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Reconstruction of large sacral defects following tumour resection: a report of two cases

Cheong YW, Sulaiman WA, Halim AS

J Orthop Surg (Hong Kong), 2008 Dec;16(3):351-4.
PMID: 19126905

Sacral tumours often present surgical resection and reconstruction challenges. Wide resections result in large sacral defects and neoadjuvant radiotherapy impairs wound healing. The wounds need to be covered with bulky, well-vascularised, healthy tissues. We present 2 cases where large sacral defects were reconstructed following tumour resection. Both defects were reconstructed with inferiorly based, transpelvic, pedicled vertical rectus abdominis myocutaneous flaps. This is a robust flap and carries a well-vascularised muscle bulk and skin paddle. The donor site is distant from the lesion site and is thus unaffected by both the resection and radiotherapy. This is a useful flap for reconstructing large sacral defects.
Fulltext Inconspicuous Nasoethmoidal Encephalocele Might Be Wrongly Diagnosed

Tang WJ, Wan Sulaiman WA

Arch Plast Surg, 2016 May;43(3):291-2.
PMID: 27218030 DOI: 10.5999/aps.2016.43.3.291
Usual Presentation Has Odds: Unilateral Tibial Hemimelia in One of Dizygotic Twins

Al-Chalabi MMM, Wan Sulaiman WA

Cureus, 2021 Jan 21;13(1):e12834.
PMID: 33633877 DOI: 10.7759/cureus.12834

Tibial hemimelia is a relatively rare congenital tibial longitudinal deficiency (approximately 1 per 1 million live births), unilateral or bilateral, with a relatively intact fibula. Hemimelia results from a disruption of the lower limb developmental field during embryogenesis due to slow or even abort of chondrification process, which results in leg length discrepancy. Affected leg commonly appears short and deformed with knee, ankle, and foot involvement. It may present with a variety of associated anomalies. Surgical treatment varies according to the type and degree of deformity, and reconstructive interventions are still limited. Reported cases of tibial hemimelia are very infrequent, especially tibial hemimelia in twins. Usually, the cases were in single embryo or less frequently in one of the monozygotic twins, but no reported cases regarding tibial hemimelia in one of the dizygotic twins as this article reports.
Functional Outcomes After Heel Pad Reconstruction: A Review of 7 Cases

Khai Luen K, Wan Sulaiman WA

J Foot Ankle Surg, 2017 8 27;56(5):1114-1120.
PMID: 28842095 DOI: 10.1053/j.jfas.2017.04.024

Sensate, durable heel pad reconstruction is challenging. The present study assessed the functional outcomes after heel pad reconstruction using various flap techniques at our institution. From June 2011 to June 2016 (5-year period), 7 consecutive patients underwent heel pad reconstruction for various etiologies, with 3 microvascular free flaps (42.9%; 2 musculocutaneous flaps [66.7%] and 1 contralateral medial plantar flap [33.3%]) and 4 local pedicle flaps (57.1%; 3 instep medial plantar artery flaps [75.0%] and 1 distally based reverse sural flap [25.0%]). The patient records and demographic data were reviewed, and surgically related information was obtained and analyzed. The subjective components of the American Orthopaedic Foot and Ankle Society hindfoot clinical ratings scale were used to evaluate the pain and functional outcomes. Sensation was assessed using Semmes-Weinstein monofilaments, and ulcer recurrence was recorded. The mean age of the patients was 41.7 (range 11 to 70) years, the mean defect size was 59 (range 12 to 270) cm2, and the mean follow-up duration was 22 (range 15 to 43) months. Complete flap survival was achieved without significant complications in all 7 patients. Patients treated with the sensate medial plantar artery flap recorded the highest mean American Orthopaedic Foot and Ankle Society score of 57.3 (maximum score of 60) and experienced a return of deep sensation at 6 (range 6 to 24) months and protective sensation at 1 year. This was followed by the reverse sural flap and the musculocutaneous flap. No recurrent heel ulceration was observed in our series of patients. In conclusion, the sensate medial plantar flap is a satisfactory method for coverage of small- to moderate-size heel defects.
Mutation screening of IRF6 among families with non-syndromic oral clefts and identification of two novel variants: review of the literature

