MyMedR

Successful treatment of very large congenital infantile fibrosarcoma

Hamidah A, Reena M, Halim ARA, Ibrahim S, Eguchi M, Zarina AL, et al.

Pediatr Int, 2011 Oct;53(5):768-770.

PMID: 21955012 DOI: 10.1111/j.1442-200X.2011.03358.x

Matched MeSH terms: Fibrosarcoma/congenital*; Hemangioma/congenital; Soft Tissue Neoplasms/congenital*

Fulltext Congenital mature cystic teratoma of the lateral neck presenting as cystic hygroma: a rare case report with literature review

Win TT, Razy N, Hamid S, Ubramanian AB, Ramalinggam G

Turk Patoloji Derg, 2014;30(3):220-4.

PMID: 24715558 DOI: 10.5146/tjpath.2013.01220

Teratoma of the neck is a rare extra-gonadal teratoma. Although it can be seen in adult, most of the cases present in fetal life or early childhood. Grading and classification of teratoma is important for management and prognosis. Decision making between mature and immature teratoma is sometime challenging if it is not composed of extensive unusual neuroectodermal tissue component. We reported a rare case of huge congenital mature cystic teratoma composted of various neuroectodermal tissues in an 8-month-old boy; it was located at the lateral neck and mimicked congenital cystic hygroma. A literature review was done to grade and classify the tumour.

Matched MeSH terms: Head and Neck Neoplasms/congenital; Teratoma/congenital; Lymphangioma, Cystic/congenital

Research on quality of life in female patients with congenital adrenal hyperplasia and issues in developing nations

Zainuddin AA, Grover SR, Shamsuddin K, Mahdy ZA

J Pediatr Adolesc Gynecol, 2013 Dec;26(6):296-304.

PMID: 23507003 DOI: 10.1016/j.jpag.2012.08.004

Congenital adrenal hyperplasia (CAH) is the commonest cause of ambiguous genitalia for female newborns and is one of the conditions under the umbrella term of "Disorders of Sex Development" (DSD). Management of these patients require multidisciplinary collaboration and is challenging because there are many aspects of care, such as the most appropriate timing and extent of feminizing surgery required and attention to psychosexual, psychological, and reproductive issues, which still require attention and reconsideration, even in developed nations. In developing nations, however, additional challenges prevail: poverty, lack of education, lack of easily accessible and affordable medical care, traditional beliefs on intersex, religious, and cultural issues, as well as poor community support. There is a paucity of long-term outcome studies on DSD and CAH to inform on best management to achieve optimal outcome. In a survey conducted on 16 patients with CAH and their parents in a Malaysian tertiary center, 31.3% of patients stated poor knowledge of their condition, and 37.5% did not realize that their medications were required for life. This review on the research done on quality of life (QOL) of female patients with CAH aims: to discuss factors affecting QOL of female patients with CAH, especially in the developing population; to summarize the extant literature on the quality of life outcomes of female patients with CAH; and to offer recommendations to improve QOL outcomes in clinical practice and research.

Matched MeSH terms: Adrenal Hyperplasia, Congenital/etiology; Adrenal Hyperplasia, Congenital/psychology*; Adrenal Hyperplasia, Congenital/therapy

Prevalence and outcome of peripartum cardiomyopathy in Malaysia

Chee KH, Azman W

Int J Clin Pract, 2009 May;63(5):722-5.

PMID: 18028390

Peripartum cardiomyopathy (PPCM) is an uncommon form of congestive heart failure, afflicting obstetric patients around the time of delivery. The epidemiology of PPCM is infrequently reported. To the best of our knowledge, there has been no report from Asia.

Matched MeSH terms: Heart Defects, Congenital/epidemiology*; Cardiomyopathies/congenital

Congenital rubella syndrome: a review of laboratory data from 2002 to 2011

Saraswathy TS, Rozainanee MZ, Asshikin RN, Zainah S

Southeast Asian J Trop Med Public Health, 2013 May;44(3):429-35.

