groups (P<0.05, P<0.001 respectively). The mean level of lactobacilli in the HAEC (5.51±0.65) and HD groups (5.87±0.78) was significantly lower than that in the control group (6.39±0.56) (P<0.05). But there was no difference in log numbers of lactobacilli between HAEC and HD groups (P>0.05).

Conclusions: The scarcity of bifidobacteria and lactobacilli in HAEC patients may result in a decrease in epithelial barrier function and be a predisposing factor in the development of HAEC. This decline suggests that treatment with probiotics or prebiotics may be beneficial in these individuals. Further research will focus on whether probiotics can decrease the incidence of HAEC.

Key words: bifidobacteria; enterocolitis; Hirschsprung's disease; lactobacilli

                  World J Pediatr 2009;5(3):201-205

  [Abstract] [Full Text] [PDF]  
Expression and clinical significance of heparanase in neuroblastoma
  Li-Duan Zheng, Qiang-Song Tong, Shao-Tao Tang, Zhi-Yong Du, Yuan Liu,Guo-Song Jiang, Jia-Bin Cai
  Author Affiliations: Department of Pathology (Zheng LD) and Department of Pediatric Surgery (Tong QS, Tang ST, Du ZY, Liu Y, Jiang GS, Cai JB), Union Hospital of Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, China

Corresponding Author: Qiang-Song Tong, PhD, MD, Department of Pediatric Surgery, Union Hospital of Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, China (Tel: 86-27-63776478; Email: qs_tong@hotmail.com)

doi:10.1007/s12519-009-0039-9

Background: Previous studies indicate that heparanase (HPA), an endoglycosidase involved in tumor angiogenesis and metastasis, is up-regulated in a variety of malignancies. However, the expression of HPA in neuroblastoma (NB), one of the most common extra cranial solid tumors in children, remains unknown. This study was undertaken to explore the expression and clinical significance of HPA in NB.

Methods: Immunohistochemical staining was applied to detect the expression of HPA in 42 cases of NB. The relationships among HPA expression, international neuroblastoma staging system (INSS) stages, histopathological classification, and postoperative survival of the NB patients were analyzed.

Results: The expression rate of HPA in NB was 61.9% (26/42), mainly in the cytoplasm of neuroblastoma cells. The expression rates of stage 1-2, stage 3-4 and stage 4S were 35.7%, 80.0% and 62.5%, respectively. The differences between stage 1-2 and stage 3-4 were significant (P<0.01). The expression of HPA was significantly higher in the NB cases that had one of the histopathological factors: age more than 1 year (P<0.01), poorer differentiation (P<0.01), and higher mitosis karyorrhexis index (P<0.01). The survival time of HPA-negative patients was significantly longer than that of HPA-positive patients (P<0.05).

Conclusion: Although these results indicate that heparanase might be correlated with development and progression of NB, a larger series of patients with a longer follow-up are probably needed to strengthen its role in assessment of NB prognosis.

Key words: heparanase; neuroblastoma; risk; survival

World J Pediatr 2009;5(3):206-210

  [Abstract] [Full Text] [PDF]  
Expression and significance of TGF-β1/Smad signaling pathway in children with IgA nephropathy
  Wei Wu, Xiao-Yun Jiang, Qiao-Ling Zhang, Ying Mo, Liang-Zhong Sun, Shu-Mei Chen
   

Background: IgA nephropathy (IgAN) exhibits an indolent but slowly progressive course, and about 30% of children with IgAN are found to deteriorate to end-stage renal failure characterized by overaccumulation of extracellular matrix, diffuse glomerular sclerosis, and tubulointerstitial fibrosis. The TGF-β/Smad signaling pathway plays an important role in glomerulosclerosis and tubulointerstitial fibrosis. The present study aimed to elucidate the significance of expressions of TGF-β1, phosphorylated Smad3 (p-Smad3), Smad7 and fibronectin (FN) in the renal tissue of children with IgAN.