Salahshourifar I, Wan Sulaiman WA, Halim AS, Zilfalil BA

Eur J Med Genet, 2012 Jun;55(6-7):389-93.
PMID: 22440537 DOI: 10.1016/j.ejmg.2012.02.006

Non-syndromic oral clefts share the main clinical features of Van der Woude Syndrome (VWS), with the exception of the lower lip pit. Thus, about 15% of VWS cases are indistinguishable from cases with non-syndromic oral clefts. IRF6 mutations are the major cause of VWS; however, variants in this gene show strong association with non-syndromic oral clefts, with a higher increased risk among cases with cleft lip only (CLO). A total of 39 individuals, including 16 patients with CLO and 23 patients with a family history of cleft, were examined for IRF6 mutations in the present study. Seven variants, including five known (c.-75-4 A>; G, c.-73T>; C, c.459G>; T 5, c.820G>; A, and c.1060 + 37C>; T) and two novel (c.-75-23G>; C and c.1380G>; T), were found. Both novel variants were inherited from non-affected parents and we did not find also in the 120 control chromosomes. In silico analysis revealed that both c.1380G>; T and c.-75-23G>; C variants may disrupts a putative exonic splicing enhancer and intronic splicing binding site for SC35, respectively. Taken together, the presence of deleterious IRF6 variants in patients with non-syndromic oral clefts could be most likely an evidence for VWS. While, IRF6 variants could, at best, contribute to clefting as part of a complex inheritance pattern, with both additional genes and environmental factors having a role.
Contribution of MSX1 variants to the risk of non-syndromic cleft lip and palate in a Malay population

Salahshourifar I, Halim AS, Wan Sulaiman WA, Zilfalil BA

J Hum Genet, 2011 Nov;56(11):755-8.
PMID: 21866112 DOI: 10.1038/jhg.2011.95

Oral clefts are clinically and genetically heterogeneous disorders that are influenced by both genetic and environmental factors. The present family-based association study investigated the role of the MSX1 and TGFB3 genes in the etiology of non-syndromic oral cleft in a Malay population. No transmission distortion was found in the transmission disequilibrium analysis for either MSX1-CA or TGFB3-CA intragenic markers, whereas TGFB3-CA exhibited a trend to excess maternal transmission. In sequencing the MSX1 coding regions in 124 patients with oral cleft, five variants were found, including three known variants (A34G, G110G and P147Q) and two novel variants (M37L and G267A). The P147Q and M37L variants were not observed in 200 control chromosomes, whereas G267A was found in one control sample, indicating a very rare polymorphic variant. Furthermore, the G110G variant displayed a significant association between patients with non-syndromic cleft lip, with or without cleft palate, and normal controls (P=0.001, odds ratio=2.241, 95% confidence interval, 1.357-3.700). Therefore, these genetic variants may contribute, along with other genetic and environmental factors, to this condition.
Contribution of variants in and near the IRF6 gene to the risk of nonsyndromic cleft lip with or without cleft palate in a Malay population

Salahshourifar I, Sulaiman WA, Zilfalil BA, Halim AS

Am J Med Genet A, 2011 Sep;155A(9):2302-7.
PMID: 21834040 DOI: 10.1002/ajmg.a.34169

Several studies have shown evidence for the contribution of interferon regulatory factor 6 (IRF6) variants to the risk of nonsyndromic oral clefts in Asians; however, this has not included the Malay population. The current study attempts to address this research gap using allele and haplotype transmission disequilibrium analyses. The results showed a strong transmission distortion for multiple haplotypes to patients with nonsyndromic cleft lip with or without cleft palate. Haplotypes carrying the 243 bp allele of D1S2136 and common alleles at the rs861019 and rs2235371 were over-transmitted to patients. By contrast, haplotypes consisting of the 251 bp allele of D1S2136 and the rare allele at rs2235371 were more under-transmitted. Furthermore, several variants and haplotypes showed excess maternal transmission, but none of them attained statistical significance in maternal relative risk analyses. In contrast, a significant child genotype effect was observed for several haplotypes, indicating fetal genotype could be the major genetic contribution rather than maternal genotype. The present study therefore further supports a role for IRF6 variants in clefting in this Southeast Asian population. Overall, Asian genetic backgrounds are most likely more susceptible to the haploinsufficiency of IRF6 variants. These variants may contribute to the condition either themselves, or they may be in linkage disequilibrium with other casual variants.
Maternal uniparental heterodisomy of chromosome 6 in a boy with an isolated cleft lip and palate

