Skull Base Fracture or Abuse Suspicion Based on Intraorbital Lymphangioma Secondary to Periorbital Hematoma.

Intraorbital lymphangiomas are among the orbital tumors that can cause sudden eye protrusion in children. In children with periorbital hematoma (panda eye sign), a skull fracture or abuse is likely first considered as the differential diagnosis. A 7-month-old boy presented to the ophthalmologist with complaints of swelling of the right upper eyelid, subconjunctival hemorrhage on the right ear side, and periorbital subcutaneous hemorrhage, which had appeared since the morning of the day before the visit. The eyeball did not protrude. Based on the interview and clinical findings, right eyeball contusion was suspected. The patient was then followed up for observation. Later, during the physical examination, the abovementioned symptoms were noted. Hence, the patient was admitted for a close examination based on the suspicion of skull base fracture and abuse. Contrast-enhanced magnetic resonance imaging (MRI) after admission revealed a multifocal cystic structure within the right intraorbital muscular cone. Thus, he was diagnosed with right intraorbital lymphangioma. Intraorbital lymphangioma may not show ocular protrusion, and this disease should be considered in cases where abuse is suspected, considering the periorbital subcutaneous hemorrhage.

Clinical application of a lung sound analysis in infants with respiratory syncytial virus acute bronchiolitis.

BACKGROUND: Objective investigation of the characteristics of acute bronchiolitis in infants is important for its diagnosis and treatment. METHODS: Lung sound data of 50 patients diagnosed with respiratory syncytial virus (RSV) acute bronchiolitis (m:f = 29:21, median of age 7 months), 20 patients with RSV acute respiratory tract infections without acute bronchiolitis (m:f = 10:10, 5 months) and 38 age-matched control infants (m:f = 23:15, 8 months) were analyzed using a conventional method and compared. Furthermore, the relationships between lung sound parameters and clinical symptoms (clinical score, length of hospital stay and SpO2 level) in the bronchiolitis and the non-bronchiolitis patients were examined. RESULTS: Results of lung sound analysis showed that the inspiratory sound power of patients with RSV respiratory tract infections was low and the expiratory sound power was high compared with those of the controls. When the patients with RSV respiratory tract infections were divided into the bronchiolitis and non-bronchiolitis groups, the expiratory/inspiratory ratio of the bronchiolitis patients was greater than that of the non-bronchiolitis patients. There was no difference in the clinical symptoms, clinical score and length of hospital stay between the bronchiolitis and non-bronchiolitis patients, except for the SpO2 level on admission. CONCLUSION: Lung sound analysis confirmed that patients with RSV acute bronchiolitis present with marked airway narrowing. Considering these results as a characteristic of acute bronchiolitis, it would be meaningful to reflect it in the improvement of diagnosis, treatment and subsequent management.

Effect of wheeze and lung function on lung sound parameters in children with asthma.

BACKGROUND: In children with asthma, there are many cases in which wheeze is confirmed by auscultation with a normal lung function, or in which the lung function is decreased without wheeze. Using an objective lung sound analysis, we examined the effect of wheeze and the lung function on lung sound parameters in children with asthma. METHODS: A total of 114 children with asthma (males to females = 80: 34, median age 10 years old) were analyzed for their lung sound parameters using conventional methods, and wheeze and the lung function were checked. The effects of wheeze and the lung function on lung sound parameters were examined. RESULTS: The patients with wheeze or decreased forced expiratory flow and volume in 1 s (FEV1) (% pred) showed a significantly higher sound power of respiration and expiration-to-inspiration sound power ratio (E/I) than those without wheeze and a normal FEV1 (% pred). There was no marked difference in the sound power of respiration or E/I between the patients without wheeze and a decreased FEV1 (% pred) and the patients with wheeze and a normal FEV1 (% pred). CONCLUSIONS: Our data suggest that bronchial constriction in the asthmatic children with wheeze similarly exists in the asthmatic children with a decreased lung function. A lung sound analysis is likely to enable an accurate understanding of airway conditions.

