This report presents a case of ring chromosome 7 syndrome with bilateral cleft lip and palate. A ... more This report presents a case of ring chromosome 7 syndrome with bilateral cleft lip and palate. A four-year-old boy presented with bilateral cleft lip and palate, microcephaly, clenched toes, cafe-au-lait spots, a history of epilepsy, and severe intellectual disability. Genetic karyotyping revealed 46 XY r(7) (p22q36). His cheiloplasty and delayed palatoplasty were successful. A review of 22 previous r(7) patients revealed that 22.7% had cleft lip and/or palate. This case demonstrates the importance of a multidisciplinary evaluation for cleft patients, particularly those with syndromic features and global developmental delay.
Supplemental material, sj-tif-1-cpc-10.1177_10556656211069820 for Repair of Protruding Bilateral ... more Supplemental material, sj-tif-1-cpc-10.1177_10556656211069820 for Repair of Protruding Bilateral Cleft Lip and Palate With Staged Premaxilla Setback Osteotomy, Cheiloplasty, and Palatoplasty in Trisomy 17p Patient: A Review of Syndromic Clinical Characteristic by Kristaninta Bangun, Jessica Halim and Vika Tania in The Cleft Palate-Craniofacial Journal
<i>Objective</i>Chromosome 17 duplication is correlated with an increased risk of dev... more <i>Objective</i>Chromosome 17 duplication is correlated with an increased risk of developmental delay, birth defects, and intellectual disability. Here, we reported a female patient with trisomy 17 on the whole short arm with bilateral complete cleft lip and palate (BCLP). This study will review the surgical strategies to reconstruct the protruding premaxillary segment, cleft lip, and palate in trisomy 17p patient.<i>Case Presentation</i>The patient had heterozygous pathogenic duplication of chromosomal region chr17:526-18777088 on almost the entire short arm of chromosome 17. Beside the commonly found features of trisomy 17p, the patient also presented with BCLP with a prominent premaxillary portion. Premaxillary setback surgery was first performed concomitantly with cheiloplasty. The ostectomy was performed posterior to the vomero-premaxillary suture (VPS). The premaxilla was firmly adhered to the lateral segment and the viability of philtral flap was not compromised. Two-flap palatoplasty with modified intravelar veloplasty (IVV) was performed 4 months after.<i>Conclusion</i>Successful positioning of the premaxilla segment, satisfactory lip aesthetics, and vital palatal flap was obtained from premaxillary setback, primary cheiloplasty, and subsequent palatoplasty in our trisomy 17p patient presenting with BLCP. Postoperative premaxillary stability and patency of the philtral and palatal flap were achieved. Longer follow-up is needed to evaluate the long-term effects of our surgical techniques on inhibition of midfacial growth. However, the benefits that the patient received from the surgery in improving feeding capacity and facial appearance early in life outweigh the cost of possible maxillary retrusion.
Wound contracture is a challenging problem to deal with. By understanding the phases of wound hea... more Wound contracture is a challenging problem to deal with. By understanding the phases of wound healing, attempts can be performed to prevent abnormal wound healing that can lead to scar contracture. This article will attempt to provide a simple summary of wound contracture and how to prevent it.Although most wounds can be expected to heal uneventfully, infection, dehiscence, and delayed healing continue to be problems, associated with signi!cant morbidity, mortality, and economic cost. Wound contracture is one of morbidities that caused by delayed healing and incorrect management of wound.
Background : The techniques of cleft palate repair has faced significant evolutions. More importa... more Background : The techniques of cleft palate repair has faced significant evolutions. More importantly, no surgical protocol has been significantly associated with lower incidence of post-operative complications such as fistula formation, velopharyngeal insufficiency, or speech hypernasality. As cleft width is a strong predictor of outcomes, The Lima Protocol was introduced to select suitable surgical technique based on the degree of severity measured by palatal index. This article aimed to evaluate the efficacy of the newly established Lima Protocol in lowering post-operative complications in primary cleft palate repair surgery in Cleft and Craniofacial Center, Cipto Mangunkusumo Hospital, Jakarta, Indonesia.Case Presentation : This study is a case series featuring 6 patients with cleft palate treated with primary palatoplasty. Surgical techniques were selected based on the degree of severity measured by Palatal Index. The average age of performing primary palatoplasty was 14 months...
Chromosome 17 duplication is correlated with an increased risk of developmental delay, birth defe... more Chromosome 17 duplication is correlated with an increased risk of developmental delay, birth defects, and intellectual disability. Here, we reported a female patient with trisomy 17 on the whole short arm with bilateral complete cleft lip and palate (BCLP). This study will review the surgical strategies to reconstruct the protruding premaxillary segment, cleft lip, and palate in trisomy 17p patient. The patient had heterozygous pathogenic duplication of chromosomal region chr17:526-18777088 on almost the entire short arm of chromosome 17. Beside the commonly found features of trisomy 17p, the patient also presented with BCLP with a prominent premaxillary portion. Premaxillary setback surgery was first performed concomitantly with cheiloplasty. The ostectomy was performed posterior to the vomero-premaxillary suture (VPS). The premaxilla was firmly adhered to the lateral segment and the viability of philtral flap was not compromised. Two-flap palatoplasty with modified intravelar velo...
