Applications of nuclear magnetic resonance in paediatric surgery: magnetic resonance cholangiopancreatography and new sequences.

The aim of the study was to evaluate fast magnetic resonance cholangio-pancreatography (MRCP) sequences as an alternative and safe investigation method for neonatal and children's pancreaticobiliary diseases. Between January 2000 and December 2000, five children (age: 1 month 14 years; mean: 7 years) affected by pancreaticobiliary diseases or already operated for biliary pathologies were studied. Patients were evaluated by 1.5 T magnet single shot T2-weighted sequences (1 image per s, TR = infinite, TE = 150-180 ms). T1-weighted conventional sequences were obtained to study parenchymal tissue. No patient needed general anaesthetic. Only in one case was sedation necessary. Fast MRCP sequences provided very precise information on biliary tract anatomy. They revealed the intra and extrahepatic bile ducts, the gallbladder, the common bile duct and the bilio-pancreatic junction in all cases investigated. MRCP allowed us to evaluate Roux-en-Y type bilio-enteric anastomosis as accurately as percutaneous transhepatic cholangiography (PTC). In addition MRCP was the only reliable study in evaluating Roux-en-Y type anastomosis where ultrasonography (US) and endoscopic retrograde cholangiography (ERCP) could not be used. In conclusion MRCP is an accurate and non-invasive method with which to investigate the anatomy of the pancreaticobiliary tract in children. It could become the investigation of choice after US in the case of biliary and pancreatic diseases.

Long-term evaluation of esophageal function in patients treated at birth for esophageal atresia.

Dysphagia, gastroesophageal reflux (GER) and esophageal metaplasia are reported with various incidence in the long term follow-up of patients treated at birth for esophageal atresia (EA). To evaluate the long term outcomes 26 patients treated at birth for EA with Tracheo Esophageal Fistula (TEF) were examined 8-28 (mean 15.8) years later by clinical evaluation, including barium meal, fiberoptic upper GI endoscopy, 24 hour ambulatory two-channel pH-monitoring and stationary esophageal manometry. 50% of patients complained of dysphagia. Mild esophagitis was found in 20% of patients but GER was detected in only 16.7% of the cases. By morphological X-ray, esophageal anomalies were detected in 31% of cases without significant functional relevance. Hundred percent of patients had a disorganized peristaltic esophageal activity and a low amplitude of the esophageal contractions was observed in 58% of them. In our series, esophageal dismotilty seems to be the main consequence of EA without any relevant disturbance of normal nutritional habit.

[True splenic cysts. Two clinical cases]. / Le cisti spleniche vere. Due casi clinici.

Two case reports of epidermoid cyst of the spleen are presented. Only 700 cases have been reported so far. Although splenic cysts are benign lesions and could involve spontaneously, the risk of rupture of a large cyst is high, so a diameter of 5 cm was suggested as the higher limit for nonsurgical treatment. For many years the surgical treatment of splenic epidermoid cysts was splenectomy: however recognition of the short and long-term complications of radical splenectomy had led to the practice of splenic preservation (partial splenectomy, total cystectomy, laparoscopic cyst decapsulation).

Video-assisted thoracoscopic resection of pulmonary sequestration in an infant.

Pulmonary sequestration is a congenital anomaly of lung parenchyma that can be definitively treated only with surgical resection. We report a case of an intralobar sequestration of the right lower pulmonary lobe in an infant successfully treated with video-thoracoscopic surgical removal of the involved lobe at 6 months of age.

Congenital renal damage associated with primary vesicoureteral reflux detected prenatally in male infants.

To assess the course of vesicoureteral reflux, we performed cystography, renal scintigraphy, and urography in all neonates with the prenatal diagnosis of renal pelvic dilation and revealed the presence of primary reflux (grades I to V) in 27 cases. Higher grades of reflux were associated with congenital renal damage, as shown by reduced tracer uptake during scintigraphy. Reflux was diagnosed more frequently in male infants (male/female ratio, 6:1), in many of whom bladder abnormalities were found by cystography. In another group of seven infants, in whom the reflux was associated with other urologic abnormalities, there was no sex prevalence. We conclude that severe primary reflux associated with hydronephrosis usually affects male infants and may be due to abnormal embryologic development of the male urethra, and that the kidney damage is primary and not the result of urinary tract infections. This pattern differs from that of vesicoureteric reflux diagnosed at an older age, which is observed most commonly in female patients.

