2017年 · 第130卷第21期

Scoliosis is a three-dimensional (3D) spinal deformity involving one or more spine curvatures with vertebral rotation. In the past 30 years, Chinese spine surgeons adopted international advanced technology and concepts, took advantage of abundant Chinese patient resources with severe deformity, and developed novel ideas and techniques, which promoted the development of the diagnosis and treatment for scoliosis.

With the increasing frequency of human social activity, the amount of data generated daily is also increasing exponentially. The recording medium of information has changed from the oldest carved stone to the current semiconductor carrier, which also pushes forward the progress of society. This also moves society into the big data era (BDE), in which all things can be data. BD has helped people make many breakthroughs in political decision-making, economic policy, and more, and thus affects the human knowledge system and way of life.^[1]The choice is no longer dependent on subjective judgment using imagination, but a large digital network in series consisting of habitual movement, physical movement, consumption, or medical record. In the medical field, many public databases are funded by the government and open to all researchers. BD for use in genomics, oncology, and proteomics provides resources and information that make healthcare and disease prevention more personalized and the digitalization of medicine unstoppable.^[2]The convergence of digital and medical is inevitable, and the current changes have appeared to be a good start.

Sacroiliac (SI) screw fixation is a demanding technique, with a high rate of screw malposition due to the complex pelvic anatomy. TiRobot™ is an orthopedic surgery robot which can be used for SI screw fixation. This study aimed to evaluate the accuracy of robot-assisted placement of SI screws compared with a freehand technique.

Thirty patients requiring posterior pelvic ring stabilization were randomized to receive freehand or robot-assisted SI screw fixation, between January 2016 and June 2016 at Beijing Jishuitan Hospital. Forty-five screws were placed at levels S1 and S2. In both methods, the primary end point screw position was assessed and classified using postoperative computed tomography. Fisher’s exact probability test was used to analyze the screws’ positions. Secondary end points, such as duration of trajectory planning, surgical time after reduction of the pelvis, insertion time for guide wire, number of guide wire attempts, and radiation exposure without pelvic reduction, were also assessed.

Twenty-three screws were placed in the robot-assisted group and 22 screws in the freehand group; no postoperative complications or revisions were reported. The excellent and good rate of screw placement was 100% in the robot-assisted group and 95% in the freehand group. The P value (0.009) showed the same superiority in screw distribution. The fluoroscopy time after pelvic reduction in the robot-assisted group was significantly shorter than that in the freehand group (median [Q₁, Q₃]: 6.0 [6.0, 9.0] s vs. median [Q₁, Q₃]: 36.0 [21.5, 48.0] s; χ² = 13.590, respectively, P < 0.001); no difference in operation time after reduction of the pelvis was noted (χ² = 1.990, P = 0.158). Time for guide wire insertion was significantly shorter for the robot-assisted group than that for the freehand group (median [Q₁, Q₃]: 2.0 [2.0, 2.7] min vs. median [Q₁, Q₃]: 19.0 [15.5, 45.0] min; χ² = 20.952, respectively, P < 0.001). The number of guide wire attempts in the robot-assisted group was significantly less than that in the freehand group (median [Q₁, Q₃]: 1.0 [1.0,1.0] time vs. median [Q₁, Q₃]: 7.0 [1.0, 9.0] times; χ² = 15.771, respectively, P < 0.001). The instrumented SI levels did not differ between both groups (from S1 to S2, χ² = 4.760, P = 0.093).

Accuracy of the robot-assisted technique was superior to that of the freehand technique. Robot-assisted navigation is safe for unstable posterior pelvic ring stabilization, especially in S1, but also in S2. SI screw insertion with robot-assisted navigation is clinically feasible.