A Boy Presenting with a Fever and Pancytopenia Diagnosed with Systemic Lupus Erythematosus without a Positive Anti-ds-DNA Antibody Result or Hypocomplementemia: A Case Report.

BACKGROUND: Hematological involvement, including anemia, leukopenia, lymphopenia, and thrombocytopenia, is one of the most common manifestations of childhood-onset systemic lupus erythematosus (cSLE). Specifically, relatively severe forms of hematological involvement, such as macrophage activation syndrome (MAS) and thrombotic microangiopathy, occur in the course of the disease. Positivity for anti-double stranded-DNA (ds-DNA) antibody and hypocomplementemia are important as not only criteria of diagnosing cSLE but also in the determination of the disease activity. CASE REPORT: A 13-year-old boy without pre-existing disease was referred to our hospital chiefly complaining of a fever for > 7 days, long-lasting malaise, nausea, and non-malar face rash. His blood examination showed pancytopenia and hyperferritinemia, but positive results for anti-ds-DNA antibody and hypocomplementemia were not recognized. Bone marrow aspiration revealed no evidence of malignant diseases, hemophagocytic lymphohistiocytosis, or MAS. A renal biopsy for the differential diagnosis of proteinuria and hematuria revealed class IIIa +V lupus nephritis, leading to the diagnosis of cSLE. CONCLUSIONS: It is important for cSLE to be considered in patients with pancytopenia, even those without positive anti-ds-DNA antibody findings or hypocomplementemia, and for aggressive approaches to be adopted for the differential diagnosis, including a renal biopsy.

Breath sound analyses of infants with respiratory syncytial virus acute bronchiolitis.

INTRODUCTION: The reliability of a breath sound analysis using an objective method in infants has been reported. OBJECTIVE: Breath sounds of infants with respiratory syncytial virus (RSV) acute bronchiolitis were analyzed via a breath sound spectrogram to evaluate their characteristics and examine their relationship with the severity. SUBJECTS AND METHODS: We evaluated the inspiratory and expiratory breath sound parameters of 33 infants diagnosed with RSV acute bronchiolitis. The sound powers of inspiration and expiration were evaluated at the acute phase and recovery phase of infection. Furthermore, the relationship between the breath sound parameters and the clinical severity of acute bronchiolitis was examined. RESULTS: Analyses of the breath sound spectrogram showed that the power of expiration as well as the expiration-to-inspiration sound ratio in the mid-frequency (E/I MF) was increased in the acute phase and decreased during the recovery phase. The E/I MF was inversely correlated with the SpO2 and positively correlated with the severity score. CONCLUSION: In infants with RSV acute bronchiolitis, the sound power of respiration was large at the acute phase, significantly decreasing in the recovery phase. In 61% of participants, nonuniform, granular bands were shown in the low-pitched region of the expiratory spectrogram.

Anti-cyclic Citrullinated Peptide Antibody-positive Girl with Chronic Polyarthritis Later Diagnosed with Juvenile Systemic Lupus Erythematosus: A Case Report.

BACKGROUND: Arthritis is one of the earliest symptoms of juvenile systemic lupus erythematosus (SLE) but is unusual in cases presenting with chronic arthritis or deforming/erosive arthritis. Overlap of juvenile idiopathic arthritis (JIA) and juvenile SLE is a rare clinical condition known as "rhupus" syndrome. The clinical and serological characteristics of rhupus syndrome in children remain to be established. In addition, no studies regarding anti-cyclic citrullinated peptide (CCP) antibody in juvenile SLE or juvenile rhupus syndrome have been reported. CASE REPORT: A 12-year-old girl suffered from polyarthralgia lasting for one week. She was tentatively diagnosed with polyarticular JIA because of her symptom of chronic arthritis and a positive result for anti-CCP antibody. After six months of follow-up for JIA, she presented with a fever, malar rash, and worsening of arthralgia. Laboratory examinations revealed hypocomplementemia and a positive result for anti-double-stranded DNA antibody. She was diagnosed with juvenile SLE. CONCLUSIONS: It is important to note that patients with chronic arthritis, as well as those with anti-CCP antibody-positive polyarthritis, should be carefully followed for their clinical and serological condition, considering the possibility of them developing juvenile SLE.

