[Clinical characteristics and surgical effect observation of congenital aniridia combined with cataract].

Objective: To analyze the clinical manifestations, surgical approaches and postoperative prognosis for the cases of congenital aniridia combined with cataract. Methods: In this retrospective case series, 26 patients diagnosed with congenital aniridia combined with cataract were collected from Zhongshan Ophthalmic Center from February 2002 to August 2016. The Clinical data were collected to analyze the clinical features, surgical approaches and postoperative prognosis. T-test was used for statistical analysis. Results: Twenty-six bilateral congenital aniridia patients were included in the case series, with 50% male cases. The average age for the first visit was (8.72±8.06) years old. Hereditary patients constitutes 30.8% (8/26) of the total number. The proportions for bilateral and unilateral cataracts were 88.5% (23/26) and 11.5% (3/26) respectively, and 49 eyes suffering from both congenital aniridia and cataract were therefore included in the final analysis. The most common morphology cataract subtypes were lamellar cataract (24.5%, 12/49), posterior subcapsular cataract (22.4%, 11/49), and total cataract (18.4%,9/49). The observed ocular comorbidities included nystagmus (36.7%, 18/49), vitreous opacity (28.6%, 14/49), foveal hypoplasia (20.4%, 10/49), ametropia (12.2%, 6/49), exotropia (12.2%, 6/49), congenital glaucoma (12.2%, 6/49), esotropia (4.1%, 2/49), congenital ptosis (4.1%, 2/49), lens ectopia (4.1%, 2/49), scleral staphyloma (2.0%, 1/49) and pigmentary degeneration of retina (2.0%, 1/49). 30.6% (15/49) eyes were performed the cataract extraction surgery. The percentage of postoperative best corrected visual acuity (BCVA) for ≥0.1 and ≥0.3 were 93.3% (14/15) and 20.0% (3/15) respectively. Evaluated ocular pressure (33.3%, 5/15), severe posterior capsular opacification (PCO) (13.3%, 2/15) and choroidal hemorrhage combined with choroidal detachment (6.7%, 1/15) were detected as the postoperative complications. Followed by cataract extraction, 80.0% (12/15) eyes were sequentially performed the intraocular lens implantation, while, 20.0% (3/12) eyes remained aphakia due to ocular comorbidities. Conclusions: Congenital aniridia combined with cataract are rare diseases, calling for the precious retrospective researches. This disorder tended to affect both eyes and occurred hereditary. The clinical courses of the cases presented progressive features. Ocular comorbidities were the crucial factors to influence the surgical approaches and postoperative prognosis. (Chin J Ophthalmol, 2017, 53: 821-827).

The Epidemiology and Characteristics of Q fever and Co-infections with Scrub Typhus, Murine Typhus or Leptospirosis in Taiwan: A Nationwide Database Study.

Q fever (QF) is a worldwide zoonosis associated with outbreaks. Only a few nationwide studies regarding the surveillance and epidemiology of human QF have been reported. Although QF is endemic in Taiwan, a nationwide database investigation of the epidemiology and characteristics of QF and its associations with scrub typhus (ST), murine typhus (MT) and leptospirosis (LS) has never been reported. We analysed nationwide databases of suspected QF, ST, MT and LS cases from October 2007 to December 2014 obtained from the Centers for Disease Control, Taiwan. A total of 468 (4.2%) QF cases were identified among 11 109 suspected QF cases. QF cases were mainly distributed in the southern and Kaohsiung-Pingtung regions but rarely in the eastern region. Compared to non-QF cases, QF cases had significantly higher percentages of males (88.7 versus 66.2%) and high-risk occupations (farming, animal husbandry or veterinary medicine) (16.2 versus 10.5%). But the percentages of specific animal contact, including cattle (0.6 versus 0.8%) and goats (0.9 versus 1.0%), were low in both. The majority of suspected QF cases (89.4%) were simultaneously suspected with ST, MT or LS, and the combinations of suspected diseases differed between regions. The number of suspected QF cases from the eastern region decreased since 2009, which was not observed in other regions. A total of 1420 (12.8%) cases had confirmed diseases, including QF (453, 4.1%), QF+ST (7, 0.06%), QF+MT (4, 0.04%), QF+LS (4, 0.04%), MT (186, 1.7%), ST (545, 4.9%), ST+LS (11, 0.1%) and LS (210, 1.9%). Compared to cases of unknown disease, QF cases had larger percentages of high-risk occupations (16.2 versus 9.6%) but similar histories of animal contact (29.8 versus 25.1%). QF is an endemic disease in southern Taiwan. It is difficult to differentiate QF from ST, MT or LS only by high-risk occupations and history of animal contact, and co-infection of QF with these diseases should be considered.