Methods: Forty-six children with IgAN were divided into 3 groups according to their clinical features: isolated hematuria group (IH group, 8 patients), hematuria and proteinuria group (HP group, 24), and nephritic syndrome group (NS group, 14). Patients were also divided into three groups according to their pathologic grade: grade I+II (22 patients), grade III (12) and grade IV (12) groups. Five normal renal specimens were used as the control group. The expression of TGF-β1, p-Smad3, Smad7 and FN in renal biopsy specimens was detected by two-step PowerVisionTM. The degrees of renal tubular injury and interstitial fibrosis were scored according to the Katafuchi semi-quantitative criteria.

Results: The expression of TGF-β1, p-Smad3, Smad7 and FN in children with IgAN was significantly higher than that in the control group (in glomeruli: P<0.05, P<0.01, P<0.05 and P<0.01, respectively; in tubulointerstitium: P<0.05, P<0.05, P<0.01 and P<0.05, respectively) and the highest expression levels were found in the NS and grade IV groups (P<0.05, P<0.01). The expression levels of the four proteins were not only positvely correlated with each other, but also with the grade of renal tubular injury and renal interstitial fibrosis (P<0.05).

Conclusion: The TGF-β1/Smad signaling pathway plays an important role in the progress of glomerular sclerosis, renal tubular injury and interstitial fibrosis in children with IgAN.

Key words: glomerulonephritis; IgA nephropathy; Smad protein; transforming growth factor  

                  World J Pediatr 2009;5(3):211-215

  [Abstract] [Full Text] [PDF]  
Melatonin protects against oxidative damage in a neonatal rat model of bronchopulmonary dysplasia
  Li Pan, Jian-Hua Fu, Xin-Dong Xue, Wei Xu, Ping Zhou, Bing Wei
   

Author Affiliations: Department of Pediatrics, Shengjing Hospital of China Medical University, Shenyang 110004, China (Pan L, Fu JH, Xue XD, Xu W, Wei B); Department of Pediatrics, The 2nd Affiliated Hospital of Harbin Medical University, Harbin 150086, China (Zhou P)

Corresponding Author: Xin-Dong Xue, MD, Department of Pediatrics, Shengjing Hospital of China Medical University, Shenyang 110004, China (Tel: +86-24-83955509; Fax: +86-024-83955509; Email: xdxue@163.com)

doi:10.1007/s12519-009-0041-2

Background: Oxidative stress plays an important role in the pathogenesis of bronchopulmonary dysplasia (BPD). Melatonin (MT) has direct and indirect free radical detoxifying activity. The present study was to investigate whether treatment with MT would attenuate hyperoxia-induced lung injury and the effect of MT on imbalance of oxidants/antioxidants in the lung of neonatal rats.

Methods: BPD was induced by exposure to hyperoxia in neonatal rats (n=90). The rats were divided randomly into three groups (n=30 each): air-exposed control group, hyperoxia-exposed group, and hyperoxia-exposed MT-treated group. Lung specimens were obtained respectively on day 3, day 7, and day 14 after exposure (n=10 each). Activities of superoxide dismutase (SOD), glutathione peroxidase (GSH-Px), and catalase (CAT), and levels of myeloperoxidase (MPO), nitrite/nitrate, and malondialdehyde (MDA) were assayed. Histopathologic changes were observed in the tissues stained with hematoxylin and eosin and Masson's trichrome stain.

Results: Increased levels of MPO, nitrite/nitrate, and MDA in the hyperoxia-exposed rats were significantly reduced by MT (P<0.05). Activities of GSH-Px, SOD, and CAT which did not change after exposure to hyperoxia were increased by MT (P<0.05). Furthermore, BPD associated histopathological alterations such as reduced total number of alveoli and interstitial fibrosis were obviously abated in the MT-treated group.