A Family Case of COVID-19 Pneumonia with Different Chest CT Features and Duration of SARS-CoV-2 Shedding: a Case Report from Japan.

Coronavirus disease 2019 (COVID-19) pneumonia in children characteristically has a milder clinical presentation, with milder inflammatory biomarkers and radiological findings. Accumulating evidence indicates a difference in chest computed tomography (CT) features and the duration of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) shedding between children and adults. Here, we report a family case of COVID-19 pneumonia in which 2 brothers (aged 14 years and 2 years) had different findings. On admission, the 2-year-old brother had few symptoms with no signs of pneumonia, whereas the older brother had presented with pneumonia on admission. Both were positive for SARS-CoV-2 infection on polymerase chain reaction and showed obvious characteristic signs of COVID-19 pneumonia on chest CT. However, CT findings in the younger brother were nonspecific and similar to those of other pneumonias. The older brother required longer treatment because of the longer shedding period of SARS-CoV-2 detected in nasopharyngeal samples. Both boys were discharged without complications. This family case suggests that the clinical features of COVID-19 pneumonia might differ between younger and older children.

Associations of complementation group, ALDH2 genotype, and clonal abnormalities with hematological outcome in Japanese patients with Fanconi anemia.

Fanconi anemia (FA) is a genetically and clinically heterogeneous disorder that predisposes patients to bone marrow failure (BMF), myelodysplastic syndromes (MDS), and acute myeloid leukemia (AML). To study which genetic and phenotypic factors predict clinical outcomes for Japanese FA patients, we examined the FA genes, bone marrow karyotype, and aldehyde dehydrogenase-2 (ALDH2) genotype; variants of which are associated with accelerated progression of BMF in FA. In 88 patients, we found morphologic MDS/AML in 33 patients, including refractory cytopenia in 16, refractory anemia with excess blasts (RAEB) in 7, and AML in 10. The major mutated FA genes observed in this study were FANCA (n = 52) and FANCG (n = 23). The distribution of the ALDH2 variant alleles did not differ significantly between patients with mutations in FANCA and FANCG. However, patients with FANCG mutations had inferior BMF-free survival and received hematopoietic stem cell transplantation (HSCT) at a younger age than those with FANCA mutations. In FANCA, patients with the c.2546delC mutation (n = 24) related to poorer MDS/AML-free survival and a younger age at HSCT than those without this mutation. All patients with RAEB/AML had an abnormal karyotype and poorer prognosis after HSCT; specifically, the presence of a structurally complex karyotype with a monosomy (n = 6) was associated with dismal prognosis. In conclusion, the best practice for a clinician may be to integrate the morphological, cytogenetic, and genetic data to optimize HSCT timing in Japanese FA patients.

Hematopoietic stem cell transplantation for inborn errors of metabolism: A report from the Research Committee on Transplantation for Inborn Errors of Metabolism of the Japanese Ministry of Health, Labour and Welfare and the Working Group of the Japan Society for Hematopoietic Cell Transplantation.