Association between polymorphisms in the csrRS two-component regulatory system and invasive group A streptococcal infection.

The csrRS two-component regulatory system is an important element in the pathogenesis of group A Streptococcus (GAS). The main goal of this study is to understand the association between csrRS polymorphisms and GAS infection. We sequenced the csrRS genes from 172 clinical isolates, including 81 invasive and 91 noninvasive isolates, and then employed phylogenetic analyses to determine the consequences of the csrRS polymorphisms. In total, 13 and 26 polymorphic loci were detected in the csrR and csrS genes, respectively. These polymorphisms constituted 14 csrR and 25 csrS alleles, producing two CsrR and seven CsrS variants, respectively. Three invasive isolates contained an indel in csrS, but no indel was identified in csrR. The frequency and distribution of polymorphisms in csrR and csrS was significantly different between the invasive and noninvasive infection isolates (p < 0.001). For CsrR, only one noninvasive isolate was identified to have a V29I mutation. The amino acid substitutions in CsrS included S32P (0.6 %), E265G (0.6 %), E265K (0.6 %), I332V (1.7 %), and N498K (82.6 %). Isolates with an N498K single mutation were more likely to be associated with invasive infections (p < 0.001). The dN/dS ratio indicated that both csrR and csrS were under purifying selection. The fixation index suggested a moderate evolutionary differentiation of the csrR and csrS alleles between invasive and noninvasive isolates. The identification of these genetic differences within the csrRS loci will provide a better understanding of the pathogenesis of GAS.

Risk factors for extra-pulmonary tuberculosis compared to pulmonary tuberculosis.

BACKGROUND: Tuberculosis (TB) continues to be a major global health problem. Extra-pulmonary TB (EPTB) manifests with protean symptoms, and establishing a diagnosis is more difficult than pulmonary TB (PTB). SETTING: A university-affiliated hospital in southern Taiwan. OBJECTIVE: To analyse the risk factors for EPTB compared with PTB. DESIGN: This retrospective study compared patients with EPTB and PTB in southern Taiwan by analysing their demographic data and clinical underlying diseases. Risk factors for EPTB were further analysed. RESULTS: A total of 766 TB patients were enrolled in this study, with 102 (13.3%) EPTB and 664 (86.7%) PTB cases. Of the 766 patients, 3% of PTB patients had EPTB, while 19.6% of EPTB patients also had PTB. The most frequently involved EPTB site was the bone and joints (24.5%). The incidence of EPTB vs. PTB decreased significantly for each decade increase in patient age. Multivariate logistic regression analysis showed that being female, not being diabetic, having end-stage renal disease and not smoking were independent risk factors for EPTB. CONCLUSION: This study defines the risk factors for EPTB compared with PTB. Awareness of these factors is essential for physicians to have a high index of suspicion for accurate and timely diagnosis.

Hepatic mesenchymal hamartoma.

Hepatic mesenchymal hamartoma (HMH) is a rare liver tumour in childhood. It is believed to be a benign tumour with a good prognosis if the tumour is excised. Only a few cases with local relapse or metastasis have been reported. We reviewed 134 patients with space-occupying liver lesions, from January 1990 to December 1999, and four patients with HMH were found. Abdominal mass and/or distension were the common initial presentations. Three of our four cases were diagnosed in the first year of life. The liver function tests and tumour markers were non-specific. Case 1 underwent tumour enucleation and another three cases underwent tumour excision. One patient died. No tumour recurrence was noted in the surviving patients.

Use of parenteral testosterone prior to hypospadias surgery.

