The purpose of this review is the current understanding of proximal junctional kyphosis (PJK) and proximal junctional failure (PJF) following adult spinal deformity (ASD) surgery. We carried out a systematic search of PubMed for literatures published up to September 2017 with “proximal junctional kyphosis,”“proximal junctional failure,” and “adult spinal deformity” as search terms. A total of 98 literatures were searched. The 37 articles were included in this review. PJK is multifactorial in origin and likely results from variable risk factors. PJF is a progressive form of the PJK spectrum including bony fracture, subluxation between UIV and UIV+1, failure of fixation, neurological deficit, which may require revision surgery for proximal extension of fusion. Soft tissue protections, adequate selection of the UIV, prophylactic rib fixation, hybrid instrumentation such as hooks, vertebral cement augmentation at UIV and UIV+1, adequate selection material of rods and age-appropriate spinopelvic alignment goals are strategies to minimize PJK and PJF. The ability to perform aggressive global realignment of spinal deformities has also led to the discovery of new complications such as the PJK and PJF. Continuous research on PJK and PJF should be proceeded in order to comprehend the pathophysiology of these complications.
Adult* ; Congenital Abnormalities* ; Humans ; Kyphosis* ; Ribs ; Risk Factors

Calvarium protects the brain, the most important organ. The defect of calvarium results in not only deformity but also fatal injury from the trauma. The cranial bone defects result from 1) removal of bone flap for intracranial decompression or infection 2) fracture 3) excision of tumor 4) craniectomy for craniosynostosis. The goals of cranioplasty are to protect the brain from trauma and make the aesthetically acceptable contour. From 1990 to 1995, we experienced twelve cases of cranioplasty using autogenous bone graft; 5 cases with rib bone, 3 cases with iliac bone, 2 cases with calvarial bone, and 2 cases with rib and calvarial bones. The result was very excellent without any significant complications.
Brain ; Congenital Abnormalities ; Craniosynostoses ; Decompression ; Ribs ; Skull ; Transplants*

The constricted ear was suggested by Tanzer for the purpose of obviating the confusion involving lop ear, cup ear and prominent ear as defect whose helix turns down, and scapha and fossa triangularis are narrowed. The constricted ear has a spectrum of severity and therefore, requires a graded surgical approach. Tanzer has described the degree of deformities of the constricted ear as falling into three groups. For the correction of constricted ear, there are numerous techniques but we have had difficulties in adopting these techniques in various type. We also describe the various constricted ear as the Tanzer's classification and adopted three methods to each type, banner flap(group I), concha cartilage graft (group II) and rib cartilage graft(group III) for reducing postoperative deformity and confusion in correcting the ear deformities. Constricted ear repairs must be individualized to accomodate each specific deformity. We corrected 22 cases of constricted ear in 20 patients using each optimal method described above according to the degree of deformities. Mild deformities need only reshaping and adjusting of existing tissues, moderate deformities need additional skin and severe deformities require a cartilage graft. For correction of constricted ear, accurate identification of the severity of deformity is essential. The results were satisfactory and we report our experience with relative literatures.
Cartilage ; Classification ; Congenital Abnormalities ; Ear* ; Humans ; Ribs ; Skin ; Transplants

Congenital Abnormalities ; Fractures, Bone ; Osteotomy ; Ribs ; Skeleton ; Transplants

2 cases of Sprengel s deformity are presented as the first report of the country. This relatively uncommon congenital deformity was found not only scapula involved but also the adjacent spine and ribs as well. Even the patients are adult surgical intervention was tried because the disability was prominent with high scapula, and good range of the shoulder joint motions is restored.
Adult ; Congenital Abnormalities ; Humans ; Ribs ; Scapula ; Shoulder Joint ; Spine

Binder syndrome is a rare maxillofacial abnormality. Yet, once presented, it often needs to be addressed surgically. To suit this purpose, various surgical techniques have been developed. This paper is a case report of a staged nasal reconstruction in a Binder syndrome patient using rib bone and cartilage graft and forehead flap. At the same time, preoperative and postoperative anthropomorphometric measurements were compared to assess the efficiency of the proposed surgical technique.
Cartilage* ; Forehead* ; Humans ; Maxillofacial Abnormalities ; Nose ; Ribs* ; Transplants*

OBJECTIVETo investigate the characteristics and incidence of the thoracic deformities in patients with microtia.