Thoracolumbar junction (TLJ) is the transitional area between the lower thoracic spine and the upper lumbar spine. Vertebral compression fractures and proximal junctional kyphosis following spine surgery often occur in this area. Therefore, the study of development and mechanisms of thoracolumbar junctional degeneration is important for planning surgical management. This study aimed to review radiological parameters of thoracolumbar junctional degenerative kyphosis (TLJDK) in patients with lumbar degenerative kyphosis and to analyze compensatory mechanisms of sagittal balance.

From January 2016 to March 2017, patients with lumbar degenerative kyphosis were enrolled in this radiographic study. Patients were divided into two groups according to thoracolumbar junctional angle (TLJA): the non-TLJDK (NTLJDK) group (TLJA <10°) and the TLJDK group (TLJA≥10°). Complete spinopelvic radiographic parameters were analyzed and compared between two groups. Pearson or Spearman correlation coefficients and independent two-sample t-test or Mann-Whitney U-test were used.

A total of 77 patients with symptomatic sagittal imbalance due to lumbar degenerative kyphosis were enrolled in this study. There were 34 patients in NTLJDK group (TLJA <10°) and 43 patients in TLJDK group (TLJA ≥10°). The median angle of lumbar lordosis (LL) in the NTLJDK or TLJDK groups was 23.40° (18.50°, 29.48°) or 19.50° (13.30°, 24.55°), respectively. The median TLJAs in all patients and both groups were -11.20° (-14.60°, -4.80°), -3.70° (-7.53°, -1.73°), and -14.30° (-17.45°, -13.00°), respectively. In the NTLJDK group, LL was correlated with thoracic kyphosis (TK; r = -0.400, P = 0.019), sacral slope (SS; r = 0.681, P< 0.001), and C7-sagittal vertical axis (r = -0.402, P = 0.018). In the TLJDK group, LL was correlated with TK (r = -0.345, P = 0.024), SS (r = 0.595, P < 0.001), and pelvic tilt (r= -0.363, P = 0.017). There were significant differences in LL, TLJA, TK, SS, and pelvic incidence (PI) between two groups.

Although TLJDK is common in patients with lumbar degenerative kyphosis, it might be generated by special characteristics of morphology and biomechanics of the TLJ. To maintain sagittal balance, pelvis back tilt might be more important in patients with TLJDK, whereas thoracic curve changes might be more important in patients without TLJDK.

Intralesional excision with curettage is the standard method of giant cell tumor (GCT) treatment, but the ideal filling material after curettage remains controversial. The purpose of this study was to compare the oncological and functional outcomes which underwent cementation or bone grafting after GCT curettage around the knee.

We reported 136 cases with GCTs in distal femur or proximal tibia who accepted curettage from five clinical centers during the last 15 years. All patients were divided into two groups according to filling materials. Recurrence-free survival proportions were used to evaluate oncological outcomes while the Musculoskeletal Tumor Society (MSTS) 93 functional score was used to evaluate functional outcomes. Other parameters including surgical complication, general condition, and radiological classification had been analyzed. The valid statisitical data was analyzed using SPSS 13.0 software.

After GCT curettage, 86 patients (63.2%) accepted bone grafting while 50 patients (36.8%) accepted cementation. There was no statistical difference in age, gender, tumor location, radiological classification, fixation, follow-up time, and MSTS 93 functional score between cementation group and bone grafting group. The recurrence-free survival proportions showed that the recurrence rate in bone grafting group was higher than it in cementation group (P = 0.034). Surgical complication was lower in cementation group than that in bone grafting group but without statistically significant difference (P = 0.141).

Parameters including patients’ age, gender, tumor location, and radiological classification did not affect surgeons’ treatments in cavity filling after GCT curettage. Cementation should be recommended because of easy usage, the similar postoperative knee function with bone grafting, and the better local tumor control than bone grafting.

Wide resection margins of osseous tumors are associated with a low incidence of local recurrence, making accurate measurement of the intraosseous extent of primary malignant long bone tumors is crucial. We compared the intraosseous tumor extent assessed by magnetic resonance imaging (MRI) with the gross specimen to evaluate the accuracy of MRI.

