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.

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.

[Congenital dilatation of the choledocus. Diagnostic considerations and therapeutic indications]. / La dilatazione congenita del coledoco. Considerazioni diagnostiche ed indicazioni terapeutiche.

From 1964 to 1979, 7 children affected by congenital choledocal cyst were operated on. A cysto-duodenostomy was performed in all cases but one, in whom an excision of the cyst was directly carried on. In the light of the this experience Authors conclude that direct excision is preferable rather than a cysto-duodenostomy and furthermore possible to be arrived at basically by means of ultrasound. This latter one is considered so far the best way to reach a pre-operative conceptualization of the pathology.

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.

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.

[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.

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.

Perinatal ultrasound monitoring: early detection and treatment of congenital uropathy.

In childhood, delay in the diagnosis of congenital obstructive or refluxing urinary tract pathology can contribute to progressive renal failure. Ultrasound examination is a safe and reliable means of investigating the urinary system pre- and post-natally. We describe 13 children investigated with ultrasound during the perinatal period. In 10, pathology was identified in utero, confirmed after birth and treated surgically within 1 month. This indicates that perinatal ultrasonography is a valuable technique.

[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).

The management of hydrocolpos.

Three cases of hydrocolpos observed in the Milan Department of Obstetrics and Gynecology and of Pediatric Surgery are described. Two of them were cases of simple imperforated hymen whereas the third had a vaginal obstruction associated with microcephaly and polydactily. The diagnosis was based on vaginography and urography and the treatment on resection of the vaginal membrane in two cases and in laparatomic Leadbetter-Politano's operation in the third. The Authors stress the importance of a very early diagnosis for a definitive treatment and a good prognosis and also the interest of a follow-up of these patients.

Reliability of ultrasound for the diagnosis of hypertrophic pyloric stenosis.

An ultrasound (US) examination might be the best way to study the pylorus and its pathological states once the reliability of this technique has been demonstrated. In order to evaluate the reliability of a sonographic diagnosis of hypertrophic pyloric stenosis (HPS), 17 randomly chosen patients with HPS were examined with both ultrasonography and radiography and then operated on or followed up clinically. Rank correlation between the size and morphological features of the pylorus was found in babies with HPS using US. No false positives or negatives were obtained, and all true positives and negatives were detected. Because of this and the safety of the US technique we wish to emphasize that it should be considered the method of choice in screening infants with severe vomiting. Whenever HPS is suspected from clinical observation, ultrasonography should precede a radiological examination before surgery.

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.

Difficulties in "real-time" ultrasound diagnosis of fetal urological anomalies.

In the fetus of a diabetic woman, at the 24th week of pregnancy urological anomalies (cystic kidney malformations) were found with real-time ultrasound. At the 34th week these findings were confirmed and interpreted to be due to a lower urinary obstruction. Therefore a preterm Caesarean section was performed. In the newborn the left multicystic kidney was removed. The indications for the US screening in pregnancy and difficulties in the interpretation of urological findings of fetal and newborn ultrasonograms are discussed.

Perinatal management of exomphalos diagnosed in late pregnancy.

The intrauterine diagnosis of a huge exomphalos, which was aspirated by transuterine puncture 1 hour prior to caesarean section, is reported. The authors emphasize that this prenatal procedure is able to minimize the risk of rupturing the exomphalos sac.

Malformation of the fetal urinary system. A new obstetrical approach.

A case of prenatal diagnosis of prune belly syndrome is described. The ultrasound presumptive diagnosis was that of low urinary tract obstruction. Neonatal mortality with this defect is estimated to be approximately 50% and mainly arises from kidney damage. In order to achieve further information for obstetrical management, intrauterine bladder evacuation was performed and fetal urine production rate monitored by ultrasound. This approach is proposed as a new method of diagnosis and temporary therapy for those fetal malformations determining urinary stasis.

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.