Laparoscopy or retroperitoneoscopy for pediatric patients with adrenal masses?

Minimally invasive approach to the adrenal gland was first reported in 1992. Since then, the experience with the laparoscopic technique for adrenal disease in children has been limited. We report our experience with minimally invasive adrenal surgery in children. Two young girls (2 and 4 years old) with a left adrenal mass were operated using minimally invasive surgery (MIS) in our Unit. Ultrasonography and MRI showed in the oldest a 2 x 3 cm adrenal mass, while in the youngest a 5.5 x 5 cm adrenal tumor was found. According to the pre-existing literature, we approached the smallest lesion via retroperitoneoscopy, and the largest one laparoscopically. The operating time was 110 minutes for retroperitoneoscopy and 75 minutes for laparoscopy. No major intra or postoperative complications occurred. There were no conversions to open surgery. Postoperative hospital stay was 5 days for both patients. In both cases, the anatomo-pathological result was an adenoma. Minimally invasive adrenalectomy is a safe and feasible procedure in children with good results. For lesions smaller than 3-4 cm retroperitonescopy is feasible, while for tumors larger than 5 cm, due to malignancy risk, the laparoscopic approach is indicated. To keep oncologic criteria it is important to avoid tumor rupture and to extract the specimen in an endobag.

Imperforate hymen causing congenital hydrometrocolpos.

A 3-day-old girl in good health was referred to our department for the evaluation of an abdominal mass detected at birth. Prenatal ultrasound (US) examinations had shown no anomaly. US examination revealed the presence of a hypoechoic and corpusculated cystic formation of about 8 × 5 × 4 cm located in the mid region of the abdomen. The uterus was not visible and the kidneys were normal with no sign of hydronephrosis. The ovaries were normal. Physical examination confirmed US findings revealing the presence of a curved membrane which covered the vaginal opening. Based on these findings, the patient was diagnosed to have hydrometrocolpos. Hymenectomy was performed and about 100 ml of milky fluid was subsequently removed by aspiration. The patient presented no other congenital anomalies and US follow-up showed a normal structure of the uterus.

Principales características del pilomatricoma múltiple y su asociación con una mutación de la betacatenina / Principal features of multiple pilomatricoma and its association with beta-catenin mutation

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Endoscopic fibrin sealing of congenital pyriform sinus fistula.

Pyriform sinus fistula is a very rare branchial apparatus malformation, often appearing in the form of a cervical inflammatory process (abscess or suppurative thyroiditis), especially in infants. Failure to diagnose this lesion may result in unexpected recurrence. A case of recurrent suppurative thyroiditis caused by pyriform sinus fistula in a 9-year-old girl is reported. In the latency period of infection, the fistula tract was identified by a barium meal contrast study. Direct endoscopy showed the fistula internal orifice at the apex of the left pyriform fossa. The fistula was completely obliterated by injection of fibrin glue. Suppurative thyroiditis is reported mainly in the pediatric literature, and the reported case is the first to be managed endoscopically by injection of fibrin adhesive.

[Transanal endorectal pull-through with or without laparoscopic assistance? Development of an experimental model]. / Abbassamento endorettale transanale con o senza assistenza laparoscopica? Sviluppo di un modello sperimentale.

The transanal approach is a relatively new technique for surgery of Hirschsprung's disease. The purpose of this experimental study was to evaluate and compare the transanal endorectal pull-through when it takes place with laparoscopic assistance, according to Soave-Georgeson procedure and without laparoscopic assistance. We used two separated groups of animals. In the 1st group the transanal endorectal pull-through was performed by retrograde transanal mucosectomy with laparoscopic devascularization of the colon and in the 2nd group the transanal endorectal pull-through was performed without laparoscopic assistance. In the 1st group the transitional zone of the colon was determined laparoscopically while in the 2nd group during the perineal devascularization of the bowel. In both groups end to end coloanal anastomosis was performed in the same way. The postoperative course was uneventful, no severe long-term problems were observed. Four months after operation the experimental subjects of the 1st group had 3-4 discharges in 24 hours. While the experimental subjects of the 2nd group had 5-7 discharges in 24 hours. Manometric resting anal sphincters pressure in the 1st group averaged 77 +/- 16 mmHg compared with 74 +/- 15 mmHg in the 2nd group. The transanal colectomy technically can be performed with or without laparoscopic assistance. However the advantages of laparoscopic assistance are the following: 1) more exact determination of transitional zone; 2) easier separation of peritoneal reflection, 3) better control of final position of the colon; 4) minor retraction on the perineal muscles.

