ABSTRACT
Two male patients with bifid rib-basal cell nevus-jaw cyst syndrome (BCNS) were admitted to Department of Stomatology, the First Affiliated Hospital of Bengbu Medical College due to radiological findings of multiple low density shadows in the jaw. Clinical and imaging findings showed thoracic malformation, calcification of the tentorium cerebellum and falx cerebrum as well as widening of the orbital distance. Whole exon high-throughput sequencing was performed in two patients and their family members. The heterozygous mutations of c.C2541C>A(p.Y847X) and c.C1501C>T(p.Q501X) in PTCH1 gene were detected in both patients. Diagnosis of BCNS was confirmed. The heterozygous mutations of PTCH1 gene locus were also found in the mothers of the two probands. Proband 1 showed clinical manifestations of low intelligence, and heterozygous mutations of c.C2141T(p.P714L) and c.G3343A(p.V1115I) were detected in FANCD2 gene. Proband 2 had normal intelligence and no FANCD2 mutation. The fenestration decompression and curettage of jaw cyst were performed in both patients. Regular follow-up showed good bone growth at the original lesion, and no recurrence has been observed so far.
Subject(s)
Humans , Male , Basal Cell Nevus Syndrome/diagnosis , Mutation , Nevus , Patched-1 Receptor/genetics , Pedigree , Ribs/abnormalitiesABSTRACT
Objective@#To investigate the clinical effect of free lower abdominal perforator flaps assisted by color Doppler ultrasound in repairing oral maxillofacial soft tissue defects.@*Methods@#From June 2018 to March 2019, five patients with oral cancer underwent surgical treatment. There were 2 males and 3 females, age from 45-69 years old. At the same time, free lower abdominal flaps pedicled with superficial circumflex iliac artery or superficial inferior epigastric artery were used to repair oral maxillofacial soft tissue defects under preoperative color Doppler ultrasound evaluation and localization.@*Results@#All the five cases survived. Three cases were repaired with superficial inferior epigastric artery flap and two cases were repaired with superficial circumflex iliac artery flaps. These flaps ranged from 5 cm ×6 cm to 7 cm×9 cm. The follow-up period ranged from 3 to 12 months, with an average of 6 months. The flaps were soft, without obvious swelling, hairless growth, scarless contracture and limited mouth opening. All donor and recipient areas were healed in one stage.@*Conclusions@#The lower abdominal perforator flaps assisted by color Doppler ultrasound for repairing oral and maxillofacial soft tissue defects are characterized by accurate selection and localization of blood vessels, convenient extraction of skin flaps and minimal morbidity of donor site. It is a good method for repairing oral and maxillofacial soft tissue defects.
ABSTRACT
To evaluate the clinical effect of digital assisted chimeric deep circumflex iliac artery perforator flap (DCIAPF) in the reconstruction of mandibular composite defects. Methods From January, 2018 to January, 2019, 6 cases of mandibular tumor patients with postoperative defect within side were treated. Preoperative CTA was used to evaluate the deep branches of spin iliac artery.Digital simulation software and 3D printing technolo-gy was taken, vascularized iliac flap of the design guide of bone was made, and the rebuilding effect was simulated. DCIAPF was used to repair the defect of lower jawbone. The donor sites were sutured directly. The patients were fol-lowed-up in outpatient department for 3-6 months to evaluate the recovery of the patient′s shape, jaw height and oc-clusal function, as well as the complications in the donor area. Results Postoperation pathological examination re-sults: ameloblastoma in 2 cases, 4 cases of gingival cancer. The length of cut out ilium was 6.0-13.0 cm, carrying the flap area of 3.0 cm×1.0 cm-6.0 cm×5.0 cm.Six cases of DCIAPF and iliac bone flap survived.The shape, mandibular height and occlusal function were satisfactory.And no obvious complications were found in the donor area. Conclu-sion The blood supply of DCIAPF is rich with enough bone mass and height. The position of terminal skin perfora-tors is invariant. The complications of donor sites is less. With the help of digital technology, the accuracy of mandibular defect repair and the 3-dimensional wound repair can be realized, and provides an advantage condition for subsequent dental implant.It is one of the ideal method of reconstruction of mandibular defect.
ABSTRACT
Objective To evaluate the clinical effect of mandibular osteomuscular flap pedicled with temporalis and radial forearm free flap to repair palatomaxillary defects. Methods From March 2008 to March 2011,nine patients with palatomaxillary defects following malignant tumor ablation,were repaired with mandibular osteomuscular flap pedicled with temporalis and radial forearm free flap. According to Brown's classification for the maxillectomy defect,eight cases were type Ⅱ B and 1 case was type Ⅱ C. There were 6 males and 3 females with an average age of 57 years(range,34-68 years). Results All the 9 osteomuscular flaps and forearm flaps survived.The patients were followed up for 12 to 24 months with an average of 14 months. The patients acquired satisfactory appearance and complete functional restoration except 1 case of chondrosarcoma of recurrence was encountered 6 months after operation. Conclusions Palatomaxillary defects were reconstructed by mandibular osteomuscular flap pedicled with temporalis and radial forearm free flap immediately.It is easy to elevate,safe,and there were no donor site problems.This is an ideal method of repairing the palato maxillary defects.