Surgical correction of genital defects was formerly proposed when the size of the penis was sufficient to permit easy surgical repair. To enlarge penile size, temporary stimulation with testosterone or dihydrotestosterone cream has been used; however, the results were not only inconsistent, but absorption was also variable. We report our experience with parenteral testosterone as an adjunct to reconstructive genital surgery in 25 patients aged 6-18 months from July 1999 to December 2000, including 8 with penile hypospadias, 15 with penoscrotal hypospadias, and 2 with perineal hypospadias. Each had a penis that was significantly smaller than usual. Testosterone enanthate 25 mg was given i.m. once per month for a total of three doses before surgical repair. Penile length and glans circumference were measured before therapy and at operation. Side effects such as the development of pubic hair and acne were monitored. Bone age was checked 1 year later. An increase in penile length (from 19.8 +/- 2.4 mm to 23.8 +/- 2.0 mm) and glans circumference (from 27.4 +/- 1.4 mm to 37.84 +/- 2.6 mm) was apparent in all except 2 patients (P < 0.001 for both, paired t-test). Four patients had a significant increase in either penile length or glans circumference after the initial dose so that no further injections were required. No definite secondary effects were found. Preoperative parenteral testosterone therapy thus causes a significant increase in penile length and glans circumference without apparent side effects. We suggest that this therapy prior to microphallic hypospadias repair is appropriate.

Hepatic Schistosomiasis japonica in a patient with gallstones and bile duct stones--a case report.

Schistosomiasis, a common parasitic disease in many countries, is found as imported cases in Taiwan. Responsible for human infections are five species, one of which, Schistosoma japonicum, is currently endemic in China and South-east Asia. Chronic infection with S. japonicum may lead to the development of liver fibrosis, calcification and portal hypertension. Under investigation by sonography and computed tomography (CT) scan, a peculiar "turtle-back" appearance of liver fibrosis and calcification may be found. Herein, we report a case referred to our department due to jaundice. The sonography of liver showed typical "turtle-back" appearance. Gallstones and bile duct stones were also found in this case. Surgical interventions with percutaneous transhepatic biliary drainage (PTBD), cholecystectomy and choledocholithotomy were performed to relieve the obstructive jaundice and remove the stones. There were no parasitic eggs in the extracted stones or in drained bile juice. However, deposits of calcified S. japonicum eggs in liver parenchyma and portal tracts were identified in liver biopsy. No special treatment was given for the schistosomiasis japonica because the calcified parasitic eggs were the sequelae of past infection.

Effect of N(G)-nitro-L-arginine methyl ester on intestinal permeability following intestinal ischemia-reperfusion injury in a rat model.

Subclinical intestinal ischemia-reperfusion injury (IRI) causes an increase in mucosal permeability and may represent an early event in the pathogenesis of necrotizing enterocolitis in premature infants. Previous studies suggested that continuous, endogenous formation of nitric oxide (NO) maintains the mucosal integrity of the intestine, thus protecting the gut from injuries from blood-borne toxins and tissue-destructive mediators. This study was undertaken to assess whether the inhibition of NO production causes an increase in intestinal permeability in rats following IRI. Sprague-Dawley rats weighing 200-300 g were divided into 4 groups: (1) untreated group (normal control); (2) ischemia-reperfusion group; (3) early N(G)-nitro-L-arginine methyl ester (L-NAME), a specific inhibitor of NO production, treatment group, and (4) late L-NAME treatment group. Transient IRI was induced by 30-min occlusion, followed by reperfusion of the isolated ileal loop. The L-NAME was administered 15 min before and after mesenteric ischemia as a 25-mg/kg bolus. Fluorescein isothiocyanate-dextran (FITC-D) was used to quantitatively assess the alteration in mucosal permeability of the intestine. There was no significant increase in the portal vein FITC-D level among normal controls, ischemia-reperfusion group and late L-NAME-treated group, but there was an approximately 6-fold increase in the early L-NAME treatment group. The pathological features of the intestine following IRI include denudation of the villus epithelium and reduction of villus height, associated with marked inflammatory cell infiltration over the lamina propria. These results suggest that endogenous NO may play a role in the protecting intestinal integrity after IRI.

Biliary atresia at the dawn of a new century.

