Nonparasitic splenic cysts in children: a multicentric study.

PURPOSE: Nonparasitic splenic cysts (NPSCs) are uncommon in children. The aim of this multinational and multicentric study was to present the authors' experience as well as the changing trends in the management of NPSCs over the last 25 years. MATERIAL AND METHODS: From 1981 to 2005, 50 children or adolescents were surgically treated for NPSCs in 6 paediatric surgical centres in four European countries. The medical records of these 50 patients with NPSCs were reviewed retrospectively. RESULTS: Twenty-six male and 24 female patients were operated on. Age at surgery ranged from 1 to 17 years (mean 11.9). Seventeen patients were symptomatic. Six total (4 open and 2 laparoscopic) and 26 partial (22 open and 4 laparoscopic) splenectomies were performed. Laparoscopic fenestration or deroofing and open cystectomy was carried out in 9 patients, respectively. Histological findings revealed the lesion to be an epidermoid cyst (n = 28), a pseudocyst (n = 15) or a mesothelial cyst (n = 2). In 5 patients haemangioma or lymphangioma was the pathological diagnosis. At a mean follow-up of 2.9 years, residual cysts were found in 8 laparoscopically treated patients, 4 of whom required re-do laparoscopy or open surgery. CONCLUSIONS: Over the last two decades, the surgical treatment of NPSCs has changed from a formerly customary total splenectomy to spleen-conserving procedures, such as total cystectomy with or without partial splenectomy or partial cystectomy. These therapeutic modalities can be performed laparoscopically, if technically possible. Fenestration or deroofing of the cyst resulted in a high recurrence rate (7/9).

Cavernous transformation of the portal vein causing jaundice, presenting in the form of Wilson's disease.

The following is a case review of portal vein cavernous malformation presenting with intermittent cholestasis and jaundice in a 4 year old child. Correct assessment was supported by radiology, later laparoscopy, yet hindered by histopathology representative Wilson's disease and elevated urinary copper excretion. During surgical procedure the stenosis of the common bile duct secondary to extremely dilated portal vein reticulation was solved by Roux-en-Y choledochojejunostomy. After a one-year follow up the child remains asymptomatic.

Changes in gastric myoelectrical activity in hypertrophic pyloric stenosis and after surgical correction.

The changes of gastric myoelectrical activity were investigated in 20 infants by cutaneous electrogastrography (EGG) before and after the surgical correction of infantile hypertrophic pyloric stenosis (IHPS). The dominance of 2-4 cycles per minute (CPM) "slow waves" is typical of the healthy gastric function. The shift of the dominant frequencies towards the slower frequency (0-2 CPM) is defined as bradygastria, whereas a shift towards the more frequent waves (4-10 CPM) is called tachygastria. Unlike with healthy infants, the electrogastrogram showed pathologic patterns in 85% (18 out of 20) of IHPS patients. In all except two of these infants with pathologic electrical patterns, the frequency of the waves significantly shifted towards tachygastria. The effect of feeding on the gastric myoelectrical activity could only be studied in limited (9/20) cases because of recurring vomiting during the preoperative period. In IHPS infants, a significant increase in the bradygastria group was observed in the postprandial period compared with healthy infants. Three to 5 days after surgical repair (pyloromyotomy) and the reintroduction of feeding in gradually increasing amounts, the gastric myoelectrical activity showed physiologic patterns again, showing that the pyloric function was back to normal. Cutaneous EGG is a useful, noninvasive method to obtain indirect information about the motor function of the stomach and might be further applicable to pediatric gastric motility disorders.

The use of axillary skin crease incision for thoracotomies of neonates and children.

UNLABELLED: Because of the complications due to "standard" postero-lateral thoracotomy, i.e. winged scapula, scoliosis etc., different muscle-sparing approaches have been published. In 1998 Bianchi et al published their work on axillary skin crease incision for neonates, primarily for the treatment of oesophageal atresia. AIM OF THE STUDY: To assess the usefulness of axillary skin crease incision in paediatric surgery. METHODS: Data of 26 cases with axillary skin crease incision were reviewed and the results compared to those of postero-lateral incisions. RESULTS: Axillary skin crease incision in both neonates and children (up to the age of 15 years) was used to treat both pulmonary and mediastinal lesions and both benign and malignant diseases. The authors performed 17 operations in neonates (8 oesophageal atresia with tracheo-oesophageal fistula, 8 patent ductus arteriosus, 1 congenital cystic adenomatoid malformation) and 9 operations in children (3 neuroblastoma, 1 teratoma, 5 pulmonary operations including lobectomies). The authors were able to perform all operations with unrestricted access through the axillary skin crease incision when the 3rd or 4th intercostal space was necessary for the thoracotomy. The authors compared patients operated with oesophageal atresia through an axillary skin crease incision with patients operated through a postero-lateral incision - seven neonates each. There were no significant differences in the results regarding duration of procedure, duration of postoperative ventilation or the incidence of anastomosis stricture. CONCLUSIONS: Although further long-term studies are necessary to evaluate the axillary skin crease incision, the authors believe that axillary skin crease incision should become the standard method for 3rd and 4th intercostal space thoracotomies in neonates and children.

