• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.24 no.2
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• pp.145-153
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• 2021

Purpose: Children treated for Hirschsprung disease (HD) are adversely affected by fecal incontinence and soiling. This can be detrimental to their physical, psychosocial quality of life (QoL) and impacts the normal functioning of their family. QoL studies in HD are predominantly from developed countries. We measured general quality of life, impact on family and functional bowel status using validated questionnaires in HD children in a developing country. Methods: Patients with HD, treated in a tertiary paediatric institution in India between 2010 and 2017, were identified. Patients and/or their proxy completed the Pediatric Quality of Life and Family Impact Module questionnaires. Functional outcomes were assessed using Rintala's score. Results: A 86 children and their parents participated in the study. Majority had rectosigmoid disease (67.4%) and underwent Soave's endoanal pull through (74.4%). A 21% of patients had low Rintala score indicating poor functional bowel outcomes. Only 11% of children had poor QoL scores. Family functioning outcomes were also severely affected in the same subgroup of patients. There was statistically significant correlation between Rintala score and QoL scores (p-value<0.001). Disease severity, type of surgery, and duration of follow-up did not have a statistically significant impact on the QoL. Conclusion: QoL in children with HD was comparable to the general population. Bowel dysfunction affects a notable number of children and was the most significant determinant of poor QoL.

• Archives of Plastic Surgery
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• v.42 no.2
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• pp.223-225
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• 2015

The closure of any circular or asymmetric wound can result in puckering or an excess of tissue known as a 'dog ear'. Understanding the mechanism of dog ear formation is a fundamental requirement necessary to facilitate an appropriate treatment. Many solutions have been reported in the literature, but in all cases, the correction entails the extension of the scar and the sacrifice of the dermal plexus. Here, we propose a novel technique of dog ear correction by using a three-bite suture that sequentially pierces the deep fascial plane and each dog ear's margin, thus allowing for flattening the dog ear by anchoring the over-projecting tissue to the deep plane. The three-bite technique proved to be a fast, easy, and versatile method of immediate dog ear correction without extending the scar, while maintaining a full and complete local skin blood supply.

• Journal of Korean Neurosurgical Society
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• v.64 no.3
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• pp.329-339
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• 2021

It has been recognised for over a century that the events of gastrulation are fundamental in determining, not only the development of the neuraxis but the organisation of the entire primitive embryo. Until recently our understanding of gastrulation was based on detailed histological analysis in animal models and relatively rare human tissue preparations from aborted fetuses. Such studies resulted in a model of gastrulation that neurosurgeons have subsequently used as a means of trying to explain some of the congenital anomalies of caudal spinal cord and vertebral development that present in paediatric neurosurgical practice. Recent advances in developmental biology, in particular cellular biology and molecular genetics have offered new insights into very early development. Understanding the processes that underlie cellular interactions, gene expression and activation/inhibition of signalling pathways has changed the way embryologists view gastrulation and this has led to a shift in emphasis from the 'descriptive and morphological' to the 'mechanistic and functional'. Unfortunately, thus far it has proved difficult to translate this improved knowledge of normal development, typically derived from non-human models, into an understanding of the mechanisms underlying human malformations such as the spinal dysraphisms and anomalies of caudal development. A paediatric neurosurgeons perspective of current concepts in gastrulation is presented along with a critical review of the current hypotheses of human malformations that have been attributed to disorders of this stage of embryogenesis.

• Proceedings of the Korean Society of Veterinary Clinics Conference
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• 2009.04a
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• pp.201-206
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• 2009

• Journal of Korean Neurosurgical Society
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• v.61 no.3
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• pp.343-351
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• 2018

Diffuse intrinsic pontine glioma (DIPG) is a deadly paediatric brain cancer. Transient response to radiation, ineffective chemotherapeutic agents and aggressive biology result in rapid progression of symptoms and a dismal prognosis. Increased availability of tumour tissue has enabled the identification of histone gene aberrations, genetic driver mutations and methylation changes, which have resulted in molecular and phenotypic subgrouping. However, many of the underlying mechanisms of DIPG oncogenesis remain unexplained. It is hoped that more representative in vitro and preclinical models-using both xenografted material and genetically engineered mice-will enable the development of novel chemotherapeutic agents and strategies for targeted drug delivery. This review provides a clinical overview of DIPG, the barriers to progress in developing effective treatment, updates on drug development and preclinical models, and an introduction to new technologies aimed at enhancing drug delivery.