Biliary atresia is a unique disorder in pediatric surgery. The incidence is 1 in 3,500 to 20,000 live births, but the disorder accounts for more than half of the patients requiring liver transplantation. After Kasai operation, the 5- and 10-year survival rate without the need of transplantation are 37-48% and 18-28%, respectively. An infectious pathogen, particularly the virus, has long been implicated as the causative agent of biliary atresia, but none has ever been identified. Ductal plate malformation is likely to be important in the pathogenesis of biliary atresia. Earlier diagnosis is associated with a better prognosis and biliary atresia should be considered in the differential diagnosis of prolonged jaundice in any infants older than 2 weeks of age. The experience of the surgeon and surgical decade are determinant in achieving success of the Kasai operation. Postoperative cholangitis affects more than 45% of the patients receiving Kasai operation and the afflicted patients are at risk of cessation of bile flow and exacerbation of preexisting liver cirrhosis. Hepatic fibrosis starts at the moment when biliary atresia develops and the ongoing inflammation will end up with liver cirrhosis and hepatic failure in most patients, including some with adequate bile drainage. The inhibition of liver cirrhosis in mice or rats by gene therapy, such as telomere gene delivery, transduction with the hepatocyte growth factor gene or blockade of TGF-beta signaling, may provide a new strategy to rejuvenate the ailing liver associated with biliary atresia in the future.

99mTc-sulphur-colloid and heat-denatured 99mTc-labelled red cell scans demonstrating a giant intrapelvic spleen in a girl after splenectomy.

A 17 x 12 x 5-cm giant intrapelvic mass in a 14-year-old girl is reported. This mass developed 6 years after a splenectomy for splenic torsion. The heat-denatured 99mTc-labelled red cell scan and 99mTc-sulphur-colloid scan confirmed the specific red cell sequestration function and reticuloendothelial activity in the giant intrapelvic spleen. The size and development of the giant intrapelvic spleen are unusual. The usefulness of functional images to diagnosis the nature of the intrapelvic mass is well demonstrated.

Overview of migraine.

Migraine, one of the most common incapacitating headaches, afflicts 23 million adults in the United States. There is a controversy in the literature on the theoretical perspective of migraine, the efficacy of treatment strategies, measurement, and correlates. Thus, more research is needed on these issues. A wide range of treatments is available for migraine. In addition to terminating migraine headache, appropriate migraine care includes preventing its attacks and improving patients' quality of life by administrating pharmacologic preparations in conjunction with a variety of alternative methods.

Sonographic features related to volvulus in neonatal intestinal malrotation.

This 3 year prospective study evaluated the sensitivity and specificity of abdominal ultrasonography and color Doppler ultrasonography in 31 neonates with suspected malrotation or malrotation with volvulus. Water instillation was used to detect duodenal dilatation, edema, and malrotated bowels. Twenty patients with ultrasonographic characteristics of inversion of the superior mesenteric artery and superior mesenteric vein were later surgically proved to have malrotation. Nine of these 20 patients also had volvulus. Sonographic features suggestive of volvulus included duodenal dilation with tapering configuration (8 of 9 cases, 89%), fixed midline bowel (8 of 9 cases, 89%), whirlpool sign (8 of 9 cases, 89%), and dilation of the distal superior mesenteric vein (5 of 5 cases, 100%). The sensitivity and specificity of duodenal dilation with tapering configuration for detecting volvulus were 89% and 92%, respectively; of fixed midline bowel, 89% and 92%; of whirlpool sign, 89% and 92%; and of dilation of distal superior mesenteric vein, 56% and 73%. The results of this study indicate that ultrasonographic features of inversion of the superior mesenteric artery and superior mesenteric vein could aid in the diagnosis of malrotation, and certain sonographic features can also be used to evaluate volvulus, a condition requiring emergent operation.

Treatment and outcome of congenital diaphragmatic hernia.

BACKGROUND AND PURPOSE: Congenital diaphragmatic hernia (CDH) is a challenging condition and is associated with a high mortality rate; optimal therapy remains unclear. This retrospective study describes the clinical characteristics of treatment and outcome in 48 infants with CDH. METHODS: Twenty-eight male (58%) and 20 female (42%) infants with CDH were treated from 1987 through 1998. The goals of the ventilator strategy were permissive hypercapnea (PaCO2 < or = 55 mm Hg) and avoidance of hyperventilation. Infants were initially ventilated with an intermittent mandatory rate of 40 to 60 per minute, peak inspiratory pressure of 20 to 25 cm H2O, and positive end-expiratory pressure of 5 cm H2O. High-frequency positive pressure ventilation was used if hypoxemia or severe hypercapnea (PaCO2 > 60 mm Hg) occurred. Most infants underwent repair after 3 days of age and only four infants underwent early repair within 24 hours of birth. A prophylactic chest tube was placed in the ipsilateral hemithorax postoperatively in all patients treated before 1996. The severity of respiratory distress was estimated by alveolar-arterial oxygen difference, oxygenation index, and alveolar-arterial ratio. RESULTS: Forty-six patients presented with Bochdalek CDH, and two with Morgangni CDH. Antenatal diagnosis was made in 10 cases. Respiratory distress was the major manifestation and usually occurred immediately after birth. Six cases were diagnosed several months after birth and presented mainly with gastrointestinal symptoms. Eleven patients died before surgery and 37 patients underwent surgical repair. Two infants died postoperatively because of congestive heart failure and tension pneumothorax, respectively. The overall mortality rate was 27%. The major causes of mortality were severe respiratory failure, persistent pulmonary hypertension, pneumothorax, and associated anomalies. CONCLUSION: Nearly 75% of patients in this series survived. This suggests that noninvasive respiratory care combined with delayed surgery may be an acceptable strategy for the treatment of CDH, and can be used in most medical institutions without equipment for extracorporeal membrane oxygenation therapy.