Long-term outcome of congenital cystic adenomatoid malformation.

To determine the long-term outcome of congenital cystic adenomatoid malformation (CCAM), the records of all patients with CCAM treated in three Hungarian paediatric surgical centers between 1977 and 1996 were reviewed. Patients were followed for up to 20 years following diagnosis and treatment. In 20 patients CCAM was diagnosed postnatally and in 3 prenatally. Biodata including the operative procedures are presented. Follow-up findings between 18 months and 20 years after diagnosis showed better height and weight growth in patients operated upon in later childhood compared with those operated upon in infancy. The older the patient at the time of diagnosis, the better was the long-term outcome.

[Renal candidiasis following treatment of infantile osteomyelitis]. / Csecsemökori osteomyelitis kezelését követó renalis candidiasis.

Systemic candidiasis with renal complications is a rather rare phenomenon in young infants. Authors report on a 4.5 month-old baby (preterm) who, during an antibiotic therapy of wide spectrum--because of osteomyelitis--acquired a mycotic infection causing bilateral pyelon and pyeloureteral obstruction. In addition to systemic antimycotic therapy surgical intervention was needed to eliminate the mycotic bezoar.

[Usefulness of ultrasound in the diagnosis of vesico-ureteral reflux in neonates and infants]. / Az ultrahangvizsgálat alkalmazhatósága újszülött- és csecsemökori vesicoureteralis reflux gyanúja esetében.

PURPOSE: Postnatal ultrasound (US) findings were assessed in an infant population with primary vesicoureteral reflux (VUR) and the findings of voiding cystourethrography (VCUG) were compared with those of sonography. MATERIALS AND METHODS: Between 1988 and 1997 55 babies (43 boys and 12 girls) with primary VUR were diagnosed. US criteria for suspected VUR included unexplained or transient fullness of the collecting system, pelvic wall thickening and signs of renal dysplasia. RESULTS: Among 55 patients reflux was unilateral in 32 cases, and bilateral in 23 patients, altogether 78 refluxing units were investigated. VUR Grade III-V, were detected in 62 units (79%). At least one US anomaly was present in 66 units (85%). No ultrasound anomaly was found in 12 renal units (15%). In 34 cases (44%) conservative treatment was performed, 40 renal units (51%) underwent ureteric reimplantation, and in 4 (5%) cases nephrectomy was carried out. CONCLUSIONS: The correct detection and grading of reflux is not reliable by sonography, although with careful US examination of the neonatal urinary tract in 85% of VUR cases at least one sonographic abnormality has been found. However, the US negative group, (15%) showed severe reflux on VCUG in a few cases. Therefore, all neonates with ultrasound pathology of unknown origin detected prenatal or postnatal, should undergo VCUG to exclude VUR.

The role of ultrasound in the follow-up of postoperative changes after pyeloplasty.

BACKGROUND: Pyeloplasty is an established treatment for pelviureteric junction (PUJ) obstruction. The postoperative change in the size of the renal pelvis and the kidney parenchyma are variable. OBJECTIVE: To document the changes in renal pelvic size and renal parenchymal thickness following pyeloplasty and to establish that improvement of both parameters are good markers for improved urine flow. MATERIALS AND METHODS: A group of 267 newborns and young infants with suspected PUJ obstruction were investigated by ultrasound. Pyeloplasty was performed on 102 babies, and 165 patients were followed conservatively. Postoperative ultrasonography at 6 and 12 months was available in 88 patients. RESULTS: One year after surgery, the renal pelvis was smaller in 76 % of the cases. The renal parenchyma was normal or had increased in 92 % of cases. CONCLUSION: Resolution of hydronephrosis after surgery is relatively slow, but renal parenchymal growth is rapid. Mild postoperative pelvic dilatation is frequent and does not indicate continued obstruction.

[Diagnosis of neonatal liver hemangioma]. / Az újszülöttkori májhaemangioma diagnosztikája.

The hepatic hemangiomas of infancy are rare, benign vascular tumours, differentiated in their morphologic and clinical appearances. They may manifest either as asymptomatic liver masses, or with the potentially fatal complication of congestive heart failure. They can be large in infancy, act as arteriovenous fistulas and may result in massive arteriovenous shunt. Multiple imaging modalities have been employed in the diagnosis, including ultrasonography (US), computed tomography (CT), radionuclide studies, angiography, magnetic resonance (MR) imaging. Generally the US, Doppler and CT appearances of hepatic hemangiomas may be diagnostic. Biopsy in often needed for diagnosis, especially if classic signs and symptoms are lacking. The diagnostic possibilities and difficulties in three patients are discussed. Different US images were present in all three cases, with atypical CT appearance in one. Doppler signals were lacking in two cases. Sonographic appearance of infantile hepatic hemangioma is variable, non-specific, but Doppler US is useful as a non-invasive method in the follow-up of hemangiomas.

[Postoperative ultrasonic studies in infants undergoing surgery for pyeloureteral obstruction]. / Posztoperatív ultrahangvizsgálatok pyeloureteralis obstructio miatt operált csecsemöknél.

