Meng-Luen Lee

Modified arterial switch operation by spiral reconstruction of the great arteries in transposition

BACKGROUND Spiral relationship of the normally related great arteries (SRGA) has never been reconstructed in an arterial switch operation. METHODS From March 1998 to April 1999, 9 consecutive cases of transposition of the great arteries (TGA) family (from 2 days to 1.6 years old) underwent arterial switch operations with SRGA at our hospital. Two had a congenitally corrected TGA (plus atrial redirection). Lecompte maneuver was not used in all. The posterior wall of pulmonary trunk was not divided but three were reattached, two of whom had had previous pulmonary trunk banding. Thus the wall was shared between the great arteries facing each other. RESULTS All survived the operation. Supraaortic stenosis was balloon-dilated in 2 cases of early series, but technical modifications later were able to avoid it. Angiogram showed smooth flow into SRGA without upward and anterior tilting of the pulmonary bifurcation. All great and coronary arteries were patent. All were doing well on follow-up (16.5 +/- 4.2 months). CONCLUSIONS We concluded that the techniques to relocate the coronary arteries using common wall and in situ switch could also be applied to pulmonary arterial reconstruction, so that SRGA can be resumed in TGA.

Clinical features, acute complications, and outcome of Salmonella meningitis in children under one year of age in Taiwan

BackgroundSalmonella meningitis remains a threat to children below two years of age in both developing and developed countries. However, information on such infections has not been well characterized. We analyzed data related to twelve years of experience in order to clarify the comprehensive features of Salmonella meningitis in our patients, including admission characteristics, acute complications, and long-term outcome.MethodsThe records of patients with spontaneous Salmonella meningitis from 1982 to 1994 were retrospectively reviewed. The long-term outcome was prospectively determined for survivors at school age by the developmental milestones reported by their parents and detailed neurological evaluation along with intelligence, hearing, visual, speech and language assessments.ResultsOf the twenty-four patients, seizures were noted in fifteen (63%) before admission and thirteen (54%) during hospitalization. Acute complications mainly included hydrocephalus (50%), subdural collection (42%), cerebral infarction (33%), ventriculitis (25%), empyema (13%), intracranial abscess (8%), and cranial nerve palsy (8%). Three patients (13%) died during the acute phase of Salmonella meningitis. The twenty-one survivors, on whom we followed up at school age, have sequelae consisting of language disorder (52%), motor disability (48%), intelligence quotient < 80 (43%), epilepsy (33%), sensorineural hearing loss (17%), visual deficits (10%), abducens nerve palsy (5%), microcephaly (5%), and hydrocephalus (5%). Overall, good outcome was noted in six (28.6%) of twenty-one survivors, mild sequelae in three (14.2%), moderate in six (28.6%), and severe in six (28.6%).ConclusionSalmonella meningitis in neonates and infants had a wide spectrum of morbidity and acute complications, leading to a complicated hospital course and subsequently a high prevalence of permanent adverse outcome. Thus, early recognition of acute complications of Salmonella meningitis and a follow-up plan for early developmental assessment of survivors are vital.

Epidemiology of measles in Taiwan: dynamics of transmission and timeliness of reporting during an epidemic in 1988-9.

We analysed nation-wide reported measles cases during the 1988-9 epidemic and found that longer duration and wider spread were two major characteristics of the outbreak. All the 22 county/city index cases were reported with a delay of > 4 days and 64% were aged 5-14 years. This epidemic occurred mainly among 5-14-year-old school-children (59%), infants under 1 year (19%), and pre-school children (18%). The overall attack rate was 0.63 cases per 10,000 population, with the highest attack rate (7.4 cases per 10,000 population) occurring in infants. Among 280 confirmed cases < 15 months of age, 9-month-old infants (42 cases) had a higher risk of measles and peaked at 10 months (49 cases). This epidemic started in March 1988 among 5-9-year-old children in the northern suburban area, then spread to Taipei City and neighbouring counties or cities. It continued to spread from the northern to southern and western areas during the summer vacation and New Year holidays. Multiple logistic regression analysis showed that the delay of measles reporting was strongly associated with the cases infected early in the epidemic (OR = 6.9, P < 0.001) and reported from teaching hospitals (OR = 2.6, P < 0.001). The reappearance of high attack rates among 5-9-year-old children in the 1985 and 1988-9 measles epidemics in Taiwan indicated the persistence of pockets of susceptible individuals even after mass immunization.

