[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).

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.

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.

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.

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.

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.

Pneumatic reduction of intussusception in children.

Over the past decade, pneumatic reduction has been increasingly accepted as the treatment of choice for pediatric intussusception. However the effectiveness of air compared with the more traditional barium reduction of intussusception continues to be a source of concern and debate. From August 1993 to November 1994, pneumatic reduction was used to treat 75 episodes in 73 patients with proven intussusception at Chang Gung Memorial Hospital, Taoyuan. Two patients underwent air reduction twice because of recurrence following an initial successful reduction. The recurrence rate was 3%. Successful reduction was achieved in 65/75 (87%) episodes. None of the patients experienced any complications following the procedure. In two of the 10 patients in whom reduction failed, one was subsequently found to have a Meckel's diverticulum and the other a duplication cyst as a leading point. This prospective study indicates that air enema is a safe and effective form of treatment for intussusception in infants and children. Pneumatic reduction should be the treatment of choice in the initial management of intussusception.

Management of yolk sac tumor of the testis in children.

Yolk sac tumor (YST) is the most common form of malignant testicular tumor in infancy and children. However, there is no general agreement on its treatment regimen due to its rare occurrence. From 1978 through 1990, 30 cases of YST of the testis were treated at Chang Gung Children's Hospital. Thirteen (43.3%) occurred in the right testis and 17 (56.7%) in the left. Age at operation ranged from six months to five years with a mean of 1.79 years. All patients received an orchidectomy as their initial treatment. Eighteen patients with stage I disease were treated by orchidectomy alone. Fourteen (77.8%) of them were free of disease, two patients who had recurrence with retroperitoneal metastasis were successfully treated with chemotherapy. The remaining two patients died of metastasis. Seven patients with stage II disease and five with stage III disease were treated with combination chemotherapy, irradiation or debulking operation following orchidectomy. Four stage II patients (57.1%) and one stage III patient (20%) showed no evidence of disease at the time of follow-up. It was concluded that for stage I disease, orchidectomy alone followed by strict monitoring of alpha- fetoprotein achieved an 89% disease-free survival rate. Retroperitoneal node dissection or routine chemotherapy added no benefit to survival. For stage II or III disease, chemotherapy or irradiation is needed to obtain a better outcome.

Repeated technetium-99m pertechnetate scanning for children with obscure gastrointestinal bleeding.

From 1980 to 1992, we found false negative results from Technetium (Tc)-99m abdominal scans in seven children with massive gastrointestinal bleeding from ectopic gastric mucosae. Other examinations--including endoscopy, gastrointestinal series studies, angiography, and Tc-99m-labeled red blood cell scans to search for the source of the bleeding--were all in vain. Positive results were only obtained after repeated Tc-99m abdominal scans. Surgical specimens confirmed that ectopic gastric tissue was the source of bleeding in five patients with Meckel's diverticulum and two patients with enteric duplication.

Pyogenic liver abscess in children.

Our center's experience with 15 pyogenic liver abscesses in 14 children from 1979 to 1992 showed an incidence of 20 per 100,000 pediatric hospital admissions. Eight of the 15 liver abscesses were cryptogenic in origin. The clinical features and laboratory findings were non-specific. Improved imaging techniques such as real time sonography and computed tomography made early diagnosis feasible. Klebsiella pneumoniae was the most common pathogen in this study. Drainage combined with antibiotics provides the most important treatment for this disease. Before 1986, surgery was frequently used, but now percutaneous drainage is preferred. Surgery may be reserved for those who respond poorly to percutaneous drainage and medical treatment. One of the 14 patients in this study died.

Role of parenteral nutrition in preventing malnutrition and decreasing bacterial translocation to liver in obstructive jaundice.

Surgery in patients with obstructive jaundice is associated with significant infectious complications probably due to impaired immune function and malnutrition. Total parenteral nutrition (TPN) may alleviate malnutrition but may also promote bacterial translocation (BT) from the gut. To elucidate if TPN can prevent malnutrition without promotion of BT in obstructive jaundice, 40 dogs underwent laparotomy for tissue sampling and placement of a central venous line and were allocated into one of four groups: I (PO-control) received dog chow and water ad libitum; II (PO-CBDL) underwent ligation of common bile duct (CBDL) and was fed dog chow; III (TPN-control) received TPN; and IV (TPN-CBDL) underwent CBDL and received TPN. Body weight, blood samples for liver function tests and bacterial culture, and tissues from liver and mesenteric lymph nodes (MLN) for quantitative bacterial culture and for histology were obtained prior to and 2 weeks after the experiment. The incidence of BT to MLN was 40% in the PO-CBDL and TPN-CBDL animals, which was significantly different from the other two groups (0%; p < 0.05). The incidence of BT to liver was 70% (7/10) in the PO-CBDL animals, which was significantly higher than that in groups I, III, and IV (0%, 20%, 20%, respectively) (p < 0.05). The PO-CBDL animals showed a significant decrease in body weight and prealbumin compatible with malnutrition, whereas the TPN-CBDL animals showed a significant increase in alkaline phosphatase and a consistent cholestasis on histology. The data suggest that TPN can prevent jaundice-associated malnutrition and decrease BT to liver but should be administered cautiously because it may precipitate cholestasis.

