• Title/Summary/Keyword: Congenital absence

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Congenital Bochdalek hernia: report of 4 cases (선천성 Bochdalek hernia4례 보고)

  • Jin, Jae-Gwon;Park, Ju-Cheol;Yu, Se-Yeong
    • Journal of Chest Surgery
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    • v.15 no.4
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    • pp.432-439
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    • 1982
  • Congenital posterolateral diaphragmatic hernia [Bochdalek hernia] is the result of a congenital diaphragmatic defect in the posterior costal part of the diaphragm in the region of the tenth and eleventh ribs. There is usually free communication between the thoracic and abdominal cavities. The defect is most commonly found on the left [90%], but may occurs on the right, where the liver often prevents detection. The male to female ratio is 2:1. Owing to the negative intrathoracic pressure, herniation of abdominal contents through the defects occurs, with resultant collapse of the lung. Shifting of mediastinum to the opposite side and compression of the opposite lung occurs. Most often these hernias are manifestated by acute respiratory distress in the newborn. A second, but less well recognized, group of patient with Bochdalek hernia survive beyond the neonatal period, usually present at a later time with "failure of thrive, intermittent vomiting, or progressive respiratory difficulty. " The diagnosis can often be made on clinical ground from the presence of respiratory distress, absence of breath sounds on the chest presence of bowel sounds over the chest . Roentgenogram of the chest confirm the diagnosis. Obstruction and strangulation have been reported but are rare. Treatment consists of early reliable identification of these congenital diaphragmatic hernia with high risk and surgical repairment. and postoperative pharmacological management with extracorporeal membranous oxygenation [=ECMO] support in the period of intensive care. On the surgical approach, for defects on left side, an abdominal incision is preferred, because of the high incidence of malrotation and obstructing duodenal bands. In the neonate, the operative mortality may be appreciable, but, later repair almost always is successful. During the period from 1972 to 1982, 4 cases of congenital Bochdalek hernia were experienced at the Kyung-Hee University Hospital.

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A Case of Short Bowel Syndrome Due to Strangulated Congenital Internal Hernia in Children (교액성 선천성 내부 탈장에 의해 발생한 단장 증후군 1례)

  • Sim, Chang Eun;Moon, Jin Soo;Kim, Ki Hong;Kim, Han-Seong
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.7 no.2
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    • pp.268-273
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    • 2004
  • Short bowel syndrome in children, most commonly results after extensive bowel resection for necrosis of the bowel. It may be caused by several intestinal catastrophes such as volvulus, hernia and necrotizing enterocolitis. The risk factors on short bowel syndrome are the remaining length of the bowel, the age of onset, the absence of the ileo-cecal valve and the time after resection. Macro and micronutritional deficiencies are the most significant complications of short bowel syndrome. We report a 5 year-old girl, who had a strangulated congenital transmesenteric hernia leading to short bowel syndrome accompanied by iron deficiency anemia.

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Brainstem auditory evoked potential findings in a French bulldog with bilaterally congenital sensorineural deafness

  • An, Daegi;Jung, Dong-In;Kim, Ha-Jung;Kang, Ji-Houn;Chang, Dong-Woo;Yang, Mhan-Pyo;Kang, Byeong-Teck
    • Korean Journal of Veterinary Research
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    • v.53 no.4
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    • pp.265-267
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    • 2013
  • A 3-month-old, intact male French bulldog was suspected of deafness. The dog was irresponsive to environmental noises generated out of sight, but normal responses were noted for visual stimuli. No abnormalities were observed on the neurological, otoscopic, radiographic, and blood examinations. To diagnose the apparent deafness, brainstem auditory evoked potential (BAEP) was recorded in the presented dog together with a normal dog. While the BAEP from the control dog showed a normal wave consisting of 5 peaks, absence of all peaks was noted in the suspected deaf dog. Therefore the dog was definitively diagnosed as bilaterally congenital sensorineural deafness.

Congenital Atresia of the Left Internal Jugular Vein with Absence of the Left Transverse Sinus and Sigmoid Sinus (좌측 가로정맥동과 에스자정맥동의 부재를 동반한 좌측 속경정맥의 선천성 폐쇄)

  • Cho, Young-Ju;Lee, Kyung-Suk;Hwang, Byung-Eon;Yang, Yun-Su;Hong, Ki-Hwan
    • Korean Journal of Head & Neck Oncology
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    • v.27 no.2
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    • pp.226-229
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    • 2011
  • Knowledge of variations of veins of head and neck in relation to internal jugular, external jugular, anterior jugular and facial veins is important to surgeons doing head and neck surgery as well as to radiologists doing catheterization and to clinicians in general. In the current case, congenital atresia of the left internal jugular vein is reported. During neck dissection, superficial veins of neck and left external jugular vein was engorged and the left internal jugular vein was look like a fibrotic band. The left common carotid artery and vagus nerve was normal.

