International Journal of Current Research and Review
ISSN: 2231-2196 (Print)ISSN: 0975-5241 (Online)
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IJCRR - Vol 05 Issue 18, September, 2013

Pages: 60-64

Date of Publication: 25-Sep-2013


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A STUDY ON THE ASSOCIATION OF HYPERTELORISM AND POSTERIORLY PLACED PINNA IN CHILDREN WITH CONGENITAL ACYANOTIC HEART DISEASES

Author: Sumi Ghorai, Swapan Bhattacharya

Category: Healthcare

Abstract:Objective: To diagnose undetected cases of asymptomatic congenital acyanotic heart disease by measuring intercanthal distance (ICD) and angle of ear inclination (AEI). Methods: A comparative study was conducted over a period of 2011-2012 in the Department of Paediatric Medicine, Medical College Hospital, Kolkata. 100 patients (Cases: 50 patients with congenital acyanotic heart disease. Control: 50 patients without it) of age group 3 months to 12 years were examined clinically. Echocardiography was done. Intercanthal distance was measured by Vernier's sliding caliper and angle of ear inclination by photogrametry. Results: Among 50 cases, 7(14%) male and 14(28%) female patients had hypertelorism, 10(20%) male and 16(32%) female patients had posteriorly rotated pinna. Among 50 controls, 4(8%) male, and 6(12%) female patients had hypertelorism and 2(4%) male, and 6(12%) female patients had posteriorly rotated pinna. In both the group female patients had higher incidence. Conclusion: Every child with hypertelorism and obliquely placed pinna should be properly screened for congenital acyanotic heart disease to decrease the morbidity.

Keywords: Pinna, hypertelorism, Congenital acyanotic heart disease.

Full Text:

INTRODUCTION

Craniofacial anthropometry has mostly been used as a descriptive tool and has helped clinicians in the description, diagnosis and surgical treatment of abnormal skeletal and facial patterns. It is especially useful in medical genetics because many of the syndromes present at birth-such as Apert, Down, Turner etc.-involve the head region, so it can assist clinicians to objectively describe what they are seeing”.1 The diagnosis of many dysmorphic syndromes is based not only on advanced cytogenetic and molecular techniques, but also on recognition of subtle morphological anomalies in craniofacial region. Dysmorphic characters are usually reported by clinicians in descriptive terms such as “wide set eyes”,”broad nose”,”large mouth” and “posteriorly placed ear”. However, such description is subjective. Measurements taken from a patient can be compared with the values obtained in normal population and deviations from the normative values can be evaluated.2 The present study was conducted not only to compare the intercanthal distance of the controls without congenital acyanotic heart disease to cases with it but also to acquire knowledge about position of the pinna or angle of ear inclination in cases with congenital acyanotic heart disease and correlate it with the controls. In normal individual the intercanthal distance is about 2 cm to 3.6 cm and angle of ear inclination is approximately 7-120. Deviation from the normal can be indicative of congenital anomalies.

MATERIALS AND METHOD

The study was conducted on 100 patients of age group 3 months to 12 years attending the outpatient department and or, admitted in the department of Paediatric Medicine, Medical College, Kolkata over a period of 2011-2012. Thorough clinical examination of cardiovascular system was done. Echocardiography of the children were performed to confirm the diagnosis. Clinical examinations were done in presence of paediatricians and echocardiographies were done. The intercanthal distance was measured by vernier’s sliding caliper taking into account the error if any, in the instrument (Fig. 1). Profile photograph of the left side of the face of all children was taken including the entire helical rim of left ear, while keeping the head in the Frankfurt Horizontal (FH) plane. The angle of ear inclination (AEI) was directly measured on the photograph fixed on a large white paper sheet. The angle subtended between longitudinal axis(AB) of ear. It was determine as per technique given   by Farkas LG3; the line joining highest point on superior aspect of outer rim of helix to the most lowest point on the inferior border of ear lobule and vertical plane(BC) of the head/face represented by a perpendicular drawn on FH plane (FH plane: line extending from the lower border of the left orbit to the upper margin of the external auditory meatus, provided head is held erect and eyes looking straight forward) was considered as angle of ear inclination(AEI)(Fig. 2). The angle was measured with the help of a protractor upto the accuracy of one degree. Each measurement was taken twice by the same examiner. If there is large difference between the initial two measurements a third reading was taken. Among the three measurements the two nearer values were used.