Prenatal diagnosis of gastrointestinal obstruction: a correlation between prenatal ultrasonic findings and postnatal operative findings.

The impact of prenatal sonographic diagnosis of oesophageal and gastrointestinal obstructions has been analysed over a 10-year period. Three groups of patients were evaluated. The first group consisted of 46 newborns with abnormal prenatal sonograms, 41 of which were confirmed to have intestinal obstruction postnatally. The second group consisted of 17 neonates with normal prenatal sonograms who had intestinal obstruction postnatally. The third group included 56 newborns who did not undergo a prenatal sonogram but who had intestinal obstruction confirmed at surgery. Polyhydramnios without the appearance of a stomach on ultrasound was diagnostic of pure oesophageal atresia. Polyhydramnios with intestinal dilation was diagnostic of intestinal obstruction. Although surgery was performed earlier in the infants diagnosed prenatally with ultrasound, mortality was no less than in the group that did not undergo a prenatal sonogram, probably because of the high incidence of associated anomalies.

Prenatal ultrasonography and early surgery for congenital cystic disease of the lung.

With the recent advent of prenatal ultrasound as a routine screening procedure, diagnosis of congenital cystic lung disease has been made in utero, raising the possibility of elective surgery for these lesions early in infancy before the patient develops respiratory distress or potentially life-threatening infection. From 1979 to 1989 six cases of congenital lung cyst were diagnosed in utero by prenatal ultrasound and followed during pregnancy. Two of the six were not confirmed after birth because the mothers preferred an abortion. The remaining four cases were studied periodically during gestation by ultrasonography. At birth, the first infant developed respiratory distress and underwent urgent left upper lobectomy and left lower segmentectomy at age 18 hours. The other three underwent elective lobectomy at age 10 days, 3 months, and 7 months, respectively. The fourth infant had a normal chest x-ray and ultrasound at birth, and the congenital cysts were confirmed by computed tomography scan. The pathological diagnosis in all four cases was cystic adenomatoid malformation. In two cases, intraoperative measurement of pulmonary function demonstrated significant improvement after resection of the affected lobe. We conclude that congenital lung cysts can be accurately diagnosed by prenatal ultrasound "screening" as early as 18 to 24 weeks' gestation. Advantages of early diagnosis include the option of moving the mother and unborn child to a high-risk obstetrical center for urgent operation on the newborn infant if necessary. Otherwise, once the diagnosis is confirmed, surgical correction can be performed electively and safely before respiratory distress or pulmonary infection complicates the infant's growth and development.

Predictability of duration and severity of congenital hydronephrosis as a cause of smooth muscle deterioration in pyelo-ureteral junction obstruction.

Between 1989 and 1990, 31 new unilateral PUJO, prenatally diagnosed (16th-37th week of gestational age), underwent pyeloplasty between 19 and 105 days of age. The correlation between severity (V) (volume of hydronephrotic kidney) and duration (dT) of congenital hydronephrosis from its first detection in utero to surgery and histological findings were prospectively evaluated in this series. A dT longer than 70 days and a V larger than 50 ml., were considered as predictable for hypotrophy with fibrosis of the upper urinary tract smooth musculature. In conclusion, time seems to play an important role in the increase of the volume of the obstructed urinary tract to the extent of a large-scale hydronephrosis.

[Ureteric peristalsis in congenital vesico-ureteral pathology. Integrated sonographic and scintigraphic studies]. / La peristalsi ureterale nella patologia congenita del giunto uretero-vescicale. Studio integrato ecografico e scintigrafico.

Ureteric peristalsis is a key point for urinary progression. Its preoperative evaluation is important to assess indications for surgery. The study of the distal ureter is possible by means of ultrasound. Renal scintigraphy allows for a complete study of urinary progression along the ureter. A combined ultrasound and 99mTc-MAG3 preoperative work-up, has been performed in 32 urinary tracts (16 children ranging in age between 45 days and 11 years). Urinary progression occurs whenever peristaltic contraction makes the ureter lumen unpatent.

Increased sodium requirement following early postnatal surgical correction of congenital uropathies in infants.