134 newborns and young infants were investigated by ultrasound because of pelviureteric junction obstruction. Operation has been performed in 47 babies, 87 patients were followed nonoperatively. The results of the ultrasound studies were elaborated in 35 patients in 6 and 12 months postoperatively. One year after surgery pyelectasy decreased in 77 percent of the cases, the size of the renal parenchyma was normal or increased in the 91 percent of the cases. The postoperative mild dilatation of the pelvis is often observed without any sign of obstruction.

[A case of detection of pulmonary sequestration during the early stage of pregnancy and subsequent successful therapy]. / A terhesség korai szakaszában felismert és sikeresen gyógyított tüdösequestratio esete.

As result of routine ultrasound screening during the pregnancy, the number of congenital abnormalities causes severe perinatal respiratory or circulatory failure detected antenatally is rising. The severity of the extralobar pulmonary sequestration is variable. The intrauterine diagnosis and the proper prognosis is difficult because of the rarity of this abnormality. The Color-Doppler ultrasound method has a great importance in the diagnosis. With our case report we would like to help in the better understanding of this rare abnormality.

[Neonatal primary hyperparathyroidism]. / Ujszülöttkori primer hyperparathyreosis *ikeres mútéti megoldása kisdedkorban.

The authors report on a case of severe primary hyperparathyroidism with clinical signs from birth. The boy was admitted because of poor somatomental development, hypotony, hepatosplenomegaly and osseous abnormalities, resembling those of rachitis. Laboratory data showed the typical findings of primary hyperparathyroidism. The authors performed parathyroidectomy with simultaneous heterotopic parathyroid tissue autotransplantation. Histology revealed parathyroid chief cell hyperplasia. Because of the hypercalcaemia and clinical signs persisting after the operation they removed the parathyroid autografts. Since this later was ineffective they performed a left sided neck dissection on the side of the excessive parathormone production. The child became hypocalcaemic necessitating calcium and vitamin D administration. He is now 17 months after the last operation. His somatomental development is accelerated. In connection with the case the authors surveyed the literature of this rare entity.

[Obstructive ectopic ureterocele: review of the literature in the light of the authors' own experience]. / Obstructiv, ectopiás ureterokele: irodalmi áttekintés, saját eseteink tükrében.

The epidemiology, clinical presentation, evaluation and methods of surgical management of ectopic ureteroceles was studied. The prenatal detection of hydronephrosis of the upper pole of duplex systems allows early surgical correction, and this effectively decreases the risk of urinary tract infection, urosepsis and irreversible loss of renal function. Yet a precise prenatal diagnosis of accompanying ureterocele is not necessary for efficient postnatal management.

[Primary neonatal liver cyst]. / Ujszülöttkori primer máj-cysta.

The authors found with neonatal ultrasound screening a cyst in the epigastrium sized 45 x 18 x 36 mm. The newborn was operated at age of 7 days. The cyst was in the right lobe of the liver and after extirpation healed.

[Extrahepatic cholestasis in infancy and childhood: the role of ultrasonography]. / Extrahepaticus cholestasis csecsemö- és gyermekkorban: az ultrahangvizsgálat jelentösége.

Of 23 children with extrahepatic cholestatic syndromes, who were studied with sonography, there were found to have biliary atresia, choledochal cyst, cholelithiasis, stenosis of bile duct, gallbladder duplication, cholecystitis. Ultrasonography is the most useful initial investigation of choice, and in most of the cases the only investigation.

[Pelvic masses in childhood: ultrasonographic imaging]. / Kismedencei terimék gyermekkorban: az ultrahangkép sajátosságai.

The echographic appearance of pelvic masses in children was examined in 38 patients. Sonography was correct in determining the site of origin in all cases. Cystic uterine masses and cystic ovarian masses were the most specific, representing hydrometrocolpos and benign ovarian cysts. A nonspecific sonographic pattern was encountered with complex masses, which proved to be ovarian teratomas, hemorrhagic ovarian cysts and pelvic abscesses. Although a number of characteristic features of teratomes have been described, these signs were seen very rarely in children.

[Importance of pre- and post-natal ultrasonic examination in neonatal hydronephrosis]. / A prae- és postnatalis ultrahangvizsgálat jelentösége az újszülöttkori hydronephrosisban.

The widespread use of obstetric and neonatal sonography has resulted an increasing number of neonatal hydronephrosis, and some of them are asymptomatic. From November 1987 to May 1990 49 neonates with hydronephrosis or multicystic dysplastic kidney were seen. The most common conditions found were obstruction of the distal ureter (10), multicystic dysplatic kidney (12). Fifty-three percent of the cases were discovered on fetal screening during obstetric sonography, seven of them were asymptomatic at birth, but 4 of the asymptomatic babies underwent operation in the neonatal period. Nine of the postnatal diagnosed cases were incidental findings. The major benefit of prenatal echography is to allow early recognition of major uropathic conditions before postnatal infection worsens the prognosis. A correct postnatal ultrasound diagnosis was established in all cases.