The involvement of two or more systems and the severity of associated anomalies significantly influence mortality in esophageal atresia.

PURPOSE: The aim of this study was to examine the influence of associated anomalies in babies born with esophageal atresia (EA). METHODS: A retrospective review of the records of 41 consecutive cases of esophageal atresia managed over an 11-year period was undertaken. RESULTS: A higher incidence of associated anomalies was seen in those babies with lower birth weights. Although all five (100%) babies with EA who weighed less than 1,800 g had associated anomalies, those who weighed 1,800 to 2,500 g and more than 2,500 g were associated with 67% (10 of 15) and 43% (9 of 21) anomalies, respectively. The most common system in which anomalies occurred was the cardiovascular system (37%) followed by gastrointestinal (24%), musculoskeletal (17%), genitourinary (7%), chromosomal (5%), and others (12%). All 17 (41%) babies with no associated anomalies survived. Four of the 10 babies who had two or more systems involvement died, whereas only one of 31 babies with less than two systems involvement died; the difference between these two groups was highly significant (Fisher's Exact test, P = .009). The overall mortality rate was 12%. Three of the deaths were associated with severe anomalies that were incompatible with life such as bilateral renal agenesis, trisomy 18, and complex cardiac anomalies. CONCLUSION: The association of two or more system anomalies and the severity of associated anomalies influence mortality in esophageal atresia.

Treatment of biliary atresia by portoenterostomy and liver transplantation: The Queen Mary Hospital, Hong Kong experience.

Ninety three out of 96 patients with biliary atresia (BA) underwent Kasai 1 portoenterostomy and 11 children subsequently underwent 12 liver transplantation (LTX) procedures which included 8 living related liver transplants (LRLT), 3 reduced-size liver transplants (RSLT) and 1 orthotopic liver transplantation (OLT). During a follow-up period of 3-206 months (mean, 73 months) after portoenterostomy and 3-63 months after LTX, 50% of 96 patients are alive and well with total clearance of jaundice while 12% are mildly jaundiced, 10% are deeply jaundiced and 28% have died. Two of the 3 patients who did not undergo portoenterostomy and 25 from the portoenterostomy group have died. Of the latter group, 22 deaths occurred before, and 3 after the introduction of LTX therapy respectively. Of the 68 long-term survivors, 32 are less than 5, and 36 are 6-17 years old. Complete clearance of jaundice was achieved in 55% of patients irrespective of whether portoenterostomy was initially performed at < 10 or between 10 and 12 weeks. Portoenterostomy performed beyond 13 weeks was associated with declining results. We conclude that (1) portoenterostomy combined with liver transplantation, when indicated, has given patients with BA a much better prognosis and (2) pediatric LTX in our institute is a well established procedure with 100% patient and 88% primary graft survival.

Childhood intussusception: ultrasound-guided Hartmann's solution hydrostatic reduction or barium enema reduction?

A comparison was made of the efficacy of ultrasound guided Hartmann's solution hydrostatic reduction on 23 patients (US group) with the same number of consecutive patients in whom hydrostatic reduction was done by barium enema (BE group) under fluoroscopy for childhood intussusception. The US group was diagnosed by ultrasound scan and reduction was attempted under the guidance of ultrasonography with Hartmann's solution at 100 mm Hg pressure. Excluded were patients older than 12 years, patients in shock, patients with peritonitis, bowel perforation, and gross abdominal distension as well as recurrent intussusception of more than three episodes. There were three patients excluded in this group. The diagnosis of intussusception and complete reduction were confirmed by gastrografin enema. This US group had three recurrences (3 of 26, 11.5%), one lead point (1 of 23, 4.4%), and 19 successful reductions (19 of 26, 73%). Incidentally, there were also three patients excluded in this period of barium enema reduction. There was only one recurrence (1 of 24, 4.2%), one leadpoint (1 of 23, 4.4%), and 12 successful reductions (12 of 24, 50%) in these 23 BE patients. The success rates for the ileo-colic intussusceptions with Hartmann's solution reduction and barium enema reduction were 91% (19 of 21) and 55% (12 of 22), respectively (P = .00865). There was no complication in either group, and the accuracy of diagnosing a complete reduction was 100% in both forms of reduction. Hence, ultrasound-guided hydrostatic reduction for childhood ileocolic intussusception is preferred because it is safe, accurate, has a higher success rate, and can avoid radiation exposure risk.

