• Journal of Chest Surgery
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• v.36 no.9
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• pp.683-686
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• 2003

Isolated right ventricular hypoplasia is a rare clinical entity. We describe a case of right ventricular hypoplasia, single atrium and spongy myocardium of left ventricle. The volume of right ventricle was half the volume of left ventricle and z-value of tricuspid valve was -4 preoperatively The patient, 6-year-old boy, underwent atrial partitioning with 3 mm fenestration, Postoperative course was smooth and he tolerated the biventricular state well during follow-up. Follow-up catherterization was done 27 months later The tricuspid valve grew well (z-value= -0.4) and atrial septal fenestration is closed spontaneously. This article reports a case of successful biventricular repair in a patient with isolated right ventricular hypoplasia.

• Clinical and Experimental Pediatrics
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• v.45 no.6
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• pp.804-808
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• 2002

We report a case of trisomy 22 in a liveborn male infant which was confirmed by fluorescence in situ hybridization(FISH), macrocultures and GTG-banding, and RHA-banding procedures of peripheral white blood cells. The infant showed lung hypoplasia, which is a unique presentation, with other clinical manifestations of previously reported cases of trisomy 22, such as intrauterine growth retardation, cleft palate, micrognathia, large atrial septal defect, limb anomalies, imperforate anus, and hypospadias. Our report gives weight to the previously reported observation that pulmonary hypoplasia may be associated in trisomy 22.

• Archives of Craniofacial Surgery
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• v.19 no.1
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• pp.48-50
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• 2018

Bony anomaly caused by lip tie is not many reported yet. There was a case of upper lip tie wrapping into the anterior premaxilla. We represent a case of severe upper lip tie of limited lip motion, upper lips curling inside, and alveolar hypoplasia. Male patient was born on June 3, 2016. He had a deep philtral sulcus, low vermilion border and deep cupid's bow of upper lip due to tension of short, stout and very tight frenulum. His upper lip motion was severely restricted in particular lip eversion. There was anterior alveolar hypoplasia with deep sulcus in anterior maxilla. Resection of frenulum cord with Z-plasty was performed at anterior premaxilla and upper lip sulcus. Frenulum was tightly attached to gingiva through gum and into hard palate. Width of frenulum cord was about 1 cm, and length was about 3 cm. He gained upper lip contour including cupid's bow and normal vermilion border after the surgery. This case is severe upper lip tie showing the premaxillary hypoplasia, abnormal lip motion and contour for child. Although there is mild limitation of feeding with upper lip tie child, early detection and treatment are needed to correct bony growth.

• Archives of Craniofacial Surgery
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• v.15 no.2
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• pp.94-97
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• 2014

Unilateral nostril hypoplasia is an extremely rare congenital malformation of unknown etiology, and only a few cases have been reported in literature. Owing to variability and complexity of the deformity, surgical correction of unilateral nostril hypoplasia represents one of the most significant reconstructive challenges to reconstructive plastic surgeons. We report a 7-year-old Vietnamese child with nasal and periocular deformity resembling a craniofacial cleft. Grossly, the right nostril was patent but with alar rim deformity, and the left nostril was not readily identifiable. A dystopic medial canthus was present on the left side as well. Closer inspection and palpation of the left side of nose revealed a patency through the soft tissue and underlying bony structure, Thus, a new alar rim were reconstructed with an irregularly shaped Z-plasty to create patency on the involved side. Simulatneously, a second Z-plasty was performed to address the medial canthal deformity. Postoperative appearance and function was sastisfactory at one-year follow up visit. In the treatment of patients with nostril hypoplasia, a careful preoperative physical examination is a prerequisite, and Z-plasty can be a valuable option for surgical correction.

• Archives of Plastic Surgery
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• v.50 no.2
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• pp.177-181
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• 2023

Digital extensor hypoplasia (DEH) is a rare malformation that presents with loss of active finger extension at the metacarpophalangeal (MCP) joints. Descriptions of optimal treatment and outcomes in this population are sparse. We describe successful operative treatment of a child with DEH involving the extensor digitorum communis, extensor digiti minimi, and the extensor indicis proprius tendons. The 5-year-old male patient was referred for severe limitation on bilateral finger extension since birth. He had been previously diagnosed with arthrogryposis and managed conservatively. Due to lack of improvement, magnetic resonance imaging was done evidencing hypoplasia/aplasia of the extensor tendons. The patient underwent successful tendon transfers using extensor carpi radialis longus to the common extensor tendons, and one hand required an additional tenolysis procedure. 2 years postoperatively, his MCP position and finger extension are markedly improved, and he is able to grip objects without limitation or difficulty. The patient returned to full activity without restriction.