Prenatally detected tumor mass in the adrenal gland.

BACKGROUND/PURPOSE: Screening programs using urinary vanillylmandelic acid have detected neuroblastomas in early infancy with some success. With the widespread use of ultrasonography in modern obstetric practice, use of ultrasonography to screen for fetal neuroblastoma seems to be reasonable and practical. METHODS: Seven fetuses had suprarenal masses detected by maternal ultrasound scan at 32 to 37 weeks' gestation between 1993 and 1998. They were delivered normally if the pregnancy was uncomplicated, especially if it was without maternal preeclampsia or fetal hydrops. Each mass was further confirmed by ultrasound scan, computed tomography, or magnetic resonance imaging in the neonatal period. Tumor excision was performed at the age of 6 to 38 days of life. RESULTS: The size of the masses measured ranged from 2.0x2.0 cm to 4.5x4.5 cm. The diagnosis was adrenal hemorrhage in 1 neonate, Evan's stage I neuroblastoma in 3, and stage IV-S neuroblastoma in 3. All of the specimens with a diagnosis of neuroblastoma showed a favorable histology by the Shimada classification system. Infants with stage I disease were treated with tumor excision only, and they had survived without disease by 14, 18, and 25 months of follow-up. One infant with stage IV-S neuroblastoma was treated further with minimal chemotherapy and has survived without disease at the 66-month follow-up examination. Another child with stage IV-S neuroblastoma has survived with local recurrence and increasing liver metastasis and was still on chemotherapy at the 2-month follow-up examination. The third child with stage IV-S disease presented with massive hepatomegaly and bone marrow involvement, and disseminated intravascular coagulopathy had developed. The patient died on the 5th day of life without surgical intervention. CONCLUSIONS: The increasing use of obstetric ultrasonography has made the prenatal screening of neuroblastomas possible. The prognosis of infants with a suprarenal mass may be improved with this early detection and early surgical intervention.

Repair of hypospadias complications using the tubularized, incised plate urethroplasty.

BACKGROUND/PURPOSE: Secondary procedures to correct complications after hypospadias repair remain challenging especially for "hypospadias cripples." The tubularized, incised plate urethroplasty was first introduced by Snodgrass for the repair of primary hypospadias in 1993. The authors used this procedure to correct the complications after hypospadias repair in patients who had no abundant local skin flaps to be used for a neourethra. METHODS: Six patients underwent tubularized, incised plate urethroplasty for the correction of complications of hypospadias repair performed the previous year, including a large urethrocutaneous fistula (n = 1) and disruption of the neourethra (n = 5). Prior surgical procedures included transverse island tube urethroplasty in 4 cases and 2-stage urethroplasty in 2 cases. The average patient age at the time of secondary procedure was 4.6 years (range, 1 to 12 years). RESULTS: The mean follow-up period was 6 months (range, 2 months to 1 year). All the patients obtained a functional neourethra with a vertical, slitlike meatus. A small fistula developed in one child and mild meatal retraction in another. CONCLUSIONS: The tubularized, incised plate urethroplasty offers few complications and good cosmetic results. The authors recommend its use for patients who have had repeated surgeries for hypospadias repair, especially those in whom only limited local skin flaps can be utilized for a neourethra.

Aminoguanidine attenuates hemodynamic and microcirculatory derangement in rat intestinal ischemia and reperfusion.