Salahshourifar I, Halim AS, Sulaiman WA, Zilfalil BA

Am J Med Genet A, 2010 Jul;152A(7):1818-21.
PMID: 20583164 DOI: 10.1002/ajmg.a.33526

We describe a chromosome 6 uniparental disomy (UPD6) in a boy, discovered during a screening for the genetic cause of cleft lip and palate. In the medical literature, almost all documented cases of UPD6 are paternal in origin, and only four were maternal. We present here a report of complete maternal chromosome 6 uniparental heterodisomy. Haplotype analysis was performed using highly polymorphic short tandem repeat (STR) markers that span both arms of chromosome 6. Analysis of these markers revealed the presence of two maternal alleles but no paternal allele, indicating an instance of maternal uniparental heterodisomy. Chromosome analysis of peripheral blood lymphocytes confirmed a normal male karyotype. Advanced maternal age at the time of the infant's birth and heterodisomy of markers around the centromere favors a meiosis-I error. No specific phenotype has been reported for maternal UPD6. Therefore, the cleft lip and palate in the present case probably occurred due to other risk factors. This report provides further evidence that maternal UPD6 has no specific clinical consequences and adds to the collective knowledge of this rare chromosomal finding.
Use of dermal regeneration template (INTEGRA) in reconstructive burn surgery

Sasidaran R, Dorai AA, Sulaiman WA, Halim AS

Med J Malaysia, 2008 Jul;63 Suppl A:29.
PMID: 19024967

We present our two year experience with a dermal regeneration template (INTEGRA) in burn reconstructive surgery for contracture release as well as a reconstructive tool for management of soft tissue loss.
Fulltext Rapidly Growing Huge Lower Back Malignant Peripheral Nerve Sheath Tumour

Abuzarifa N, Al-Chalabi MMM, Wan Sulaiman WA

Cureus, 2021 Mar 05;13(3):e13712.
PMID: 33842103 DOI: 10.7759/cureus.13712

Malignant peripheral nerve sheath tumours (also called neurofibrosarcomas) are a rare, highly aggressive soft tissue sarcomas that arise from the peripheral nerves or cells associated with the nerve sheath, such as Schwann cells, peri-neural cells and fibroblasts. It is representing 10% of all soft tissue sarcomas in which it is considered as an extremely rare malignancy, especially in patients with neurofibromatosis type I. In the general population, it affects approximately 1 in 100,000 people. This article is reporting a 56-year-old Malay female patient who is a known case of neurofibromatosis type I for 20 years, presented with a lower back, pruritic, gradually increasing swelling during the last five months. Last month before the presentation, the lesion rapidly grows, reaching a size of (15×15 cm), accompanied by foul-smelling discharge and pain exacerbated with movement. Although no history of preceding trauma or accident, the mass bleeds within contact. In conclusion, only a few cases of giant malignant peripheral nerve sheath tumours reported in the literature describing its location and growth progression. We present a massive, extremely rapid growth of cutaneous exophytic malignant peripheral nerve sheath tumours over the lower back.
Heel Pad Reconstruction With Medial Plantar Flap

