• Korean Journal of Cleft Lip And Palate
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• v.16 no.1
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• pp.9-18
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• 2013

Distraction osteogenesis is useful treatment which the gradual separation of cut bone edges results in the generation of new bone. It is effective treatment for correcting maxillofacial deformities. Patients with cleft lip and palate usually have maxillary hypoplasia due to scarring of lip and palate. To correct these deformities, we chose to use a 2-jaw orthognathic surgery or distraction osteogenesis. But despite improvements in surgical techniques for maxillofacial deformities, postoperative stability still leaves the question of when relapse may occur. This case report describes the Relapse after treatment of maxillary hypoplasia with cleft lip and palate by Rigid External distraction system over a 2-year treatment and follow-up period. In addition, we reviewed related articles about the influence of the occlusal stability on postoperative stability in patients with cleft lip and palate correction with Distraction osteogenesis.

• Journal of the korean academy of Pediatric Dentistry
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• v.11 no.1
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• pp.131-143
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• 1984

150 rats weighting about 150gm were devided into control group of 80 and experimental group of 70. Control group was subdivided into the irradiated vitamin D injection group and X-ray irradiated group. Experimental group was given 2.0mg ergocalciferol by four intramuscular injection prior to X-ray irradiation with single 800 rads and 1,500 rads respectively. Experimental animals from each group was sacrificed after 1, 3, 7, 14, and 28 days and their incisors were investigated by histopathological examination. The results were as follows; 1. In the irradiated groups, it showed dentin hypoplasia and formation of dentinoid substance caused by degeneration of odontoblast at the early stage. Especially, 1,500 rads group which was severely effected showed formation of osteoid dentin at the apical portion and severe injuries of dental papilla at the first week. 2. In the vitamin D2 administration group, it showed thinned dentin layer at the early stage but, taking time, predentin and dentin layer was thickened. At the fourth week, dentin was chiefly composed of interglobular dentin, especially in the lingual portion. 3. Using in combination of overdose vitamin D2 administration and X-ray irradiation, it effected severely odontoblast, undifferentiated mesenchymal cells around tooth germ and pulp tissue. At the early stage, dentin layer was thinned but, taking time, it was thickened and composed of interglobular dentin caused by calcification of predentin layer. 4. In 800 rads irradiation after the overdose vitamin D2 administration, it showed formation of osteoid dentin in the lingual portion at the first week. In the 1,500 rads irradiation after the overdose vitamin D2 administration, it showed formation of osteoid dentin and degeneration of ameloblast in both buccal and lingual portion at the first week, and enamel hypoplasia caused by edema and loss of polarity of ameloblasts at the second week. 5. By the entire experiment, the overdose vitamin D2 administration and X-ray irradiation effected severely odontoblasts, undifferentiated mesenchymal cells of dental papilla, and primitive cells of tooth germ among the dental tissue. Especially using combination of overdose vitamin D2 administration and X-ray irradiation also effected ameloblasts, resulting in enamel hypoplasia.

• Archives of Plastic Surgery
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• v.34 no.3
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• pp.346-351
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• 2007

Purpose: As a rare congenital anomaly, Poland's syndrome has been known to show hypoplasia in breast and nipple, absence of pectoralis major muscle, and aplasia or deformity of rib or costal cartilage which has been reported to be more common in male. However, most patients who are seeking operation are female patients having one-side deformity. In the field of plastic surgery, the major surgical indications could be asymmetric chest wall depression in man or breast hypoplasia in woman. There are many reconstruction options according to the degree of patient's deformity: a prosthetic implant, breast implant with or without tissue expander, latissimus dorsi musculocutaneous pedicled flap with or without implant and/or tissue expander, and free tissue transfer with or without tissue expander. Methods: The authors have treated 4 patients(2 male, 2 female) who had a diagnosis of Poland's syndrome. According to the degree of patient's deformity, all patients underwent correction of breast asymmetry and unilateral anterior thoracic hypoplasia with one-staged or two-staged reconstruction. Results: All patents were satisfied with the results and there occurred no specific complications. Conclusion: The authors propose the treatment plan for patient with Poland's syndrome, according to the degree of patient's deformity. In case of male patient with mild deformity, the prosthetic implant or latissimus dorsi musculocutaneous pedicled flap will simulate the missing pectoralis and improve the contour deformity. In case of female patient with moderate to severe breast asymmetry and upward displaced nipple areolar complex (NAC), NAC can be lowered with tissue expander, breast can be enlarged with autologous free flaps or latissimus dorsi musculocutaneous pedicled flap with implant.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.30 no.3
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• pp.246-250
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• 2004

Uni- or bilateral mandibular hypoplasia can be associated with various syndromes or is acquired after early traumatic or inflammatory disease in the temporomandibular joint(TMJ). Early treatment is necessary to avoid consequent impairment of midfacial growth. The standard treatment of these malformations consists of the application of bone grafts which can lead to unpredictable growth, but the new procedure of bone lengthening which was presented by McCarthy et al. represents a limited surgical intervention and therefore open up a new perspective of treatment, especially in younger children with severe deformities. Patients with hemifacial microsomia and facial asymmetry have a vertically short maxilla, a tilted occlusal plane, and a short mandible. A 14-years-old boy with facial asymmetry, who was fractured on both condyle and mandibular symphysis before 8 years ago, was treated by mandibular ramus lengthening, symphysial widening and surgically assisted rapid palatal expansion with corticotomy. After allowing 1 week for the healing of the periosteum, the distraction was performed at the rate of 0.5-1.0mm per day for 7 days on maxilla and 14 days on mandible. The device was maintained on maxilla and mandible for 12 weeks following distraction. The difference in ramus and mandibular transverse deficiency were corrected and facial asymmetry was improved with complex distraction osteogenesis.