METHODSIn Plastic Surgery Hospital, we conducted a retrospective study of the clinical and radiographical data of 300 patients with microtia from March 2013 to October 2014. Pearson χ2 test was used to analyze the relationship among deformities of ribs and spine, as well as microtia.

RESULTSA total of 78 (26.0%) patients were documented with rib deformities, 26 patients (8.7%) had spinal deformities, and 17 patients (5.7% )had both. The incidence of rib deformities in microtia I, II, and III was 7.1% (2/28), 26.7% (62/232) and 35.0% (14/40) respectively. The incidence of spinal deformities in microtia I, II, and III was 3.6% (1/28), 6.5% (15/232) and 25.0% (10/40 respectively. The patients with microtia III were found to have a higher incidence of ribs and spinal deformities than those with microtia II, patients with microtia II were found to have a higher incidence of ribs and spinal deformities than those with microtia I (P < 0.05).

CONCLUSIONSThe incidence of ribs and spinal deformities is high in patients with microtia. The poorer one auricle developed, the higher the incidence of thoracic deformities.

Biomedical Research ; Congenital Microtia ; epidemiology ; Humans ; Incidence ; Retrospective Studies ; Ribs ; abnormalities ; Spine ; abnormalities

Jarcho-Levin syndrome (JLS) is a condition manifested by malformations of vertebral bodes and related ribs. There are two major subtypes spondylocostal dysostosis and spondylothoracic dysostosis, with different survival rates, associated malformations, and inheritance patterns. We have experienced an autopsy case of a premature female fetus with multiple congenital anomalies. She was 30 weeks of gestational age, born as the second baby of twins and expired shortly after birth. A post-mortem examination revealed multiple abnormalities including cervicothoracic hemivertebrae, a diminished number of right-sided ribs, and pulmonary hypoplasia with left diaphragmatic hernia. In addition, there were anomalous rotation of the foregut, unfused pancreas and anomalous drainage of the superior vena cava. Chromosomal analysis showed 46, XX, del(4)(q ter).
Abnormalities, Multiple/genetics/*pathology ; Autopsy ; Chromosome Deletion ; Chromosomes, Human, Pair 4 ; Female ; Humans ; Infant, Newborn ; Ribs/*abnormalities ; Spine/*abnormalities ; Syndrome

Thanatophoric dysplasia is the most common lethal congenital chondrodysplasia with characteristic features of narrow thorax, short rib, severe platyspondyly, short bowed limbs and skull deformity, etc. It is not a hereditary disorder and there is usually no family history of dysplasia. We experienced a case of thanatophoric dysplasia at 38 weeks of gestation with antenatal sonographic and abdominal radiographic findings of small thorax, short bowed extremities with surrounding thickened soft tissues and marked platyspondyly. Soon atter delivery, the baby died and post-mortem radiographs showed the characteristic findings of thanatophoric dysplasia.
Congenital Abnormalities ; Extremities ; Humans ; Pregnancy ; Ribs ; Skull ; Thanatophoric Dysplasia* ; Thorax ; Ultrasonography

Craniofacial cleft is a rare congenital anomaly with a wide range of clinical manifestation and severity of deformity. In 1976, Tessier announced classification system on the basis of anatomical observation derived from clinical finding or operative dissection. Nowadays, this system is in common use because it is in accordance with terminology and observational finding and clinical manifestation is accordant with operative finding. Median facial cleft(No. 0-14 facial cleft) has a wide range of congenital malformation from a midline cleft upper lip to orbital hypertelorism, among which the bifid nose is frequently associated with hypertelorism. The manifestation of a bifid nose is variable from a simple central groove at the nasal tip to a complete clefting of the osteocartilaginous framework. In consequence, the planning of correction of the bifid nose must be individualized. We contrived correction of bifid nose using rib bone graft containing small amount of costal cartilage with maneuver of 2mm incision on nasal root skin together with fixation with 9mm miniscrew through an open approach in two No. 0-14 facial cleft patients with mild hypertelorism and bifid nose. With this method we could obtain satisfactory results in the standpoint of function as well as aesthetics. We think that this method is appropriate for correction of bifid nose of mild median facial cleft.
Cartilage ; Classification ; Congenital Abnormalities ; Esthetics ; Humans ; Hypertelorism ; Lip ; Nose* ; Orbit ; Ribs* ; Skin ; Transplants*