Technetium-99m pertechnetate scan for ectopic gastric mucosa in children with gastrointestinal bleeding.

The detectability of ectopic gastric mucosa by Technetium (Tc)-99m pertechnetate abdominal scanning was investigated by reviewing 62 such scans in 52 pediatric patients with gastrointestinal hemorrhage. The histologic diagnoses and clinical conditions were correlated to the findings of the scans. There were 21 true-positives, 35 true-negatives, one false-positive and five false-negative studies. The overall sensitivity of this scan for the detection of ectopic gastric mucosa was 81%, the specificity was 97% and the accuracy was 90%.

New observations in a child with angiofollicular lymph node hyperplasia (Castleman's disease) originated from the mesenteric root.

Localized plasma cell type Castleman's disease (CD) is an unusual pathologic entity. It is frequently associated with clinical and laboratory characteristics and rarely occurs in children. Total surgical excision results in cure in all aspects. To make early diagnosis of mesenteric CD is not easy, especially for children. An 11-year-old Taiwanese boy was recently evaluated for anemia and delayed growth. His clinical findings included a syndrome of severe hypochromic microcytic anemia, neutropenia, thrombocytosis, hypoferremia, hypergammaglobulinemia, and growth failure. Radiological examinations (abdominal ultrasound, small intestinal series, and computerized tomography) identified hepatosplenomegaly, nephromegaly, and huge masses in the middle abdomen with precaval, celiac, and paraaortic lymph nodal enlargement. However, detailed physical examination failed to detect a mass. At laparotomy a double-fist-sized confluent mass was found arising from the mesenteric root. Most masses were discrete and were excised individually. The pathologic diagnosis was plasma-cell type angiofollicular lymph node hyperplasia (Castleman's disease). Seven weeks after surgery, he had an episode of acute hepatitis B. Postoperatively, he exhibited a dramatic growth spurt; the hemoglobin, red blood cell indices, serum iron, and immunoglobulins returned to normal in 2 months. Neutropenia, which has not been previously related to mesenteric CD, was an unexpected finding in our case; however, it resolved spontaneously 3 months after the surgery, suggesting its causal relationship with the tumor.

The efficacy of Kasai operation for biliary atresia: a single institutional experience.

To assess the efficacy of Kasai operation for biliary atresia, 60 cases during a 14-year period (1976 to 1989) at this institution were reviewed. Thirty-four (56.7%) underwent operation within 90 days of life, 11 (18.3%) between 90 and 120 days and 15 (25.0%) beyond 120 days. Fifty-six underwent hepaticoportojejunostomy and 4 underwent hepaticoportocholecystostomy. Follow-up was 1 to 14 years (mean, 7.2 years). The postoperative follow-up of these 60 patients was categorized into five groups: group A (16 patients, 26.7%) was characterized by survival greater than 3 years and without jaundice; group B (3 patients, 5.0%) was defined as survival greater than 3 years, but with jaundice; group C (11 patients, 18.3%) was defined by survival but follow-up less than 3 years, (this group was further divided into C1 [7 patients, 11.7%] if anicteric and C2 [4 patients, 6.7%] if icteric); group D (2 patients, 3.3%) was patients who underwent liver transplantation; and group E (28 patients, 46.7%) was the group of patients who died at time of review. Group A and group C1 were interpreted as success after Kasai operation; hence, the potential success rate in this series was 38.3% (23 patients). They were long-term survivers, and most of them performed normal activities appropriate for their ages. We conclude that approximately one third of patients with biliary atresia would benefit from or be cured by this operation, which should be the first attempt in the treatment of this disease entity.

Persistent third branchial apparatus.

Neck sinuses, cysts or fistulae arising from the third branchial apparatus, have seldom been reported. Between 1979 and 1989, 16 cases were diagnosed as persistent third branchial apparatus based on a fistula open or in proximity to the pyriform sinus. There were 8 boys and 8 girls whose ages ranged from newborn to 13 years. Esophagogram was performed in 6; 4 showed a fistula tract leading to the pyriform sinus. Others were demonstrated at surgery. The main presentations were suppurative thyroiditis (5), lateral neck fistula (5), cyst (3), mass (1), abscess (1), and esophageal stricture (1). This was interpreted as a spectrum of disease that in the newborn may present as a cyst, and later in childhood as a fistula in the lower neck or recurrent suppurative thyroiditis if the fistula ends in the thyroid gland. It is imperative to search for this internal communication to the pyriform sinus to make a correct diagnosis and to avoid development of esophagocutaneous fistula postoperatively. With more awareness of this disease entity, the noted incidence should increase.