Langat AS, Wan Sulaiman WA, Mat Johar SFN

Cureus, 2021 Mar 19;13(3):e13987.
PMID: 33884238 DOI: 10.7759/cureus.13987

The heel of the foot is covered by highly specialized thick, glabrous skin containing fibroadipose tissue with numerous fibrous septae traversing the subcutaneous tissue, which acts as a shock-absorbent and prevents shearing of the skin. The loss of heel pad would cause interruption of the propelling function of the foot during walking. Therefore, heel pad reconstruction is an important procedure for wound closure in the acute phase and also functional reconstruction in delayed cases. We report a case of heel pad deformity in a patient who presented to us with left heel pain and inability to fully bear weight, which has caused her walking difficulty, following a road traffic accident. She sustained a degloving injury of the left foot and an open fracture of left calcaneum with ruptured Tendon Achilles in which the wound was initially addressed with failed reverse sural flap and the wound was allowed to heal by secondary intention. Delayed heel reconstruction was carried out with a propeller medial plantar flap and split skin graft. Postoperatively, the patient had improved functional and esthetic outcome.
An Intriguing Case of Purple Urine Bag Syndrome

Wan Sulaiman WA, Hoo FK, Inche Mat LN

Am J Med Sci, 2017 May;353(5):e9.
PMID: 28502345 DOI: 10.1016/j.amjms.2016.11.022
Fulltext Integra: An Alternative Option for Reconstruction of Extensive Finger Defects With Exposed Bones

Hamdan AM, Al-Chalabi MMM, Wan Sulaiman WA

Cureus, 2021 Jul;13(7):e16223.
PMID: 34367823 DOI: 10.7759/cureus.16223

Extensive soft tissue injuries with exposed joints, bones, and tendons due to trauma of the hand -particularly fingers- necessitate soft tissue reconstruction and coverage. However, these injuries are challenging; therefore, various management options for traumatic skin loss over fingers are widely performed. These options comprise wound care permitting wound contracture without surgical intervention, full or split-thickness skin grafting, skeletal shortening and primary closure, and various types of flaps. We present a case of successfully placed Integra over the exposed phalangeal bones followed by split-thickness skin grafting a few weeks later, with a good outcome. We conclude that Integra is an alternative, safe and effective method for reconstructing severely traumatized fingers with exposed bone, tendon, or joint without sacrificing outcome.
Fulltext Expression profiling of genes related to endothelial cells biology in patients with type 2 diabetes and patients with prediabetes

Moradipoor S, Ismail P, Etemad A, Wan Sulaiman WA, Ahmadloo S

Biomed Res Int, 2016;2016:1845638.
PMID: 27781209 DOI: 10.1155/2016/1845638

Endothelial dysfunction appears to be an early sign indicating vascular damage and predicts the progression of atherosclerosis and cardiovascular disorders. Extensive clinical and experimental evidence suggests that endothelial dysfunction occurs in Type 2 Diabetes Mellitus (T2DM) and prediabetes patients. This study was carried out with an aim to appraise the expression levels in the peripheral blood of 84 genes related to endothelial cells biology in patients with diagnosed T2DM or prediabetes, trying to identify new genes whose expression might be changed under these pathological conditions. The study covered a total of 45 participants. The participants were divided into three groups: group 1, patients with T2DM; group 2, patients with prediabetes; group 3, control group. The gene expression analysis was performed using the Endothelial Cell Biology RT(2) Profiler PCR Array. In the case of T2DM, 59 genes were found to be upregulated, and four genes were observed to be downregulated. In prediabetes patients, increased expression was observed for 49 genes, with two downregulated genes observed. Our results indicate that diabetic and prediabetic conditions change the expression levels of genes related to endothelial cells biology and, consequently, may increase the risk for occurrence of endothelial dysfunction.
Primary Bone Leiomyosarcoma in Neurofibromatosis Type 1: Extremely Rare Concurrency