Serum electrolyte equilibrium and plasma aldosterone concentrations were monitored in 19 infants who had severe obstructive uropathy or grade 5 vesico-ureteral reflux and were undergoing surgical correction in the first 2 months of life. Before surgery high plasma aldosterone levels were observed in 8 patients, but serum sodium and potassium concentrations were normal. Plasma concentrations of aldosterone were elevated in all patients during the week following surgery and 7 patients developed severe hyponatraemia, hyperkalaemia and weight loss despite very high plasma aldosterone concentrations. As a consequence 5 infants were infused with sodium chloride (4 mEq/kg per day) before and for 36 h after surgery; this prevented metabolic imbalance. We conclude that infants undergoing surgical correction of uropathies may require a high sodium intake to maintain electrolyte balance and adequate growth.

Duration and severity of congenital hydronephrosis as a cause of smooth muscle deterioration in pyelo-ureteral junction obstruction.

A potential relationship between duration and severity of obstructive hydronephrosis and the histological structure of the upper urinary tract smooth musculature has been studied in a series of prenatally diagnosed cases. Between 1981 and 1988, fifty-five monolateral pyelo-ureteral junction obstructions (PUJO), prenatally diagnosed (16th-37th week of gestational age), underwent pyeloplasty between 4 and 90 days of life. Gestational age at the diagnosis, delta t (duration of hydronephrosis from first detection in utero to surgery) and V (volume of the hydronephrotic kidney) were related to the frequency of muscle hypotrophy and fibrosis of both pelvis and ureter just above and below the junction. A close correlation between duration (delta t) and the severity of the involvement of the smooth musculature of the upper urinary tract was found. The larger the volumes (V), the more severe the muscular damage. In the long-term follow-up, poor results were obtained only in 5 of the 19 newborn who had severe hypotrophy and fibrosis of the pelvis. In conclusion, in PUJO, the shorter the delta t and the lower the V, the better one expects the smooth musculature of the upper urinary tract to be.

Persistent tubular resistance to aldosterone in infants with congenital hydronephrosis corrected neonatally.

Renal function of 18 infants who had undergone surgery in the neonatal period because of severe congenital hydronephrosis was followed up for 5 to 36 months (mean +/- SD 21 +/- 10 months). In all cases the diagnosis was made prenatally by sonography and confirmed at birth by intravenous urography. Creatinine clearance developed normally in all the children. Eight had a reduction in maximal urinary concentrating ability after intranasal DDAVP; this defect was transient and resolved after 4 to 5 months in all but one child, in whom it persisted. However, other tubular abnormalities were present. Throughout the observation period, patient serum potassium concentrations were significantly higher than normal, paralleled by a significant increase in plasma aldosterone concentration but with normal excretion fraction of sodium and potassium. There were no disturbances of acid-base balance. These findings may be accounted for by a persistent partial reduced sensitivity of the distal tubule to the action of aldosterone despite normal renal function. This alteration is usually mild, but may constitute a persistent metabolic risk despite successful surgical intervention.

Perinatal management of fetal hydronephrosis with normal bladder.

This report covers 30 cases of prenatal diagnosis of uni- or bilateral hydronephrosis not associated with an overdistended bladder. Oligohydramnios was observed only in four cases while polyhydramnios occurred in three affected fetuses. In no case was fetal urine aspirated or drained prenatally. Early delivery was performed in four cases with oligohydramnios. Sixteen newborns required surgical correction of the lesion as it was confirmed by urography, following at least two sonographic examinations which confirmed a moderate to severe hydronephrosis after birth. The most frequent lesion was pyelo-ureteric junction obstruction (12 cases). Thirteen cases were normal at follow-up, and in two of these the dilation cleared up during intrauterine life. One case of severe hydronephrosis proved to be a multicystic kidney. In this series isolated hydronephrosis, both uni- or bilateral did not result in fetuses being at high risk for survival (only one infant died after surgery) nor as regards to associated malformations and perinatal morbidity. Provided a properly timed surgical correction was performed, renal function resulted to be good at follow-up.

Antenatal ultrasound diagnosis of congenital malformations of the urinary tract: results and criticism.