A case of restrictive dermopathy with complete chorioamniotic membrane separation caused by a novel homozygous nonsense mutation in the ZMPSTE24 gene.

A Case of Restrictive Dermopathy With Complete Chorioamniotic Membrane Separation Caused by a Novel Homozygous Nonsense Mutation in the ZMPSTE24 Gene Ming Chen, Hsiang-Hsu Kuo, Yi-Chen Huang, Yu-Yuan Ke, Shun-Ping Chang, Chih-Ping Chen, Dong-Jay Lee, Meng-Luen Lee, Mei-Hui Lee, Tze-Ho Chen, Chia-Hsiang Chen, Hui-Mei Lin, Chin-San Liu, and Gwo-Chin Ma* Department of Genomic Medicine, Changhua Christian Hospital, Changhua, Taiwan Department of Medical Research, Changhua Christian Hospital, Changhua, Taiwan Department of Obstetrics and Gynecology, Changhua Christian Hospital, Changhua, Taiwan Department of Obstetrics and Gynecology, National Taiwan University Hospital, Taipei, Taiwan Department of Medical Genetics, National Taiwan University Hospital, Taipei, Taiwan Department of Obstetrics and Gynecology, Puli Christian Hospital, Nantou, Taiwan Department of Pediatrics, Puli Christian Hospital, Nantou, Taiwan Department of Pediatrics, Changhua Christian Hospital, Changhua, Taiwan Department of Obstetrics and Gynecology, Mackay Memorial Hospital, Taipei, Taiwan Department of Medical Research, Mackay Memorial Hospital, Taipei, Taiwan Department of Neurology, Vascular Biology and Genomics Center, Changhua Christian Hospital, Changhua, Taiwan Institute of Biochemistry and Biotechnology, Chung-Shan Medical University, Taichung, Taiwan

Restoring the spiral flow of nature in transposed great arteries

OBJECTIVES The great arteries are in a non-spiral relationship in their transposition, even after arterial switch operation (ASO) with the Lecompte manoeuvre. We aim to restore the natural spiral great arteries and compare such ASO with conventional ASO with the Lecompte manoeuvre to clarify the functional implications of the spiral great arteries. METHODS The early survivors of ASO (n=130) were included in this retrospective study. In spiral ASO (n=48), the main pulmonary artery ran alongside the aorta and gave rise to its branches posteriorly. Patients who underwent non-spiral ASO with the manoeuvre (n=82) were compared. The survival, re-operation-free ratio and the anatomic details for re-intervention after ASO were reviewed. RESULTS Average follow-up was 5.6 + or - 3.4 years (range: 2 months to 11 years). The estimated 10-year survival was similar (92.6% spiral vs 92.1% non-spiral, respectively). Significant pulmonary stenosis (PS) (>40mmHg) was noted in five (10.4%) in the spiral group and seven patients (9.7%) in the non-spiral (p=NS). The re-operation-free survival at 10 years was not significantly different (87.4% vs 90.1%). The reasons for re-operation were subvalvular PS (n=3) and residual defect (n=2) in the spiral group, while supravalvular PS (n=3), neo-aortic regurgitation (n=1) and aortic neo-coarctation (n=2) in the non-spiral group. Supravalvular PS and aortic neo-coarctation that occurred in the non-spiral group were not seen after spiral ASO. CONCLUSIONS Intermediate-term results of spiral ASO were satisfactory. Transposition is not a mere reversal of the great arteries; therefore, recognition of non-spiral relationship should be appreciated. Spiral reconstruction would be beneficial to reduce supravalvular PS and neo-aortic kinking. Further recognition of spiral function and refined modification might justify the application of spiral ASO in the future.

