Nien-Lu Wang

Clinical Spectrum of Alimentary Tract Duplication in Children

UNLABELLED Alimentary tract duplication is a rare congenital malformations. Sixteen patients admitted to our pediatric ward were enrolled in a ten-year study over the most recent decade. In this paper, we trace their history and retrospectively analyze sex, age, clinical presentations, duplication size and location, presence of ectopic tissue, complications and additional associated anomalies and prognosis. The duplications were cystic in all cases except one tubular duplication of the colon. The cases included duplication of the stomach in 3 cases (19%); ileum in 12 cases (63%); appendix in 1 case (5%); colon in 3 cases (16%); and rectum in 1 case (5%). The most frequent symptom was intermittent vomiting, half of which occurred before 1 year of age. Other symptoms included abdominal pain (50%), abdominal distension (30%), palpable mass (20%), peritoneal sign (13%), bloody stool (6%), fever (6%), and constipation (6%). The outcome was good, except in 2 patients, 1 of whom died of a perforation of the sigmoid colon duplication, and another who was complicated with multiple congenital anomalies. CONCLUSION In our series, the average time period from the initial onset of symptoms to the diagnosis was 6.9 days, with only one exception (a 17-year-old girl who had habitual constipation). The constipation in the single exception was not a specific presentation, which may have delayed the diagnosis. Pediatric patients with unexplained gastrointestinal symptoms must be diagnosed for the possibility of alimentary tract duplication, even though it rarely occurs. Ultrasonography is helpful in diagnosing a duplication, as it will identify a three-layered image representing the duplication cyst, common wall, and outer bowel wall. These features have proven useful for establishing the correct diagnosis or ruling out alimentary tract duplication where surgery was indicated anyway.

Neonatal gastric perforation: report of 15 cases and review of the literature.

BACKGROUND Gastric perforation among neonates is a rare but frequently fatal condition of uncertain etiology. The aim of this study was to review the clinical course of neonatal gastric perforation and to evaluate possible prognostic factors. METHODS We retrospectively analyzed the medical records of 15 patients with neonatal gastric perforation over a 19-year period. Another 97 patients described in the medical literature, for whom the gestational ages and birth weights were clearly stated, were also reviewed. RESULTS In our series, there were three girls and 12 boys, nine of whom were full-term infants and six preterm infants. The most common initial manifestations were poor activity, abdominal distension, and respiratory distress. The overall mortality was 47% (7/15). Prematurity was the only statistically significant risk factor; 83% (5/6) of premature infants died compared with 22% (2/9) of term babies (p < 0.05). Combining our series with the patients reported in the literature, there were a total of 50 premature infants and 62 term infants. Gastric perforation occurred on postnatal days 2-7 and presented with nonspecific manifestations. The mortality was significantly higher in premature than in term infants (31/50, 62% vs. 16/62, 26%; p < 0.001). A trend towards higher mortality in infants with lower birth weights was observed (>2500 g, 28%; 1501-2500 g, 52%; 1000-1500 g, 60%; <1000 g, 100%). Infants with birth weights <2500 g had a significantly higher mortality than infants with birth weights >2500 g (32/58, 55% vs. 15/54, 28%; p<0.05). CONCLUSION Neonatal gastric perforation is associated with high mortality, particularly in premature infants. There is also a trend towards higher mortality in lower-birth-weight infants.

Experience with primary laparoscopy-assisted endorectal pull-through for Hirschsprung's disease

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.

Gastro-intestinal bleeding caused by leiomyoma of the small intestine in a child with neurofibromatosis.

Abstract Gastro-intestinal bleeding is an uncommon presentation in children with neurofibromatosis. Gastro-intestinal involvement caused by jejunal leiomyoma has only been described in adults. To the best of our knowledge, this is the first paediatric case of jejunal leiomyoma associated with neurofibromatosis. We present a 10-year-old girl with a 9-month history of anaemia and low gastro-intestinal bleeding. Abdominal sonography and small bowel series showed a submucosal mass in the proximal jejunum. On surgery, a submucosal tumour was excised and histological examination suggested a diagnosis of “smooth muscle tumour of undetermined malignant potential”. There were no recurrence of symptoms for 4 years after the operation. Conclusion Jejunal leiomyoma should be considered in a child with neurofibromatosis presenting with gastro-intestinal bleeding.

Sonogram before and after pyloromyotomy: the pyloric ratio in infantile hypertrophic pyloric stenosis.

