• Korean Journal of Radiology
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• v.22 no.10
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• pp.1690-1696
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• 2021

Objective: To describe the anatomic locations and imaging features of posterior lung herniation in unilateral pulmonary agenesis and aplasia, focusing on radiograph-CT/MRI correlation. Materials and Methods: A total of 10 patients (seven with pulmonary agenesis and three with pulmonary aplasia, male: female = 1:9, mean age 7.3 years, age range from 1 month to 20 years) were included. Chest radiographs (n = 9), CT (n = 9), and MRI (n = 1) were reviewed to assess the type of lung underdevelopment, presence of anterior and posterior lung herniation, bronchus origin, supplying artery, and draining vein of the herniated lung. Results: Pulmonary agenesis/aplasia more commonly affected the left lung (n = 7) than the right lung (n = 3). Anterior lung herniation was observed in nine of the 10 patients. Posterior lung herniation was observed in seven patients with left pulmonary agenesis/aplasia. Two patients showed posterior lung herniation crossing the midline but not beyond the aorta, and five patients showed the posteriorly herniated right lower lobe crossing the midline to extend into the left hemithorax farther beyond the descending thoracic aorta through the space between the esophagus and the aorta. This anatomical configuration resulted in a characteristic radiographic finding of a radiolucent area with a convex lateral border and a vertical medial border in the left lower lung zone, revealing a tongue-like projection on CT and MRI. Conclusion: Posterior lung herniation occurs in unilateral left lung agenesis/aplasia. Approximately 70% of the cases of posterior lung herniation reveal a unique radiolucent tongue-like projection in the left lower lung zone on imaging studies, which is caused by the extension of the posteriorly herniated right lung farther beyond the descending aorta.

• Journal of Veterinary Clinics
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• v.34 no.4
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• pp.283-286
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• 2017

A 4-month-old, female Maltese dog was referred with continuous heart murmur. Patent ductus arteriosus was diagnosed via radiography and echocardiography. The patient was untreated because of client's refusal. After 13 months, the dog was referred again with seizure and salivation. Laboratory examination revealed increased liver enzymes, hyperammonemia and decreased total cholesterol and total protein. Microhepatica was identified on abdominal radiography. CT angiography showed a shunt vessel that originated from the portal trunk to the prehepatic caudal vena cava and patent ductus arteriosus connecting proximal descending aorta with the main pulmonary artery. No portal vasculature toward liver is observed after shunt vessel. The patient was diagnosed as concurrent patent ductus arteriosus and congenital extrahepatic portosystemic shunt with suspected portal vein aplasia. In human, cardiac malformations are frequently observed in patients with congenital extrahepatic portosystemic shunt with portal vein aplasia. This report described concurrent patent ductus arteriosus and congenital extrahepatic portosystemic shunt with suspected portal vein aplasia in a dog.

• Childhood Kidney Diseases
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• v.12 no.1
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• pp.111-115
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• 2008

Vitamin D-dependent rickets(VDDR) is a rare autosomal disorder, characterized by hypocalcemia, hypophosphatemia, increased alkaline phosphatase, secondary hyperparathyroidism and many other clinical features. Type I VDDR is due to congenital defects of renal 1${\alpha}$-hydroxylase, the enzyme responsible for the conversion of 25-(OH)D3 to 1,25-$(OH)_2D3$. Type II VDDR arise from target organ resistance to 1,25-$(OH)_2D3$. Unilateral renal aplasia is generally thought to result from a lack of induction of the metanephric blastema from the ureteral bud, which may be secondary to ureteral bud maldevelopment and/or to a problem with the formation of the mesonephric duct. The incidence of unilateral renal aplasia is approximately 1/500-3,200. Type 1 VDDR associated with unilateral renal aplasia has not been reported yet. Thus we report a case of a 3 month old female infant diagnosed as type 1 VDDR with unilateral aplasia of kidney.

• Archives of Plastic Surgery
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• v.33 no.5
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• pp.672-675
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• 2006

Purpose: The purpose of this study is to document the surgical methods used in infants with aplasia cutis congenita treated with allogenic dermal matrix and cultured epithelial autografts. Methods: The large defects in both lower legs were replaced with allogenic dermal matrix to avoid the postoperative hypertrophic scar contracture and a full-thickness skin biopsy was taken from right groin area simultaneously. We sent the specimen to a commercial laboratory for culture and obtained cultured epithelial autografts($Holoderm^{(R)}$) after 2 weeks, placed it over the allogenic dermal matrix. Results: The skin-defected area were nearly epithelialized after 2 weeks and there were no significant problem on during 6 months follow-up. Conclusion: The surgical method using allogenic dermal matrix and cultured epithelial autograft provided an excellent coverage of large skin defects of infant with aplasia cutis congenita.