Liver transplantation in children: the experience of Queen Mary Hospital, Hong Kong.

Seven living-related liver transplants (LRLT) and two reduced-size liver transplants (RSLT) were performed on eight children who suffered from end-stage liver disease, having previously undergone one to three abdominal operations. Their ages at initial transplantation ranged from 8 months to 11 years (mean 35 months, median 12 months). Excluding the two older children aged 7 and 11 years, respectively, the rest of the children weighed 6 to 9.5 kg (mean 7.3 kg) at the time of the initial transplantation. Seven left lateral segments (S2 + 3) and two left lobes (S2 + 3 + 4) were used; of these the smallest graft had a graft-to-recipient body weight ratio of 0.9%. The volunteer living donors were four mothers, two fathers and one sister who were selected after medical and psychiatric evaluations, and their suitability was confirmed by hematological, biochemical, and radiological criteria. During a follow-up period of 3 to 30 months, all eight children are alive and well with normal liver function, one of them having undergone a retransplant LRLT because of hepatitis of undetermined etiology following a RSLT 1.5 years earlier. All seven donors had an uneventful postoperative course and were discharged on day 4 to 7 postoperatively. They have all resumed normal day-to-day activities. There were no complications in the donor group. A variety of complications occurred in the recipients, all of which were overcome. Operating microscope was used to perform all the arterial anastomoses using microvascular techniques. This method has proven to be a major factor in preventing arterial thrombosis even with the smallest of arterial anastomosis where a 1.5-mm diameter recipient artery was anastomosed to a 2.5-mm diameter donor hepatic artery.

Ileoileocolic intussusception in children: diagnosis and significance.

The ileoileocolic type of childhood intussusception is difficult to diagnose pre-operatively and is associated with increased morbidity. This study describes the clinical and imaging features of 10 consecutive ileoileocolic intussusceptions diagnosed ultrasonically in 10 patients over a 36 month period. Ultrasound-guided hydrostatic reduction using Hartmann's solution was attempted in all 10 patients. Clinical and imaging features were compared with those of 28 ileocolic intussusceptions in 25 patients diagnosed and treated using the same methods during the same period. Most of the clinical and plain radiographic features of the patients with the ileoileocolic and ileocolic types of intussusception were similar. The two types of intussusception had the classical doughnut or pseudokidney, or both, signs on pre-reduction ultrasound scans. During the reduction process, when surrounded by fluid within the caecal lumen, the ileoileocolic type of intussusception had a typical complex appearance due to frond-like loops of intussuscepted small bowel. This finding was present in all cases. The hydrostatic reduction rate was only 10% (1/10) for ileoileocolic intussusception, compared with 92.9% (26/28) for the ileocolic type. All unsuccessfully-reduced cases underwent surgery, with surgical confirmation of the intussusception type in all cases. Only one patient was found to have a lead point, caused by a Meckel's diverticulum. In conclusion, the diagnosis of ileoileocolic intussusception can be made pre-operatively and these patients require surgical management.

Horseshoe kidney with bilateral single system ectopic ureters.

A horseshoe kidney, associated with bilateral single system ectopic ureters in a 9-month-old boy, is reported. The right ureteric orifice was located near the midline of a deformed trigone while the grossly dilated left ureter inserted into the posterior urethra. Imaging defined this rare combination of urological anomalies prior to surgical treatment.

Sonographically guided hydrostatic reduction of childhood intussusception using Hartmann's solution.

