• Journal of the Korean Orthopaedic Association
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• v.53 no.6
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• pp.530-539
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• 2018

Purpose: We aimed to evaluate the magnetic resonance imaging (MRI) findings of congenital postaxial polydactyly of the foot. Materials and Methods: Three-hundred and forty-seven feet of 288 patients who underwent congenital postaxial polydactyly or polysyndactyly correction were divided into five subtypes according to the radiographic shapes of deformity origins (widened metatarsal head, bifid, fused duplicated, incompletely duplicated, or completely duplicated). MRIs were assessed to determine whether they unrevealed areas were fused or separated. MRI was also used to assess cases with radiographic phalangeal aplasia. Results: Huge variations were noted in MRIs. Fusion or separation at the base or head between original and extra digits were observed, and MRI effectively depicted phalangeal aplastic areas. Conclusion: MRI evaluations of congenital postaxial polydactyly of the foot are useful for determining the anatomical statuses which were not visualized by plain radiography (level of evidence: 3).

• Korean Journal of Biological Psychiatry
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• v.5 no.2
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• pp.283-287
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• 1998

The mother was 24 years old, primipara, and had been taking carbamazepine 400mg(serum concentration $5.0-8.5{\mu}g/ml$) during pregnancy without any clinical seizures. A male baby with physical malformation was delivered on week 39. The malformation is extradigit(polydactily) on X-ray of right foot and left mild hydronephrosis on ultrasonography and renal scan with radioactive material. We reported this rare case and reviewed related articles about teratogenic effect of carbamazepine, mechanism of action and prevention of teratogenesis.

• Neonatal Medicine
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• v.16 no.1
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• pp.85-88
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• 2009

The amniotic band syndrome is an uncommon congenital fetal abnormality, presumably due to fetal entanglement in strands of ruptured amniotic sac. The defects caused by this syndrome vary from simple limb defects to major visceral and craniofacial defects. We cared for a newborn infant with this syndrome, who showed constriction rings of the right leg and right axilla, right club foot, thoracic scoliosis, polydactyly, absence of the right thumb and aplasia cutis of the scalp. We report this case with a brief review of the literature.

• Neonatal Medicine
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• v.16 no.1
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• pp.89-93
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• 2009

Maternal diabetes is known to have teratogenic effects which increase the risk for congenital anomalies, such as caudal dysplasia, cardiac defects, hydronephrosis, and small left colon syndrome. Infants of diabetic mothers have a 10-fold higher frequency of anomalies in the central nervous system and a 5-fold higher frequency of congenital heart defects. However, jejunal atresia combined with multiple anomalies of the face, ears, and hands has rarely been reported. Herein we report a neonate born to a diabetic mother, who had hemifacial microsomia, displacement of the lacrimal ducts, polydactyly of the right hand, microtia of the right ear and proximal jejunal atresia presenting as bile regurgitation on the 1st day of life.

• Journal of Korean Foot and Ankle Society
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• v.3 no.1
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• pp.53-57
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• 1999

Polydactyly is the most common congenital deformity of the foot. The authors present an unusal case of polydactyly of the foot in an otherwise healthy adult male. The patient has an mixed type of polydactyly composed of polysyndactyly of the first toe, Y shaped second metatarsal and polysyndactyly with the fusion to the forth toe of the fifth toe. Meticulous. preoperative plan was prepared and performed at the operation. Main procedures were as follows : 1) Excision of extradigit of first toe and first metatarsocuneiform joint fusion. 2) Excision of lateral bud of second metatarsal and plantar-medial osteotomy of the medial bud. 3) Metatarsal head resection arthroplasty of third & forth metatarsophalangeal joint and 4) Excision of medial polydactyly of the fifth toe and syndactyly release and split thickness skin graft. Postoperatively, The forefoot width was reduced from 11.5 to 9.5cm and the pain was relieved.

• Journal of Korean Foot and Ankle Society
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• v.5 no.2
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• pp.120-128
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• 2001

Purpose: This study was undertaken to review the precise morphological patter'ns of polydactyly of the foot and to evaluate outcomes of surgical interventions. Materials and Methods: The records of patients who had polydactyly of foot from 1983 to 2000 were reviewed retrospectively. 29 duplicated toes(24 patients) were analyzed. All cases were evaluated and classified with Temtamy and McKusick's axial pattern, Watanabe's morphologic pattern and level of duplication. Clinical evaluation was performed according to Phelps and Grogan's. Mean age at surgery was 5.7 years(range: 10 months$\sim21$ years). All the patients were subsequently evaluated during mean 17 months(range: 13 months-$\sim5$ years). Results: Preaxial polydactyly was seen in 3 cases, central polydactyly in 3 cases, postaxial polydactyly in 22 cases and remaining 1 case was multiple my involvement. Only 2 patients have familial history and the most common associated anomaly was hand deformity(20.8%). Clinical results were excellent in 14 cases, good in 5 cases, poor in one. 17 patients(70%) were operated before 6 years olds and they had better results than those of who were operated after 6 years olds. Conclusion: The Method of surgical correction for the polydactyly should be individualized by its morphological pattern because the purpose of operation was to give comfort in wearing shoes and patient's psychological relief. This study showed that polydactyly of the foot could be corrected surgically with good results in most cases, and the better results would be achieved if the operation is performed before preschool age according to its individualized pattern of duplication.

