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Vascular rings are a diagnostic and therapeutic challenge for pediatricians. Many diagnostic modalities contribute to the detection of these rare congenital vascular anomalies. The role of flexible bronchoscopy is still being debated. We present 3 cases to emphasize the usefulness of pediatric flexible bronchoscopy (PFB) in the early diagnosis and postoperative evaluation of vascular rings. In patient 1, PFB was performed before conventional techniques were available. A right aortic arch with a retroesophageal aortic diverticulum and mirror-image branching was later confirmed. In patients 2 and 3, pulmonary artery slings could not be detected clearly by imaging studies before PFB was performed. PFB was arranged again postoperatively for these 2 patients, because of difficulty weaning patient 2 from ventilator support and persistent respiratory distress in patient 3. In conclusion, we expect that more vascular rings will be diagnosed using PFB. This instrument is also useful in making a decision for surgery, and for detecting associated tracheobronchial anomalies preoperatively, assuring appropriate correction intraoperatively, and monitoring the condition of vascular rings postoperatively.

A cystic hygroma was found in a full-term (39 week-old) male newborn delivered by cesarian section. Polyhydramnios was diagnosed by sonography at 21 weeks of gestation. Clinical manifestations at birth, such as respiratory distress, tachypnea and chylothorax were successfully relieved by the insertion of chest tube and medical therapy. Other conditions, which included mild hydronephrosis of left kidney, thickening of aortic and pulmonary valves with mild mitral and tricuspid valves regurgitation, subsided within six months. The cystic hygroma regressed to a webbed neck. In general, the features and clinical manifestations of the present case resembled the criteria of Noonan syndrome including various congenital heart defects; webbed neck; chest deformity; a characteristic facial appearance comprising of a broad forehead, ocular hypertelorism, antimongoloid slant of palpebral fissures, low set ears; and bilateral undescended testis etc., suggested by Noonan and other subsequent authors. The outcome of this infant was satisfactory following medical and surgical therapy (chest tube, orchiopexy) and a special dietary regimen.

Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) is a rare congenital disorder characterized by a massively enlarged urinary bladder without mechanical outlet obstruction and microcolon, as well as a hypoperistaltic bowel with normal ganglion cell distribution. We report one such case to discuss the findings of antenatal ultrasound and the radiologic and pathologic features of this condition.

A 2-day-old newborn was treated with tolazoline for persistent pulmonary hypertension due to meconium aspiration syndrome. Hypoxemia improved during continuous tolazoline infusion, but gastrointestinal bleeding occurred. After treatment with cimetidine, there was a rapid deterioration with decreased oxygen saturation and arterial PO2 values. Tolazoline induces a dilatation of the pulmonary vascular system by stimulating H2 receptors. Cimetidine, an H2 blocker, may partially abolish the effect of tolazoline. However, given the clinically significant result to the interaction between tolazoline and cimetidine we report, the use of cimetidine in tolazoline induced upper gastrointestinal hemorrhage should deserve more attention.

Hydrometrocolpos is a rare congenital anomaly and serious life threatening condition in the newborn infant due to its long-term compression sequelae and associated congenital anomalies. Prenatal diagnosis of hydrometrocolpos by sonogram allows appropriate management during the prenatal and neonatal period. The combination of hydrometrocolpos and polydactyly is the cardinal hallmark feature of McKusick-Kaufman Syndrome. We present a case of congenital hydrometrocolpos due to vaginal atresia combined with polydactyly of both feet, mild atrial septum defect, bilateral hydronephrosis, fetal ascites and polyhydramnios. Pathogenesis and treatment of hydrometrocolpos and its associated congenital anomalies are discussed.

Candida infection contracted in utero and manifested at birth is a rare event. Two newborn infants are presented with generalized maculopapular rashes at birth. The eruption soon became vesicles or pustules; no other manifestations were found. Candida albicans was isolated from the skin lesion. After topical antifungal application, the lesions became fine and desquamated in the recuperative period. No complication was noted during the course. The diagnosis of congenital cutaneous candidiasis relies on its clinical course and positive culture result of the skin lesion. No systemic antifungal therapy is recommended except for the high risk infants.

Edwards syndrome is the second most common autosomal trisomy syndrome, resulting from an extra chromosome in chromosome 18. Recently we encountered two cases with the following representative features: intrauterine growth retardation, polyhydramnios, low-set ears, micrognathia and clenched hands. Both cases were female newborns and had an abnormal karyotype 47, XX, 18.

Neonatal adrenal hemorrhage is frequently associated with large fetal size, birth trauma owing to difficult labor or delivery, perinatal asphyxia, fetal hypoxia, septicemia or coagulation defect. A case of giant baby with perinatal asphyxia and birth injury complicated with adrenal hemorrhage is presented. The baby was admitted at five days of life due to profound jaundice. Right adrenal hematoma was found by abdominal sonography. The hematoma resolved gradually under supportive management by three months of age as documented by serial sonography. No complication was found. We conclude that abdominal sonography is the modality of choice for initial diagnosis and follow-up of neonatal adrenal hemorrhage, and conservative management is the mainstay of treatment.