• Clinical and Experimental Pediatrics
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• v.52 no.1
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• pp.1-5
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• 2009

Children are not small adults and there is a need to carry out specific trials that cannot be performed in adults. In general, children (minors) are unable to consent but their assent should be obtained using age appropriate information. Institutional Review Board (IRB) need paediatric expertise to balance the benefits and risks of research in children. The lack of consent has implications on the design, analysis and the choice of comparators used in the trials, which should only be performed by trained investigators with paediatric experience. Pain, fear, distress and parental separation should be prevented and minimised when unavoidable. The children requires even more careful review. Children represent a vulnerable population with developmental, physiological and psychological differences from adults, which make age- and development- related research important for their benefit. Finally, criteria for the protection of children in clinical trials therefore need to be laid down. Specific protection should be defined for research performed in children, at all stages and ages.

• Korean Journal of Head & Neck Oncology
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• v.24 no.2
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• pp.207-210
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• 2008

The purpose of this paper is to review our experience with mucoepidermoid carcinoma(MEC), a rare tumour in minor salivary glands, in a paediatric patients. 15-year-old boy was noted to have a irregular round mass appearing atthe soft palate just to the right of the mid-line. A computed tomographic(CT) scan showed a palatal mass limited to soft palate with no bony erosion. The lesion was curetted and debulked. Pathology was reported as an intermediate-grade mucoepidermoid carcinoma, and the patient was considered to radiation therapy institution for further treatment. To date, patients remain free of disease. Wide local excision is the treatment of choice for low to intermediate grade MEC of the minor salivary glands in paediatric patients.

• Imaging Science in Dentistry
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• v.38 no.4
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• pp.219-223
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• 2008

Purpose : The purpose of this study was to determine the prevalence of sinus opacification among dental paediatric patients. Materials and methods : Two hundred and eight Cone Beam Computed Tomography (CBCT) scans of dental patients under the age of 18 were reviewed for sinus opacification. Patients with any sinus-related signs or symptoms were excluded. Results : The overall prevalence of sinus opacification was 48.1 %. The ethmoid (28.4%) and maxillary (27.8%) sinuses were most frequently affected. There were no statistically significant differences for both age and gender. Conclusion : The high prevalence of sinus opacification in asymptomatic children emphasizes the necessity of clinical correlation. (Korean J Oral Maxillofac Radiol 2008; 38 : 219-23)

• Journal of Korean Neurosurgical Society
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• v.64 no.2
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• pp.151-188
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• 2021

Spinal dysraphic lesions due to focal nondisjunction in primary neurulation are commonly encountered in paediatric neurosurgery, but the "fog-of-war" on these conditions was only gradually dispersed in the past 10 years by the works of the groups led by the senior author and Prof. Kyu-Chang Wang. It is now clear that limited dorsal myeloschisis and congenital spinal dermal sinus tract are conditions at the two ends of a spectrum; and mixed lesions of them with various configurations exist. This review article summarizes the current understanding of these conditions' embryogenetic mechanisms, pathological anatomy and clinical manifestations, and their management strategy and surgical techniques.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.21 no.2
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• pp.86-92
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• 2018

Purpose: Celiac disease is a common non-communicable disease with varied presentations. Purpose of this study was to find the duodeno-endoscopic features in celiac disease and to compare duodeno-endoscopic and histological findings between typical and atypical celiac disease in children. Methods: Hospital based observational study was conducted at Sir Padampat Mother and Child Health Institute, Jaipur from June 2015 to May 2016. Patients were selected and divided in two groups- typical and atypical celiac disease based upon the presenting symptoms. Upper gastrointestinal endoscopy and duodenal biopsy was performed for serology positive patients. Results were analysed using appropriate statistical test of significance. Results: Out of 101 enrolled patients, 47.5% were male. Age ranged from 1 to 18 years. Study showed that 54.5% were typical and 45.5% were atypical. Patients presenting with atypical symptoms were predominantly of older age group. On endoscopy, scalloping, mosaic pattern, reduced fold height and absent fold height; and in histology, advanced Marsh stage were significantly higher in the typical group. Conclusion: Awareness of atypical presentations as well as duodeno-endoscopic features may have considerable practical importance for the diagnosis of celiac disease in children. Scalloping, mosaic pattern, reduced fold height and nodularity are main endoscopic markers of celiac disease in children. Endoscopic markers of duodenal mucosa may be important in early diagnosis of celiac disease, in children subjected to endoscopy for atypical presentations or indication other than suspected celiac disease.