A total of 255 patients with primary malignant tumors in the long bones were included. Using MRI, we defined the length of tumor as the distance from the articular surface to the boundary between abnormal and normal marrow signal. The extent of the abnormal intraosseous signal was measured on unenhanced T1-weighted (T1WI) magnetic resonance images after chemotherapy. All gross surgical specimens were sectioned, and tumor extent was measured. Wilcoxon signed-rank test was used to test the differences between MRI and gross specimen findings. Spearman’s correlation analysis was used to test the correlation between groups.

Median tumor length by gross specimen (112 mm; range, 45–300 mm) was longer than that by MRI (108 mm; range, 45–304 mm; Z = -6.916, P < 0.001). Of 255 images, tumor length was accurately represented on 27 T1WI magnetic resonance images, overestimated on 79 images, and underestimated on 149 images. The median difference between imaging and gross specimen measurements was 2.0 mm (range: 1.0–15.0 mm) for the 79 cases where tumor length was overestimated, and 5.0 mm (range: 1.0–18.0 mm) for the 149 cases where tumor length was underestimated. The Spearman correlation demonstrated a high correlation of tumor length on gross specimen with the tumor length on MRI (R = 0.99, P < 0.01).

We conclude that preoperative MRI could be a useful method in determining intramedullary malignant bone tumor boundaries and may serve as an accepted assessment method of long bone tumors before limb-sparing surgery.

Total knee arthroplasty (TKA) may be associated with serious complications that adversely affect outcomes and increase the likelihood of disability. However, few studies with sufficient sample size have reported postoperative complications following TKA among Chinese patients. This study aimed to evaluate complications of TKA within 30 postoperative days and to identify the related risk factors. Methods: A retrospective complication-based analysis of TKA using the arthroplasty registry between 2008 and 2013 was performed by summarizing complications of TKA within 30 postoperative days. Multivariate logistic regression was used to identify the predicting factors for complications 30 days after operation.

A total of 1542 patients underwent 2254 primary TKA between January 2008 and December 2013. A total of 137 complications occurred within 30 days after operation with an incidence rate of 6.1%. The incidence rate of major systemic complications within postoperative 30 days was 2.3%, with cardiovascular and respiratory complications as the most common complications. The incidence rates of deep venous thrombosis (DVT) and local complications were 2.4% and 1.0%, respectively. The 30-day postoperative mortality rate was 0.1% (3/2254). Multivariate logistic regression analyses identified body mass index (BMI) of ≥30.0 kg/m² (odds ratio [OR]: 1.47) and age ≥80 years (OR: 1.87) as significant risk factors for postoperative systemic complications. A BMI of ≥30.0 kg/m² was a significant risk factor for DVT (OR: 2.86) and other complications (OR: 2.11). The comorbidity of diabetes was a risk factor for postoperative mortality (OR: 19.20).

This study highlighted complications with cardiac and respiratory origins as the most common complications within 30 postoperative days following primary TKA. The BMI of ≥30.0 kg/m² and age ≥80 years were significant risk factors for 30-day postoperative complications.

Accurate evaluation of the plain radiography of lower limb is critical for preoperative planning of total knee arthroplasty (TKA). We aimed to investigate the effect of femoral lateral bowing and rotation on the radiographic measurements of distal femoral condyle resection thickness (DRT) and the distal femoral resection valgus angle (FVA).

We analyzed 246 three-dimensional femoral models generated from computed tomography images of 123 patients, acquiring projected contours in seven positions – 20° and 10° internal rotation; 0° rotation; 10°, 20°, 30°, and 40° external rotation – for each model. Medial and lateral condyle DRTs, femoral shaft lateral bowing angle (FBA), and distal FVA were determined for each position. Linear mixed effect model was used to determine the effect of degree of femur rotation on repeated measurements of DRT or FVA.