PMID: 24050074

Rubella infection in pregnant women during the first trimester of pregnancy can lead to fetal anomalies, commonly known as congenital rubella syndrome (CRS). The objective of our study was to analyze the serological test results among infants suspected of having CRS aged < or = 12 months compared with their clinical status. Between January 2002 and December 2011, 3,279 serum samples from infants aged < or = 12 months from government hospitals in Malaysia were examined for rubella specific IgM and IgG antibodies using a Axsym, automated analyzer (Abbott Laboratories). Forty-eight samples were positive for rubella specific IgM antibodies and 494 samples were positive for rubella specific IgG antibodies. These were then age stratified and their clinical history reviewed for any CRS symptoms. Fifteen of 38 rubella IgM positive infants (39.5%) aged < 3 months, had a clinical appearance compatible with CRS. However, only 1 IgM positive infant aged 3 to 6 months and one infant aged 7 to 11 months had clinical appearance compatible with CRS. The most common abnormal findings in these cases were congenital heart defects and cataracts. Forty-eight point eight percent of IgM positive cases and 53.1% of IgG positive cases, had inadequate information in the chart to determine the presence of CRS. Clinical findings and timely laboratory diagnosis to determine the presence of CRS are important in infants born with congenital defects. Physicians should also be aware of the appropriate interpretation of these findings.

Matched MeSH terms: Rubella Syndrome, Congenital/blood; Rubella Syndrome, Congenital/diagnosis*; Rubella Syndrome, Congenital/epidemiology

Fulltext Complete congenital third branchial fistula: does the theoretical course apply?

Aneeza WH, Mazita A, Marina MB, Razif MY

Singapore Med J, 2010 Jul;51(7):e122-5.

PMID: 20730387

The course of a third branchial fistula is derived from its embryological origin, in accordance with the branchial apparatus theory. Treatment of this condition requires complete removal of the tract in order to avoid recurrence; however, this can pose a risk to the surrounding structures. We report the case of a complete third branchial fistula as well as a literature review on its theoretical course and management.

Matched MeSH terms: Congenital Abnormalities/radiography; Congenital Abnormalities/surgery; Cutaneous Fistula/congenital*

OHVIRA syndrome: clinical implications of a delayed diagnosis

Mohd Adzlan F, Mohd K, Ahmad N, Ramli R

BMJ Case Rep, 2024 May 22;17(5).

PMID: 38782440 DOI: 10.1136/bcr-2024-259861

Obstructed Hemi Vagina with Ipsilateral Renal Agenesis (OHVIRA) syndrome is a rarely encountered müllerian duct anomaly. Delayed diagnosis is common due to normal onset of puberty and menstruation. We report a case of a woman in her early 20s with a background history of multiple emergency department visits, ward admissions and surgeries for chronic abdominal pain. She was reviewed at 1 month postlaparotomy for recurrent pelvic abscess and was finally diagnosed to have an OHVIRA syndrome, 11 years after her first clinical presentation. Excision of the vaginal septum completely resolved her symptoms. We are reporting this case to highlight the clinical implications resulting from the delayed diagnosis, to look into factors contributing to the delay and to highlight the importance of having a high index of suspicion to diagnose this unique condition.

Matched MeSH terms: Congenital Abnormalities/diagnosis; Congenital Abnormalities/surgery; Kidney Diseases/congenital

Fulltext Ebstein's malformation of the tricuspid valve--a report of six cases

Danaraj TJ, LaBrooy EB

Singapore Med J, 1960 Mar;1(1):16-32.

PMID: 13719549

Six cases of Ebstein's malformation are reported. The notable clinical features seen in four patients were gross enlargement of the
heart, venous congestion and marked hepatosplenomegaly. There was complete heart block in one patient and auricular fibrillation in the remaining three. The unusual pathologic features in the four cases that came to necropsy were the large pericardial effusions associated with the cardiomegaly, the absence of an atrial septal defect or patent foramen ovale, the gross enlargement of liver with cirrhotic changes and the large spleen. Histologic examination of heart muscle revealed certain unusual features which are described.

Matched MeSH terms: Heart Defects, Congenital*

Fulltext The entrapped twin: a case of fetus-in-fetu

Yaacob R, Zainal Mokhtar A, Abang Jamari DZH, Jaafar N

BMJ Case Rep, 2017 Sep 23;2017.