A total of 216 patients with IEM were treated by allogeneic HSCT in Japan from 1985 until 2010. The results of UCBT have improved, and the OS rate of UCBT (81.9%) was not different from those of RBMT (87.2%) or UBMT (73.9%) in 2000-2010. However, EFS rates in RBMT (73.2%) and UBMT (62.2%) were better than that in UCBT (49.5%), and the difference between RBMT and UCBT was significant (p = 0.01). The EFS rate of patients conditioned by RIC (74.6%) was comparable or slightly better than in those who underwent MAC with irradiation (57.9%) or without irradiation (54.2%) in 2000-2010. A more pronounced trend was observed toward differential EFS for UCBT in 2000-2010: RIC (62.9%), MAC with irradiation (20.0%), and MAC without irradiation (42.1%). The difference between RIC and MAC with irradiation was significant (p < 0.03). In summary, we report a Japanese registry analysis of HSCT for IEM with improving survival in UCBT. The introduction of RIC after 2000 was considered to contribute to this improvement. UCBT could be recommended for those who lack an HLA-identical sibling donor.

Aortic valve repair with autologous pericardium for traumatic aortic valve regurgitation.

We present a case of successful aortic valve repair for traumatic aortic valve regurgitation. A 26-year-old male who had a history of motor-cycle accident months prior to admission, was referred to our hospital for surgical treatment of severe aortic valve regurgitation. Intraoperative inspection revealed a tear in noncoronary cusp, with otherwise preserved valvular anatomy. Aortic valvuloplasty was successfully performed with closure using an autologous pericardium patch. Intraoperative transesophageal echocardiogram confirmed absence of residual regurgitation.

Early diagnosis of asparaginase-associated pancreatitis based on elevated serum elastase-1 levels: Case reports.

L-asparaginase (L-asp) is a well-known anticancer agent used in the treatment of acute lymphoblastic leukemia (ALL) in children. However, it is also known to induce several acute complications, such as acute pancreatitis. This is a presentation of two pediatric acute lymphoblastic leukemia (ALL) cases of asparaginase-associated pancreatitis (AAP) diagnosed at an early stage based on elevated serum elastase-1 levels, in the presence of normal serum amylase levels. Early diagnosis and treatment of AAP, although imperative, is occasionally difficult if only standard diagnostic procedures are followed. Elastase-1 is a potentially useful marker for the early diagnosis of AAP. Therefore, the measurement of elastase-1 levels, in addition to amylase and lipase levels, is recommended in L-asp-treated patients.

Long-term efficacy of hematopoietic stem cell transplantation on brain involvement in patients with mucopolysaccharidosis type II: a nationwide survey in Japan.

Hematopoietic stem cell transplantation (HSCT) has not been indicated for patients with mucopolysaccharidosis II (MPS II, Hunter syndrome), while it is indicated for mucopolysaccharidosis I (MPS I) patients <2 years of age and an intelligence quotient (IQ) of ≥ 70. Even after the approval of enzyme replacement therapy for both of MPS I and II, HSCT is still indicated for patients with MPS I severe form (Hurler syndrome). To evaluate the efficacy and benefit of HSCT in MPS II patients, we carried out a nationwide retrospective study in Japan. Activities of daily living (ADL), IQ, brain magnetic resonance image (MRI) lesions, cardiac valvular regurgitation, and urinary glycosaminoglycan (GAG) were analyzed at baseline and at the most recent visit. We also performed a questionnaire analysis about ADL for an HSCT-treated cohort and an untreated cohort (natural history). Records of 21 patients were collected from eight hospitals. The follow-up period in the retrospective study was 9.6 ± 3.5 years. ADL was maintained around baseline levels. Cribriform changes and ventricular dilatation on brain MRI were improved in 9/17 and 4/17 patients, respectively. Stabilization of brain atrophy was shown in 11/17 patients. Cardiac valvular regurgitation was diminished in 20/63 valves. Urinary GAG concentration was remarkably lower in HSCT-treated patients than age-matched untreated patients. In the questionnaire analysis, speech deterioration was observed in 12/19 patients in the untreated cohort and 1/7 patient in HSCT-treated cohort. HSCT showed effectiveness towards brain or heart involvement, when performed before signs of brain atrophy or valvular regurgitation appear. We consider HSCT is worthwhile in early stages of the disease for patients with MPS II.