RESULTS

The study was conducted on 100 patients, 50 cases and 50 controls. Out of those 20 were male and 30 were female in both the group. The age range was 3months to 12 years. In this study 7(14%) male and 14(28%) female patients had hypertelorism and 10(20%) male and 16(32%) female patients had posteriorly rotated pinna among the 50 cases with Congenital acyanotic heart disease (Table1). Whereas, 4(8%) male,10(12%) female patients had hypertelorism and 2(4%) male,6(12%) female patients had posteriorly rotated pinna out of 50 patients without Congenital acyanotic heart disease(table 2).  

DISCUSSION

The values for AEI reported by Farkas LG4 ranged between 6.3°-35.6° (mean 20°) in normal girls and 9.3°-31.2° (mean 21°) in boys. However, he had not examined the heart and has therefore, not commented on any co-existing cardiac abnormality.

Walia  BNS et al5 reported that 18 of the 20 subjects with congenital heart disease having angle of ear inclination more than 12° on photographic measurement, and concluded the mean AEI in heart defects was more than two times as compared to children who had normal ears and normal hearts. In our study 26(52%) of 50 patients with congenital acyanotic heart disease had AEI more than 12°. Of these 26 subjects 10(38.46%) were male,16(61.54%) were female.

Nair S et al6  reported one case of congenital acyanotic heart disease - Ventricular septal defect(VSD), Atrial septal defect(ASD),Patent ductus arteriosus( PDA) with pulmonary hypertension with hypertelorism, low set dysmorphic ears and other facial dysmorphism.

In our study 7(14%) male and 14(28%) female patients with heart disease had hypertelorism.

Noonan’s syndrome is one of the multiple congenital anomaly syndrome which is related with hypertelorism, cardiac defect and many other anomalies as per study of Bertola DR et al7. Our study did show co-relation between heart disease and hypertelorism, as there are 21 subjects who had increased intercanthal distance out of 50 patients with acyanotic heart disease. From the above discussion it can be said that, hypertelorism and obliquely placed pinna is associated with congenital acyanotic heart diseases. During embryologic development, external ear appears in  the region around the first pharyngeal groove in the 2nd month and interventricular septum also develops at about the same time. It is possible that the same agent/stimulus acting at this period distorts the development of both the regions simultaneously8.

Neural crest cells are essential for formation of much of the craniofacial region and they also  contribute to the conotruncal endocardial cushions, which septate the outflow tract of the heart into pulmonary and aortic channels. So, this might be reason for infants to have cardiac abnormalities with craniofacial defects.9

CONCLUSION

From the above discussion it has been proved that there is certainly a relationship between hypertelorism and congenital acyanotic heart disease. Though, of 50 patients without heart disease 4(8%) male,6(12%) female  patients had hypertelorism and 2(4%) male,6(12%) female patients had posteriorly rotated pinna ,that is far less common than in subjects with congenital acyanotic heart disease.

Our data suggest that any child whose pinna appears obliquely placed and eyes look apart on clinical examination should be carefully investigated for coexistence of unrecognized congenital cardiac anomalies.                                  

ACKNOWLEDGEMENT

The authors of this article gratefully acknowledge the inspiration and help received from the scholars whose articles have been cited in the reference section. The authors pay their gratitude to authors/editors/publishers of all those articles/journals/books from where the reviews and literatures for the discussion have been collected.