PMID: 28942402 DOI: 10.1136/bcr-2017-220801

Fetus-in-fetu (FIF) is a rare entity in which malformed parasitic twin grows inside the body of its twin. It is most commonly presented with mass in the abdomen. We present a case of a 15-year-old boy who presented with abdominal mass since infancy. Radiological investigations are suggestive of FIF. Intraoperatively, malformed fetus in a sac was found and excised. Postoperatively the patient recovers well and was put on follow-up.

Matched MeSH terms: Congenital Abnormalities/surgery

Congenital arhinia - First published case in Malaysia

Ng RL, Rajapathy K, Ishak Z

Med J Malaysia, 2017 10;72(5):308-310.

PMID: 29197888 MyJurnal

Congenital arhinia is one of the rare craniofacial malformation that may cause severe respiratory distress at birth due to upper airway obstruction. Our patient, whose abnormalities were only detected after delivery in our centre, is the first reported case of congenital arhinia in Malaysia. Contrary to popular belief that neonates are obligate nasal breather, our patient adapted well to breathing through mouth before an elective tracheostomy was performed on day four of life.

Matched MeSH terms: Congenital Abnormalities/physiopathology*

Fulltext ECMO in children post cardiac surgery-opportunity for redress

Alwi M

Anatol J Cardiol, 2017 12;18(6):431-432.

PMID: 29256879 DOI: 10.14744/AnatolJCardiol.2017.26116

Matched MeSH terms: Heart Defects, Congenital*

Protein loosing enteropathy after Fontan procedure

Vijayaraghavan R, Thay WY

Med J Malaysia, 2018 06;73(3):175-176.

PMID: 29962503 MyJurnal

Fontan and Baudet described the procedure in 1971 and was very useful to improve the quality of life in many complex cyanotic heart diseases. It has gone through various modifications since then to improve the outcome. The mortality was reported as 2.1% and survival rate of at 5 years, 10 years, 15 years and 20 years were 91%, 80%, 73%and 69% respectively. Though it was a useful palliative procedure to improve the quality of life, it has complications which may affect the morbidity and mortality like Protein loosing enteropathy ,reduced exercise capacity thromboembolism. The patients with protein losing enteropathy present with Pleural effusion, ascites, and edema and they need periodic replacement of Albumin and frequent reviews and close follow up in the management to reduce the mortality and to improve the quality of life .These complications we come across and they need our support in the management at district level hospitals.

Matched MeSH terms: Heart Defects, Congenital/surgery

Fulltext Prevalence and risk factors of gestational diabetes mellitus in Asia: a systematic review and meta-analysis

Lee KW, Ching SM, Ramachandran V, Yee A, Hoo FK, Chia YC, et al.

BMC Pregnancy Childbirth, 2018 Dec 14;18(1):494.

PMID: 30547769 DOI: 10.1186/s12884-018-2131-4

BACKGROUND: Gestational diabetes mellitus (GDM) is a of the major public health issues in Asia. The present study aimed to determine the prevalence of, and risk factors for GDM in Asia via a systematic review and meta-analysis.

METHODS: We systematically searched PubMed, Ovid, Scopus and ScienceDirect for observational studies in Asia from inception to August 2017. We selected cross sectional studies reporting the prevalence and risk factors for GDM. A random effects model was used to estimate the pooled prevalence of GDM and odds ratio (OR) with 95% confidence interval (CI).