Conclusions: MT can reverse oxidants/antioxidants imbalance in damaged lung tissue and thus exert a beneficial effect on hyperoxia-induced lung disease in neonatal rats. With regard to humans, there may be a protective effect of MT on BPD.

Key words: antioxidants; bronchopulmonary dysplasia; free radicals; lung damagemelatonin

World J Pediatr 2009;5(3):216-221
  [Abstract] [Full Text] [PDF]  
Constitutional retinoblastoma gene deletion in Egyptian patients
  Amal M Mohammed, Alaa K Kamel, Saida A Hammad, Hanan H Afifi,Zeinab El Sanabary, Mostafa Ezz El Din
   

Author Affiliations: Department of Human Cytogenetics (Mohammed AM, Kamel AK, Hammad SA) and Department of Clinical Genetics (Afifi HH), National Research Centre, Cairo, Egypt; Department of Ophthalmology, Faculty of Medicine, Cairo University, Egypt (El Sanabary Z, Ezz El Din M)

Corresponding Author: Amal Mahmoud Mohamed, 33 El-Bohoos street, Department of Human Cytogenetics, Division of Human Genetics and Genome Research, National Research Centre, Dokki, 12311, Cairo, Egypt (Tel: +202-33389438; Fax: +202-33370931; Email: amalmahmoud15@yahoo.com)

doi:10.1007/s12519-009-0042-1

Background: Retinoblastoma is a neuroblastic tumor of childhood with an incidence of 1: 20 000. Retinoblastoma gene (Rb1) is a tumor suppressor gene that is located on the long arm of chromosome 13 at region 14. This study was to evaluate the constitutional monoallelic Rb1 deletion among retinoblastoma families.

Methods: Nine families with an affected Rb proband were evaluated. Clinical examination, pedigree analysis, and complete eye examination were given to patients, their sibs and parents. Standard cytogenetic and fluorescence in situ hybridization (FISH) analyses were carried out for all of them. Also, two sib fetuses were tested for Rb1 deletion.

Results: No dysmorphic features were detected in any patient. Developmental milestones were within normal limit except in one proband who had a mild delay. The age of onset ranged from one month to 4 years. Positive family history was found in two families. In one, the father and 3 sibs had retinoblastoma, and in the other, 2 sibs were affected, but the parents were free. Chromosomal study revealed no abnormalities in all parents and sibs. Two patients had mosaic chromosome 13 abnormalities, 46,XY/46,XY,del(13)(pter→q14:) and 46,XX/46,inv(13)(q14q22). FISH analysis detected mosaic Rb1 deletion in two patients and excluded Rb1 deletion in two fetuses.

Conclusions: The detection of genetic alterations affecting the Rb1 locus is important for the establishment of carriers, and prenatal and presymptomatic diagnosis. The search for deleted Rb1 mosaic cell lines is important for genetic counseling. Germline mutation may be considered as genetic transmission method of the Rb1 gene.

Key words: familial retinoblastoma; mosaic cell line; mutation; retinoblastoma gene (Rb1)

                   World J Pediatr 2009;5(3):222-225

  [Abstract] [Full Text] [PDF]  
Case reports:
Delayed presentation of congenital diaphragmatic hernia with intrathoracic gastric volvulus
  Ramazan Karabulut, Zafer Türkyılmaz, Kaan Sönmez, Suleyman Cuneyt Karakus, Apdullah Can Basaklar
   

Background: Gastric volvulus (GV) occurs when the stomach abnormally rotates around one of its axes and is a rare upper gastrointestinal obstruction. We present an unusual case of intrathoracic GV associated with delayed manifestation of congenital diaphragmatic hernia.

