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  1. Khoo ET, Tan JA, Al-Chalabi MMM, Mat Zain MA, Wan Sulaiman WA
    Cureus, 2023 Jun;15(6):e40512.
    PMID: 37461779 DOI: 10.7759/cureus.40512
    Juvenile hyaline fibromatosis (JHF) is a rare, hereditary disease characterized by abnormal hyaline deposits within the skin, soft tissues, joints, and bones. The condition itself is often debilitating, with no curative treatment available. A definitive diagnosis is established by genetic testing. However, the hallmarks of gingival hypertrophy, subcutaneous scalp nodules, and joint contractures can be used as a clinical guide when genetic testing is unavailable. Here, we report an unusual case of a five-year-old child clinically diagnosed with juvenile hyaline fibromatosis with atypical nodules exclusively confined to the perioral region.
  2. Mohd Said MT, Halim AS, Mat Zain MA, Koh KL
    Cureus, 2023 May;15(5):e39312.
    PMID: 37346202 DOI: 10.7759/cureus.39312
    Webbed neck deformity is a congenital anomaly that exists in several syndromes. Various techniques for surgically correcting the webbed neck deformity have been described in the literature, each comes with its own advantages and disadvantages. The aim of surgery is to achieve normal neck contour and symmetrical hairline, avoid excessive scarring over the anterior and lateral neck, and limit recurrence. In this report, we described our experience in managing a case of Turner syndrome with bilateral webbed neck deformity using the modified five-flap Z-plasty technique.
  3. Heng SSL, Ganandran T, Gan YH, Mat Zain MA, Basiron N, Wan Sulaiman WA
    J Plast Reconstr Aesthet Surg, 2023 Oct;85:187-192.
    PMID: 37499560 DOI: 10.1016/j.bjps.2023.07.008
    INTRODUCTION: Tessier cleft 7 are rare craniofacial clefts. Live-birth incidence varies from 1/80,000 to 1/300,000, with the incidence of 1 in 120 craniofacial clefts among Asians. Its clinical presentation varies widely in severity, thus, complicating diagnosis and contributing to the lack of consensus regarding its surgical management. The aim of this study is to describe clinical findings, types of Tessier cleft 7 soft tissue repair, and its outcomes in Kuala Lumpur Hospital.

    METHODS: This retrospective study reviewed records of children operated from January 2001 to July 2019. Data regarding concurrent congenital anomalies, complications, type of surgery, etc., were collected from outpatient records, operative notes, and clinical photographs.

    RESULTS: Twenty-eight children were treated, and 33 clefts (5 bilateral) were repaired. The male-to-female ratio was 1.3:1 (16 males and 12 females). Twenty-three patients had unilateral clefts (82.14%), with 14 right (60.86%) and 8 left (34.78%). Bilateral clefts were less common (17.86%). Twenty-three patients (82.14%) were Malay, 3 (10.71%) Chinese, 1 (3.57%) Indian, and 1 (3.57%) Cambodian. Eleven medical records were untraceable (discontinued due to duration of inactivity). There were 10 straight-line repairs, 5 Z-plasties, and 1 W-plasty performed; 3 cases did not detail the type of repair. One child required scar revision, and 1 had hypertrophic scarring requiring corticosteroid injection-no disturbances in speech or oral incompetency while eating were reported. Duration of follow-up ranged from 3 to 14 years.

    CONCLUSION: Our center has a higher rate of Tessier cleft 7 attendance. Straight-line cutaneous repairs combined with inferior vermilion mucosal flap can be used with low rates of complication and revision surgery.

  4. Dharmaraj B, Diong NC, Shamugam N, Sathiamurthy N, Mohd Zainal H, Chai SC, et al.
    Indian J Thorac Cardiovasc Surg, 2021 Jan;37(1):82-88.
    PMID: 33442211 DOI: 10.1007/s12055-020-00972-7
    Chest wall resection is defined as partial or full-thickness removal of the chest wall. Significant morbidity has been recorded, with documented respiratory failure as high as 27%. Medical records of all patients who had undergone chest wall resection and reconstruction were reviewed. Patients' demographics, length of surgery, reconstruction method, size of tumor and chest wall defect, histopathological result, complications, duration of post-operative antibiotics, and hospital stay were assessed. From 1 April 2017 to 30 April 2019, a total of 20 patients underwent chest wall reconstructive surgery. The median age was 57 years, with 12 females and 8 males. Fourteen patients (70%) had malignant disease and 6 patients (30%) had benign disease. Nine patients underwent rigid reconstruction (titanium mesh for sternum and titanium plates for ribs), 6 patients had non-rigid reconstruction (with polypropylene or composite mesh), and 5 patients had primary closure. Nine patients (45%) required closure with myocutaneous flap. Complications were noted in 70% of patients. Patients who underwent primary closure had minor complications. In total, 66.7% of patients who had closure with either fasciocutaneous or myocutaneous flaps had threatened flap necrosis. Two patients developed pneumonia and 3 patients (15%) had respiratory failure requiring tracheostomy and prolonged ventilation. There was 1 mortality (5%) in this series. In conclusion, chest wall resections involving large defects require prudent clinical judgment and multidisciplinary assessments in determining the choice of chest wall reconstruction to improve outcomes.
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