Annular pancreas combined with distal stenosis. A report of four cases and review of the literature.

BACKGROUND: Congenital duodenal obstruction (CDO) in combination with more distal duodenal obstructions is a rare anomaly occurring in 4% of neonates with duodenal atresia. The experience of two European Pediatric Centers in treatment of congenital double duodenal obstruction (CDDO) is reported and the pertinent literature is reviewed. MATERIALS AND METHODS: During the last 15 years a total of 86 neonates were operated upon for CDO at the department of pediatric surgery of "St. Sophia" Children's Hospital of Athens in Greece and the department of pediatric surgery of "Federico II" Children's University Hospital of Naples in Italy; four of them had a CDDO. These ones presented with nonbilious vomiting and the plain film of the abdomen showed the typical "double bubble". Our cases with CDDO presented annular pancreas causing complete obstruction of the second part of the duodenum and dilatation of the duodenum distal to this obstruction due to an additional congenital stenosis (two cases) or a membranous web (two cases). A diamond shaped duodeno-duodenal (DDD) anastomosis was carried out to relieve the proximal obstruction and a Heinecke-Mikulicz plasty was used to relieve the distal stenosis. RESULTS: All patients with double obstruction underwent successful surgery with no complications. The postoperative course was uneventfuL An upper gastrointestinal barium study at one month postoperatively showed no blind loop, megaduodenum, anastomotic stenosis or malfunction. CONCLUSIONS: a) The combination of duodenal atresia with annular pancreas and distal duodenal stenosis or web is extremely rare. b) Patency of the duodenum distal to the usual obstruction should always be checked in order to avoid misdiagnosis of this combination.

Is the risk of hypertension an indication for prophylactic nephrectomy in patients with unilateral multicystic dysplastic kidney?

OBJECTIVE: Surgery is still proposed by some as the treatment of choice for unilateral multicystic dysplastic kidney (UMCDK) because of the potential complications of hypertension, infection and malignant change. The purpose of this study is to demonstrate that the risk of hypertension does not justify routine nephrectomy. MATERIAL AND METHODS: We report 41 cases of UMCDK, treated between 1980 and 2001, 28 of whom were male (68%). Twenty-one patients were nephrectomized (51%). Twenty patients (49%) underwent clinical and ultrasound follow-up. Blood pressure was controlled every 3-4 months (over a period ranging from 9 months to 6 years) and an ultrasound scan was performed every 6 months during the first 2 years, and annually thereafter. RESULTS: In the patients treated non-operatively we observed a progressive spontaneous involution of their multicystic dysplastic kidneys and the total absence of complications such as hypertension or malignancy. CONCLUSION: It is reasonable to conservatively manage patients with UMCDK by means of repeated ultrasound examinations and blood pressure control.

[Varicocele at a prepubertal age. Adolescent varicocele]. / Il varicocele nell'età pre-adolescenziale. Adolescential varicocele.

Varicocele is a dilatation of the spermatic veins, particularly on the left side, and represents one of the most frequent causes of male infertility. Its frequency in pre-adolescence varies from 10% to 15%. Even today varicocele pathogenesis is not clear. In the period from August 1990 to August 1999, 35 males between 10 and 16 years of age were treated surgically in our Institution via interruption of the internal spermatic vein using Palomo's technique. Patients that reached the age of 18 after surgery have had a spermiogramm. The problem of varicocele therapy in pre-adolescence has not yet been resolved with regard to surgical therapy and follow-up.