A new embryologic linkage between chromosome 22q11 deletion and a right ductus from a right aortic arch in a neonate with DiGeorge syndrome

a , b c d e * Meng-Luen Lee , Wun-Tsong Chaou , Yu-Mei Wang , Williams Fang , Ing-Sh Chiu Department of Pediatrics, Division of Pediatric Cardiology, Changhua Christian Hospital, Changhua 50050, Taiwan Division of Pediatric Neurology, Changhua Christian Hospital, Changhua 50050, Taiwan Pediatric Endocrinology and Metabolism, Changhua Christian Hospital, Changhua 50050, Taiwan Laboratory of Medical Genetics, Changhua Christian Hospital, Changhua 50050, Taiwan Department of Surgery, Division of Cardiovascular Surgery, College of Medicine, National Taiwan University Hospital, Taipei 10002, Taiwan

Exchange Transfusion Using Peripheral Vessels Is Safe and Effective in Newborn Infants

OBJECTIVES. The purpose of this study was to compare the efficiency and safety of exchange transfusion by using peripheral arteries and veins with that of conventional exchange transfusion via the umbilical vein in treating neonatal pathologic hyperbilirubinemia. PATIENTS AND METHODS. We retrospectively reviewed the medical charts of all neonates who had undergone exchange transfusion at our institution from January 1995 to December 2006. Causes of jaundice, efficiency of exchange transfusion in lowering serum bilirubin concentrations, adverse events, and outcomes were recorded. Data were compared between neonates who had undergone exchange transfusion via the peripheral arteries and veins method and those who had undergone exchange transfusion via the umbilical vein method. Data were also compared between stable neonates (body weight > 1500 g without medical problems other than jaundice) and unstable neonates. RESULTS. A total of 123 exchange-transfusion procedures were performed in 102 neonates in the 12-year study period: 24 were performed via the umbilical vein method and 99 via the peripheral vessels method. A total of 87 procedures were performed in 75 stable neonates and 36 in 27 unstable neonates. There was no significant difference in reduction of serum bilirubin level from circulation or the duration of procedures between the 2 methods. Eight neonates died before discharge, but none of the deaths seem to have been attributable to the exchange-transfusion procedure. Severe adverse events occurred more commonly in the umbilical vein group than the peripheral arteries and veins group in the stable neonates. All of the severe and minor events resolved completely without noticeable sequelae before discharge. CONCLUSIONS. Exchange transfusion using peripheral arteries and veins is efficient and effective in reducing serum bilirubin from circulation and is associated with few adverse events. This method should be considered for all neonates requiring exchange transfusion for treatment of neonatal hyperbilirubinemia.

Late-onset Enterobacter cloacae sepsis in very-low-birth-weight neonates: experience in a medical center.

OBJECTIVE The objective of this study was to review the early clinical profiles and outcomes of very-low-birth-weight (VLBW) neonates with late-onset sepsis caused by Enterobacter cloacae. METHODS We reviewed the medical records of VLBW neonates whose blood and/or cerebral spinal fluid yielded E. cloacae after 3 days of hospitalization in our neonatal intensive care unit. RESULTS From January 1997 to December 2006, a total of 29 episodes of E. cloacae infection occurred in 28 VLBW neonates. The onset of E. cloacae infection ranged from 4 to 70 days (27.4 +/- 9.6) days after birth. The most common symptoms and signs of sepsis were desaturation (58.6%), tachycardia (58.6%), apnea (55.2%), unstable body temperature (48.3%), and decreased activity (44.8%). The infected neonates had an average of four kinds of symptoms in each sepsis episode. The most common laboratory findings in VLBW neonates with E. cloacae sepsis were thrombocytopenia (65.5%), C-reactive protein > or = 1 mg/dL (55.2%), band-form neutrophils > or = 5% (41.4%), and leucopoenia (20.7%). Empirical intravenous antibiotic therapy comprising piperacillin (or piperacillin and tazobactam) and gentamicin (or amikacin) was successful in the early treatment of 24 episodes of E. cloacae sepsis in 25 patients. Three neonates (10.7%) died due to E. cloacae sepsis. Four neonates (14.3%) developed E. cloacae meningitis, and two of them developed brain abscesses. CONCLUSIONS E. cloacae infection in VLBW neonates usually presents with nonspecific symptoms and signs. Early recognition of sepsis and empirical combination of piperacillin (or piperacillin and tazobactam) and gentamicin (or amikacin) may be useful for treatment of sepsis caused by this highly virulent pathogen.

Persistent fifth aortic arch associated with 22q11.2 deletion syndrome.

