Urologic abnormalities in Menkes' kinky hair disease: report of three cases.

Menkes' kinky hair disease is a rare congenital disorder of copper metabolism with X-linked recessive inheritance. It is well known that it is frequently associated with urologic abnormalities. The authors experienced three such cases, but each of them was different. Multiple bladder diverticula, right vesico-ureteral reflux, and right hydronephrosis were noted on the first baby boy. In the second case, hematomas in the left kidney, pelvis, ureter and the adipose capsule, which were thought to be the abnormality of vessels, were noted in the neonatal period. In the third body, multiple bladder diverticular were noted at the age of 1 year. One year, 10 months later, a spontaneous rupture at the huge diverticulum occurred.

New operative method for tracheal compression in congenital heart disease.

A new technique is described for repairing tracheal compression caused by ascending aorta associated with corrected transposition of the great arteries and pulmonary atresia in a 113-day-old infant. Surgical repair for the tracheal compression was accomplished by suspending the pericardium around the aortic arch to the underside of the sternum. This method seems to be useful for some patients with tracheal compression caused by the ascending aorta.

[A case of thoracic aortic aneurysm associated with atypical coarctation and kinking of the aortic arch].

The aneurysm associated with atypical coarctation and kinking of the aortic arch is rare. Authors experienced such a case and performed operation using bypass technique as below. A 17-year-old male admitted to the authors' hospital to undergo operation of the enlarging thoracic aneurysm. He was observed from his childhood diagnosed as the coarctation and kinking of the aortic arch. The chest X-ray films showed a mass shadow in the left superior mediastinum. It had been also diagnosed as having a small post-stenotic dilation of aortic arch. Recently the mass on the X-ray enlarged. He was diagnosed as an aneurysm located on aortic arch using MR-imaging and aortogram. The aneurysmectomy was performed because of the possibility of the aneurysmal rupture. Under the general anesthesia, extra-anatomical bypass was made between ascending aorta and descending aorta, and another one between the bypass and left common carotid like as T-shape, because the proximal aneurysm was very near located on the origin of left common carotid. The aneurysm was excised and woven Dacron graft was implanted. The post-operative course was uneventful. This case suggests the necessity of deliberate clinical observation using roentgenography for a case of kinking of the aortic arch.

[A case report of cor triatriatum benefit of MRI for preoperative diagnosis and surgical method].

Cor triatriatum is uncommon in all congenital heart diseases. It is a malformation resulting in a separation of the left atrium or right atrium into two chambers due to a congenitally abnormal diaphragm. We wish to present a case of cor triatriatum in which MRI was found most useful for preoperative diagnosis and surgical procedure. A 2-year-old girl was transferred to us for severe pulmonary congestion as shown on chest X-ray. Echocardiography showed abnormal diaphragm in the left atrium. MRI demonstrated clearly the relationship between left pulmonary vein and the abnormal diaphragm. Therefore we should preoperatively determine type I A according to the Lucas and Schmidt's classification. Cardiac catheterization showed moderate pulmonary hypertension and confirmed cor triatriatum. The resection of the abnormal diaphragm was performed under extracorporeal circulation with moderate hypothermia. The postoperative course was uneventful. MRI is a very useful non-invasive technique in making a diagnosis and in choosing the appropriate surgical procedure for cor triatriatum.

[A case of tetralogy of Fallot associated with isolation of a subclavian artery and partial DiGeorge syndrome].

A month old girl was referred to our hospital for heart murmur and congestive heart failure. After the UCG and angiography, clinical diagnosis was tetralogy of Fallot, right aortic arch, isolation of left subclavian artery (Victorica type I), and a patent ductus arteriosus that originated from left subclavian artery. The direction of blood flow in the patent ductus arteriosus was from a subclavian artery to pulmonary artery, so she was in the condition of pulmonary over flow and appeared congestive heart failure, although she associated with tetralogy of Fallot. With intraoperative and postoperative examinations, we diagnosed the association of partial DiGeorge syndrome. Isolation of subclavian artery is uncommon, but this anomaly was classified by Victorica. Embryology of this anomaly was explained with hypothesis of double arch system. We had seen no reports of DiGeorge syndrome associated with isolation of a subclavian artery. But DiGeorge Syndrome is hypoplasia of branchiogenic organs, and subclavian arteries is differentiated from brachial arteries. So we supposed that this two lesions have the possibility of combination.

[A case of the successful treatment of pulmonary artery pseudoaneurysm after PA banding].

Pulmonary artery pseudoaneurysm after PA banding is a rare complication and its mortality is very high. The authors successfully operated on this sort of lesion. To our knowledge, this is the first successful case in Japanese literature. The second of twins was found to be suffering asplenia, dextro cardia, TAPVC, atrioventricular septal defect, corrected TGA and PDA. On the 19th day of life, increasingly pulmonary congestion forced us to operate. We performed PDA ligation, correction of TAPVC, together with extrathoracically adjustable PA banding. 5 months following this operation, spike fever and swelling of anterior thorax were noted. PA angiography was performed, and it showed that the pulmonary artery had been cut through by the band and a pseudoaneurysm had developed. The PA banding was removed and an end-to-end anastomosis of the PA was performed using cardiopulmonary bypass and deep hypothermia with surface cooling. The diameter of the PA anastomosis was designed to be one half that of her aorta. Staphylococcus aureus was cultured from the specimen of the PA band. It was thought to have contributed to the development of this aneurysm. The post operative course was uneventful.

Recurrent perforations of viscus due to ventriculoperitoneal shunt in a hydrocephalic child.

In this report, we describe the first case of recurrent perforations of viscus due to ventriculoperitoneal shunt for the treatment of hydrocephalus. Based on our own experience and a survey of literature, we suggest that perforation of the gastrointestinal tract should be suspected in patients with ventriculoperitoneal shunt, particularly when abdominal symptoms or ventriculitis are presented. To manage this complication, at the first perforation in the present case, a fibrin sealant was effective for closure of the perforated stomach wall. At the second perforation, when laparotomy was performed, we could not find any abnormality in the abdominal cavity except for a sheath and slight omental adhesions near it.