Fifty-five congenital malformations of the urinary tract (25%) were among 220 antenatally diagnosed malformations. Accuracy of diagnosis was 63.8% (44 cases); 20 infants had to be operated on. Good morphological and functional results could be obtained in 82% of the cases. Prenatal diagnosis of congenital uropathies provides early treatment burdened with less risk.

[Consequences from prenatal sonography of urinary tract abnormalities]. / Konsequenzen aus der pränatalen Sonographie von Harnwegsfehlbildungen.

At the Gynaecologic Clinic of the University of Milan/Italy from 1979-1983, pathological changes of the urinary tract were diagnosed in 55 foeti on the occasion of routine sonography of pregnants. In 23 children prenatal diagnosis was confirmed. 5 neonates had another disease than the disease of the urinary tract assumed during the prenatal period. In 19 neonates the first diagnosis had to be rescinded because of a second examination showing normal findings. 8 neonates could not be examined a second time. Advantages of an intrauterine diversion of urine could not be verified. Therefore, the following diagnostic and therapeutic consequences from the prenatal examination by sonography are recommended: Immediate check and analysis of prenatal findings in the neonatal period by further examinations. Short-term checks to clarify indication for operation. Avoidance of early complications and other kidney changes by early therapy.

The diagnostic value of perinatal ultrasound monitoring for foetal abdominal mass.

In the past two years seven cases of foetal abdominal mass were observed prenatally and operated on within 30 days of life. In four of seven cases no abdominal mass was ever palpable neither at birth nor under anaesthesia just before laparotomy, whereas in all cases it was both sonographically and surgically detected. The authors stress the importance of perinatal ultrasound monitoring to cope early with clinically undetectable abdominal mass. Furthermore, they emphasise that the necessary condition for definition of a mass should by now be that it is palpable and seen via ultrasound, but a perinatal ultrasound image of mass is sufficient to warrant careful detailed clinical evaluation.

Correlation between fetal and maternal serum bile acid concentrations.

Serum concentrations of different bile acids (BA) were determined by radioimmunoassay in 56 human fetuses and mothers. Serum was obtained immediately after legal abortion, performed between the 14th and the 21st wk of gestation. Conjugated cholic (CCA) and chenodeoxycholic acid (CCDCA) concentrations were determined in 33 cases, conjugated lithocholic (CLCA) and deoxycholic acid (CDCA) in 20, and sulfolithocholyglycine (SLCG) in 15. In fetal blood, mean concentrations of CCA (0.80 +/- 0.40 mumol/liter), CCDCA (4.50 +/- 2.70 mumol/liter), and CLCA (1.70 +/- 1.04 mumol/liter) were significantly higher than those in the mother (CCA 0.34 +/- 0.17 mumol/liter; CCDCA 0.79 +/- 0.34 mumol/liter; CLCA: 0.70 +/- 0.30 mumol/liter; p less than 0.001); fetal serum levels of CDCA (0.46 +/- 0.32 mumol/liter) and SLCG (0.15 +/- 0.09 mumol/liter) were lower than in the mothers (CDCA 1.20 +/- 0.80 mumol/liter, p less than 0.001; SLCG 0.40 +/- 0.30 mumol/liter, p less than 0.01). There was no correlation between levels of BA and gestational age. Serum total protein and albumin concentrations were both reduced in 10 fetuses as compared with the mothers. These data support the concept of a state of physiologic cholestasis during development and suggest that placental transfer of primary BA occurs mostly in the fetal to maternal direction. This transfer could be facilitated by the reduced fetal plasma albumin concentration, since BA in free solution diffuse more easily through the placenta. There is evidence of lithocholic acid synthesis in the fetal liver, while deoxycholic acid appears to be mostly of maternal origin. Finally, sulfation of BA is poorly developed at this age of gestation.

The usefulness of ultrasound in the post operative management of hydronephrosis.

The renal tracts of thirty-four children aged between 6 months and 12 years, with vesico-ureteric reflux or obstructive hydronephrosis were examined by ultrasound (US) pre and post operatively, in an attempt to determine the validity of US as an alternative means of diagnostic imaging. The results show the technique to be both accurate and reproducible and we therefore believe that US is a reliable procedure for monitoring urinary tract morphology in patients with hydronephrosis or in the post operative assessment of vesicoureteric reflux.