BACKGROUND Chromosome 22q11.2 deletion is frequently associated with conotruncal malformations and aortic arch anomalies. This study investigated the association of chromosome 22q11.2 deletion with clinical manifestations in four pediatric patients with persistent fifth aortic arch. METHODS Four patients with persistent fifth aortic arch treated between July 1997 and June 2004 were included in this retrospective study. There were two girls and two boys, aged 2 days to 11.3 years, with persistent fifth aortic arch and cardiac conotruncal malformations. Chart recordings, plain chest films, two-dimensional and Doppler echocardiograms, cardiac catheterization with angiograms, surgical findings, and cytogenetic study were analyzed. RESULTS Clinically, all four patients had the cardinal phenotypic features of 22q11.2 deletion syndrome, including cardiovascular malformations (conotruncal malformations and aortic arch anomalies), abnormal facies, thymic hypoplasia, canopy anomaly of the palate (high-arched palate, rather than cleft palate), and hypocalcemia (or hypoparathyroidism). All four patients were confirmed to have chromosome 22q11.2 deletion. CONCLUSION Congenital conotruncal malformations, including tetralogy of Fallot with pulmonary atresia or stenosis, and aortic arch anomalies including a persistent fifth aortic arch or a right aortic arch, should lead to suspicion of chromosome 22q11.2 deletion when manifested together with any one of the other four cardinal phenotypic features.

Transarterial occlusion of patent ductus arteriosus with Gianturco coils in pediatric patients: a preliminary result in central Taiwan

OBJECTIVE We wish to present the preliminary result of transarterial occlusion of patent ductus arteriosus (PDA) with Gianturco coils in pediatric patients in central Taiwan. MATERIALS AND METHODS We attempted occlusion of PDA with Gianturco coils in a total of 26 consecutive patients, 13 infants and 13 children, 23 female and three male, between July 1 1997 to September 30 1998. Median patient age was 2.57 years (from 0.25 to 14.02 years old). Median patient weight was 10.8 kg (4.0 to 36.0 kg). Premature babies with PDA, full-term babies who were less than three months old and patients who had other congenital heart disease were not included in this study. All PDAs were approached transarterially from the femoral artery. Coils were selected to provide a helical diameter that was twice or more the minimum ductus diameter and a length approximating five loops. In five patients who had a PDA diameter > or =3.5 mm, we used a snare technique to assist coil delivery beforehand, and to test coil stability, or to retrieve coil that had migrated to the pulmonary artery afterwards. Physical auscultation, chest radiographs and echocardiography with color Doppler were done in all patients within 24 h, and one, two, three, six and 12 months after coil occlusion. RESULTS The median ductus minimum diameter was 2.3 mm (range, 1.0 to 4.7 mm). Fifteen patients had the megaphone type (type A), four had the window type (type B), five had the tubular type (type C), one had the aneurysmal type (type D) and one had the elongated conical type (type E). Twenty-one patients underwent single coil occlusion and five had multiple coils occlusion. Twenty-one patients had immediate angiographic closure of the ductus and disappearance of heart murmur at 15 min after the procedure. Dark-brown urine (hemoglobinuria) was found in one patient, 10 h after the first procedure, due to a mild residual ductal shunt. Two more coils were implanted in a second procedure that was performed within 24 h, and the ductus was completely occluded. The dark-brown urine regressed. At one month follow-up, four patients had mild residual ductal shunts, which were completely occluded by one more coil in three patients and by two more coils in the other patient. Malpositioned coils were deployed in five patients immediately after the procedure. In total, the closure rate at 15 min, within 24 h, and at one, two, three, six and 12 months were 81, 85, 85, 100, 100, 100 and 100%, respectively. In one year of follow-up, there was no instance of coil migration, ductus reopening or stenosis of the left pulmonary artery. CONCLUSIONS Transarterial occlusion of PDA, with a Gianturco coil having approximately five loops, can be effectively achieved in patients with a minimum ductus diameter up to 4.7 mm. In patients with a ductus of more than 3.5 mm, the snare-assisted technique was employed advantageously to control coil delivery with accuracy and stability. Coil malposition or migration can be easily retrieved using a 10-mm Nitnol snare catheter. Hemoglobinuria, due to intravascular hemolysis, may regress within 24 h after the second attempt at coil implantation.