Yasuhiro Kitayama

Experience in tracheobronchial reconstruction with a costal cartilage graft for congenital tracheal stenosis

Although successful surgical management of congenital tracheal stenosis has been reported, it is still controversial as to the best operative procedure. Eleven infants with congenital tracheal stenosis were evaluated to confirm the efficacy of tracheobronchial reconstruction with costal cartilage graft. Symptoms ranged from recurrent respiratory infection to severe respiratory failure. All infants had other congenital anomalies in addition to tracheal stenosis. Notably, five infants had pulmonary artery sling and four infants had patent ductus arteriosus. Definitive diagnosis was made by bronchoscopy, results of which showed complete tracheal rings in all patients with severely compromised tracheobronchial lumens. Five infants had elongated stenosis involving nearly the whole length of the trachea, and five infants had segmental stenosis involving nearly one half the length of the trachea. One infant had bilateral stenosis of the main bronchi. Early experience included two deaths from problems related to the repair. The involvement of the carina and the distal portion of the trachea was associated with increased complications and a higher mortality rate. Currently, our preferred technique facilitated by extracorporeal membrane oxygenation (ECMO) includes carinal reconstruction with a thin-wall intraluminal stent. Bronchoscopy is essential for accurate intraoperative incision of the trachea, post-operative airway management for several weeks, and removal of the intraluminal stent.

Prenatal diagnosis of abdominal wall defects and their prognosis

To determine whether associated anatomic features may be useful in predicting fetal prognosis, 43 fetuses who had ultrasonographic evaluation for abdominal wall defects during a 13-year period were reviewed. Thirty-one fetuses had omphalocele and were classified into three groups according to fetal ultrasonography results: ruptured omphalocele with exposed liver, giant omphalocele with exposed liver, and small omphalocele without liver herniation. Twelve fetuses had gastroschisis. Ten of the 12 fetuses with gastroschisis survived; one died in utero. Nine of the 12 with a small omphalocele survived. Ten of 12 fetuses with giant omphalocele survived, but six of the seven with ruptured omphalocele died of pulmonary hypoplasia and respiratory insufficiency. Ruptured omphalocele recognized in utero was accompanied by intrauterine growth retardation and liver herniation and frequently was associated with deformity of the spine, diaphragmatic defects, vesicointestinal fissure, and meningocele. These results indicated that, together with the deformity of the spine, rupture and absence of the covering membrane with an exposed liver (noted through fetal ultrasonography) may suggest a poor prognosis because of pulmonary hypoplasia.

Nitric oxide inhalation therapy for an infant with persistent pulmonary hypertension caused by misalignment of pulmonary veins with alveolar capillary dysplasia

Misalignment of pulmonary veins with alveolar capillary dysplasia (MPV) has been reported to be a rare cause of persistent pulmonary hypertension of the newborn (PPHN) and to be fatal despite extracorporeal membrane oxygenation (ECMO). A full-term female neonate with PPHN was brought to the hospital for ECMO therapy at 2 days of age. On the 14th day of life, she was extubated early after the second run of ECMO, and underwent nitric oxide (NO) inhalation therapy in the incubator. She died of catheter-related sepsis on the 61st day of life. After autopsy findings revealed MPV, the longest survival with this disease was documented. NO inhalation therapy in the incubator may provide time for lung transplantation.

Prolonged preoperative stabilization using high-frequency oscillatory ventilation does not improve the outcome in neonates with congenital diaphragmatic hernia

Abstract Although delayed repair with preoperative stabilization using high-frequency oscillatory ventilation (HFOV) has been advocated in neonates with congenital diaphragmatic hernia (CDH), improved survival has not been reported. We compared survival between neonates undergoing delayed repair after prolonged stabilization for more than 48 h using HFOV and those undergoing immediate repair following short stabilization using HFOV in subjects with high-risk CDH. Patient selection was made by two criteria: fetuses with the lung/thorax transverse area ratio (L/T) below 0.26 and neonates who presented with respiratory distress significant enough to require intubation within 6 h after delivery. Selection of the strategies differed by era and medical center. Stabilization failed in 11 of 18 patients with delayed repair and only 7 patients (38.9%) in this group survived, but 18 of 23 patients (78.3%) in the immediate-repair group survived (P < 0.05). Although lower values of Apgar scores, best postductal PO2 (BPtDPO2), gestational weeks, and L/T and more frequent patch repair were observed in the delayed than in the immediate repair group, significant differences in survival among the subpopulations were also observed in the prenatally diagnosed group, including the group with L/T < 0.15 and the group that required ECMO, the group with the best preductal PO2 (BPrDPO2) ≥ 100 torr, and the group with BPtDPO2 < 100 torr. These results indicate that prolonged stabilization for more than 48 h using HFOV does not improve survival in patients with high-risk CDH.

Clinical significance of the lung/thorax transverse-area ratio in fetuses with cystic lung disease

Abstract We examined the clinical significance of the lung-thorax transverse-area ratio (L/T) in fetuses with cystic lung disease (CLD). The transverse section of the thorax was analyzed at the level of the 4-chamber view of the heart. L/T was calculated as the bilateral normal lung area divided by the thoracic area. Within a 9-year period, ultrasonography was performed in 15 fetuses with CLD, 13 of which were histologically diagnosed: congenital cystic adenomatoid malformation of the lung (CAM; n = 8), pulmonary sequestration (n = 4), and bronchial atresia (n = 1). Although the initial L/T measurement (29.6 ± 4.2 weeks) showed significantly lower values than in the controls, the final measurement (36.4 ± 1.6 weeks) indicated that the L/T in CLD excluding CAM was significantly higher than that in CAM. Six patients with a final L/T of less than 0.21 required mechanical ventilation immediately after birth. Two of these patients died, and the autopsy findings indicated pulmonary hypoplasia. In these cases, a significant correlation was observed between the maximal value of L/T and preductal alveolar-arterial oxygen difference (A-aDO2), although the final L/T was not correlated to A-aDO2. These results indicate that serial measurements of L/T may be useful in the diagnosis of CLD and may help to predict postnatal respiratory conditions.

