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A case of argyria secondary to the prolonged use of nasal drops that contained mild silver protein is presented. The patient was thought to be cyanosed immediately after a total knee replacement. Argyria, its differentiation from cyanosis and its prevention are discussed. The need for a careful drug history is noted.
A six-month old male infant with severe atopic dermatitis was admitted with hypoalbuminemia, oliguria and cyanosis of the extremities. There was marked edema and generalized eczema with foul, yellowish exudates. The patient's major clinical manifestations were attributed to the loss of albumin through the skin. Although atopic dermatitis is a common disease in children, here we want to show that systemic disturbances may arise from such condition, describe the total care given the patient, and emphasize the wholistic approach in managing cases of severe atopic dermatitis. Intensive treatment was instituted and the patient was discharged after three weeks and remained in a stable condition.
Isolation of an abnormal methemoglobin from two families exhibiting dominantly transmitted cyanosis have permitted the recognition of two different pigments of the hemoglobin M type. It is possible that the abnormal properties which characterize the acidic methemoglobin derivatives result from a crevice configuration of the heme, with two Feprotein bonds.
A 73 year old woman presented with profound central cyanosis and a history of a minor stroke. She had normal heart morphology, normal pulmonary artery pressure, and a normal coronary angiography. A patent foramen ovale (PFO) with a massive right to left shunt was demonstrated at the atrial level, with normal pulmonary venous saturations and PO2 values. This rare, age related case of right ventricular diastolic dysfunction in a normotensive patient revealed a generous PFO allowing a pronounced right to left shunt.
Twenty seven months after pulmonary artery banding a boy aged two and a half developed rapidly progressive cyanosis. A periarterial abscess was found at the site of the band. Repair of the pulmonary artery and closure of the ventricular septal defect were complicated by profoundly low cardiac output and prolonged dependence on a ventilator. This near fatal complication would have been avoided by primary closure of the ventricular septal defect.
This report investigated the origin of H(2)S in a newborn boy with sulfhaemoglobin induced cyanosis, who died because of multiple organ failure. Frozen material was collected and studied after death. The results suggest that enzymes had been released from deteriorating organs into the blood and abdominal fluid, and that the reaction of one of these enzymes with sulfur containing amino acids might have resulted in increased H(2)S concentrations. It is hypothesised that this release of enzymes resulted from a haemolysin produced by an invasive haemolytic Escherichia coli that was found in the blood and organs of this patient.
A patient with cyanosis in an uncomplicated ostium secundum atrial septal defect without pulmonary hypertension is described. There were no anatomic abnormalities in right-sided cardiac valves or venous return and no evidence of right ventricular hypoplasia or hypertrophy; however, the diastolic pressure curve suggested a decreased compliance. We suggest that this unusual finding may be a result of intrinsically abnormal right ventricular compliance.
Cardiac fibromas are rare lesions which occur more often in infants and children than in adults. These tumors are benign proliferations of connective tissue most often found in the left ventricular myocardium or septum. In an 8-month-old infant with cyanosis and progressive exertional dyspnea, a huge cardiac tumor obstructing the right ventricular outflow tract (RVOT) was diagnosed by means of 2-dimensional echocardiography and cardiac catheterization. At surgery, a whitish gray solitary tumor measuring 5.0 x 4.5 cm could be well visualized. It was nearly totally resected, and the RVOT was reconstructed with an Equine pericardial patch. Histologic examination classified the tumor as a fibroma. Although surgical mortality in cardiac fibroma with RVOT obstruction is extremely high, early diagnosis and prompt excision of the tumor is mandatory in relieving its dangerous symptoms.
Atrioventricular septal defects account for 4% of congenital cardiac malformations and over 50% of cardiac defects seen in Down syndrome. Clinical presentation is marked by congestive heart failure early in infancy. Cyanosis is rarely found in infants and suggests irreversible pulmonary hypertension or associated cardiac defects as tetralogy of Fallot, double outlet right ventricle, Ebstein anomaly, persistent left superior vena cava draining in the left atrium (Barbero Marcial, personal communication). Children with Down's syndrome is particularly difficult to assess because they often suffer from upper airways obstruction, which may contribute to the increased pulmonary vascular resistance determined at cardiac catheterization. This association of factors becomes a challenge for operability and, we will report one such case.
