MyMedR

Displaying all 11 publications

Abstract:

Sort:

Non-syndromic craniosynostosis treated by frontal orbital advancement: A case report

Khoo KW, Arman Zaharil MS, Mohd Ali MZ

Med J Malaysia, 2018 12;73(6):423-424.
PMID: 30647221

Craniosynostosis is a premature pathologic fusion of one or more cranial vault sutures leading to abnormally-shaped skull. It can occur in isolated event (non-syndromic), or it can occur in conjunction with other anomalies in welldefined patterns (syndromic). The diagnosis rests on clinical examination and confirmation is generally on the computed tomography scan. The need for surgery is both for cosmetic and functional reasons. Here we describe a case of nonsyndromic craniosynostosis that was treated with frontal orbital advancement (FOA). The potential benefits of FOA need to be carefully weighed against the potential complications when deciding for any surgical intervention.

Matched MeSH terms: Craniosynostoses/surgery*
Bohring-opitz syndrome - A case of a rare genetic disorder

Visayaragawan N, Selvarajah N, Apparau H, Kamaru Ambu V

Med J Malaysia, 2017 08;72(4):248-249.
PMID: 28889139 MyJurnal

The diagnostic challenge of Bohring-Opitz Syndrome, a rare genetic disorder has haunted clinicians for ages. Our patient was born at term via caesarean-section with a birth weight of 1.95 kilograms. She had mild laryngomalacia, gastroesophageal reflux disease and seizures. Physical signs included microcephaly, hemangioma, low set ears, cleft palate, micrognatia and the typical BOS posture. Chromosomal analysis showed 46 xx -Bohring-Opitz Syndrome overlapped with C- syndrome. Goal-directed holistic care with integration of parent/carer training was started very early. She succumbed to a Respiratory- Syncitial-Virus and Pseudomonas pneumonia complicated with sepsis at the age of two years and 11 months.

Matched MeSH terms: Craniosynostoses/complications; Craniosynostoses/diagnosis; Craniosynostoses/genetics*; Craniosynostoses/therapy
Intracranial Volume Post Cranial Expansion Surgery Using Three-Dimensional Computed Tomography Scan Imaging in Children With Craniosynostosis

Sulong S, Alias A, Johanabas F, Yap Abdullah J, Idris B

J Craniofac Surg, 2019 8 14;31(1):46-50.
PMID: 31403510 DOI: 10.1097/SCS.0000000000005810

BACKGROUND: Craniosynostosis is a congenital defect that causes ≥1 suture to fuse prematurely. Cranial expansion surgery which consists of cranial vault reshaping with or without fronto-orbital advancement (FOA) is done to correct the skull to a more normal shape of the head as well as to increase the intracranial volume (ICV). Therefore, it is important to evaluate the changes of ICV after the surgery and the effect of surgery both clinically and radiologically.
OBJECTIVE: The aim of this study is to evaluate the ICV in primary craniosynostosis patients after the cranial vault reshaping with or without FOA and to compare between syndromic and nonsyndromic synostosis group, to determine factors that associated with significant changes in the ICV postoperative, and to evaluate the resolution of copper beaten sign and improvement in neurodevelopmental delay after the surgery.
METHODS: This is a prospective observational study of all primary craniosynostosis patients who underwent operation cranial vault reshaping with or without FOA in Hospital Kuala Lumpur from January 2017 until Jun 2018. The ICV preoperative and postoperative was measured using the 3D computed tomography (CT) imaging and analyzed. The demographic data, clinical and radiological findings were identified and analyzed.
RESULTS: A total of 14 cases (6 males and 8 females) with 28 3D CT scans were identified. The mean age of patients was 23 months. Seven patients were having syndromic synostosis (4 Crouzon syndromes and 3 Apert syndromes) and 7 nonsyndromic synostosis. The mean preoperative ICV was 880 mL (range, 641-1234 mL), whereas the mean postoperative ICV was 1081 mL (range, 811-1385 mL). The difference was 201 mL which was statistically significant (P 1.0). However, there was 100% (n = 13) improvement of this copper beaten sign. However, the neurodevelopmental delay showed no improvement which was statistically not significant (P > 1.0).
CONCLUSION: Surgery in craniosynostosis patient increases the ICV besides it improves the shape of the head. From this study, the syndromic synostosis had better increment of ICV compared to nonsyndromic synostosis.