FBA significantly affected the FVA and DRT (Pearson’s R = 0.767 and -0.408, respectively; P < 0.000). Samples were divided into three groups according to the FBA measured in neutral position: FBA <0°: DRT 3.75 ± 1.30 mm, FVA 4.53°± 1.27°; FBA >0° but <3°: DRT 3.39 ± 1.31 mm, FVA 5.92°± 1.31°; FBA >3°: DRT 2.22 ± 1.31 mm, FVA 7.37°± 1.31°. From simulated 20° internal rotation to 40° external rotation in each femoral model, the average variation ranges of radiographically measured DRT, FVA, and FBA were 0.50 ± 0.28 mm, 2.93° ± 0.96°, and 10.33°± 1.90°, respectively, with no significant differences among the FBA groups. The degree of femoral rotation significantly affected the FVA (F = 62.148, P < 0.000), whereas there was no effect on condyle resection thickness (F = 0.4705, P = 0.494).

Axial femoral rotation has less effect on radiographic measurements of differences in the DRT than on those of the distal FVA.

Lateral compartmental osteoarthritis (LCOA), a major complication after medial mobile-bearing unicompartmental knee arthroplasty (UKA), is highly associated with the increased stress of the lateral compartment. This study aimed to analyze the effects on the stress and load distribution of the lateral compartment induced by lower limb alignment and coronal inclination of the tibial component in UKA through a finite element analysis.

Eight three-dimensional models were constructed based on a validated model for analyzing the biomechanical effects of implantation parameters on the lateral compartment after medial Oxford UKA: postoperative lower limb alignment of 3° valgus, neutral and 3° varus, and the inclination of tibial components placed in 4°, 2° valgus, square, and 2° and 4° varus. The contact stress of femoral and tibial cartilage and load distribution were calculated for all models.

In the 3° valgus lower limb alignment model, the contact stress of femoral (3.38 MPa) and tibial (3.50 MPa) cartilage as well as load percentage (45.78%) was highest compared to any other model, and was increased by 36.75%, 47.70%, and 27.63%, respectively when compared to 3° varus. In the condition of a neutral position, the outcome was comparable for the different tibial tray inclination models. The inclination did not greatly affect the lateral compartmental stress and load distribution.

This study suggested that slightly varus (undercorrection) lower limb alignment might be a way to prevent LCOA in medial mobile-bearing UKA. However, the inclination (4° varus to 4° valgus) of the tibial component in the coronal plane would not be a risk factor for LCOA in neutral position.

The lateral pillar of the femoral head is an important site for disease development such as osteonecrosis of the femoral head. The femoral head consists of medial, central, and lateral pillars. This study aimed to determine the biomechanical effects of early osteonecrosis in pillars of the femoral head via a finite element (FE) analysis.

A three-dimensional FE model of the intact hip joint was constructed from the image data of a healthy control. Further, a set of six early osteonecrosis models was developed based on the three-pillar classification. The von Mises stress and surface displacements were calculated for all models.

The peak values of von Mises stress in the cortical and cancellous bones of normal model were 6.41 MPa and 0.49 MPa, respectively. In models with necrotic lesions in the cortical and cancellous bones, the von Mises stress and displacement of lateral pillar showed significant variability: the stress of cortical bone decreased from 6.41 MPa to 1.51 MPa (76.0% reduction), while cancellous bone showed an increase from 0.49 MPa to 1.28 MPa (159.0% increase); surface displacements of cortical and cancellous bones increased from 52.4 μm and 52.1 μm to 67.9 μm (29.5%) and 61.9 μm (18.8%), respectively. In addition, osteonecrosis affected not only pillars but also adjacent structures in terms of the von Mises stress and surface displacement levels.

This study suggested that the early-stage necrosis in the femoral head could increase the risk of collapse, especially in lateral pillar. On the other hand, the cortical part of lateral pillar was found to be the main biomechanical support of femoral head.