Abualjubain IJ, Al-Chalabi MMM, Wan Sulaiman WA

Cureus, 2021 Dec;13(12):e20811.
PMID: 35141069 DOI: 10.7759/cureus.20811

Neurofibromatosis type 1 (NF1) is a complex autosomal dominant, multisystem genetic disease affecting about 1 in 3500 individuals. Plexiform neurofibromas represent a rare variant (30%) of NF1 in which the spread of tumor cells along nerve fascicles leads to a diffuse mass of thickened nerve fibers. Affected patients with NF1 have a greater chance of developing soft tissue sarcomas than the general population. Leiomyosarcoma is one of the most frequent soft tissue sarcomas, seldom observed in patients with NF1. Herein we report a rare concurrency of bone leiomyosarcoma in a patient with a plexiform neurofibroma, adding to the few reported cases of leiomyosarcomas in patients with NF1. Our case is a 14-year-old male who is a known case of NF1 and presented with a four-month history of pain and swelling on the medial side of the right knee. Imaging and biopsy confirmed the diagnosis of leiomyosarcoma. Based on the authors' knowledge and search, this is the first reported case of plexiform neurofibroma with a primary bone leiomyosarcoma, representing an extremely rare concurrency. Patients with such uncommon tumors should be assessed regularly, and continuous follow-up is essential.
Fulltext Prevention of firecracker injuries via the digital platform: A Malaysian experience

Mat Johar F, Halim AS, Wan Sulaiman WA, Muhamad H

Burns, 2021 11;47(7):1683-1687.
PMID: 33715940 DOI: 10.1016/j.burns.2021.02.007

INTRODUCTION: Health campaigns include physical campaigns in schools, community halls, or community malls. They can also be broadcast via television, newspaper, and radio. We launched a health campaign on social media platforms that have a powerful impact in this digital era.
METHODS: A three-dimensional short animation was developed for the Burn and Blast Injury Awareness campaign. It was launched during the fasting month and Eid 2020 when firecracker-related injuries are highly incident in Malaysia. The video was launched primarily on Universiti Sains Malaysia (USM) and Wau Animation Sdn Bhd social media platforms. Each party shared the video on the top 3 social media platforms, which are Facebook, Instagram, and YouTube. The numbers of viewers, reaches, and shares, and demographic data were captured at 1 month after the release.
RESULTS: We recorded 29,585 views, 60,920 reach, and 874 shares from the USM and Wau Animation platforms alone. The USM Facebook platform showed predominant female viewership (60%), whereas the Wau Facebook platform showed predominant male viewership (66%). In both platforms, the viewers were aged 18-34 years.
CONCLUSION: Health awareness campaigns on digital platforms are powerful because the message spreads faster, and it is also safe during the pandemic.
Fulltext Unusual Location of Bone Tumor Easily Misdiagnosed: Distal Radius Osteosarcoma Treated as Osteomyelitis

Al-Chalabi MMM, Jamil I, Wan Sulaiman WA

Cureus, 2021 Nov;13(11):e19905.
PMID: 34976513 DOI: 10.7759/cureus.19905

Despite being the most common primary malignant bone tumor in children and adolescents, the presence of osteosarcoma at the wrist is infrequent; only less than 1% of osteosarcomas arise in the distal radius. The clinical presentation may mimic common musculoskeletal problems or benign lesions such as osteomyelitis, and a high index of suspicion is necessary so that the treating surgeon does not miss such lesions. We reported a case treated initially as osteomyelitis before being diagnosed as distal radius osteosarcoma. We conclude that an unusual location of osteosarcoma may be easily misdiagnosed, and therefore, osteosarcoma should be considered one of the main differential diagnosis in such cases until proven otherwise.
Perception of the most perfect female breast shape among Malays, Chinese, and Indians community

Hamzan MI, Wan Sulaiman WA, Ismail NN

J Plast Surg Hand Surg, 2022 Feb;56(1):30-37.
PMID: 33840334 DOI: 10.1080/2000656X.2021.1908313