BACKGROUND: Nitric oxide (NO) participates in the regulation of hemodynamic and microcirculatory changes in intestinal ischemia and reperfusion (I/R). However, the nature of the involvement of an inducible NO release has been controversial. This study evaluates the impact of an inducible NO synthase inhibitor, aminoguanidine, used as a treatment in a rat intestinal I/R model. METHODS: We investigated the hemodynamics by measuring the mean arterial pressure (MAP), and the microcirculatory responses of the intestine and liver to systemically administered aminoguanidine by use of laser-Doppler flowmetry (LDF), in vivo microscopy, and flow cytometry. RESULTS: During the 30-min ischemia of the selected 20-cm ileal segment, no MAP change was noted. At reperfusion, a marked decrease of MAP was noted and the lowest levels were noted 3 hours after reperfusion (67 +/- 4% vs. 99 +/- 5% in sham-operated control animals). A marked decrease in liver perfusion as measured by LDF was noted 1 hour after reperfusion and remained low at 5 hours (72 +/- 4% vs. 97 +/- 3% in sham-operated control animals). A marked decrease in intestine perfusion was noted by using LDF 1 hour after reperfusion and remained low at 5 hours (43 +/- 3% vs. 92 +/- 4% in sham-operated control animals). The flow velocity of the postcapillary venules of the intestine was markedly decreased (1.01 +/- 0.62 vs. 2.67 +/- 0.34 mm/s in sham-operated control animals) at 5 hours after reperfusion. The flow velocity of the postsinusoidal venules of the liver was also markedly decreased (1.01 +/- 0.62% vs. 2.67 +/- 0.34% in sham-operated control animals). Leukocyte-endothelial interaction (adhesion) was increased in the postcapillary venules of the intestine (54 +/- 12 vs. 6 +/- 4/microm2 in sham-operated control animals) and in the postsinusoidal venules of the liver (32 +/- 8 vs. 2 +/- 2/microm2 in sham-operated control animals). Concomitantly, the granulocyte count was increased (9.1 +/- 0.6 vs. 2.1 +/- 0.3% of total circulating leukocytes in sham-operated control animals), with an increase of CD 11b expression. Aminoguanidine administration (1 mg/kg) 0.5 hour before ischemia and 1 hour after reperfusion significantly increased MAP, increased intestine and liver perfusion, decreased adhesion, and decreased circulating granulocytes and CD 11b expression. CONCLUSION: Inhibition of an inducible NO release by aminoguanidine in intestinal I/R can attenuate hemodynamic and microcirculatory derangement.

Anorectal malformations--update 1998.

Anorectal malformation is a common congenital anomaly. Its correction still challenges the wisdom and expertise of the pediatric surgeon. From 1977 through 1997, approximately 400 cases of this anomaly were treated at this institution. Prior to 1985, cut-back anoplasty and translocation anoplasty were the treatments of choice for the low anomalies, and abdominoperineal pull-through anoplasty or sacroperineal anoplasty (and their modifications) preferred for the intermediate or high defects. Since 1985, posterior sagittal anorectoplasty (PSARP) has been adopted exclusively as the operative procedure for the correction of the entire spectrum of anorectal malformations. Two hundred patients have undergone this procedure either primarily (N = 166) or secondarily (N = 34). PSARP is a procedure which enables the surgeon to repair the pelvic anatomy under direct vision, maximally utilizing all the existing continence muscles and hence resulting in better fecal continence. With minimal, limited or maximal division of the continence muscles under one same skin incision, the entire spectrum of this defect can be repaired. The result of cosmesis is generally regarded as better, and the immediate postoperative complications are fewer. In spite of all efforts, 10 to 30% of patients still suffer from total fecal incontinence which is intractable to all kinds of medications and enemas. A bowel management program with large volume enemas, either through the anus or a Malone appendicostomy, retrogradely or antegradely, to wash out stool in the colon and rectum may be necessary to keep the patient clean and more socially acceptable. Biofeedback therapy offers another alternative to improve continence with the development of better computer systems and manometry equipment. This review provides a new concept of the fecal continence mechanism and an operative procedure for constructing a new anorectum based on this concept for the treatment of patients with anorectal malformations.

Ultrasonographic study of the sternocleidomastoid muscle in the management of congenital muscular torticollis.