[Bilobectomy in a brazilian infant suffering from extensive monolateral pulmonary TBC] / Bilobectomia in un divezzo brasiliano affetto da estesa tubercolosi polmonare monolaterale.

As there has recently been a significant increase in childhood tuberculosis, we considered an important contribution the clinical case of an infant from Brazil, who underwent right bilobectomy to treat a previously poorly cured enlarged pulmonary tubercular infiltrate.

[Vascular emergencies in childhood]. / Le urgenze vascolari in età pediatrica.

Vascular urgencies in childhood are rare and various in presentation. Authors report their experience regarding a wide range of vascular urgencies occurring in pediatric age. A number of cases of neonatal gangrene of extremities, vascular trauma, complicated vascular dysplasia, arterial and venous thrombosis both peripheric and visceral are described. Etiopathogenetic diagnostic and therapeutic features of these lesions are discussed.

Neonatal congenital epulis.

Congenital epulis is not a common lesion. 2 rare neonatal multiple cases are reported. The treatment of choice of these lesions is radical excision during early infancy.

[Congenital cystic adenomatoid malformation of the lung]. / La malformation adénomateuse kystique congénitale du poumon.

Congenital cystic adenomatoid malformation of the lung (CCAM) has been reported with increasing frequency since it was first described in 1949. In a review of the literature by Halloran and co-workers in 1972, 61 cases were reported. After ten years, we collected from the literature 279 cases. The malformation is characterized by a multicystic mass of lung tissue with bronchiolar proliferation and alveolar impairment. The clinical picture of this pulmonary malformation varies with the age at presentation. In the neonatal period the respiratory distress syndrome is often very serious and may require an urgent treatment. In the older infant, the unrecognized disease has more often a chronic course characterized by recurrent respiratory infections and failure to thrive. Sometime, the malformation may be completely silent and be disclosed during a routine examination of the thorax for others reasons. Both types of presentations of the cystic adenomatoid malformation require a surgical therapy and the lobectomy is the treatment of choice. The prognosis following resection in the older patients is generally good, while in the neonatal period the remaining hypoplastic pulmonary tissue may represent a problem in the postoperative ventilatory management. Two cases of congenital cystic adenomatoid malformation observed in a 5 months old and in a 10 months old infants are reported. The clinical, pathologic and radiologic features of this pulmonary malformation are discussed. The hypothesis of a future "in utero" correction of the most severe cases, in order to prevent the pulmonary hypoplasia of the healthy parenchyma, is also suggested.

Infected and ruptured urachal cyst causing peritonitis.

Urachal cysts are rare and usually as asymptomatic until they become infected. The route the infection follows is uncertain. A very unusual case is reported of a newborn female afflicted with a posteriorly ruptured urachal cyst causing incapsulated peritonitis, in which we obtained a visualization of the cyst during fistulography

[Gastroesophageal reflux and peptic esophagitis in childhood. What is the role of traditional radiology?]. / Reflusso gastro-esofageo ed esofagite peptica in età pediatrica. Qual è il ruolo della radiologia tradizionale?

The authors studied 52 patients aged from 1 month to 8 years with symptoms consistent with gastro-esophageal reflux and esophagitis. The employed methods were radiological examination, esophageal manometry, acid reflux test, endoscopy. The gastro-esophageal reflux was diagnosed in 38 children. The authors compare the results of radiological examination to those of the other methods. The analysis of the results led the authors to believe that the association of every methods is necessary to define the diagnostic picture. However, the authors emphasize that the conventional X-rays examination is the first stage that cannot be substituted in the management of children with non acute gastro-esophageal disease.

Abdominothoracic gastric teratoma in a female newborn infant.

Gastric teratoma is a rare, benign tumor. The 47th case, reported here, is the first in which an abdominothoracic localization has been found.

Epicardial oesophageal duplication with hiatal hernia in a case of Turner's syndrome.

The authors of this paper report the first case of epicardial oesophageal duplication causing hiatal hernia in a patient afflicted with Turner's syndrome, and they discuss its possible etiology.