RESULTS: Eighty-four studies with STROBE score ≥ 14 were included in our analysis. The pooled prevalence of GDM in Asia was 11.5% (95% CI 10.9-12.1). There was considerable heterogeneity (I2 > 95%) in the prevalence of GDM in Asia, which is likely due to differences in diagnostic criteria, screening methods and study setting. Meta-analysis demonstrated that the risk factors of GDM include history of previous GDM (OR 8.42, 95% CI 5.35-13.23); macrosomia (OR 4.41, 95% CI 3.09-6.31); and congenital anomalies (OR 4.25, 95% CI 1.52-11.88). Other risk factors include a BMI ≥25 kg/m2 (OR 3.27, 95% CI 2.81-3.80); pregnancy-induced hypertension (OR 3.20, 95% CI 2.19-4.68); family history of diabetes (OR 2.77, 2.22-3.47); history of stillbirth (OR 2.39, 95% CI 1.68-3.40); polycystic ovary syndrome (OR 2.33, 95% CI1.72-3.17); history of abortion (OR 2.25, 95% CI 1.54-3.29); age ≥ 25 (OR 2.17, 95% CI 1.96-2.41); multiparity ≥2 (OR 1.37, 95% CI 1.24-1.52); and history of preterm delivery (OR 1.93, 95% CI 1.21-3.07).

CONCLUSION: We found a high prevalence of GDM among the Asian population. Asian women with common risk factors especially among those with history of previous GDM, congenital anomalies or macrosomia should receive additional attention from physician as high-risk cases for GDM in pregnancy.

TRIAL REGISTRATION: PROSPERO (2017: CRD42017070104 ).

Matched MeSH terms: Congenital Abnormalities/epidemiology*

Contrast echocardiogram in diagnosing rare type of persistent left superior vena cava

Koay HS, Anis M, Mokhtar SA

Med J Malaysia, 2018 12;73(6):413-414.

PMID: 30647217

We report a rare case of persistent left superior vena cava (PLSVC) with direct drainage into the left atrium in a 3-yearsold boy who had been electively admitted for hypospadias repair, when he was noticed to have finger clubbing and mild hypoxia but was otherwise asymptomatic. The diagnosis of PLSVC can be made without an invasive tool as direct drainage of PLSVC into the left atrium be visualised using transthoracic echocardiography (TTE) by injecting agitated saline into the left arm.

Matched MeSH terms: Heart Defects, Congenital/diagnosis

Paediatric cataract surgery in Hospital Kuala Lumpur - A 5-year review of visual outcomes

Chew FLM, Qurut SE, Hassan I, Lim ST, Ramasamy S, Rahmat J

Med J Malaysia, 2019 02;74(1):15-19.

PMID: 30846656

INTRODUCTION: Paediatric cataract surgery is challenging with reported post-operative visual acuity (VA) of 0.3LogMar or better varying between 33% to 68% of patients.

OBJECTIVE: The aim is to document the post-operative refraction, VA and complications of non-traumatic pediatric cataract surgery performed in a tertiary referral center in Malaysia.

METHODOLOGY: This retrospective study reviewed case notes of all consecutive patients aged 12 years and below who underwent cataract surgery from January 2010 to December 2015. Patients were recruited if they had a minimum of six months post-operative follow-up. Exclusion criteria included traumatic cataract, central nervous system abnormalities, incomplete medical records or pre-existing ocular pathology. Subjects were divided into two groups based on refraction at one month. Subjects with refraction within 1- dioptre of the targeted spherical equivalent were in the success group and the rest were in the failure group.

RESULTS: A total of 111 subjects were recruited (65 subjects in success group and 46 subjects in the failure group). Mean age at surgery was 33.14 (SD: 33.47) months. The success group had significantly longer axial length (p:0.0045, CI: 0.566-0.994, OR: 0.750). At final review, 44.1%(49/111) subjects had visual acuity of 0.3LogMar or better. The success group had better final mean VA in comparison to the failure group (p:0.034, CI:1.079-7.224, OR: 2.791).

CONCLUSION: The outcome of non-traumatic paediatric cataract surgery was acceptable with 58.6% achieved targeted refractive correction at 1-month post-operative period. Longer axial length was associated with better refractive outcome. Capsule related complications was the most common intra-operative complication.

Matched MeSH terms: Cataract/congenital

Congenital lobar emphysema: a case with bronchial septum

Saim L, Mohamad AS, Ambu VK

Int J Pediatr Otorhinolaryngol, 1994 Jan;28(2-3):241-6.