The etiology of Kienböck’s disease is controversial, and the blood supply is a possible pathogenic factor. The red lead (Pb₃O₄) angiography with micro-computed tomography (micro-CT) of lunate to investigate intralunate arteries has rarely been reported. This study aimed to investigate a new, reasonable, and simplified technique to study the intraosseous arterial pattern of normal lunates.

This study investigated the intraosseous arterial pattern of six normal cadaveric lunates through Pb₃O₄ injection and three-dimensional reconstruction with micro-CT. The intraosseous arteries of all specimens were clearly enhanced. The data of enhanced arteries and nutrient foramina were shown as median (Q₁, Q₃) and analyzed with Wilcoxon signed-rank test.

The mean number of total nutrient foramina was 2.00 (1.75, 2.00) on the palmar side and 3.50 (2.50, 4.25) on the dorsal side. The number with enhanced arteries on the palmar side was 1.00 (0.75, 2.00) and on the dorsal side was 3.50 (1.75, 4.00). There were no significant differences between the number of nutrient foramina on the palmar and dorsal sides of the lunates, no matter the total number or enhanced arteries. The intraosseous arterial pattern in normal lunates can be classified into three types: the dominate stems from the palmar side, from the dorsal side, and from both sides with anastomosis.

The Pb₃O₄ angiography with micro-CT is a simplified, quicker, and reliable method to study intraosseous arteries.

Current treatments for scoliosis have some defects and complications. To study spinal deformities and test novel scoliosis treatments, many animal models of scoliosis have been developed. These models applied a single load to the spine and could not precisely modulate the spinal growth in different dimensions. In this study, we applied posterior tethering in various directions with the application of nickel-titanium (NT) coil springs in dog’s spine to modulate spinal growth in the coronal, sagittal, and transverse planes and create a scoliosis model possess curves that mimic adolescent idiopathic scoliosis (AIS) three dimensionally.

Scoliosis was surgically induced in eight 8-week-old female dogs (weight: 1.95–2.30 kg) using bone screws and NT coil springs. The deformity was induced through the placement of posterior NT coil springs that tethered the spine by bone screw fixation. All dogs were monitored with serial radiographs to document changes in deformities.

All experimental animals developed scoliotic curves convex to the left in the lumbar segment. The mean coronal Cobb angle was 18.0° immediately postoperatively and 54.5° at 22 weeks. The mean lordosis increased from 6.2° postoperatively to 35.0° at final follow-up. Apical axial rotation increased from 4.5° postoperatively to 31.2° at 22 weeks.

With the application of NT springs in dogs that allowed posterior tethering in various directions, lumbar spinal deformity was achieved in three planes: coronal, sagittal, and transverse planes. Notably, the lumbar spine in surgically treated dogs developed lordoscoliosis with obvious rotation and the curves mimic AIS three dimensionally well. This method allows lumbar scoliosis to develop without deep dissection of muscle and maintains the essential anatomical elements along the spinal curve. Moreover, the spinal growth modulation technique could yield information that would provide a basis for developing novel early-stage treatments for children with scoliosis.

This review aimed to provide a current recommendation to multidisciplinary physicians for early detection, diagnosis, and treatment of corticosteroid-induced osteonecrosis of the femoral head (ONFH) based on a comprehensive analysis of the clinical literature.

For the purpose of collecting potentially eligible articles, we searched for articles in the PubMed, Cochrane Library, Embase, and CNKI databases up to February 2017, using the following key words: "corticosteroid" , "osteonecrosis of the femoral head" , "risk factors" , "diagnosis" , "prognosis" , and "treatment" .

Articles on relationships between corticosteroid and ONFH were selected for this review. Articles on the diagnosis, prognosis, and intervention of earlier-stage ONFH were also reviewed.