We have experienced a case of coronary sinus atrial septal defect (ASD) with ventricular septal defect (VSD). Cardiac catheterization revealed a step-up of oxygen saturation in right ventricle, L-R shunt 70.3%, R-L shunt 6.9% and pulmonary hypertension (PH). Since diagnosis of coronary sinus ASD was difficult, the operation was performed under diagnosis of VSD. Although the operation was success, cyanosis appeared after the operation. Contrast echocardiography using subcostal 4 chamber view showed coronary sinus ASD with a large Thebesian valve and the right-to-left shunt of the ASD. The Thebesian valve seemed to lead the venous return flow to the left atrium. Coronary sinus ASD was closed using a Xenomedica patch.
Echocardiography is a useful and reliable tool for the clinical assessment of the cyanotic neonate. It can be applied rapidly without compromising even the critically ill neonate. Hypoplastic left and right heart syndromes can be diagnosed or excluded echocardiographically. The diagnostic exclusions of total anomalous pulmonary venous drainage and complete transposition of the great arteries in the neonate with cyanosis remain difficult challenges for the echocardiographer because of the variability of their anatomic and hemodynamic presentations.
We describe a patient with a diagnosis of heterotaxia and independent drainage of the suprahepatic veins into the venous atrium who underwent total extracardiac cavopulmonary connection in which hepatic vein drainage remained directly into the atrium. In the immediate postoperative period she showed increasing cyanosis that suggested substantial right-to-left shunt. Surgical ligation of the suprahepatic veins resolved the complication effectively without signs of hepatic congestion or portal hypertension.
Four term babies, three boys and one girl, became cyanotic shortly after birth. In two cases the cause was maternal use of drugs: methemoglobinaemia due to use of a chromate and depression of the nervous system due to clomipramine. They recovered. A third child suffered cerebral infarction and was left with decreased left-arm function. The fourth child had a hypoplastic left heart and died. Central cyanosis in a newborn should be cause for concern. A thorough case history should be taken and extensive physical examination carried out. In case of doubt additional diagnostic tests should be performed.
We report the case of a newborn presenting a cyanosis after the birth with a good general state. Congenital methemoglobinemia is a rare disease which is characterized by a brutal appearance, in early infancy, of a bluish skin color not regressing with oxygen inspiration, and by a good general state. It is due to the recessive autosomal NADH-cytochrome b5 reductase (EC. 1.6.2.2) deficiency. This enzyme normally allows the reduction of the physiologically formed methemoglobinemia. Two forms of congenital methemoglobinemia have to be distinguished: the benign form (type I) and the severe form (type II).
Primary cardiac tumours are uncommon entities, and myxoma is the most common, comprising about 50% of all these neoplasms. A case of a 43-year-old woman with right atrial myxoma and a shunt through a patent foramen ovale producing severe cyanosis is reported.
A well but cyanotic newborn was found to have a mutant gamma-globin chain, leading to a functionally abnormal fetal hemoglobin. A single amino acid substitution was found in a site consistent with known adult M hemoglobins. This patient showed no clinical evidence of cyanosis at 5 weeks of age as gamma-chain synthesis was replaced by beta-chain synthesis. A sibling born 20 months later was also cyanotic and the same mutant hemoglobin was found.
During investigation of chronic cyanosis in a 25 year old male, after excluding pulmonary and cardiac causes, methemoglobinemia was suspected. Investigation of the activity of methemoglobin reductase clenched the diagnosis of homozygous cytochrome b5 reductase deficiency in a case of recessive congenital methemoglobin type I (absence of neurologic symptoms).
A case of cyanosis during general anaesthesia in a patient suffering from congenital deficiency of metahaemoglobin-reductase (diaphorase) is reported. The anaesthesiological technique and the probable triggering cause are analysed. The investigation carried out in members of the family to obtain confirmation of the genetic origin of the enzymatic deficiency is also reported.