OBJECTIVE: Currently, the standard methods for therapeutic reduction of intussusception in children involve considerable ionizing radiation. This study tested the effectiveness of sonographically guided hydrostatic reduction of intussusception using Hartmann's solution, a fluid with near-physiologic composition. SUBJECTS AND METHODS: Between March 1, 1994, and January 31, 1996, all children clinically suspected of having intussusception were evaluated by sonography. Those with positive findings on sonography were entered into the study and underwent confirmatory sonographically guided meglumine diatrizoate enema. During continuous sonographic monitoring, we used Hartmann's solution for attempted reduction of intussusception. Criteria for successful reduction were disappearance of the intussusceptum and passage of fluid through the ileocecal valve. Another sonographically guided meglumine diatrizoate enema was used to confirm successful reduction. RESULTS: We detected 25 consecutive intussusceptions in 22 patients. The patients were 12 girls and 10 boys, with a mean age of 14 months (range, 1-72 months). Sonograms revealed in all patients doughnut or pseudokidney signs or both. The sites of intussusception were the transverse colon (17 of 25), hepatic flexure (4 of 25), ascending colon (2 of 25), splenic flexure (1 of 25), and descending colon (1 of 25). Other findings were dilated fluid-filled small bowel (11 of 25) and free intraperitoneal fluid (9 of 25). The success rate of our sonographically guided attempts at hydrostatic reduction was 76% (19 of 25). Success was proven by meglumine diatrizoate enema in all 19 patients. The mean time of the reduction procedure was 18 min (range, 2-45 min). No complications occurred. All six patients in whom hydrostatic reduction was unsuccessful underwent surgery. Five of these patients had ileoileocolic intussusceptions. On sonography, when surrounded by fluid, ileoileocolic intussusceptions had a typically complex, fronded appearance. The remaining patient in whom hydrostatic reduction was unsuccessful had ileocolic intussusception. Of six ileoileocolic intussusceptions, one was hydrostatically reduced and a second was converted into an ileoileal intussusception before requiring surgery. The other four intussusceptions were surgically treated. CONCLUSION: Our data suggest that sonographically guided hydrostatic reduction with Hartmann's solution can be used to treat ileocolic intussusception and to diagnose ileoileocolic intussusception.

Associated anomalies in patients with undescended testes.

Out of 252 patients with undescended testes admitted under our care between January 1984 and September 1992, 70 had bilateral undescended testes and 182 had unilateral undescended testes. Fifty-one (20 percent) patients had at least one associated anomaly. Thirteen (25 percent) patients had more than one anomaly. The anomalies included 20 patients with urogenital disorders, eight with gastrointestinal tract anomalies, seven with abdominal wall defects, and seven with cardiovascular anomalies. In addition there were 11 patients with intersexual disorders, six patients with autosomal disorders and six patients with well-recognized syndromes. We found that the incidence of intersexual disorders among the patients with undescended testes was 4.6 percent, but the incidence was much higher (55 percent) in the group of patients with both undescended testes and hypospadias. Patients with bilateral undescended testes had a much higher incidence (46 percent) of associated anomalies than those with unilateral undescended testes (10 percent).

Cutaneous stoma in the roux limb of hepaticojejunostomy (hepaticocutaneous jejunostomy): useful access for intrahepatic stone extraction.

A cutaneous stoma in the Roux limb of hepaticojejunostomy (hepaticocutaneous jejunostomy) was used for stone extraction in two children who had hepatolithiasis (14.5 and 15.5 years, respectively) after operation for choledochal cysts. In constructing the hepaticocutaneous jejunostomy, a short, straight proximal limb from the skin to the bilioenteric anastomosis is mandatory. It provides a pathway, superior to the T-tube tract, for repeated stone extraction, which can be performed under sedation, thus obviating repeat laparotomies. The stoma allows flexible choledochoscopy, balloon dilatation of intrahepatic duct strictures, and extraction of intrahepatic stones using grasping forceps, baskets, and balloons. The electrohydraulic lithotriptor may be applied to fragment larger stones.

Does the amniotic fluid protein absorption contribute significantly to the fetal weight?

OBJECTIVE: This study was carried out to evaluate the significance of amniotic fluid protein ingestion and absorption on fetal growth. METHODOLOGY: Neonates with small bowel atresia during a 30 year period were studied retrospectively. RESULTS: There were 56 patients enlisted, 17 with duodenal atresia, 18 with jejunal atresia and 21 with ileal atresia. The percentage of mothers with polyhydramnios and the percentage of premature babies decreases as the intestinal atresia becomes more distal. The mean gestational age and the mean birthweight increase as the intestinal atresia becomes more distal. On the other hand, the percentage of the neonates with birthweight below the 50th and the 10th percentiles do not differ significantly as the intestinal atresia becomes more distal. CONCLUSIONS: It appears that the variation of birthweights in babies with different levels of small bowel atresia may be due to the difference in gestation caused by polyhydramnios. The effect of amniotic fluid protein absorption on fetal bodyweight could not be demonstrated clinically in this study.

Hemihypertrophy with a liver cyst: a case report.

An 8-month-old girl with a right-sided hemihypertrophy was found to have a large abdominal mass. At exploration, the mass, arising from the antero-inferior edge of the right lobe of the liver, was pedunculated and measured 17 cm x 12.5 cm x 12.5 cm. This was excised and histology confirmed it to be a benign single cyst of the liver.