Methods: A 16-month-old female infant presented with a history of projectile non-bilious vomiting for 2 days and mild hematemesis for the last day. Physical examination showed epigastric fullness and pain with abdominal palpation. Complaints of the patient disappeared on the 2nd day after hospital admission. On the 6th day non-bilious vomiting started again and an epigastric mass was palpable. Contrast study of the stomach after oral barium administration showed the mesenteroaxial volvulus of the stomach. At laparotomy, the association of non-necrotic intrathoracic GV with intrathoracic spleen was confirmed. Moreover, the diaphragm presented a giant posterolateral hernia of the left dome. Diaphragmatic repair was performed in addition to gastropexy and splenopexy.

Results: The postoperative course was uneventful and the child was discharged on the 5th post-operative day. On follow up after one month, clinical examination and plain abdominal X-ray were normal.

Conclusions: GV is a clinical emergency which can be life-threatening for children. Upper gastrointestinal study and CT scan with contrast meal are helpful in the diagnosis of the lesion. We emphasize prompt surgical therapy to avoid gastric necrosis.

Key words: congenital diaphragmatic hernia; gastric volvulus; wandering spleen

                   World J Pediatr 2009;5(3):226-228

  [Abstract] [Full Text] [PDF]  
Papillary thyroid carcinoma presenting with upper respiratory tract obstruction and pulmonary metastases
  Youssef Al-Tonbary, Khaled Zalata, Rasha El-Ashry, Ashraf Fouda
   

Background: Thyroid cancer is rare in children especially before the age of 10 years. Upper airway obstruction and pulmonary infiltration are rare manifestations of such tumor.

Methods: An 8-year-old school girl was admitted to Mansoura University Children's Hospital for a papillary thyroid carcinoma manifested by severe upper respiratory tract obstruction. CT scan of the chest revealed multiple miliary shadows in both lungs.

Results: Total thyroidectomy was performed and pathological examination confirmed the diagnosis of papillary carcinoma of the thyroid gland. The patient received ablative dose of Iodine 131 and replacement therapy of L-thyroxine.

Conclusion: Thyroid cancer, although rare, should be considered for differential diagnosis of upper airway obstruction and pulmonary metastases.

Key words: pulmonary miliary metastasis; thyroid cancer; upper respiratory tract obstruction

                   World J Pediatr 2009;5(3):229-231

  [Abstract] [Full Text] [PDF]  
Clinical experience:
Pediatric surgery and pediatric surgical oncology
  Jin-Zhe Zhang
   

Pediatric surgery and pediatric surgical oncology

Jin-Zhe Zhang

Beijing, China

Author Affiliations: Department of Pediatric Surgery, Beijing Children's Hospital, Capital Medical University, Beijing 100045, China (Zhang JZ)

Corresponding Author: Jin-Zhe Zhang, MD, Honorary Fellow of the Royal College of Surgeons (HonFRCS), Member of Chinese Academy of Engineering (MCAE), Professor and Senior Consultant of Pediatric Surgery, Beijing Children's Hospital, Beijing 100045, China (Email: jinzhezhang@sina.com)

doi:10.1007/s12519-009-0045-y

D

espite major advances in pediatric surgery, skills at the operating table should not be neglected. Surgical technique requirements differ in children of different ages, and many techniques are special in pediatric surgery. The following is a brief account of my experience showing the peculiarities of surgery in children (more illustrative in newborns) and suggestions for up-grading pediatric surgical techniques.

Peculiarities in pediatric surgery

The abdominal cavity of a baby is relatively small, while the tumor is much bigger. A large enough transverse incision should be made and then all intestines are placed out of the abdomen, making the abdominal cavity nearly empty to expose the tumor fully as much as possible.

In severely distended intestine of a newborn (The intestinal wall looks like a transparent plastic film), its seromuscular coat may burst into bivalves suddenly following even a negligible injury, leaving an isolated bare mucosal tube bulged out from the broken intestine coat, which will naturally lose its viability (Fig. 1). In order to avoid peritoneal contamination due to puncture leakage, a fine needle connected to a suction can be used. Complete decompression should be achieved until the intestinal wall recovers its normal thickness and color. Sometimes, a little bit antibiotics may be injected before pulling the needle, and additional fine suture is rarely necessary.