• Journal of Yeungnam Medical Science
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• v.9 no.2
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• pp.422-426
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• 1992

One case of Congenital Cutis Aplasia is presented. The defect involved includes full-thickness skin defect of scalp and cranium. The patient was treated with debridement of dirty necrosed crust which covered exposed dura mater and with double opposing rotation flap including pericranium for bone regneration. The donor site was covered with skin graft from right thigh. During operation, the superficial temporal arterty was found to be short and weak. And after operation, the margin of flap were congested and finally necrotized. The necrotic wound was treated with conservative management. The vascular impairment is thought to be main course of Congenital Cutis Aplasia. So we conclude that the treatment of choice is conservative management or careful flap surgery for coverage of defect area.

• Journal of Yeungnam Medical Science
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• v.10 no.2
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• pp.493-505
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• 1993

The congenital cutis aplasia mainly occurs in head, sometimes involving the skull and dura mater. It's cause and the rate of falling ill are not known yet, it is the disease that rate of death is high by the infection, such as, the injured vascular hemorrhange of meningitis. Craniosynostosis is the disease the appears the skull as well as the facial deformity with growing, has from the developmental difficiency, visual distibance, motor disturbance, convulsion to the neurologic impairment of mental retardation, and accompanies the each characteristic deformity following the suture fused. Satisfactory results was achieved by local flap surgery and conservative treatment on the infant, diagnosed as the congenital cutis aplasia, case 1 Also successful treatment experiences of craniosynostosis(oxycephaly, brachycephaly, trigonocephaly, cloverleaf deformity) through the frontal bone advancement and the barrel stave asteotomy, were reviewed & pursues and investigates the intracranial volume of before and after of surgery, and then reports with the literature investigation.

• Journal of Chest Surgery
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• v.50 no.2
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• pp.119-122
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• 2017

Pure red cell aplasia (PRCA) and hypogammaglobulinemia are paraneoplastic syndromes that are rarer than myasthenia gravis in patients with thymoma. Good syndrome coexisting with PRCA is an extremely rare pathology. We report the case of a 50-year-old man with thymoma and PRCA associated with Good syndrome who achieved complete PRCA remission after thymectomy and postoperative immunosuppressive therapy, and provide a review of the pertinent literature.

• Korean Journal of Veterinary Research
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• v.57 no.4
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• pp.253-255
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• 2017

A 2-year-old, female Pomeranian dog was referred for dyspnea. Thoracic radiographs revealed left-sided mediastinal shift, increased soft tissue opacity in the caudal aspect of left thorax with loss of the left diaphragmatic silhouette, and dorsal elevation of mediastinal structures and heart from the sternum by lung tissue. The left main bronchus was visualized as an air-bronchogram and observed to abruptly discontinue at the level of the 10th rib. Thoracic computed tomography (CT) revealed absence of the left lung parenchyma and left pulmonary vessels with a rudimentary left main bronchus. The case was congenital pulmonary aplasia diagnosed via radiography and CT.

• Korean Journal of Veterinary Research
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• v.59 no.3
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• pp.171-173
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• 2019

Two dogs presented with vomiting and head pressing. In both dogs, a large vessel was revealed in computed tomography (CT) angiography, which was found to leave the portal vein (PV) cranial to the splenomesenteric confluence and enter the pre-hepatic caudal vena cava cranial to the right renal vein. The flow of portal blood to the liver was not identified. Based on CT angiography, the dogs were suspected to have congenital PV aplasia with portocaval shunting. Diagnostic imaging of potential malformations for PV continuation should be conducted before attempting shunt closure.

• Journal of Veterinary Clinics
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• v.32 no.2
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• pp.196-199
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• 2015

A 7-year-old intact female dachshund (Dog 1) and a 17-year-old intact male Yorkshire terrier (Dog 2) were presented for evaluation of mammary gland tumor and a regular checkup, respectively. Incidentally, segmental caudal vena cava aplasia was detected on CT images in both dogs. Absent postrenal segment (Dog 1) or discontinuation of caudal vena cava (Dog 2) were detected, but prerenal segment of caudal vena cava was continued to the dilated azygos vein. Segmental CVC aplasia should be considered when an abdominal vascular anomaly was evaluated with CT angiography in dogs.