Matched sibling donor stem cell transplantation for Fanconi anemia patients with T-cell somatic mosaicism.

SCT from HLA-identical sibling donors is generally associated with an excellent survival in FA patients if performed prior to the development of MDS or leukemia. However, the optimal conditioning regimen has not been defined. We report here our experience with 15 Japanese FA patients who underwent HLA-matched sibling donor SCT. The aim of this study is to compare radiation-based conditioning to Flu-based conditioning for FA patients in a Japanese population where the T-cell somatic mosaicism is higher than in the Caucasian population. Eight patients (a-group) received a radiation-based conditioning (500-600 cGy of thoracoabdominal/TBI) with CY dose modification (20-120 mg/kg), and ATG; two patients exhibited rejection. Seven patients (b-group) received CY (40 mg/kg), 150-180 mg/m(2) of Flu, and ATG. Durable engraftment was demonstrated in all patients. In FA patients, Flu-based conditioning may allow stable engraftment in matched sibling donor transplantation without radiation, even in patients with T-cell somatic mosaicism.

Decreased serum testosterone levels in long-term adult survivors with fatty liver after childhood stem cell transplantation.

Fatty liver and male gonadal dysfunction are potential late effects of therapy in adult survivors treated with stem cell transplantation (SCT) in childhood. Obesity and metabolic syndrome also are associated with low serum testosterone levels in the general population. However, the relationship between the degree of fatty liver and changes in serum testosterone levels in adult survivors has not been fully studied. We reviewed the clinical records of 34 male patients who received allogeneic SCT in childhood or adolescence. The median age at SCT was 10.0 years, and the median follow-up after SCT was 15.9 years. All but one patient showed no tendency toward overweight/obesity during the follow-up period. Fatty liver was diagnosed by ultrasound in 15 patients at 4 to 20 years after SCT. Patients who received cranial radiation therapy before SCT were more likely to develop fatty liver and insulin resistance. Moreover, fatty liver was statistically associated with decreased serum testosterone levels, whereas nonfatty liver was not (median, 527 ng/dL [range, 168-944 ng/dL] versus 302 ng/dL [165-698 ng/dL]; P < .0001). Changes in testosterone levels after SCT are affected not only by primary gonadal dysfunction but also by subsequent development or exacerbation of fatty liver.

High incidence of radiation-induced cavernous hemangioma in long-term survivors who underwent hematopoietic stem cell transplantation with radiation therapy during childhood or adolescence.

Radiation-induced cavernous hemangioma (RICH) is a late complication of cerebral radiation therapy. Long-term survivors of hematopoietic stem cell transplantation (HSCT) who underwent radiation therapy could be at increased risk for RICH. We investigated records of 68 patients who underwent HSCT during childhood or adolescence and were assessed by magnetic resonance imaging (MRI), including T2*-weighted imaging of the brain, annually for 5 years over a range of 6 to 29 years after HSCT. We developed a scoring and grading system for RICH to monitor the process and the progress of radiologic changes. Among the 68 patients investigated, 28 (41.2%) were diagnosed with CH. All 28 patients had received total body irradiation as a conditioning treatment for HSCT and/or cranial radiation therapy before HSCT as part of the treatment of their primary disease. RICH was diagnosed in none of the patients who did not receive radiation (n = 19), in 46.2% of those who received 6 to 12 Gy (n = 39), and in all of those who received 18 to 36 Gy (n = 10). Total RICH scores were correlated with higher radiation doses. Careful and long-term evaluation with MRI, including T2*-weighted imaging, is necessary for HSCT recipients who received radiation therapy before and/or during HSCT.

Cryosurgical left-sided maze procedure in patients with valvular heart disease: medium-term results.