At the critical moment when a rapid closure of the abdomen requires for resuscitation, the wound should be closed in a few minutes. Commonly, the patient's condition is very poor and anesthesia is inadequate. It is wise to leave all the intestines outside the abdomen undisturbed. Running suture from one end of the wound is used to close most parts of abdominal wound until a small opening (about 2-3 stitches untied) is left behind. Then the outside intestine is sent in its natural sequence into the deep part of the abdominal cavity, and finally the rest stitches are tied (Fig. 2).

The largest chest wound is in the 6th intercostal space (ICS, cut-in directly without rib resection) of neonates for tumors of all locations in the thoracic cavity (Fig. 3A).

Subcutaneous binding of the upper and lower marginal ribs of the wound by 4 heavy stitches to close the wound tightly air-proved (Fig. 3B). No drainage is preferred because the chest wall is too thin to keep the drainage air tightly. If necessary, daily aspiration may be required.

A small catheter in the small urethra may serve as a plug causing obstruction. Suprapubic cystostomy is preferred when necessary, particularly in the urethra surgery.

"S" curved incision is preferred for laminectomy in small kids in order to prevent the direct exposure of the cord in case of wound disruption. The accurate number of the vertebra is difficult to obtain by palpation on the back of a small kid. A pre-placed nail on the spinal process nearby the selected vertebra (location of tumor) in a plain film may help to count the number of vertebrae accurately with the retention nail as a landmark during operation (Fig. 3C).

Special attention to tumor-removal

Do not touch the tumor before knowing the followings: tendency of bleeding? distention to surrounding vessels? active oozing? capsule broken or high tension? definite anatomical location and organs? any organ or big vessels adhered? If necessary and possible, pre-placed temporary tourniquets on feeding vessels are preferred (Fig. 3D).

A perfect plan should be made so that the procedure can be stopped at any step, and the abdomen may be closed in a few minutes. Sharp dissection under direct vision should be the principal technique for mobilizing a tumor.

Sharp scalpel gently cuts in the surface of the vessel or nerve along its long axis. The outer fibrous layer lays open and the organs are exposed totally free.

In soft adhesion, tumor is movable. In order to protect the tumor from squeezing or injury, sharp dissection under direct vision and/or finger break is advised. Special attention should be paid to any vessel or nerve being caught in the adhesion.

In hard adhesion, tumor is not movable. Sharp dissection under vision is the only choice. Although there is rarely big vessel or nerve being caught, some neighboring organs might be caught or stretched.

In bony adhesion, tumor is usually fixed to the bony structure, e.g., the vertebra or pelvis, and hard to raise. A periosteal elevator should be held steadily and closely against the bony surface. The instrument should be pushed slowly and the distance of every advancement should be controlled as short as possible. Any injury to surrounding structures and cutting into the bony tissue should be avoided. A metal finger-tip periostotome designed in 1980 may serve as a reference (Fig. 3E), which is like the thimble for sewing needle and worn outside the glove on the fore-finger with a window on the palm side allowing free palpation and a metal finger-nail on the back serving as the periosteal elevator.

In case of a huge tumor such as Wilms' tumor, the under surface may be adherent to big vessels underneath. Forceful lift may cause tearing of tissues and hemorrhage. In order to put it under direct vision, a combined thoraco-abdominal incision may be advisable. If available, a malleable laryngoscope and a mini-ultrasonic probe may be inserted into the narrow space of the undersurface of a huge tumor to make the dissection under vision. If the tumor accidentally breaks during or before operation, all the dislodged tissues and the residual tumor should be cleansed as possible. After a thorough lavage, some proper chemotherapeutic drugs and metal markers or Titanium clips might be placed in the tumor bed for continuous treatment and as land-marks for later radiation therapy respectively.