• Korean Journal of Cleft Lip And Palate
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• v.12 no.1
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• pp.21-32
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• 2009

Generally, an adult cleft lip or/and palate patient shows some amount of maxillary deficiency due to limitation of bony growth caused by heavy scars resulted from previous operations such as a cheiloplasty and/or a palatoplasty at an early child age. To solve the problem, advancement of the maxilla is usually required during orthognathic surgery. However, severe tensional force resulted from heavy scars on the palate and/or the lip, as well as the bony defect at the cleft area limited sufficient advancement of the maxillary segment and finally caused relapse of the reposed maxilla. Therefore, distraction osteogenesis of the maxilla was introduced for the successful maxillary advancement inthose kinds of patients. As both hard and soft tissues can be simultaneously and gradually extended with this technique, tensional force caused by heavy scars opposed to forward movement of the maxilla can be reduced to an extent not to develop severe relapse of the advanced maxilla. Since distraction osteogenesis of the maxilla was applied as one of standard protocols for the treatment of the patients with severe maxillary hypoplasia dueto cleft lip and/or palate, the devices for the distraction was improved to control the vectors of distraction with better and more stable. We have treated a 23-year-old male cleft patient with a severe maxillary hypoplasia using a newly developed a maxillary distraction device and a RP model for a pre-operative simulation surgery. As a result, we could successfully move the maxilla as we designed pre-operatively and also reduce much of operation time. Therefore, we report of the case to share our experience with colleagues.

• Journal of Chest Surgery
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• v.40 no.1 s.270
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• pp.60-62
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• 2007

Pulmonary hypoplasia is an entity of pulmonary agenesis. Pulmonary agenesis is a rare congenital anomaly, usually diagnosed soon after birth. It is commonly associated with other anomalies, mainly of the cardiovascular systems. Although it may hasten the death of a child, sometimes it is compatible with normal growth. We report a right lateral thoracotomy approach for mitral valve repair in a young woman with pulmonary hypoplasia, in whom preoperative computed tomography showed severe right side shifting of the mediastinum and total collapse of the right lung.

• Journal of the Korean Society of Radiology
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• v.81 no.4
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• pp.979-984
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• 2020

Cri-du-chat syndrome is a rare genetic disorder in which the patient presents with a characteristic high-pitched monotonous cry and recurrent aspiration pneumonia, attributed to abnormalities in the larynx, epiglottis, and nervous system. The most prominent brain MRI findings are the presence of pontine and cerebellar hypoplasia, which primarily involve posterior cranial fossa structures. Although atrophy of supratentorial structures were also a common radiological finding, it was considered to be a secondary change due to pontine hypoplasia. Here, we present the case of a three-month-old patient presenting with cri-du-chat at our institution. The patient also showed the presence of prominent pontine hypoplasia similar to previously reported cases; however, contrary to other cases, there was a general delayed myelination of brain instead of decreased myelination of anterior limb of internal capsule. Since the larynx, pons, and cerebellum all originated from similar notochord level, which suggests anomaly in early stage of development, laryngeal, and brain anomaly characteristically observed in the cridu-chat syndrome.

• Tuberculosis and Respiratory Diseases
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• v.71 no.2
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• pp.126-133
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• 2011

Unilateral pulmonary artery hypoplasia (UPAH) is a rare disease in adults and is frequently accompanied by a congenital cardiac anomaly at a young age. The diagnosis is usually based on computed tomography (CT), angiography, and magnetic resonance imaging (MRI). However, no reports are available on retrograde flow in patients with UPAH. We describe a 68-year-old man with isolated UPAH and retrograde blood flow. He was admitted for dyspnea on exertion for the past 23 years. His diagnosis was delayed, as his symptoms and signs mimicked his underlying pulmonary diseases, such as emphysema and previous tuberculous pleurisy sequelae. A discrepancy was detected between the results of a ventilation-perfusion scan and the CT image. This was resolved by MRI, which showed retrograde blood flow from the right to the left pulmonary artery. Using MRI, we diagnosed this patient with isolated pulmonary artery hypoplasia and retrograde flow.

• Neonatal Medicine
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• v.18 no.1
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• pp.158-162
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• 2011

Preterm infants with oligohydramnios after preterm premature rupture of membranes can present with severe respiratory distress immediately after birth, and the most common cause is pulmonary hypoplasia. Unlike infants with pulmonary hypoplasia, some cases have shown dramatic improvement with aggressive ventilatory support during the initial 1-2 days of distress: those patients have been defined as having dry lung syndrome. It is assumed that oligohydramnios leads to functional pulmonary hypoplasia by compression of the fetal lungs: some of the improvement in dry lung syndrome may thus have resulted from inflation of compressed lung tissue and increase of lung compliance. We report two incidences of dry lung syndrome that were treated successfully with high inflation pressure and inhaled nitric oxide (NO): these are the first dry lung syndrome cases to be reported in Korean infants.

• Journal of Chest Surgery
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• v.22 no.4
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• pp.672-676
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• 1989

We experienced a case of congenital tricuspid stenosis. The patient was 5 years old boy. The 2D-echo 4 cardiac catheterization with cineangiogram revealed as Tricuspid Stenosis, associated with functioning patent foramen ovale & mild R.V hypoplasia. The lesion was successfully corrected by TV commissurotomy & closure of PFO. Postoperatively, moderate degree of tricuspid regurgitation was remained.