This report presents a case of ring chromosome 7 syndrome with bilateral cleft lip and palate. A ... more This report presents a case of ring chromosome 7 syndrome with bilateral cleft lip and palate. A four-year-old boy presented with bilateral cleft lip and palate, microcephaly, clenched toes, cafe-au-lait spots, a history of epilepsy, and severe intellectual disability. Genetic karyotyping revealed 46 XY r(7) (p22q36). His cheiloplasty and delayed palatoplasty were successful. A review of 22 previous r(7) patients revealed that 22.7% had cleft lip and/or palate. This case demonstrates the importance of a multidisciplinary evaluation for cleft patients, particularly those with syndromic features and global developmental delay.
Supplemental material, sj-tif-1-cpc-10.1177_10556656211069820 for Repair of Protruding Bilateral ... more Supplemental material, sj-tif-1-cpc-10.1177_10556656211069820 for Repair of Protruding Bilateral Cleft Lip and Palate With Staged Premaxilla Setback Osteotomy, Cheiloplasty, and Palatoplasty in Trisomy 17p Patient: A Review of Syndromic Clinical Characteristic by Kristaninta Bangun, Jessica Halim and Vika Tania in The Cleft Palate-Craniofacial Journal
<i>Objective</i>Chromosome 17 duplication is correlated with an increased risk of dev... more <i>Objective</i>Chromosome 17 duplication is correlated with an increased risk of developmental delay, birth defects, and intellectual disability. Here, we reported a female patient with trisomy 17 on the whole short arm with bilateral complete cleft lip and palate (BCLP). This study will review the surgical strategies to reconstruct the protruding premaxillary segment, cleft lip, and palate in trisomy 17p patient.<i>Case Presentation</i>The patient had heterozygous pathogenic duplication of chromosomal region chr17:526-18777088 on almost the entire short arm of chromosome 17. Beside the commonly found features of trisomy 17p, the patient also presented with BCLP with a prominent premaxillary portion. Premaxillary setback surgery was first performed concomitantly with cheiloplasty. The ostectomy was performed posterior to the vomero-premaxillary suture (VPS). The premaxilla was firmly adhered to the lateral segment and the viability of philtral flap was not compromised. Two-flap palatoplasty with modified intravelar veloplasty (IVV) was performed 4 months after.<i>Conclusion</i>Successful positioning of the premaxilla segment, satisfactory lip aesthetics, and vital palatal flap was obtained from premaxillary setback, primary cheiloplasty, and subsequent palatoplasty in our trisomy 17p patient presenting with BLCP. Postoperative premaxillary stability and patency of the philtral and palatal flap were achieved. Longer follow-up is needed to evaluate the long-term effects of our surgical techniques on inhibition of midfacial growth. However, the benefits that the patient received from the surgery in improving feeding capacity and facial appearance early in life outweigh the cost of possible maxillary retrusion.
Wound contracture is a challenging problem to deal with. By understanding the phases of wound hea... more Wound contracture is a challenging problem to deal with. By understanding the phases of wound healing, attempts can be performed to prevent abnormal wound healing that can lead to scar contracture. This article will attempt to provide a simple summary of wound contracture and how to prevent it.Although most wounds can be expected to heal uneventfully, infection, dehiscence, and delayed healing continue to be problems, associated with signi!cant morbidity, mortality, and economic cost. Wound contracture is one of morbidities that caused by delayed healing and incorrect management of wound.
Background : The techniques of cleft palate repair has faced significant evolutions. More importa... more Background : The techniques of cleft palate repair has faced significant evolutions. More importantly, no surgical protocol has been significantly associated with lower incidence of post-operative complications such as fistula formation, velopharyngeal insufficiency, or speech hypernasality. As cleft width is a strong predictor of outcomes, The Lima Protocol was introduced to select suitable surgical technique based on the degree of severity measured by palatal index. This article aimed to evaluate the efficacy of the newly established Lima Protocol in lowering post-operative complications in primary cleft palate repair surgery in Cleft and Craniofacial Center, Cipto Mangunkusumo Hospital, Jakarta, Indonesia.Case Presentation : This study is a case series featuring 6 patients with cleft palate treated with primary palatoplasty. Surgical techniques were selected based on the degree of severity measured by Palatal Index. The average age of performing primary palatoplasty was 14 months...
Chromosome 17 duplication is correlated with an increased risk of developmental delay, birth defe... more Chromosome 17 duplication is correlated with an increased risk of developmental delay, birth defects, and intellectual disability. Here, we reported a female patient with trisomy 17 on the whole short arm with bilateral complete cleft lip and palate (BCLP). This study will review the surgical strategies to reconstruct the protruding premaxillary segment, cleft lip, and palate in trisomy 17p patient. The patient had heterozygous pathogenic duplication of chromosomal region chr17:526-18777088 on almost the entire short arm of chromosome 17. Beside the commonly found features of trisomy 17p, the patient also presented with BCLP with a prominent premaxillary portion. Premaxillary setback surgery was first performed concomitantly with cheiloplasty. The ostectomy was performed posterior to the vomero-premaxillary suture (VPS). The premaxilla was firmly adhered to the lateral segment and the viability of philtral flap was not compromised. Two-flap palatoplasty with modified intravelar velo...
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