References:

  1. Identifying fetal alcohol syndrome using computerized craniofacial anthropometry. Medical news today article. Date 27th September 2007. Available form: http://www.medicalnewstoday.com/releases/83589.php. [last cited on 13/08/2013.]
  2. Naglel E, Teibe U, Kapoka D. Craniofacial anthropometry in a group of healthy Latvian residents. Acta Medica Lituanica 2005;12 :47-53.
  3. Farkas LG. Anthropometry of Head and Face. 2nd edition, p-3-56.
  4. Farkas LG. Anthropometry of normal and defective ears. Clin Plast Surg 1990; 17: 213-21.
  5. Walia BNS, Bhalla AK, Dhawan A. Co-Existence Of Oblique Pinnae And Congenital Heart Disease. Indian Pediatrics  1994;31:559-63.
  6. Nair S, Varghese R, Hashim S, Scariah P. Dysmorphic features and congenital heart disease in chromosome 6q deletion. Indian Journal of Human genetics 2012;18:127-29.
  7. Bertola DR, Sugayama SMM, Albano LMJ, Kim CA ,Gonzalez CH. Noonan Syndrome: A Clinical And Genetic Study Of 31 Patients. Rev. Hosp. Clin. Fac. Med. S.Paulo 1999;54:147-50.
  8. Hamilton WJ, Mossman HW. Human Embryology. Cambridge, W. Heffer and Sons Ltd, 1972,186-88 , 289.
  9. Sadler TW. Langman’s Medical Embyology. 9th edition. Baltimore, Maryland, USA:  Lippincott Williams and Wilkins; 2004.

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One article from every issue is selected for the ‘Best Article Award’. Authors of selected ‘Best Article’ are rewarded with a certificate. IJCRR Editorial Board members select one ‘Best Article’ from the published issue based on originality, novelty, social usefulness of the work. The corresponding author of selected ‘Best Article Award’ is communicated and information of award is displayed on IJCRR’s website. Drop a mail to editor@ijcrr.com for more details.

