- Email: [email protected]
Clinical experieces of trisomy 18 with cleft lip and palate
Poster Session of defect range by measuring bone height or length on the panoramic radiographs. Those measuring methods are as follows; The preserving rate = postoperative MBH / preoperative MBH, and the rate of defect range = the length of postoperative defect / the distance between bilateral condylar heads. In 47 patients, 33 (70.2%) patients underwent MR by extraoral approach and neck dissection with or without soft tissue reconstruction. The surgical defects were closed by primary suture in 25 patients (53.2%). The others were skin grafting (17%), soft tissue reconstruction by forearm flap (14.9%), and simple tie-over (14.9%). The MF occurred in 4 patients (8.5%). We examined their oral conditions such as surgical technique, defect area, fracture point, postoperative period, dental formula including the number of tooth, the preserving rate of MBH, and the rate of defect range. On the Eichner’s classification, all 4 fractured patients were included in the B-group that had at least one molar occlusal support. In addition, they had more than 20 teeth, and most of their preserving rates of MBH were less than 0.3, while the rate of defect range had no correlation with MF. Since the MF was observed between 1.5 and 40 months after MR, it was impossible to foresee when the MF occurred. Statistically, there were no significant factors for MF in age, sex, resection area, use of denture, and irradiation. These results suggest that the preserved bone should be reinforced with titanium plates or bone transplantation when the preserving rate of MBH is less than 0.3 and the number of tooth are more than 20. Though, the patient’s oral habits such as bruxism, thickness of mandibular cortical bone2, buccal to lingual width of mandibular bone, and bone density, etc. were not examined in this study, those suggested standards in this study may be useful to prevent MF after MR of the mandible. References: 1. Murakami K, Sugiura T, Kirita T, et al: Biomechanical Analysis of the Strength of the Mandible After Marginal Resection. J Oral Maxillofac Surg 69:1798-1806, 2011 2. Kingsmill VJ, Boyde A: Variation in the apparent density of human mandibular bone with age and dental status. J Anat 192:233, 1998
POSTER 14 Clinical experieces of trisomy 18 with cleft lip and palate S. Madoka: Department of Oral-maxillofacial Surgery, Dentistry and Orthodontics, The University of Tokyo Hospital
recent progresses in medicine has enabled a significantly longer survival rates. We report two surgical cases of trisomy 18 with bilateral cleft lip and palate. Case Reports: A 16-day-old female newborn baby presented with retractive breathing and multiple anomalies since birth. Because trisomy 18 was suspected by facial features, she was transferred to our hospital for intensive care and underwent tracheotomy. Later, she was diagnosed with trisomy 18 by chromosomal test. Her parents had a strong desire to the correction of cleft lip, in view of her human dignity. We consulted with pediatricians and anesthesists repeatedly and referred to the ethical committee of our hospital. Her general conditions had stabilized at 6 months, so we performed a cheilopasty utilizing the De Haan method. Postoperative concourse was uneventful. She is well 21 months after surgery, and her parents are satisfied with their decision for daughters’ sake.The other patient was 3-year-old-male with trisomy 18, and he was referred to our hospital for treatment of bilateral cleft lip and palate. From birth, he received a tracheotomy for breathing problems. His general conditions were stable, and he received home medical care. Responding to his parents’ request, we again consulted with pediatricians and anesthesists and referred to the ethical committee of our hospital.We performed cheilopasty utilizing by the De Haan method. Postoperative concourse was uneventful. He is well 3 months after surgery and he tries to eat a baby food in stages by oral ingestion. His parents are pleased with his improvement of facial anomalies and the progression of daily activity. Conclusions: There are some reports all over the world that the intensive care for tirsomy 18, including respiratory support, cardiac inotropic agents, and gastrointestinal surgery could lead to a better prognosis. Although cheiloplasty is a method which drastically improves functions and aesthetics, it has not been discussed for trisomy 18. When we do the surgery for trisomy 18, we must thoroughly explain the risks to patients’ that conditions may turn worse and that they sometimes become mortal to family, and take informed consent from the family. Sufficient consultations with related faculties or the third party such as an ethics committee are also necessary. As life expectancy of the patients with trisomy 18 rises like in our cases, it is expected that the family will demand more active treatment to enhance the patient’s human dignity. Careful therapeutic strategy and plan should be determined in view of the situation of patients and the attitude of their family. References:
Statement of the Problem: Trisomy 18 is a common and important chromosomal disorder, displaying multiple congenital anomalies. Less than 10% of the patients survived 1 year. Surgical treatment for children of trisomy 18 is controversial due to poor prognosis. However,
AAOMS 2014
1. Kosho, T., et al. Natural history and parental experience of children with trisomy 18 based on a questionnaire given to a Japanese trisomy 18 parental support group. Am J Med Genet A. 2013 Jul;161A(7):1531-42. 2. Nishi, E., et al. Surgical intervention for esophageal atresia in patients with trisomy 18. Am J Med Genet A. 2014 Feb;164A(2):324-30.
