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  1. Jia Y, Zheng F, Zhang Q, Duan HF, Savic D, Kapelan Z
    Water Res, 2021 Oct 01;204:117594.
    PMID: 34474249 DOI: 10.1016/j.watres.2021.117594
    Hydraulic modeling of a foul sewer system (FSS) enables a better understanding of the behavior of the system and its effective management. However, there is generally a lack of sufficient field measurement data for FSS model development due to the low number of in-situ sensors for data collection. To this end, this study proposes a new method to develop FSS models based on geotagged information and water consumption data from smart water meters that are readily available. Within the proposed method, each sewer manhole is firstly associated with a particular population whose size is estimated from geotagged data. Subsequently, a two-stage optimization framework is developed to identify daily time-series inflows for each manhole based on physical connections between manholes and population as well as sewer sensor observations. Finally, a new uncertainty analysis method is developed by mapping the probability distributions of water consumption captured by smart meters to the stochastic variations of wastewater discharges. Two real-world FSSs are used to demonstrate the effectiveness of the proposed method. Results show that the proposed method can significantly outperform the traditional FSS model development approach in accurately simulating the values and uncertainty ranges of FSS hydraulic variables (manhole water depths and sewer flows). The proposed method is promising due to the easy availability of geotagged information as well as water consumption data from smart water meters in near future.
    Matched MeSH terms: Craniofacial Dysostosis*
  2. Majid ZA
    Singapore Dent J, 1988 Dec;13(1):33-5.
    PMID: 2979012
    A sporadic case of Crouzon Syndrome without mental retardation is described. The patient, an 11 year old boy with grossly carious teeth and in severe pain was referred for dental treatment. A review of literature on Crouzon Syndrome is presented.
    Matched MeSH terms: Craniofacial Dysostosis*
  3. 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: Craniofacial Dysostosis/surgery
  4. Al-Namnam NMN, Hariri F, Rahman ZAA
    Br J Oral Maxillofac Surg, 2018 06;56(5):353-366.
    PMID: 29661509 DOI: 10.1016/j.bjoms.2018.03.002
    Our aim was to summarise current published evidence about the prognosis of various techniques of craniofacial distraction osteogenesis, particularly its indications, protocols, and complications. Published papers were acquired from online sources using the keywords "distraction osteogenesis", "Le Fort III", "monobloc", and "syndromic craniosynostosis" in combination with other keywords, such as "craniofacial deformity" and "midface". The search was confined to publications in English, and we followed the guidelines of the PRISMA statement. We found that deformity of the skull resulted mainly from Crouzon syndrome. Recently craniofacial distraction has been achieved by monobloc distraction osteogenesis using an external distraction device during childhood, while Le Fort III distraction osteogenesis was used in maturity. Craniofacial distraction was indicated primarily to correct increased intracranial pressure, exorbitism, and obstructive sleep apnoea in childhood, while midface hypoplasia was the main indication in maturity. Overall the most commonly reported complications were minor inflammatory reactions around the pins, and anticlockwise rotation when using external distraction systems. The mean amount of bony advancement was 12.3mm for an external device, 18.6mm for an internal device and 18.7mm when both external and internal devices were used. Treatment by craniofacial distraction must be validated by long-term studies as there adequate data are lacking, particularly about structural relapse and the assessment of function.
    Matched MeSH terms: Craniofacial Dysostosis/complications; Craniofacial Dysostosis/surgery*
  5. 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: Craniofacial Dysostosis
  6. 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: Craniofacial Dysostosis
  7. 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: Craniofacial Dysostosis/complications*
  8. 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: Craniofacial Dysostosis
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