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A Study by Humaira Tahir et al. entitled "Comparison of First Analgesic Demand after Major Surgeries of Obstetrics and Gynecology between Pre-Emptive Versus Intra-Operative Groups by Using Intravenous Paracetamol: A Cross-Sectional Study" is awarded Best Article for Vol 14 issue 14
A Study by Monica K. entitled "Risk Predictors for Lymphoma Development in Sjogren Syndrome - A Systematic Review" is awarded Best Article for Vol 14 issue 13
A Study by Mokhtar M Sh et al. entitled "Prevalence of Hospital Mortality of Critically Ill Elderly Patients" is awarded Best Article for Vol 14 issue 12
A Study by Vidya S. Bhat et al. entitled "Effect of an Indigenous Cleanser on the Microbial Biofilm on Acrylic Denture Base - A Pilot Study" is awarded Best Article for Vol 14 issue 11
A Study by Pandya S. et al. entitled "Acute and 28-Day Repeated Dose Subacute Toxicological Evaluation of Coroprotect Tablet in Rodents" is awarded Best Article for Vol 14 issue 10
A Study by Muhammad Zaki et al. entitled "Effect of Hemoglobin Level on the Severity of Acute Bronchiolitis in Children: A Case-Control Study" is awarded Best Article for Vol 14 issue 09
A Study by Vinita S & Ayushi S entitled "Role of Colour Doppler and Transvaginal Sonography for diagnosis of endometrial pathology in women presenting with Abnormal Uterine Bleeding" is awarded Best Article for Vol 14 issue 08
A Study by Prabhu A et al. entitled "Awareness of Common Eye Conditions among the ASHA (Accredited Social Health Activist) Workers in the Rural Communities of Udupi District- A Pilot Study" is awarded Best Article for Vol 14 issue 07
A Study by Divya MP et al. entitled "Non-Echoplanar Diffusion-Weighted Imaging and 3D Fiesta Magnetic Resonance Imaging Sequences with High Resolution Computed Tomography Temporal Bone in Assessment and Predicting the Outcome of Chronic Suppurative Otitis Media with Cholesteatoma" is awarded Best Article for Vol 14 issue 06
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A Study by Ajai KG & Athira KN entitled "Patients’ Gratification Towards Service Delivery Among Government Hospitals with Particular Orientation Towards Primary Health Centres" is awarded Best Article for Vol 14 issue 04
A Study by Mbungu Mulaila AP et al. entitled "Ovarian Pregnancy in Kindu City, D.R. Congo - A Case Report" is awarded Best Article for Vol 14 issue 03
A Study by Maryam MJ et al. entitled "Evaluation Serum Chemerin and Visfatin Levels with Rheumatoid Arthritis: Possible Diagnostic Biomarkers" is awarded Best Article for Vol 14 issue 02
A Study by Shanthan KR et al. entitled "Comparison of Ultrasound Guided Versus Nerve Stimulator Guided Technique of Supraclavicular Brachial Plexus Block in Patients Undergoing Upper Limb Surgeries" is awarded Best Article for Vol 14 issue 01
A Study by Amol Sanap et al. entitled "The Outcome of Coxofemoral Bypass Using Cemented Bipolar Hemiarthroplasty in the Treatment of Unstable Intertrochanteric Fracture of Femur in a Rural Setup" is awarded Best Article Award of Vol 13 issue 24
A Study by Manoj KP et al. entitled "A Randomized Comparative Clinical Trial to Know the Efficacy of Ultrasound-Guided Transversus Abdominis Plane Block Against Multimodal Analgesia for Postoperative Analgesia Following Caesarean Section" is awarded Best Article Award of Vol 13 issue 23
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A Study by Ashish B Roge et al. entitled "Development, Validation of RP-HPLC Method and GC MS Analysis of Desloratadine HCL and It’s Degradation Products" is awarded Best Article of Vol 13 issue 21
A Study by Isha Gaurav et al. entitled "Association of ABO Blood Group with Oral Cancer and Precancer – A Case-control Study" is awarded Best Article for Vol 13 issue 20
A Study by Amr Y. Zakaria et al. entitled "Single Nucleotide Polymorphisms of ATP-Binding Cassette Gene(ABCC3 rs4793665) affect High Dose Methotrexate-Induced Nephrotoxicity in Children with Osteosarcoma" is awarded Best Article for Vol 13 issue 19
A Study by Kholis Ernawati et al. entitled "The Utilization of Mobile-Based Information Technology in the Management of Dengue Fever in the Community Year 2019-2020: Systematic Review" is awarded Best Article for Vol 13 issue 18
A Study by Bhat Asifa et al. entitled "Efficacy of Modified Carbapenem Inactivation Method for Carbapenemase Detection and Comparative Evaluation with Polymerase Chain Reaction for the Identification of Carbapenemase Producing Klebsiella pneumonia Isolates" is awarded Best Article for Vol 13 issue 17
A Study by Gupta R. et al. entitled "A Clinical Study of Paediatric Tracheostomy: Our Experience in a Tertiary Care Hospital in North India" is awarded Best Article for Vol 13 issue 16
A Study by Chandran Anand et al. entitled "A Prospective Study on Assessment of Quality of Life of Patients Receiving Sorafenib for Hepatocellular Carcinoma" is awarded Best article for Vol 13 issue 15