Bilateral diaphragmatic hernia followed by fetal ultrasonography. A report of two cases.

Two cases of bilateral congenital diaphragmatic hernia (CDH) followed by fetal ultrasonography were described. Although many cases of CDH are diagnosed by fetal ultrasonography, it is difficult to diagnose bilateral CDH in utero, which is a relatively rare and fatal condition. Two fetuses were diagnosed as having left CDH associated with severe anomalies. However, a retrospective review of fetal ultrasonography indicated elevation of the liver in the right posterior chest. Both patients died shortly after surgical repair for left CDH despite the use of extracorporeal membrane oxygenation. Diagnosis of bilateral CDH by fetal ultrasonography and the evaluation of its prognosis were discussed.

Successful excision of a congenital, prenatally diagnosed fibrosarcoma involving the entire right ovary

This report describes a congenital fibrosarcoma (CFS) of the ovary presenting as an abdominal mass on prenatal ultrasonography. Few cases of intrauterine CFS have been reported to date. When CFS develops prenatally, it usually grows rapidly, and resection is difficult at birth. We successfully resected a tumor after birth, and there has been no recurrence after 2 years. This patient is the first known survivor of CFS diagnosed prenatally.

Laparoscopic Rectopexy for Rectal Prolapse in Children

Recently the laparoscopic approach has been applied to rectal prolapse as a less invasive procedure in adults. However, there have been few reports of these laparoscopic procedures in children. We performed laparoscopic suture rectopexy in three children, two of whom had an associated developmental disorder. After the retrorectal dissection, the rectum was fixed to the presacral fascia with four 2-0 braided absorbable sutures. Additionally, oxycellulose was left in the retrorectal space as an adhesive and hemostatic agent. The postoperative course was uneventful. In two cases, the results remain good after follow-up of 18 and 27 months. In the remaining case, rectal prolapse recurred after 5 months, and eventually resection rectopexy was needed 1 year after the initial operation. Our experience shows that laparoscopic suture rectopexy is feasible and less invasive in children, as it is in adults. In selected cases, laparoscopic rectopexy could be the primary surgical intervention for the treatment of full...

Is adrenomedullin involved in the pathophysiology of persistent pulmonary hypertension of the newborn

Although adrenomedullin (ADM) is a potent vasodilating peptide reported to play a possible role in the mechanisms of fetal lung differentiation and maturation, the ADM blood level in fetuses and in neonates with persistent pulmonary hypertension (PPHN) and pulmonary hypoplasia is not known. Therefore, we examined 15 patients with PPHN: 10 with congenital diaphragmatic hernia, four with congenital cystic adenomatoid malformation of the lung, and one with misalignment of pulmonary vessels with alveolar capillary dysplasia. Eight surgical patients with neonatal conditions such as intestinal atresia served as controls. Blood samples were drawn from the umbilical artery and vein at birth, and arterial blood was drawn from patients with PPHN on the 3rd and 6th days after birth. Plasma levels of ADM were measured by radiometric assay. Plasma levels of ADM in the umbilical artery and vein were elevated in patients with PPHN compared with controls, and in all groups the levels in the umbilical vein were higher than those in the umbilical artery. The arterial levels in patients with poor prognoses were elevated on the 3rd and 6th days after birth compared with those in survivors. These results indicate that ADM may be involved in the pathophysiology of PPHN and in the mechanisms of lung differentiation and/or maturation.

Entry criteria for extracorporeal membrane oxygenation In neonates with congenital diaphragmatic hernia treated with high-frequency oscillatory ventilation

Although respiratory management with high-frequency oscillatory ventilation (HFOV) has generally been used for neonates with congenital diaphragmatic hernia (CDH), entry criteria for extracorporeal membrane oxygenation (ECMO) based on data from patients who underwent HFOV have not yet been reported. To establish entry criteria for ECMO in such patients, we retrospectively studied 36 neonates with CDH treated by HFOV in our institutions between 1986 and 1994. From the admission records, preductal and postductal arterial blood gas data and HFOV ventilation conditions for 72 h after admission were extracted. Oxygenation index (01) and alveolar-arterial oxygen gradient (A-aD02) time interval combinations were calculated. Patients were divided into two groups: candidates for ECMO (n = 22) who underwent ECMO (n = 18) or died without ECMO (n = 4); and non-candidates (n = 14), who survived without ECMO. Blood gas data in patients placed on ECMO were comparable to those in patients who died without ECMO: mean pre- and postductal OI for 4 h > 30, postductal A-aD02 ≥620 mmHg for 4 h, postductal A-aD02 ≥580 mmHg for 8 h, and postductal A-aD02 ?550 mmHg for 12 h showed better sensitivity with a specificity of more than 90% compared to entry criteria that had previously been used in our institutions: a postductal OI >40 for 4 h and postductal A-aDO2 ≥610 mmHg for 8 h. In addition, a combination of preand postductal OI >30 for 4 h indicated a sensitivity of 95.5% and a specificity of 92.9%.