e-59
POSTER 14 Clinical experieces of trisomy 18 with cleft lip and palate S. Madoka: Department of Oral-maxillofacial Surgery, Dentistry and Orthodontics, The University of Tokyo Hospital
recent progresses in medicine has enabled a significantly longer survival rates. We report two surgical cases of trisomy 18 with bilateral cleft lip and palate. Case Reports: A 16-day-old female newborn baby presented with retractive breathing and multiple anomalies since birth. Because trisomy 18 was suspected by facial features, she was transferred to our hospital for intensive care and underwent tracheotomy. Later, she was diagnosed with trisomy 18 by chromosomal test. Her parents had a strong desire to the correction of cleft lip, in view of her human dignity. We consulted with pediatricians and anesthesists repeatedly and referred to the ethical committee of our hospital. Her general conditions had stabilized at 6 months, so we performed a cheilopasty utilizing the De Haan method. Postoperative concourse was uneventful. She is well 21 months after surgery, and her parents are satisfied with their decision for daughters’ sake.The other patient was 3-year-old-male with trisomy 18, and he was referred to our hospital for treatment of bilateral cleft lip and palate. From birth, he received a tracheotomy for breathing problems. His general conditions were stable, and he received home medical care. Responding to his parents’ request, we again consulted with pediatricians and anesthesists and referred to the ethical committee of our hospital.We performed cheilopasty utilizing by the De Haan method. Postoperative concourse was uneventful. He is well 3 months after surgery and he tries to eat a baby food in stages by oral ingestion. His parents are pleased with his improvement of facial anomalies and the progression of daily activity. Conclusions: There are some reports all over the world that the intensive care for tirsomy 18, including respiratory support, cardiac inotropic agents, and gastrointestinal surgery could lead to a better prognosis. Although cheiloplasty is a method which drastically improves functions and aesthetics, it has not been discussed for trisomy 18. When we do the surgery for trisomy 18, we must thoroughly explain the risks to patients’ that conditions may turn worse and that they sometimes become mortal to family, and take informed consent from the family. Sufficient consultations with related faculties or the third party such as an ethics committee are also necessary. As life expectancy of the patients with trisomy 18 rises like in our cases, it is expected that the family will demand more active treatment to enhance the patient’s human dignity. Careful therapeutic strategy and plan should be determined in view of the situation of patients and the attitude of their family. References:
Statement of the Problem: Trisomy 18 is a common and important chromosomal disorder, displaying multiple congenital anomalies. Less than 10% of the patients survived 1 year. Surgical treatment for children of trisomy 18 is controversial due to poor prognosis. However,
AAOMS 2014
1. Kosho, T., et al. Natural history and parental experience of children with trisomy 18 based on a questionnaire given to a Japanese trisomy 18 parental support group. Am J Med Genet A. 2013 Jul;161A(7):1531-42. 2. Nishi, E., et al. Surgical intervention for esophageal atresia in patients with trisomy 18. Am J Med Genet A. 2014 Feb;164A(2):324-30.
e-59