In summary, a qualified pediatric surgeon has to be familiar with the surgical principles required for the peculiarities of the baby and the tumor. For the time being, the up-grading of tumor surgery depends on the further exploration and the improvement of laparoscopic skills, which will be the leading point of the evolution in all respects.

Funding: None.

Ethical approval: Not needed.

Competing interest: None declared.

Contributors: Zhang JZ is the single author of the paper.

Received June 5, 2008 Accepted after revision October 7, 2008

 

  [Abstract] [Full Text] [PDF]  
Correspondence:
To The Editor
  R. Shane Tubbs,Marios Loukas, Mohammadali Shoja; Chun-Quan Cai,Chang-Hong Shen
   

To The Editor

We read with interest the paper by Cai et al.[1] These authors described two cases of persistent falcine sinus. We would like to add to the authors' discussion by mentioning a recent study of ours.[2] We found that in 25 adult cadavers an extensive network of small tributaries within the falx cerebri could always be found, especially within its posterior one third (Fig.). In this posterior segment, these structures were usually more pronounced in the inferior two thirds. The portion of the falx cerebri not containing significant falcine venous sinus was termed a "safe area" for surgical consideration. These vascular channels ranged in size from 0.5 mm to 1.1 cm (mean 0.6 mm). 100% of these vessels communicated with the inferior sagittal sinus. Typing was performed based on communication of the falcine venous sinus with the superior sagittal sinus. Type I falcine sinuses had no communication with the superior sagittal sinus, Type II falcine sinuses had limited communication with the superior sagittal sinus, and Type III falcine sinuses had significant communication with the superior sagittal sinus. Seventeen of 27 (63%) specimens had communication with the superior sagittal sinus (Types II and III). Further subdivision revealed 10 Type I, 7 Type II, and 10 Type III falcine venous plexuses. As the majority of specimens in our study were found to have a plexiform venous morphology within the falx cerebri, we propose that these channels be referred to as the falcine venous plexus and not sinus.

R. Shane Tubbs

Marios Loukas

Mohammadali Shoja

Pediatric Neurosurgery

Children's Hospital

Birmingham, AL, 35233 USA

Email: Richard.Tubbs@ccc.uab.edu

References

1   Cai CQ, Zhang QJ, Yang WD, Wang CX, Shen CH. Neuroimages of persistent falcine sinus in children. World J Pediatr 2009;5:63-64.

2   Tubbs RS, Loukas M, Louis RG Jr, Shoja MM, Acakpo-Satchivi L, Blount JP, et al. Anatomy of the falcine venous plexus. J Neurosurg 2007;107:155-157.

doi:10.1007/s12519-009-0046-x

The Author Reply:

W

e would like to thank Prof. Tubbs et al from Pediatric Neurosurgery of Birmingham Children's Hospital for their interest in our paper of two cases on persistent falcine sinus in children and their comment. We regret that we missed their important paper and are grateful to them for bringing it to our attention. We reported two patients with persistent falcine sinus with an emphasis on the neuroimages.[1] Tubbs et al[2] identified the anatomical structure named falcine venous plexus in specimens, and made a detailed discussion on it. Their study on falcine venous plexus is necessary for the neurosurgeon who performs the operation involving this region.

Chun-Quan Cai, PhD

Chang-Hong Shen, MD

Department of Neurosurgery

Tianjin Children's Hospital

No. 225, Machang Road, Hexi District

Tianjin 300074, China

Email: tjpns@126.com

 

References

1   Cai CQ, Zhang QJ, Yang WD, Wang CX, Shen CH. Neuroimages of persistent falcine sinus in children. World J Pediatr 2009;5:63-64.

2   Tubbs RS, Loukas M, Louis RG Jr, Shoja MM, Acakpo-Satchivi L, Blount JP, et al. Anatomy of the falcine venous plexus. J Neurosurg 2007;107:155-157.

doi:10.1007/s12519-009-0047-9

 

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