BACKGROUND Sonography is used to diagnose infantile hypertrophic pyloric stenosis, but Little information is available about the appearance of postoperative sonographs. The purpose of this study was to evaluate the morphology of the pylorus in association with an obstruction before and after pyloromyotomy. METHODS Pyloric length, diameter, muscle thickness and intermuscular space were measured sonographically at diagnosis and daily after pyloromyotomy until discharge in 12 infants with infantile hypertrophic pyloric stenosis. The ratios of pyloric wall thickness and intermuscular space to the entire pyloric diameter were measured. RESULTS The pylorus still appeared hypertrophied after pyloromyotomy on the sonograms. The traditional measurement of linear dimensions of the pylorus was not significantly changed from the preoperative values by the time of discharge, except for muscle thickness. The intermuscular space increased from 4.8+/-0.8 mm preoperatively to 7.3+/-2.1 mm by postoperative day 3 (p=0.10). Lowes pyloric ratio at diagnosis was a mean of 0.32, decreasing to 0.29 on postoperative day 3 and 0.29 on the day of discharge (p=0.82). The alternative pyloric ratio increased significantly by postoperative day 2 (0.24+/-0.09 on day 2 vs. 0.11+/-0.07 preoperatively, p=0.02). CONCLUSION The pyloric ratio appears to be a reliable parameter in evaluating the regression of pyloric stenosis after pyloromyotomy, and also aids in the diagnosis of pyloric stenosis.

Torticollis: A Long-term Follow-up Study

To achieve better guidelines for the future management of torticollis, this study analyzed surgical and nonsurgical management of 253 torticollis patients who were treated in this hospital from 1971 to 1993. Of those, 37 cases received operation only, 78 cases were operated after failed physical therapy, and 138 cases were treated only at the Rehabilitation Department. If free neck movement was considered to be the primary goal of treatment, most parents were satisfied with the results. However, if facial and skull deformities were the serious sequelae of torticollis, then only less than half of the surgical and nonsurgical groups of patients were graded as normal. Further, 10.9% of physical therapy group and 7% of the surgical patients need further operation to release the fibrotic bundle which limited their neck movement. Therefore, it is suggested that torticollis treatment should include early interventions such as adjusting sleep position, careful planning of physical therapy and/or operation and a long term follow-up period as essential for better management of torticollis.

Cystic lymphatic malformation of the pelvis mimicking seminal vesicle cysts

A 6-year-old boy presented with fever of 2 days of duration and had blood tests demonstrating leukocytosis and impaired liver function. Abdominal sonogram incidentally revealed 2 cystic lesions within the pelvis. Further contrast-enhanced abdominal computed tomography showed a symmetrically bilobed cystic mass between the urinary bladder and rectum in the location of the seminal vesicles. The boy underwent surgical excision, and the histopathology confirmed the mass as a cystic lymphatic malformation originating from the mesentery of the sigmoid colon.

An unusual neurogenic cystic tumor

Abstract.The presacral cystic teratoma in an infant is reported. The cyst consisted solely of central nervous system tissue. This pathologic finding is extremely rare. A review of the literature reveals no previous report of a teratoma arising in this manner.

VACTERL Association Complicated with Right-sided Congenital Diaphragmatic Hernia

We describe a neonate with VACTERL association and right-sided congenital diaphragmatic hernia (CDH). Such coexistence is rare. The lack of symptoms during the early neonatal period, the absence of bowel loops herniated into the right thoracic cavity, and an unfinished surgery led to clinical and radiological diagnostic difficulties. Respiratory distress occurred when the patient was 2 months old. Chest radiology plain film revealed typical findings of right-sided CDH. The diagnosis was confirmed after surgical exploration.

Excision of Mullerian duct remnant for persistent Mullerian duct syndrome provides favorable short- and mid-term outcomes

OBJECTIVE In dealing with persistent Mullerian duct syndrome (PMDS), excision of Mullerian duct remnant (MDR) has been rarely mentioned in the past, but recent discussions have taken place. This study aimed to evaluate the operative feasibility and outcomes. MATERIALS AND METHODS Three patients with PMDS operated on with excision of MDR between 2000 and 2009 were enrolled. Medical records were retrospectively collected and reviewed. RESULTS Bilateral undescended testis was manifested in all cases. Two patients presented with incarcerated hernia, requiring emergency herniorrhaphy at the ages of 6 months and 10 days, respectively. Reconstruction comprising simultaneous MDR excision and orchiopexy was made at the age of 1 year. MDR was incidentally found in another patient during operation for undescended testis. Immediate reconstruction was accomplished. Follow-up periods were 12.0, 3.5, and 2.5 years, respectively. Worse outcomes were noted on the two testes with repeated operations for incarcerated hernias, whereas the outcomes on the other four testes with a single operation were favorable. CONCLUSIONS Excision of MDR is technically feasible, and provides favorable outcomes in cases of a single operation. For experienced surgeons, immediate reconstruction should be the priority when this abnormality is incidentally encountered at an age suitable for orchiopexy.