Matched MeSH terms: Craniosynostoses/surgery
Surgical Outcome After Less "Rigid" Fixation in Open Cranial Vault Remodeling for Craniosynostosis

Koh KL, Zain A

J Craniofac Surg, 2018 Jun;29(4):861-867.
PMID: 29438206 DOI: 10.1097/SCS.0000000000004347

: Open cranial vault remodeling techniques require rigid fixation with hardware such as plates and screws; however, complications can occur. The purpose of this study was to assess the surgical outcome after open reconstruction for craniosynostosis with less rigid fixation using nonabsorbable suture.

METHODS: Retrospective review of patients who underwent open craniofacial reconstruction for craniosynostosis at the Hospital Kuala Lumpur between January 2011 and December 2016 were performed. Demographic data, surgical complications, and postoperative aesthetic outcomes and reoperations were evaluated using Whitaker classification. Statistical analyses were performed using SPSS.

RESULTS: Thirty-four (n = 34) cases were included in this review consisting of 16 males and 18 females (ratio 1:1.25). Sixteen patients were syndromic with multiple suture synostoses: Apert syndrome (n = 8), Crouzon syndrome (n = 6), and Muenke's syndrome (n = 2). Eighteen patients were nonsyndromic: isolated single-suture craniosynostosis (n = 12) and multiple suture involvement (n = 6). Mean age of presentation was 17.4 months (4-16 months) with mean age of surgery of 23.8 months (6-68 months). Mean length of surgery was 6.1 hours (range 3-10 hours) and mean length of hospital stay was 10 days (mean 7-20 days). Mean duration of follow-up was 2.2 years (6 month-4 years). There were a total of 9 complications postoperatively: massive blood loss (n = 4), seroma (n = 2), exposure keratitis (n = 1), hand extravasation (n = 1), and occipital sore (n = 1). Analysis showed multiple suture craniosynostoses were associated with longer operative times (4.3 hours vs 6.5 hours, P = 0.0082 24 months of age, P = 0.00059). Patients were categorized as 88.2% (n = 30) Whitaker I and II and 11.8% (n = 4) Whitaker III and IV. Reoperation rates were 2.9% (n = 1). Whitaker III and IV class were higher among patients with syndromic synostosis, unicoronal and multiple suture synostoses.

CONCLUSIONS: Our technique of open cranial reconstruction with nonabsorbable suture nylon 2/0 as sole method of fixation has resulted in good aesthetic outcome with low reoperations and complications rate. Longer follow-up is needed to ascertain our long-term results.

Matched MeSH terms: Craniosynostoses/surgery*
A Novel Technique Using Customized Headgear for Fixation of Rigid External Distraction Device in an Infant With Crouzon Syndrome

Hariri F, Rahman ZA, Mahdah S, Mathaneswaran V, Ganesan D

J Craniofac Surg, 2015 Nov;26(8):e740-4.
PMID: 26594993 DOI: 10.1097/SCS.0000000000002174

Rigid external distraction device is often indicated for superior midfacial advancement in pediatric syndromic craniosynostosis patients. Even though the technique is proven reliable to treat the functional issues related to the craniofacial deformity, major complications associated with its fixation, such as intracranial pin perforation and migration have been reported. We report a novel technique of using a customized headgear to prevent intracranial pin perforation over a very thin temporal bone region in an 8-month-old infant with Crouzon syndrome who underwent monobloc Le Fort III distraction osteogenesis using a combination of bilateral internal and a rigid external distraction device. The customized headgear provides a protective platform at the temporal region thus preventing intracranial pin perforation and allows stable fixation during the early phase of consolidation period to prevent central component relapse. The headgear can be used short term when rigid external distractor is indicated in infant patient but requires close monitoring because of risks of skin necrosis and temporal region indentation.