The incidence of corticosteroid-induced ONFH was associated with high doses of corticosteroids, and underlying diseases in certain predisposed individuals mainly occurred in the first 3 months of corticosteroid prescription. The enhanced awareness and minimized exposure to the established risk factors and earlier definitive diagnosis are essential for the success of joint preservation. When following up patients with ONFH, treatment should be started if necessary. Surgical treatment yielded better results than conservative therapy in earlier-stage ONFH. The ideal purpose of earlier intervention and treatment is permanent preservation of the femoral head without physical restrictions in daily living.

Clinicians should enhance their precaution awareness of corticosteroid-induced ONFH. For high-risk patients, regular follow-up is very important in the 1^st year after high-dose prescription of corticosteroids. Patients with suspected ONFH should be referred to orthopedists for diagnosis and treatment in its earlier stage to preserve the joint.

Conventional open spinal surgery of adult scoliosis can be performed from anterior, posterior, or combined approach. Minimally invasive spine surgery (MISS) was developed for the purpose of reducing the undesirable effects and complications. This review aimed to make a brief summary of recent studies of the approach and clinical outcomes of MISS in adult scoliosis.

We conducted a systematic search from PubMed, Medline, EMBASE, and other literature databases to collect reports of surgical methods and clinical outcomes of MISS in treatment of adult scoliosis. Those reports were published up to March 2017 with the following key terms: "minimally invasive," "spine," "surgery," and "scoliosis."

The inclusion criteria of the articles were as followings: diagnosed with adult degenerative scoliosis (DS) or adult idiopathic scoliosis; underwent MISS or open surgery; with follow-up data. The articles involving patients with congenital scoliosis or unknown type were excluded and those without any follow-up data were also excluded from the study. The initial search yielded 233 articles. After title and abstract extraction, 29 English articles were selected for full-text review. Of those, 20 studies with 831 patients diagnosed with adult DS or adult idiopathic scoliosis were reviewed. Seventeen were retrospective studies, and three were prospective studies.

The surgical technique reported in these articles was direct or extreme lateral interbody fusion, axial lumbar interbody fusion, and transforaminal lumbar interbody fusion. Among the clinical outcomes of these studies, the operated levels was 3–7, operative time was 2.3–8.5 h. Both the Cobb angle of coronal major curve and evaluation of Oswestry Disability Index and Visual Analog Scale decreased after surgery. There were 323 complications reported in the 831 (38.9%) patients, including 150 (18.1%) motor or sensory deficits, and 111 (13.4%) implant-related complications.

MISS can provide good radiological and self-evaluation improvement in treatment of adult scoliosis. More prospective studies will be needed before it is widely used.

Parkinson’s disease (PD) is featured with motor disorder and nonmotor manifestations including psychological symptoms, autonomic nervous system dysfunction, and paresthesia, which results in great inconvenience to the patients’ life. The apolipoprotein (Apo) superfamily, as a group of potentially modifiable biomarkers in clinical practice, is of increasing significance in the diagnosis, evaluation, and prognosis of PD. The present review summarized the current understanding and emerging findings of the relationship between Apo superfamily and PD.

All literatures were identified by systematically searching PubMed, Embase, and Cochrane electronic databases with terms "Parkinson disease," "apolipoprotein," and their synonyms until May 2017.

We have thoroughly examined titles and abstracts of all the literatures that met our search strategy and the full text if the research is identified or not so definite. Reference lists of retrieved articles were also scrutinized for additional relevant studies.

The levels of plasma ApoA1 are inversely correlated with the risk of PD and the lower levels of ApoA1 trend toward association with poorer motor performance. Higher ApoD expression in neurons represents more puissant protection against PD, which is critical in delaying the neurodegeneration process of PD. It is suggested that APOE alleles are related to development and progression of cognitive decline and age of PD onset, but conclusions are not completely identical, which may be attributed to different ApoE isoforms. APOJ gene expressions are upregulated in PD patients and it is possible that high ApoJ level is an indicator of PD dementia and correlates with specific phenotypic variations in PD.