PURPOSE: We evaluated medium-term results of the left-sided maze procedure using cryoablation in patients with valvular heart disease. METHODS: We retrospectively evaluated 111 patients with valvular heart disease who underwent the cryosurgical left-sided maze procedure. The mean follow-up period was 36.8 ± 24.9 months, and the mean duration of atrial fibrillation was 5.6 ± 6.0 years. The primary surgical procedure was mitral valve replacement in 42 patients, mitral valve plasty in 28, aortic valve replacement in 25, and combined aortic and mitral replacement or plasty in 16. RESULTS: The 7-year actuarial survival rate was 82.9 ± 11.4% for patients in sinus rhythm and 87.0 ± 7.0% for patients with atrial fibrillation, showing no difference between the two groups (p = 0.236). At final follow-up, 86 out of 111 patients (77.5%) remained free from atrial fibrillation. Sinus rhythm was maintained in 26 of 42 patients (61.9%) in the mitral valve replacement group, 26 of 28 patients (92.9%) in the mitral valve plasty group, 15 of 17 patients (88.2%) in the aortic valve replacement group, and 18 of 24 patients (75.0%) in the combined aortic and mitral replacement or plasty group. The overall actuarial rate of freedom from atrial fibrillation at 5 years after surgery was 70.4 ± 6.0%. CONCLUSION: The cryosurgical left-sided maze procedure is a safe, simple, and excellent operation for medically refractory atrial fibrillation.

Electron beam cine CT-based evaluation of left atrial function after the maze procedure for mitral valve regurgitation.

OBJECTIVE: There has been little investigation of whether atrial function is equally restored by surgery in patients with mitral regurgitation (MR) and atrial fibrillation (AF) compared to patients in sinus rhythm (SR). PATIENTS AND METHODS: We evaluated left atrium (LA) volume-time curves obtained from electron beam tomography. The study involved 33 patients who had surgical treatment for MR with or without AF and 11 control patients. RESULTS: (1) In patients with SR, LA volume decreased significantly along with a resolution of early regurgitation postoperatively, and the reserve function was well maintained. The LA booster pump function was also well maintained before and after surgery. (2) In patients with AF that resolved after maze surgery, the LA volume was larger than that of the sinus group immediately after surgery, and it did not improve in postoperative periods. These patients had a lower reserve function and a much lower booster pump function despite restoration of SR. CONCLUSIONS: The maze procedure is suggested to be unlikely to achieve restoration of atrial function in patients with MR accompanied by AF, even if SR returns postoperatively. Because patients with SR demonstrated the same LA function as the control postoperatively, surgical indication should be considered for patients with severe MR while their atrial function and SR are maintained.

Renovascular hypertension due to bilateral renal artery stenosis treated with stent implantation in a 12-year old girl.

Renovascular hypertension (RVH) in children is a relatively rare disease, but it is important in that it is a treatable condition when properly diagnosed. Percutaneous transluminal renal angioplasty (PTRA) with or without stenting is widely applied to adult patients with RVH. However, limited information is available as to PTRA with stenting in pediatric patients. We experienced a case of RVH in a 12-year-old girl, who had severe hypertension (180/110 mmHg). Bilateral renal artery stenosis was demonstrated by 3D-CT, MR angiography and selective renal arteriography. Renal function and plasma renin activity were normal. Angiotensin blockade was refrained for fear of functional deterioration of the kidney. Medical treatment with amlodipine insufficiently lowered the pressure to 140-160/80-100 mmHg, so we performed PTRA. Stenotic lesion and pressure gradient was still present after balloon angioplasty on both sides, prompting us to place LUMINEXX® stents on both renal arteries. Blood pressure dropped dramatically after the intervention. Amlodipine was discontinued, and then, enalapril and warfarin were administered to prevent neointima and thrombus formation. Her blood pressure and renal function was stable 18 months after PTRA. Oversized self-expanding stent such as LUMINEXX® stent could be used for renal artery stenting even in pediatric patients with RVH.