Matched MeSH terms: Craniosynostoses
Fulltext Pyknodysostosis - complications after dental extraction

Bahadun, J., Nik Hussien, N.N., Yunus, N.N.N.

Ann Dent, 2007;14(1):26-30.
MyJurnal

Pyknodysostosis is a rare sclerosing bone disorder with autosomal recessive mode of inheritance. The condition is characterized by continuous bone deposition without physiological resorption, occurring mainly in the trabecular and not the cortical bone. The skeletal features include short stature, brachycephaly, open cranial sutures and fontanelles, diffuse osteosclerosis and obtuse mandibular angles. Among the oral features observed were grooved or furrowed palate and disturbances in eruption and exfoliation of teeth. Multiple fractures of long bones and osteomyelitis of the jaw are frequent complications. These are attributed to the increased brittleness and reduced vascularity of the bone as a result of continuous endosteal bone deposition. Treatment is usually successful with surgical management and sustained bactericidal antibiotic therapy, although hyperbaric oxygen has been recommended as adjunct treatment in the more refractory form.

Matched MeSH terms: Craniosynostoses
Fulltext Application of three-dimensional computed tomography in craniofacial clinical practice and research

Anderson PJ, Yong R, Surman TL, Rajion ZA, Ranjitkar S

Aust Dent J, 2014 Jun;59 Suppl 1:174-85.
PMID: 24611727 DOI: 10.1111/adj.12154

Following the invention of the first computed tomography (CT) scanner in the early 1970s, many innovations in three-dimensional (3D) diagnostic imaging technology have occurred, leading to a wide range of applications in craniofacial clinical practice and research. Three-dimensional image analysis provides superior and more detailed information compared with conventional plain two-dimensional (2D) radiography, with the added benefit of 3D printing for preoperative treatment planning and regenerative therapy. Current state-of-the-art multidetector CT (MDCT), also known as medical CT, has an important role in the diagnosis and management of craniofacial injuries and pathology. Three-dimensional cone beam CT (CBCT), pioneered in the 1990s, is gaining increasing popularity in dental and craniofacial clinical practice because of its faster image acquisition at a lower radiation dose, but sound guidelines are needed to ensure its optimal clinical use. Recent innovations in micro-computed tomography (micro-CT) have revolutionized craniofacial biology research by enabling higher resolution scanning of teeth beyond the capabilities of MDCT and CBCT, presenting new prospects for translational clinical research. Even after four decades of refinement, CT technology continues to advance and broaden the horizons of craniofacial clinical practice and phenomics research.

Matched MeSH terms: Craniosynostoses/radiography*
Crouzon Syndrome: A Case Series of Craniomaxillofacial Distraction Osteogenesis for Functional Rehabilitation

Hariri F, Abdul Rahman ZA, Bahuri NFA, Azmi MN, Abdullah NA, Ganesan D

J Oral Maxillofac Surg, 2018 03;76(3):646.e1-646.e12.
PMID: 29268076 DOI: 10.1016/j.joms.2017.11.029

Crouzon syndrome (CS) is the most common craniosynostosis syndrome and requires a comprehensive management strategy for the optimization of care and functional rehabilitation. This report presents a case series of 6 pediatric patients diagnosed with CS who were treated with distraction osteogenesis (DO) to treat serious functional issues involving severe orbital proptosis, an obstructed nasopharyngeal airway, and increased intracranial pressure (ICP). Three boy and 3 girls were 8 months to 6 years old at the time of the operation. The mean skeletal advancement was 16.1 mm (range, 10 to 27 mm) with a mean follow-up of 31.7 months (range, 13 to 48 months). Reasonable and successful outcomes were achieved in most patients as evidenced by adequate eye protection, absence of signs and symptoms of increased ICP, and tracheostomy tube decannulation except in 1 patient. Complications were difficult fixation of external stabilizing pins in the distraction device (n = 1) and related to surgery (n = 4). Although DO can be considered very technical and can have potentially serious complications, the technique produces favorable functional and clinical outcomes in treating severe CS.