• Archives of Plastic Surgery
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• v.38 no.6
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• pp.821-828
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• 2011

Purpose: The authors applied Bilhaut-Cloquet procedure to Wassel type III and IV duplicated thumb, which was limited to patients with Wassel type I, II. This procedure was applied in order to improve the growth potential, range of joint motion, joint stability and cosmetic outcome. Methods: Sixteen patients received Bilhaut-Cloquet procedures to correct duplicated thumbs from May, 2005 to December, 2010. Seven patients were Wassel type III, nine patients were type IV. This procedure was applied not only to balanced type, but also unbalanced type or convergent type. Five patients were balanced type and eleven patients were unbalanced type. Convergent type of Wassel type IV was three. Sex ratio was the same, mean age at the operation was 20.1 months old (8~52 months old). Angular deformity, joint stability and range of joint motion and cosmetic outcome were considered together and estimated in Tada score. Also, postoperative subjective satisfaction score of the parents was evaluated by a 100-points scale. Results: Mean subjective satisfaction scored 75 points at 28 months after the operation. Radiologic study showed bony union of proximal phalangeal bone and stable joint in all patients. Range of motion was mean 20 degrees in interphalangeal joint and mean 73 degrees in metacarpophalangeal joint. Tada score showed 'good' in eleven patients (68.8%), 'fair' in three patients (18.7%) and 'poor' in two patients (12.5%). In seven patients those who were able to follow up for a long term showed no significant difference in length of proximal and distal phalangeal bones compared to the opposite thumb. Conclusion: Bilhaut-Cloquet procedure can be applied not only to balanced type of Wassel type III, IV duplicated thumb, but also to unbalanced type or convergent type that focused on functional reconstruction and cosmetic improvement.

• Journal of Korean Foot and Ankle Society
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• v.6 no.1
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• pp.7-14
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• 2002

Purpose: We investigate the characteristic morphologic features and suggest proper treatment of postaxial polydactyly of the foot Materials and Methods: We analysed 37 cases of postaxial polydactyly. Mean post operative follow up period was 2 years 10 months. We analysed them according to morphological, radiological and operative findings. Patients were classified into extra 5th toe polydactyly and extra 6th toe polydactyly based on the abnormal extradigit, and subdivided into joint origin type, bone origin type and floating type based on duplication pattern. Results: 23 cases were extra 5th toe polydactyly and 14 cases were extra 6th toe polydactyly. Most common types were metatarsophalangeal joint origin type of extra 6th toe polydactyly. Compared with extra 5th toe polydactyly, extra 6th toe polydactyly originated from more proximal part and had not syndactylism. Conclusion: As the duplication level was more distal, degree of syndactylism and nail union was more severe. In case of syndactyly between 5th and 6th toe, abnormal extradigit was 5th toe.

• Childhood Kidney Diseases
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• v.6 no.2
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• pp.266-271
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• 2002

There are several diseases characterized by neurologic abnormalities and renal disease. Joubert syndrome is one of them. Joubert syndrome Is a relatively rare autosomal recessive syndrome. The most significant and constant neurologic finding is hypoplasia of the cerebellar vermis. Joubert syndrome is associated with hypotonia, retinal dystrophy, abnormal eye movement, delayed development, abnormal respiratory pattern (neonatal episodic tachypnea or apnea) and nephronophthisis. We report a boy with Joubert syndrome associated with nephrocalcinosis and agenesis of the cerebellar vermis. This patient had also abnormal eye movement, hypotonia, abnormal respiratory pattern, delayed development and chronic renal failure.

• Archives of Plastic Surgery
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• v.36 no.4
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• pp.458-461
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• 2009

Purpose: Preaxial polydactyly is the most common congenital anomaly of upper extremities. In this study, we classified 112 patients of preaxial polydactyly for investigation of recent incidences of each types. Methods: We reviewed 120 cases of preaxial polydactyly diagnosed at university hospitals of Incheon and Bucheon from 2000 to 2008. All cases were grouped using simple radiographs and all cases were classified according to the Upton modified Iowa system. Results: Among the 120 cases of preaxial polydactyly, there were 7 cases(6%) of type I, 35 cases(29%) of type II, 1 case(1%) of type III, 48 cases(40%) of type IV, 12 cases(10%) of type V, 5 cases(4%) of type VI, 12 cases(10%) of type VII polydactyly. All cases of type VII polydactyly were triphalangism. Conclusion: Comparing with Wassel's study and Upton's study, our study showed similar distribution of each types of preaxial polydactyly.