The Apo superfamily has been proved to be closely involved in the initiation, progression, and prognosis of PD. Apos and their genes are of great value in predicting the susceptibility of PD and hopeful to become the target of medical intervention to prevent the onset of PD or slow down the progress. Therefore, further large-scale studies are warranted to elucidate the precise mechanisms of Apos in PD.

Objective: The aim was to update the genetic and clinical advances of congenital muscular dystrophy (CMD), based on a systematic review of the literature from 1991 to 2017.

Aderived disaster is a common concept in natural emergencies, such as earthquakes. In recent years, the incidence of fractures caused by high-energy trauma has increased annually with socioeconomic development in China.^[1,2,3]Accordingly, derived injuries resulting from primary injuries have also been increasing considerably. However, most surgeons in the field of orthopedics and traumatology do not yet have adequate knowledge of such kind of derived injuries. We summarized our experience in managing a patient who experienced reinjury caused by a primary injury over the last decade and proposed the concept of a derived injury. In addition, we aimed to introduce a classification of derived injuries, explore their significance in orthopedic trauma, and discuss methods to minimize their severity.

Tibial plateau fractures account for 1.66–2.0% of all fractures in adults and about 8% of fractures in the elderly.^[1]Tibial plateau fractures should be considered as complex injuries representing a wide fracture spectrum: soft-tissue compromise, neurovascular damage, compartment syndrome, and ligament and meniscus tears, which remain a big challenge, even for experienced orthopedic trauma surgeons. Although the best treatment modality remains controversial, we aimed to present new developments in the treatment of tibial plateau fractures for reference.

The clinical behavior of nonossifying fibroma (NOF) appears to be a benign lesion without recurrence or canceration.^[1]Ewing’s sarcoma (ES) is usually regarded as a differential diagnosis of NOF.^[2]There has not been any report on NOF and ES appearing in the same position simultaneously. Here, we first report a case that the simultaneous onset of two entities was mistaken for the development of NOF into ES.

Immunoglobulin G4 (IgG4)-related cholangitis (IAC) is one of the IgG4-related immune-mediated diseases. Although a study has provided a preliminary insight into its pathological mechanisms, the specific pathogenesis is unknown and the incidence of IAC remains unclear.^[1]

To the Editor: Calcific discitis is a self-limiting cervical disc disease usually seen in children and uncommon in adults.^[1]It can typically be cured by conservative treatment.^[2]In this article, we reported a case of paralysis caused by thoracolumbar calcific discitis, with acute herniation.

To the Editor: A 65-year-old woman with calcinosis, Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia (CREST) syndrome was admitted to hospital because of progressive dyspnea on exertion for about 1 year. In the past, she had been diagnosed as gastroesophageal reflux disease and scleroderma for 10 years. Physical examination showed masked face, Raynaud’s phenomenon, finger sclerodactyly, and skin telangiectasia on her neck and back [Figure 1aandFigure 1b]. The titer of anticentromere antibody in serum increased to 1: 1000. Analysis of arterial gas on room air revealed the partial pressure of arterial oxygen (PaO₂) was 54 mmHg (1 mmHg = 0.133 kPa) and carbon dioxide (PaCO₂) was 32 mmHg. Echocardiogram showed severe tricuspid regurgitation and elevated pulmonary arterial pressure of 78 mmHg. Chest high-resolution computer tomography demonstrated diffuse mild ground-glass opacities in both lung fields [Figure 1c] and dilated central pulmonary artery [Figure 1d]. Open lung biopsy demonstrated characteristic more than two layers of capillaries on alveolar walls [Figure 1e], which was confirmed by CD34 immunohistochemical staining [Figure 1f]. Therefore, the patient was diagnosed as pulmonary capillary hemangiomatosis (PCH) associated with CREST syndrome, and treated by prednisone and ambrisentan. One month later, her symptoms obviously improved and repeated echocardiogram report showed pulmonary systolic pressure decreased to 46 mmHg.