Pancreatic pleural effusion: an indication for emergency distal pancreatectomy and Roux-en-Y pancreatico-jejunostomy.

A previously healthy 2-year-old boy was admitted because of shortness of breath, cough, and fever; there was minimal abdominal pain. He had recurrent right, followed by left pleural effusions, which contained markedly elevated amylase levels and high protein content. The pleural fluid amylase levels were disproportionately higher than the serum amylase levels. His abdominal signs were minimal. Surgical exploration showed a disruption of the proximal pancreatic duct. Distal pancreatectomy and Roux-en-Y pancreatico-jejunostomy were performed. After a complicated postoperative course he was discharged well and has remained so for more than 2 years.

Colonic hyperganglionosis presenting as neonatal enterocolitis and multiple strictures.

Neuronal intestinal dysplasia (NID) usually mimics Hirschsprung's disease, but rarely presents as neonatal enterocolitis. The authors report a case of colonic hyperganglionosis, which is a form of NID, presenting with postenterocolitis intestinal strictures. NID should be considered as a possible (although rare) cause of neonatal enterocolitis and may present with intestinal strictures.

A comparison of three induction agents in paediatric anaesthesia--cardiovascular effects and recovery.

We studied 30 children undergoing circumcision randomly allocated to receive either thiopentone 4 mg.kg-1, propofol 2.5 mg.kg-1 or midazolam 0.5 mg.kg-1 (n = 10) IV over 30 seconds at induction of anaesthesia. Blood pressure and pulse rate during the first 15 minutes of induction were recorded by a Finapres 2300e and a Cardiocap CM-104, and changes from preinduction baseline compared between the three induction agents and the two recording instruments. Postoperatively, blood levels of the induction agents were measured and recovery from anaesthesia was assessed by clinical criteria, mood and sedation scores and psychomotor performance. The Cardiocap data revealed no statistically significant haemodynamic differences between the three induction agents. Finapres data demonstrated that propofol caused a greater decrease in mean arterial pressure when compared to thiopentone at one minute (P = 0.01) and the MAP remained significantly lower than midazolam at five minutes (P = 0.02), illustrating an advantage of continuous over intermittent non-invasive blood pressure monitoring. The midazolam group took longer to identify themselves compared to both the propofol (P = 0.005) and the thiopentone groups (P = 0.02), but there was no difference in the groups in time to eye-opening. Psychomotor performance on awakening was significantly worse in the midazolam group compared to the propofol (P < 0.03) and thiopentone groups (P < 0.02). Most children had recovered to 80% of their best, practised, unmedicated, preoperative performance four hours after awakening, irrespective of the induction agent administered. Drug blood levels correlated weakly with both methods of psychomotor assessment (r > or = 0.6). Of the three induction agents, thiopentone caused the least haemodynamic perturbation on induction, and anaesthesia induced with midazolam caused the greatest psychomotor impairment on awakening. Within one hour patients in all drug groups were equally awake, co-operative and co-ordinated.

Pharmacokinetics of flumazenil and midazolam.

We have studied simultaneously the pharmacokinetics of flumazenil and midazolam in 12 healthy Chinese children, aged 5-9 yr, undergoing circumcision. Two hours before operation each patient received midazolam 0.5 mg kg-1 orally for premedication and 0.5 mg kg-1 i.v. during induction. Six minutes after cessation of anaesthesia, a bolus of flumazenil 10 micrograms kg-1 was given i.v., followed by an infusion of flumazenil at 5 micrograms kg-1 min-1 which was maintained until the child could identify himself. Midazolam data were consistent with a three-compartment model with a mean (SD) elimination half-life of 107 (30) min, total body clearance of 15.4 (3.2) ml min-1 kg-1 and apparent volume of distribution at steady state of 1.9 (0.6) litre kg-1. Flumazenil data were best interpreted by a monoexponential function, with a mean terminal elimination half-life of 35.3 (13.8) min, a total plasma clearance of 20.6 (6.9) ml min-1 kg-1 and apparent volume of distribution at steady state of 1.0 (0.2) litre kg-1. No unchanged midazolam was detected in the 24-h urine sample, but 5.8-13.8% of the flumazenil dose was recovered unchanged. At the time of self identification, 4.5 (1.4) min after flumazenil administration, the mean plasma concentrations of midazolam and flumazenil were 163.1 (43.7) and 29.9 (16.1) ng ml-1, respectively.