A Study by Rosa PS et al. entitled "Emotional State Due to the Covid – 19 Pandemic in People Residing in a Vulnerable Area in North Lima" is awarded Best Article for Vol 13 issue 14
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A Study by M. Muthu Uma Maheswari et al. entitled "A Study on C-reactive Protein and Liver Function Tests in Laboratory RT-PCR Positive Covid-19 Patients in a Tertiary Care Centre – A Retrospective Study" is awarded Best Article of Vol 13 issue 06 Special issue Modern approaches for diagnosis of COVID-19 and current status of awareness
A Study by Gainneos PD et al. entitled "A Comparative Evaluation of the Levels of Salivary IgA in HIV Affected Children and the Children of the General Population within the Age Group of 9 – 12 Years – A Cross-Sectional Study" is awarded Best Article of Vol 13 issue 05 Special issue on Recent Advances in Dentistry for better Oral Health
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A Study by Ghaffar UB et al. entitled "Correlation between Height and Foot Length in Saudi Population in Majmaah, Saudi Arabia" is awarded Best Article for Vol 12 issue 21
A Study by Siti Sarah Binti Maidin entitled "Sleep Well: Mobile Application to Address Sleeping Problems" is awarded Best Article for Vol 12 issue 20
A Study by Avijit Singh"Comparison of Post Operative Clinical Outcomes Between “Made in India” TTK Chitra Mechanical Heart Valve Versus St Jude Mechanical Heart Valve in Valve Replacement Surgery" is awarded Best Article for Vol 12 issue 19
A Study by Sonali Banerjee and Mary Mathews N. entitled "Exploring Quality of Life and Perceived Experiences Among Couples Undergoing Fertility Treatment in Western India: A Mixed Methodology" is awarded Best Article for Vol 12 issue 18
A Study by Jabbar Desai et al. entitled "Prevalence of Obstructive Airway Disease in Patients with Ischemic Heart Disease and Hypertension" is awarded Best Article for Vol 12 issue 17
A Study by Juna Byun et al. entitled "Study on Difference in Coronavirus-19 Related Anxiety between Face-to-face and Non-face-to-face Classes among University Students in South Korea" is awarded Best Article for Vol 12 issue 16
A Study by Sudha Ramachandra & Vinay Chavan entitled "Enhanced-Hybrid-Age Layered Population Structure (E-Hybrid-ALPS): A Genetic Algorithm with Adaptive Crossover for Molecular Docking Studies of Drug Discovery Process" is awarded Best article for Vol 12 issue 15
A Study by Varsha M. Shindhe et al. entitled "A Study on Effect of Smokeless Tobacco on Pulmonary Function Tests in Class IV Workers of USM-KLE (Universiti Sains Malaysia-Karnataka Lingayat Education Society) International Medical Programme, Belagavi" is awarded Best article of Vol 12 issue 14, July 2020
A study by Amruta Choudhary et al. entitled "Family Planning Knowledge, Attitude and Practice Among Women of Reproductive Age from Rural Area of Central India" is awarded Best Article for special issue "Modern Therapeutics Applications"
A study by Raunak Das entitled "Study of Cardiovascular Dysfunctions in Interstitial Lung Diseas epatients by Correlating the Levels of Serum NT PRO BNP and Microalbuminuria (Biomarkers of Cardiovascular Dysfunction) with Echocardiographic, Bronchoscopic and HighResolution Computed Tomography Findings of These ILD Patients" is awarded Best Article of Vol 12 issue 13 
A Study by Kannamani Ramasamy et al. entitled "COVID-19 Situation at Chennai City – Forecasting for the Better Pandemic Management" is awarded best article for  Vol 12 issue 12
A Study by Muhammet Lutfi SELCUK and Fatma entitled "Distinction of Gray and White Matter for Some Histological Staining Methods in New Zealand Rabbit's Brain" is awarded best article for  Vol 12 issue 11
A Study by Anamul Haq et al. entitled "Etiology of Abnormal Uterine Bleeding in Adolescents – Emphasis Upon Polycystic Ovarian Syndrome" is awarded best article for  Vol 12 issue 10
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A Study by Ilona Gracie De Souza & Pavan Kumar G. entitled "Effect of Releasing Myofascial Chain in Patients with Patellofemoral Pain Syndrome - A Randomized Clinical Trial" is awarded best article for  Vol 12 issue 08
A Study by Virendra Atam et. al. entitled "Clinical Profile and Short - Term Mortality Predictors in Acute Stroke with Emphasis on Stress Hyperglycemia and THRIVE Score : An Observational Study" is awarded best article for  Vol 12 issue 07
A Study by K. Krupashree et. al. entitled "Protective Effects of Picrorhizakurroa Against Fumonisin B1 Induced Hepatotoxicity in Mice" is awarded best article for issue Vol 10 issue 20
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List of Awardees

A Study by Ese Anibor et al. "Evaluation of Temporomandibular Joint Disorders Among Delta State University Students in Abraka, Nigeria" from Vol 13 issue 16 received Emerging Researcher Award


A Study by Alkhansa Mahmoud et al. entitled "mRNA Expression of Somatostatin Receptors (1-5) in MCF7 and MDA-MB231 Breast Cancer Cells" from Vol 13 issue 06 received Emerging Researcher Award


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