Patient-prosthesis mismatch may be irrelevant after aortic valve replacement with the 19-mm Perimount pericardial bioprosthesis in patients aged 65 years or older.

The prevalence of patient-prosthesis mismatch (PPM) and its influence on clinical midterm results were examined in elderly patients whose activity was supposed to be less than that of younger patients. We evaluated valve function and the effects of PPM on the midterm results of the 19-mm Carpentier-Edwards Perimount (CEP) pericardial aortic valve in patients aged 65 years or older. Between August 1996 and May 2005, 51 patients underwent aortic valve replacement with the 19-mm CEP valve. The mean follow-up was 2.4 +/- 1.8 years, involving a total of 134.4 patient-years. The mean age and body surface area at operation were 74.0 +/- 5.0 years and 1.41 +/- 0.14 m(2). There were two (3.9%) operative deaths. Three patients (5.9%) underwent enlargement of their small aortic annuli. The actuarial survival rate at 8 years, including operative mortality, averaged 90.2% +/- 4.7%. The freedom from thromboembolism, reoperation, and valve-related mortality averaged 75.0% +/- 21.7%, 97.8% +/- 2.2%, and 95.3% +/- 3.2%, respectively, at 8 years. High preoperative peak and mean transvalvular pressure gradients were significantly improved after the operation (peak, 93 +/- 35 versus 28 +/- 12 mmHg; mean, 58 +/- 19 versus 17 +/- 7 mmHg, respectively; P < 0.01). The mean left ventricular mass index was reduced from 192 +/- 44 to 142 +/- 46 g/m(2) at late follow-up (P < 0.01). The prevalence of PPM was low (17.6%) when an indexed effective orifice area of less than 0.85 cm(2)/m(2) was taken as the definition of PPM. The clinical results, postoperative pressure gradients, and reduction in left ventricular mass index were not different between the PPM and no-PPM groups. The 19-mm CEP valve produced satisfactory midterm clinical outcomes in patients aged 65 years or older whose activity was supposed to be less than that of younger patients, regardless of the presence or absence of PPM. Moderate PPM was rare and it did not adversely impact on the midterm results. The application of annulus enlargement could be limited to the small number of patients for whom the 19-mm CEP valves are not able to be inserted.

Prevalence and avoidance of patient-prosthesis mismatch in aortic valve replacement in small adults.

BACKGROUND: It is still controversial as to whether a small prosthesis should be inserted or a small aortic annulus should be enlarged to minimize patient-prosthesis mismatch (PPM). This retrospective study reviewed our strategy for avoiding PPM. METHODS: Isolated or combined aortic valve replacement was performed in 181 patients, including 24 patients (13.3%) aged less than 65 years with a small aortic annulus (< or = 21 mm) who underwent enlargement of the annulus by the Manouguian (n = 18) or Nicks (n = 6) procedure. In patients aged 65 years or more, a Carpentier-Edwards Perimount pericardial (CEP) valve was implanted with few exceptions. We assessed our strategy for avoiding PPM by comparison with published normal reference values for the indexed effective orifice area. RESULTS: A CEP valve was implanted in 53 patients, and St. Jude Medical (SJM) mechanical valves were used in 128 patients. A standard 21-mm SJM valve was only used in 4 patients and no 19-mm valves were employed. However, 19-mm CEP valves were used in 12 older patients with a small body surface area (1.43 +/- 0.14 m2). No patient receiving an SJM valve had an indexed effective orifice area of 0.85 cm2/m2 or less, and PPM developed in only 2 (3.8%) of 53 patients receiving CEP valves. Consequently, the prevalence of PPM was 1.1%. The 10-year survival rates of patients receiving CEP or SJM valves with or without annular enlargement were similar. CONCLUSIONS: The prevalence of PPM was low in patients more than 65 years old with a relatively small body size who received bioprosthetic valves. In patients less than 65 years old with a small annulus, the method of first choice for avoiding PPM is aortic annular enlargement.