PMID: 8157425

Congenital lobar emphysema has been described under various designations. Although the etiology is not clear, some form of bronchial obstruction has been documented in about 50% of the patients. A rare case of congenital lobar emphysema with a membranous septum in the left main bronchus is described. Although prompt lobectomy has been the treatment of choice in most cases, this case demonstrates the importance of early bronchoscopy to exclude treatable intra luminal abnormalities.

Matched MeSH terms: Pulmonary Emphysema/congenital*

Fulltext Congenital diaphragmatic hernia in a post-partum woman

Abdullah M

Med J Malaysia, 2003 Mar;58(1):99-101.

PMID: 14556332

Congenital diaphragmatic hernia (CDH) is rare in adults. We report a 24 year old woman presenting with shortness of breath, chest pain and nausea after the birth of her first baby. Clinical examination, plain radiography and a CT scan revealed herniation of abdominal contents into her left chest. Via a midline laparotomy, the contents were reduced and the defect repaired, using a mesh. She remains symptom-free three years since her surgery and even after a second childbirth. A brief review of the literature reporting adult diaphragmatic hernia of congenital origin accompanies this case report. We conclude that symptomatic CDH in adults usually presents as an emergency with gastrointestinal and occasionally respiratory complications. Early diagnosis and repair is essential to avoid subsequent morbidity and mortality.

Matched MeSH terms: Hernias, Diaphragmatic, Congenital*

Fulltext Congenital (Bochdalek) diaphragmatic hernia: problems in diagnosis and management

Laidin AZ, Al Rashid Z, Mohd Nor M

Med J Malaysia, 1984 Sep;39(3):185-91.

PMID: 6544919

A review of 24 children with posterolateral (Bochdalek) diaphragmatic hernia over a five-year period was carried out to highlight the problems of diagnosis and' management. Nine children were delivered in the Maternity Hospital Kuala Lumpur, giving an incidence of 1:10,000 live births which is half the expected incidence. Difficulty in diagnosis is apparent from the large number of initially misdiagnosed cases (29%) and those not detected
soon after birth (71% diagnosed after 24 hours). Less than half the babies had associated anomalies, commonest being malrotation and ipsilateral lung hypoplasia. Mortality (20.8%) appears to be related to the degree of lung hypoplasia and shunting, and the birthweight of the babies. Current evidence indicates that pulmonary hypertension is the main factor in the chain of events beginning with lung hypoplasia, which ultimately leads to their demise. Various methods to overcome this complication have been evolved which appear to give some hope for these high-risk infants.

Matched MeSH terms: Hernias, Diaphragmatic, Congenital*

Fulltext Tracheoesophageal fistula (TOF)-blind pouch intubation: a case report

Arshad R

Med J Malaysia, 1984 Jun;39(2):159-62.

PMID: 6513857

A case of Tracheoesophageal Fistula (TOF) was presented where the blind upper esophageal pouch was mistakenly intubated; in spite of this, adequate lung ventilation was possible for more than one hour. This was only noticed by the surgeon upon incision of the lower end of the pouch.

Matched MeSH terms: Tracheoesophageal Fistula/congenital*

Fulltext Congenital rubella syndrome in Malaysia

Tan DSK

Med J Malaysia, 1985 Mar;40(1):11-4.

PMID: 3831727

Of the five diseases generally recognised as causing congenital defects, viz., toxoplasmosis, rubella, cy tomegaloviral infection, herpes simplex and syphilis (TORCHES) studied in Malaysia, rubella was found to be the most important. A total of 574 children with features of congenital rubella syndrome (CRS) were examined for rubella-specific IgM (in infants four months and below), and for rubella HAl antibodies (in children six months to four-years-old), and compared with 374 normal children of the same age groups. Whereas the prevalence rate of rubella in normal children was only 1.3%, in children with CRS (multiple defects) it was 87.3%; with congenital heart disease 71.0%; with congenital cataract 64.0%; with deafness 60.1%; with rash 30.8%; with hepatomegaly 17.1%; with mental retardation 4.1 %. Congenital rubella was not important as a cause of neonatal jaundice (0.9%)
and CNS defects (0%).

Matched MeSH terms: Rubella/congenital*

Displaying publications 61 - 80 of 336 in total