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Background Zinner syndrome (ZS), the association of congenital seminal vesicle cyst (SVC) and ipsilateral kidney anomalies, is rarely diagnosed in childhood. This study aimed to assess presentation, imaging findings, management, and outcome of pediatric ZS. Methods Sixteen children with ZS were diagnosed and managed at our hospital from 2003 to 2021. We reviewed the medical records to collect data on initial symptoms, results of imaging studies, complications, operation, and follow-up. Results Ultrasound was used in all 16 cases as initial diagnostic tool. Fourteen patients were asymptomatic at diagnosis: these were transferred from obstetricians or pediatricians for evaluation of the prenatally or postnatally detected ultrasonic kidney anomalies. SVCs were incidentally noted on ultrasonography. The other two cases initially presented with urinary tract infection (UTI). Kidney anomalies included multicystic dysplastic kidney in 3 and kidney agenesis in 13 patients. Eleven (68.7%) patients had ipsilateral ectopic ureters entering SVC. Four (36.4%) patients had a reflux from urethra into SVC (urethro-cystic reflux) on voiding cystourethrography. Ten (62.5%) patients remained asymptomatic over a mean of 58 months (range, 7-216 months), two patients developed lower urinary tract dysfunction, and five patients had UTIs. Two boys needed SVC removal, and SVC had disappeared in two patients after 2.5-4 years of follow-up. Conclusions Unilateral kidney hypodysplasia with ectopic ureter inserting into the ipsilateral SVC is a characteristic sign for diagnosis of ZS. In our case series, ZS was mainly asymptomatic. Urethro-cystic reflux was associated with UTIs in young infants. SVC removal was rarely required. Graphical abstract A higher resolution version of the Graphical abstract is available as Supplementary information, Author(s): Chun-Chen Lin [sup.1] [sup.2] [sup.3] , Jin-Cherng Sheu [sup.4] , Pei-Shan Tsai [sup.2] [sup.3] [sup.5] [sup.6] , Ming-Dar Lee [sup.7] , Tsu-Hua Lin [sup.1] , Jeng-Daw Tsai [sup.1] [sup.2] [...]

Byline: Chee-Chee Koh (1,3), Jin-Cherng Sheu (1), Nien-Lu Wang (1), Hung-Chang Lee (2), Pei-Yeh Chang (1), Ming-Lun Yeh (1) Keywords: Intussusception; Recurrence; Ileopexy Abstract: Intussusception in children may recur after surgical correction. Ileopexy has popularly been used as a procedure to prevent recurrent intussusception, but its effect has not been well evaluated. The present study compared the incidence of recurrent intussusception after several different surgical procedures for intussusception in children. The charts of 278 children undergoing surgical reduction of ileocolic intussusception over 17 years were retrospectively reviewed. The incidence of recurrent intussusception was compared among the three surgical procedures: simple manual reduction, manual reduction plus ileopexy, and segmental resection. Simple manual reduction was performed in 67 (24.1%) of the 278 patients, manual reduction plus ileopexy in 186 (66.9%), and resection of the involved segment in 25 (9.0%). There were four episodes of recurrence in three (4%) patients who had had manual reduction, nine episodes in eight (4%) patients who had undergone ileopexy, and none after segmental resection. There was no statistical difference in recurrent rate among the three groups (reduction vs. ileopexy, P = 0.95 reduction vs. resection, P = 0.28 ileopexy vs. resection, P = 0.29). Ileopexy is not better than simple manual reduction in preventing recurrence of ileocolic intussusception in children. Author Affiliation: (1) Division of Pediatric Surgery, Mackay Memorial Hospital, No. 92, Chung-Shan North Road Sec. 2, Taipei, 10449, Taiwan (2) Division of Pediatrics, Mackay Memorial Hospital, Taipei, Taiwan (3) Mackay Medicine, Nursing and Management College, Taipei, Taiwan Article History: Registration Date: 19/07/2006 Accepted Date: 13/07/2006 Online Date: 05/08/2006

Byline: Jin-Cherng Sheu (1,3), Chee-Chee Koh (1,3), Pei-Yei Chang (2), Nien-Lu Wang (1), Jeng-Daw Tsai (1), Tsuen-Chiuan Tsai (1) Keywords: Ureteropelvic junction; Pyeloplasty; Outcome Abstract: We reviewed our experience over 10 years with pyeloplasty for ureteropelvic junction obstruction. Preoperative and postoperative isotope renal scans were performed to assess the surgical outcome, with particular emphasis on the postoperative change in renal function (RF). We retrospectively reviewed the medical records of 137 consecutive children with ureteropelvic obstruction operated between January 1994 and December 2003. A total of 109 kidneys in 102 patients were evaluated with renal echo and 99m.sup.Tc-diethylenetriaminepentaacetic renography both before and after Anderson--Hynes dismembered pyeloplasty. The improvement of RF >5% was noted in 67 kidneys (61.5%), the function remained stable in 30 kidneys (27.5%), and it deteriorated (>5%) in 12 kidneys (11.0%). Five patients (4.6%) required repeat pyeloplasty for either prolonged urine leakage or reobstruction with deterioration in RF. Dismembered pyeloplasty in children with ureteropelvic junction obstruction is safe. The renal functional improvement can be expected in the majority of kidneys that have impaired function at presentation. Author Affiliation: (1) Division of Pediatric Surgery, Division of Pediatrics, Mackay Memorial Hospital, No. 92, Chung-Shan North Road Sec 2, Taipei, 10449, Taiwan (2) Division of Pediatric Surgery, Chang-Gung Children's Hospital, Taipei, Taiwan (3) Mackay Medicine, Nursing and Management College, Taipei, Taiwan Article History: Registration Date: 13/04/2006 Accepted Date: 05/12/2005 Online Date: 16/05/2006