Matched MeSH terms: Craniosynostoses
An audiological evaluation of syndromic and non-syndromic craniosynostosis in pre-school going children

Goh LC, Azman A, Siti HBK, Khoo WV, Muthukumarasamy PA, Thong MK, et al.

Int J Pediatr Otorhinolaryngol, 2018 Jun;109:50-53.
PMID: 29728184 DOI: 10.1016/j.ijporl.2018.03.010

OBJECTIVE: To study the audiological outcome and early screening of pre-school going children with craniosynostosis under follow-up at the University of Malaya Medical Center(UMMC), Kuala Lumpur, Malaysia over a 10 year period.
METHODS: A retrospective descriptive cohort study on the audiological findings detected during the first hearing assessment done on a child with craniosynostosis using otoacoustic emissions, pure tone audiometry or auditory brainstem response examination. The main aim of this study was to evaluate the type and severity of hearing loss when compared between syndromic and non-sydromic craniosynostosis, and other associated contributory factors.
RESULTS: A total of 31 patients with 62 ears consisting of 14 male patients and 17 female patients were evaluated. Twenty two patients (71%) were syndromic and 9 (29%) were non-syndromic craniosynostosis. Amongst the syndromic craniosynostosis, 9 (41%) had Apert syndrome, 7 (32%) had Crouzon syndrome, 5 (23%) had Pfieffer syndrome and 1 (4%) had Shaethre Chotzen syndrome. Patients with syndromic craniosynostosis were more likely to present with all types and severity of hearing loss, including severe to profound sensorineural hearing loss while children with non-syndromic craniosynostosis were likely to present with normal hearing (p 

Matched MeSH terms: Craniosynostoses/complications*
Congenital nasal cavity stenosis in children with craniosyntosis: A report of 4 rare cases

Liew YT, Soo SS, Nathan AM, Manuel AM

Auris Nasus Larynx, 2017 Oct;44(5):635-638.
PMID: 27793496 DOI: 10.1016/j.anl.2016.10.001

Congenital bony nasal stenosis (CBNS) is a very rare but life-threatening cause of airway obstruction in neonates and infants. This review aims to assess the presentation and early airway management of 4 new cases of craniosynostosis with bilateral nasal cavity stenosis. Patients were treated with endoscopic endonasal widening of the nasal cavity and stenting. All patients were extubated well post-operatively with resolution of symptoms. They remained asymptomatic with stents in situ for at least 6 months with no complications reported. Minimally invasive endoscopic endonasal widening of the nasal cavity with stenting is an effective and safe way of addressing nasal cavity stenosis.

Matched MeSH terms: Craniosynostoses*
Fulltext Crouzon syndrome: Genetic and intervention review

Al-Namnam NM, Hariri F, Thong MK, Rahman ZA

J Oral Biol Craniofac Res, 2018 08 29;9(1):37-39.
PMID: 30202723 DOI: 10.1016/j.jobcr.2018.08.007

Crouzon syndrome exhibits considerable phenotypic heterogeneity, in the aetiology of which genetics play an important role. FGFR2 mediates extracellular signals into cells and the mutations in the FGFR2 gene cause this syndrome occurrence. Activated FGFs/FGFR2 signaling disrupts the balance of differentiation, cell proliferation, and apoptosis via its downstream signal pathways. However, very little is known about the cellular and molecular factors leading to severity of this phenotype. Revealing the molecular pathology of craniosynostosis will be a great value for genetic counselling, diagnosis, prognosis and early intervention programs. This mini-review summarizes the fundamental and recent scientific literature on genetic disorder of Crouzon syndrome and presents a graduated strategy for the genetic approach, diagnosis and the management of this complex craniofacial defect.

Matched MeSH terms: Craniosynostoses