Researchers have long tried to search for the 'perfect' aesthetic outcome of breast surgery. Although lots of anthropometric studies have been done, there is no consensus when dealing with harmony, abstract, and proportion that make up for its ideal shape. This study was convened via a survey to find the most perfect breast shape by assessing the upper pole contour and upper pole to lower pole ratio (UP:LP) preference among the Malays, Chinese, and Indians races. Hundred thirty-five individuals partook in this survey. Most of the participants generally preferred a more convex shape of upper pole contour with the Malay (50%) and Indian (35.7%) favored a more convex shape while the Chinese preferred it to be just slightly convex (42.8%). Single unmarried individuals (64.7%) significantly preferred a more convex upper pole contour (p = 0.018). The UP:LP ratio of 45:55 (p = 0.002) was the most significantly preferred proportion (37.8%) which largely comprised of the Chinese (51%), followed by Indian (21.6%) and Malay (19.6%). With these findings, using a controlled perception-preference method is a more preferred choice when describing an ideal breast shape as compare to an anthropometric measurement that might be less accurate. Therefore, breast and plastic surgeons alike need to look beyond the anthropometric numbers and should consider the other 'abstract' aesthetic shape which difficult to measure including the upper pole breast that has more convexity and the UP:LP ratio of 45:55 which showed to be the most aesthetically perfect form as agreed in the current works of literature.
Fulltext Two novel genes TOX3 and COL21A1 in large extended Malay families with nonsyndromic cleft lip and/or palate

Mohamad Shah NS, Sulong S, Wan Sulaiman WA, Halim AS

Mol Genet Genomic Med, 2019 May;7(5):e635.
PMID: 30924295 DOI: 10.1002/mgg3.635

BACKGROUND: Nonsyndromic cleft lip and/or palate is one of the most common human birth defects worldwide that affects the lip and/or palate. The incidence of clefts varies among populations through ethnic, race, or geographical differences. The focus on Malay nonsyndromic cleft lip and/or palate (NSCL/P) is because of a scarce report on genetic study in relation to this deformity in Malaysia. We are interested to discuss about the genes that are susceptible to cause orofacial cleft formation in the family.
METHODS: Genome-wide linkage analysis was carried out on eight large extended families of NSCL/P with the total of 91 individuals among Malay population using microarray platform. Based on linkage analyses findings, copy number variation (CNV) of LPHN2, SATB2, PVRL3, COL21A1, and TOX3 were identified in four large extended families that showed linkage evidence using quantitative polymerase chain reaction (qPCR) as for a validation purpose. Copy number calculated (CNC) for each genes were determined with Applied Biosystems CopyCallerTM Software v2.0. Normal CNC of the target sequence expected was set at two.
RESULTS: Genome-wide linkage analysis had discovered several genes including TOX3 and COL21A1 in four different loci 4p15.2-p16.1, 6p11.2-p12.3, 14q13-q21, and 16q12.1. There was significant decreased, p
Fulltext Long-Term Nasolabial Appearance Post-Unilateral Cleft Lip Repair in a Single Center: A Descriptive Study

Al-Chalabi MMM, Wan Sulaiman WA, Halim AS

Cureus, 2023 Jul;15(7):e41683.
PMID: 37575768 DOI: 10.7759/cureus.41683

Introduction Modern treatments still aim to keep the impact of surgical intervention low and the outcome of surgeries as good as a surgeon can. Assessing the long-term nasolabial appearance of patients who underwent cleft lip (CL) repair surgery is one of the methods of evaluating the outcomes of cleft surgery. Methods This is a retrospective cross-sectional descriptive study of data records of unilateral CL patients. The data records of all patients who underwent unilateral CL repair by the reconstructive science unit at Hospital Universiti Sains Malaysia (HUSM) within the first two years of their lives and whose current age is 14 years or above were accessed and analyzed. Results The data records of 50 patients were analyzed, including 13 (26%) males and 37 (74%) females. The surgeons opined that 28% of the patients had an acceptable nasolabial appearance, while there were 10 (20%) patients whose nasolabial appearance was considered unacceptable by the reviewing surgeons. Fifteen (30%) patients were described as having an acceptable lip appearance with secondary nasal deformity, and 11 (22%) patients had an acceptable nasal appearance with secondary lip deformity. There were no surgical modifications or postoperative complications among the patients. None of our variables reported a significant association with long-term nasolabial appearance. Conclusion The long-term evaluation of the nasolabial appearance in individuals with CL following surgical correction significantly improves the service and care provided to patients to achieve optimum results. Although our results showed no relationship between gender, age at operation, type or diagnosis of cleft, and family history and long-term nasolabial appearance, frequent assessments will enhance surgical results.

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