Congenital Left Atrial Appendage Aneurysm - A case report- (선천성 좌심방이류 - 1례 보고 -)

  • 김근직;이응배;전상훈;장봉현;이종태;김규태
    • Journal of Chest Surgery
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    • v.32 no.9
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    • pp.827-830
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    • 1999
  • Congenital left atrial appendage aneurysm is a very rare congenital cardiac abnormality. That is postulated to arise from a developmental weakness in the atrial wall in utero. Clinically, patients are often asymptomatic and are diagnosed incidentally, but supraventricular arrhythmias and systemic thromboembolism have also been reported in some cases. Surgical resection at the time of diagnosis is recommended because of the propensity for thromboembolic complications. A 13-month-old female, who was suspected preoperatively as having partial absence of pericardium with left atrial herniation through the defect, underwent surgical resection of the left atrial appendage aneurysm. Exposure through a median sternotomy showed an intact pericardium. The postoperative course was uneventful.

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A Case of Mayer-Rokitansky-K$\ddot{u}$ster-Hauser(MRKH) Syndrome with Imperforate Anus and Unilateral Renal Agenesis (편측 신장 무형성 및 쇄항을 동반한 Mayer-Rokitansky-Küster-Hauser(MRKH) 증후군 1례)

  • Kim, Tae-Hyung;Kim, Jin-Hee;Kim, Soo-Yung
    • Childhood Kidney Diseases
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    • v.10 no.2
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    • pp.233-237
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    • 2006
  • The clinical features of the Mayer-Rokitansky-K$\ddot{u}$ster-Hauser(MRKH) syndrome include normal female secondary sex characteristics, normal genitalia, congenital absence of vagina, rudimentary or bipartite uterus, normal ovarian function and normal ovulation, 46, XX, karyotype, frequent association of renal, skeletal and other congenital anomalies. We experienced a case of a premature infant with MRKH syndrome who had imperforate anus and unilateral renal agenesis.

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Taxonomy of the Genus Symphyocladia (Rhodophyta) (한국산 홍조식물 보라색 우무속(Symphyocladia)직물의 분류)

  • Lee, In-Kyu
    • Journal of Plant Biology
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    • v.38 no.3
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    • pp.297-303
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    • 1995
  • Four species of Symphyocladia growing along the coast of Korea were investigated in taxonomic point of view. Characters available for identification of the species were; difference in gross morphology, degree of cortication in vegetative axis, presence or absence of vegetative trichoblasts, and developmental pattern of stichidia. Vegetative structure and reproductive organs in developmental anatomy were similar among species. Branching pattern and occurrence of vegetative trichoblasts adopted for systematic criteria between Pterosiphonieae and Polysiphonieae, and between Symphyocladia and Pterosiphonia were no more confidential. The degree of congenital fusion of laterals with the parent axes was considered as only main character to distinguish Symphyocladia from Pterosiphonia.

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Neonatal arrhythmias: diagnosis, treatment, and clinical outcome

  • Ban, Ji-Eun
    • Clinical and Experimental Pediatrics
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    • v.60 no.11
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    • pp.344-352
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    • 2017
  • Arrhythmias in the neonatal period are not uncommon, and may occur in neonates with a normal heart or in those with structural heart disease. Neonatal arrhythmias are classified as either benign or nonbenign. Benign arrhythmias include sinus arrhythmia, premature atrial contraction, premature ventricular contraction, and junctional rhythm; these arrhythmias have no clinical significance and do not need therapy. Supraventricular tachycardia, ventricular tachycardia, atrioventricular conduction abnormalities, and genetic arrhythmia such as congenital long-QT syndrome are classified as nonbenign arrhythmias. Although most neonatal arrhythmias are asymptomatic and rarely life-threatening, the prognosis depends on the early recognition and proper management of the condition in some serious cases. Precise diagnosis with risk stratification of patients with nonbenign neonatal arrhythmia is needed to reduce morbidity and mortality. In this article, I review the current understanding of the common clinical presentation, etiology, natural history, and management of neonatal arrhythmias in the absence of an underlying congenital heart disease.

Diagnostic imaging of congenital pulmonary aplasia in a dog

  • Kim, Soochan;Choi, Hojung;Lee, Youngwon
    • 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.

Shoulder instability with congenital absence of bilateral long head of biceps tendon - A Case Report - (양측성 상완이두장건 결손과 견관절 불안정성 - 증례 보고 -)

  • Rah, Jung-Ho;Chung, Hoi-Jeong;Kim, Doo-Sup
    • Journal of the Korean Arthroscopy Society
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    • v.14 no.3
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    • pp.188-191
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    • 2010
  • Absence of long head of biceps tendon is a very rare anomaly. We encountered a 19 year-old male patient who had right shoulder subluxation. On magnetic resonance imaging, shallow intertubercular groove on right humerus was observed, and absence of long head of biceps tendon was discovered. Arthroscopically, labral rupture and absence of long head of biceps tendon were also evident. Left shoulder had a similar symptom as right 5 months after the initial development of the symptom on the right shoulder. Both MRI and arthroscopy revealed absence of long head of biceps tendon and labral ruptre. Arthroscopic labral repair was performed for both shoulders, and, postoperatively, they were fixated with abduction brace. In this case, it is considered that the absence of long head of biceps tendon triggered the instability of shoulder found in our patient.

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