• Journal of Korean Neurosurgical Society
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• v.41 no.5
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• pp.343-346
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• 2007

Hypoplasia of the internal carotid artery is a rare congenital anomaly. Agenesis, aplasia, and hypoplasia of the internal carotid artery [ICA] are frequently associated with cerebral aneurysms in the circle of Willis. Authors report two cases with congenital hypoplasia of the ICA accompanying with the aneurysms. Transfemoral cerebral angiography [TFCA] in one patient identified nonvisualization of the left ICA. Bilateral anterior cerebral artery [ACA] and middle cerebral artery [MCA] were supplied from the right ICA accompanying with two aneurysms at anterior communicating artery [AcoA] and A1 portion of the left ACA. TFCA in another patient demonstrated hypoplastic left ICA and left ACA filled from the right ICA accompanying with AcoA aneurysm. Left MCA was filled from basilar artery via posterior communicating artery [PcoA]. Skull base computed tomography [CT] in two patients showed hypoplastic carotid canal. Authors performed direct aneurysmal neck clipping. Follow up CT angiography [CTA] at one year after surgery did not show regrowth or new development of the aneurysm. In patients with hypoplastic ICA, neurosurgeons should be aware of the possibility of development of the aneurysms, presumably because of hemodynamic process. Direct aneurysmal neck clipping is a good treatment modality. After operation, regular CTA, magnetic resonance angiography [MRA] or TFCA is needed to find progressive lesion and to prevent cerebrovascular attack [CVA].

• Tuberculosis and Respiratory Diseases
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• v.46 no.1
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• pp.116-121
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• 1999

Unilateral hypoplasia of the pulmonary artery is an uncommon anomaly, which commonly develops in combination with congenital cardiovascular defects such as tetralogy of Fallot, patent ductus arteriosus and septal defect of atrium or ventricle, but may also present as an isolated lesion. We have recently experienced a case of the left pulmonary artery hypoplasia in adult by chance of during the general health screen, which diagnosed by chest X-ray, chest spiral CT, lung perfusion and ventilation scan, digital substraction angiogram and bronchoscopy, then presented hereby with the review of relevant literature.

• The Journal of Advanced Prosthodontics
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• v.1 no.1
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• pp.6-9
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• 2009

An 18 year old female with oligodontia and maxillary hypoplasia was treated using an interdisciplinary team approach involving orthodontists, maxillofacial surgeons and prosthodontists. Full mouth one-piece fixed partial dentures were the final restoration. The fixed partial dentures fabricated for the maxilla and mandible using the concept of a shortened dental arch resulted in improved esthetics and the masticatory function. This paper describes the treatment procedures for an oligodontia patient with alveolar bone hypoplasia.

• Journal of Chest Surgery
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• v.25 no.3
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• pp.321-324
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• 1992

Poland`s syndrome is very rare anomaly and typified by absence of the pectoralis major, absence or hypoplasia of the pectoralisminor, absence of costal cartilage, hypoplasia of breast and subcutaneous tissue, and brachysyndactyly. The clinical features are variable but all patients have absence of at least the sternal head of the pectoralis major muscle. The syndrome is not hereditary and is of unknown origin. Early recognition of Poland`s syndrome may give the provision of psychologic and genetic counselling for anxious parents. We have encountered a patient with this entity, and underwent successful correction.

• Journal of Korean Academy of Oral and Maxillofacial Radiology
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• v.19 no.1
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• pp.31-37
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• 1989

The author observed a rare case of radicular dentin dysplasia associated with enamel hypoplasia in a 11-year-old boy with a complaint of gum boil formation. 1. Clinically. yellowish-brown colored teeth with severe attrition and several gum boils were observed. 2. Radiographically, obliteration of pulp chamber and root canal, multiple periapical radiolucencies without obvious cause and blunt roots were observed. 3. Systemically, scalp hair and eyebrows were loose and short. And saddle nose could be also seen.