Byline: Jin-Sheng Wang (1), Hung-Chang Lee (2), Fu-Yuan Huang (3), Pei-Yeh Chang (4), Jin-Cherng Sheu (4) Keywords: Hirschsprung's disease; Unexpected death; Soave's endorectal pull-through; Thomsen antigen; T-antigen Abstract: Hirschsprung's disease is characterized by lower intestinal obstruction. In this retrospective study, we investigated the postoperative complications and the causes of death in pediatric patients with Hirschsprung's disease. Between January 1981 and June 1998, 147 patients with Hirschsprung's disease underwent surgical intervention (129 with both colostomy and Soave's pull-through procedures) at Mackay Hospital. Of 147 total patients, 83.67% had aganglionosis in the rectosigmoid, 5.44% had colon involvement, and 4.08% had total colon aganglionosis, documented pathologically after a Soave's pull-through procedure. Any post-pull-through patient who showed symptoms of fever, leukocytosis, diarrhea, and clinical sepsis was diagnosed with Hirschsprung's enterocolitis. Four patients died within 4.5 months of surgery, and one patient died more than 4 years after surgery these five patients were positive for Thomsen (T-) antigen. One patient died soon after rectal irrigation was performed in preparation for the surgery. All five of these patients had sepsis clinically, and anaerobic sepsis was attributed as the main cause of their deaths. Of interest in these cases is the presence of T-antigen, a potentially useful marker for anaerobic bacterial infection that may not be well known to most pediatricians. We recommend (1) determining the presence of T-antigen in patients with recurrent Hirschsprung's enterocolitis, (2) administering empirical antibiotics with anti-anaerobic activity as soon as possible, and (3) irrigating the colon in those patients who develop sepsis after a pull-through procedure. Author Affiliation: (1) Department of Pediatrics, Kaohsiung Municipal United Hospital and Kaohsiung Medical University, Taiwan (2) Department of Pediatrics, Mackay Memorial Hospital and Taipei Medical University, Taipei, Taiwan (3) Department of Pediatrics, Mackay Memorial Hospital, Taipei, Taiwan (4) Department of Pediatric Surgery, Mackay Memorial Hospital, Taipei, Taiwan Article History: Registration Date: 29/04/2004 Accepted Date: 03/04/2003 Online Date: 04/06/2004

Byline: Nien-Lu Wang (1), Hung-Chang Lee (2,3), Ming-Lun Yeh (1), Pei-Yeh Chang (1), Jin-Cherng Sheu (1) Keywords: Hirschsprung's disease; Laparoscopy; Endorectal pull-through Abstract: From October 1996 to July 2001, 61 patients received primary laparoscopy-assisted endorectal pull-through (LAERPT) for biopsy-proven Hirschsprung's disease (HD) at Mackay Memorial Hospital. The patients' ages at operation ranged from 7 days to 8 years. The patients were followed up for an average of 3.8 years (range 1--5.5 years). Major complications occurred in five (8.1%) patients, of whom four had surgical complications (two instances of anastomotic leakage, one colon perforation, and one delayed formation of colovesical fistula) and one had postoperative Salmonella infection-induced colonic stricture. The five patients required a diverting colostomy and a redo-pull-through procedure. Postoperative enterocolitis developed in 13 (21%) infants. All of them had enterocolitis before the diagnosis of HD was established. The majority of the episodes of postoperative enterocolitis were mild and resolved spontaneously, but four neonates were hospitalized with significant systemic manifestations, and two of them needed a second operation to solve the problem. After primary LAERPT, stooling frequency in young infants declined rapidly from 10.5+-3.2 to 4.4+-1.6 bowel movements per day in the first 3 months and more slowly thereafter. Most of these infants had regular bowel movements one to two times per day 1 year after operation. Continence evaluation of the 43 patients over 3 years of age was graded as normal in 24 (56%) patients and good in 19 (44%) patients. Of the 13 patients between 1 and 3 years of age, 11 (85%) had regular normal stools, and two (15%) had occasional soiling. From the current study, the authors concluded that primary LAERPT is a safe and effective method of managing HD with excellent continence results. Considering the complications of surgery and postoperative enterocolitis, patients with long segment aganglionosis, severe enterocolitis, or prominently dilated colon are not good candidates for primary LAERPT. Author Affiliation: (1) Department of Pediatric Surgery, Mackay Memorial Hospital, 92, Section 2, Chung-San North Rd., 10449, Taipei, Taiwan, Republic of China (2) Department of Pediatrics, Mackay Memorial Hospital, Taipei, Taiwan, Republic of China (3) Department of Pediatrics, Taipei Medical University Hospital, Taipei, Taiwan, Republic of China Article History: Registration Date: 21/10/2003 Accepted Date: 01/05/2003 Online Date: 24/01/2004

Byline: Chee-Chee Koh (1), Jin-Cherng Sheu (1) Keywords: Key wordsaIntra-abdominal lymphangioma; Hepatic lymphangioma Abstract: A patient presented with a huge, pedunculated abdominal cystic lymphangioma arising from the quadrate lobe of the liver near the round ligament. Microscopically, dilated hepatic ducts with scant liver tissue could be recognized in the main cyst. A review of the literature reveals no previous report of a lymphangioma arising in this manner or from this area. Author Affiliation: (1) Department of Pediatric Surgery, Mackay Memorial Hospital, No. 92 Chung-Shan North Road Sec 2, Taipei, Taiwan 10449, Republic of China, CN Article note: Accepted: 31 June 1999