BACKGROUND: Congenital muscular torticollis (CMT) in infancy is caused by the fibrotic change of the sternocleidomastoid muscle (SCMM). The etiology and management strategies remain controversial. METHODS: One hundred ninety-seven infants and children aged 1 month to 16 years who had CMT were examined by real-time ultrasonography of the SCMM between June 1995 and September 1996 in a prospective and longitudinal study. A total of 362 examinations were performed. There were 122 boys and 75 girls. RESULTS: The right side was involved in 117 patients (59.3%), the left side in 79 patients (40.1%), and both sides in one patient. The sonographic findings were homogeneous or heterogeneous (patchy) hyperechoic lesion within the SCMM, and all were diagnostic. The ultrasonographic appearance of the SCMM in this study has a close resemblance to the clinical course of CMT. The extent of fibrosis as represented by the cross section of lesion to muscle ratio (L/M ratio) decreased from 83.6% at 2 months to 59.9% at 9 months of age and further decreased to 40% beyond 1 year of age. This consistent decrease in fibrosis was caused by the increased normal muscle volume at the periphery and by the regenerated muscle fibers within the lesion. In this series of 197 patients, 32 (16.2%) eventually underwent surgery to release the SCMM because of persistent head tilt, chin deviation and limited range of neck motion beyond 1 year of age. The L/M ratio of the operative group was 62.7 +/- 16.0% compared with an L/M ratio of 54.5 +/- 14.2% (P = .035) for the nonoperative group at 1 year of age. The extent of fibrotic change in the cross section of the muscle was a significant factor in determining prognosis. In the longitudinal section, the fibrotic change was limited to only the lower third of the SCMM in 27 patients, and all of them recovered without operation. In 95 patients, the fibrotic lesion was limited to the middle and lower third or middle third only, and only six (6.3%) underwent operation. However, in 75 cases the entire length of muscle was involved, and 26 (34.7%) required surgical release of the contracted muscle. Whole-length muscle involvement was also important for predicting recovery without operative intervention. CONCLUSIONS: Ultrasonographic study of the SCMM is not only a valuable diagnostic tool but can also serve as a useful guideline for the treatment of infants who have congenital muscular torticollis.

Factors related to detection of blood flow by color Doppler ultrasonography in intussusception.

Color Doppler ultrasonography was performed in 125 patients with 134 intussusceptions. Color flow was present in 121 cases of intussusception (group A) and was absent in 13 cases (group B). Young age and duration of symptoms greater than 48 hr were significantly related to the nonvisualization of blood flow detected by color Doppler sonography (P < 0.05). The successful rate of air reduction was significantly higher in group A than in group B (109 of 121 versus 4 of 13). Color Doppler ultrasonography is useful to predict the reducibility of an intussuscepted intestine. The nonvisualization of blood flow by this method is not a contraindication for air reduction.

Chemical modification of surface active poly(ethylene oxide)-poly (propylene oxide) triblock copolymers.

A general route has been developed to chemically modify a series of poly(ethylene oxide)-poly(propylene oxide) triblock copolymers with molecular weights from 6500 to 14600. It is initiated by the introduction of p-nitrophenyl groups; such nitrophenyl conjugated copolymers are stable in an organic milieu and in a dry state but are seen to react easily with amino-containing molecules including small peptides. Among them, introduction of 2-pyridyl disulfide groups after coupling with 2-(2-pyridyldithio)ethylamine enables the selective attachment of thiol-containing molecules. The released thiopyridone in such thiol-disulfide reactions can be used to quantify the content of 2-pyridyl disulfide groups. In addition, a new type of modified copolymers was developed for the radioisotope (125I) labeling purpose that consists of a reaction of nitrophenyl conjugated copolymers with hydrazine and a subsequent coupling with N-succinimidyl 3-(4-hydroxyphenyl)propionate (Bolton-Hunter reagent). Adsorption studies of 125I-labeled and 2-pyridyl disulfide conjugated copolymers on polystyrene particles are consistent with previous determinations of surface coverage using other technologies, in turn indicating that this new chemical modification does not alter their surfactant properties on hydrophobic solid phase. The coating of common hydrophobic surfaces with 2-pyridyl disulfide conjugated copolymers has been demonstrated as a general and robust immobilization method to generate a high-sensitivity bioactive surface with low nonspecific binding. The optimal space between immobilized ligands can also be controlled by incubating the solid phase with solutions containing mixtures with different ratios of unmodified and modified copolymers.