Carlo Manzoni

Varicocele in pediatric patients: comparative assessment of different therapeutic approaches.

OBJECTIVES The best therapeutic approach for varicocele correction in pediatric patients is still an object of some debate. We analyzed a series of 99 patients to identify the most effective approach in terms of low recurrence rates and preservation of testicular growth. METHODS One hundred four operations were performed on 99 patients between 9 and 16 years of age (mean 13.3). The first 18 patients underwent sclerotherapy of the internal spermatic vein. Twenty others underwent inguinal varicocelectomy. In 12 patients, a modified Palomo procedure (ie, sparing of the internal spermatic artery) was performed (four laparoscopically), and 54 were treated with the original Palomo procedure (ie, sectioning of the entire spermatic cord), using laparoscopy in 20. RESULTS Three recurrences (16.6%) occurred among the patients treated with sclerotherapy. Similar rates of recurrence were observed in those who underwent inguinal varicocelectomy (3 [15%] of 20) and modified Palomo procedures (2 [16.6%] of 12). Only one recurrence occurred in the 54 patients treated with the original Palomo procedure (1.85%). CONCLUSIONS Our experience, together with a review of published reports, leads us to believe that the open Palomo procedure as originally described is the most effective approach to the correction of varicoceles in adolescents.

Contrast-enhanced gray-scale and color Doppler voiding urosonography versus voiding cystourethrography in the diagnosis and grading of vesicoureteral reflux.

The purpose of this study was to compare contrast‐enhanced gray‐scale voiding urosonography (CE‐VUS) and contrast‐enhanced color Doppler voiding urosonography (CE‐CDVUS) with voiding cystourethrography (VCUG) to verify whether the use of color Doppler imaging improves the diagnosis and grading of vesicoureteral reflux (VUR).

Invasive fetal therapies: approach and results in treating fetal ovarian cysts

Objective. To show the validity of prenatal invasive surgical intervention when a fetal ovarian cyst is diagnosed, compared to a wait and see attitude, in order to avoid possible prenatal and postnatal complications. Patients. Fourteen cases of intra-abdominal cysts monitored in our center between April 2005 and November 2010. All cases were first diagnosed in the third trimester, and were monitored for the remainder of the pregnancy and after delivery (2 months–3 years postnatally). Surgical intervention. Upon maternal and fetal cutaneous anesthesia performed trans-amniotically, the cystic fluid (mean contents 43.85 cc, DS 46.27) was extracted for cytological, biochemical, and hormonal examination. Results. Thirteen cases of intra-abdominal cysts (92.8%) were fetal ovarian cysts. Ninety-two percent of pregnancies bearing such a condition were successfully concluded (n = 12). Sixty-nine percent concluded in vaginal delivery (n = 9). None experienced maternal and/or fetal complications. Every drained cyst had an estradiol concentration higher than 10,000 pg/ml. Conclusions. The aspiration of ovarian cysts exceeding a 40 mm diameter, performed as early as possible, allows a good longitudinal treatment of this fetal affection, thus avoiding torsion, tissue necrosis, and invasive postnatal surgery, as well as giving hope of future gestational capability to the fetus/newborn.

Caustic ingestion in childhood: current treatment possibilities and their complications

In a review of 15 pediatric patients who had ingested caustic substances, the authors describe the diagnostic and therapeutic procedures to be followed as well as the complications that may occur with their use. The cases reported include 1 esophageal rupture caused by balloon dilatation and 1 recurrent stenosis treated with a silastic tutor.

Fetal ovarian cysts: postnatal evolution and indications for surgical treatment

Fetal ovarian cysts are an increasingly common finding during routine prenatal ultrasonography (US). These lesions were occasionally diagnosed in the past, but only on the basis of a palpable abdominal mass or, rarely, because a twisted ovarian cyst caused an acute abdomen. Surgery is currently recommended for neonates after prenatal diagnosis, even in asymptomatic cases, when the cyst has a diameter larger than 5 cm or echographic findings suggest torsion or intracystic hemorrhage. In our 12 cases of suspected fetal ovarian cysts, wide variations in diameter were seen in 6 during pre- and postnatal follow-up. Two of these were not confirmed at birth; 7 were treated conservatively and excluding 1 perinatal death from multiple associated anomalies and 1 case lost to follow-up, the remaining cases showed uneventful, spontaneous regression of the cyst. Postnatal US signs suggesting necrosis secondary to torsion in a previously uncomplicated cyst and requiring surgery were seen in only 3 cases. Conservative treatment must be recommended in most prenatally diagnosed cysts; indications for surgery cannot be based only on dimensions. The incidence of complications has been overemphasized and the risk of associated malignancy is negligible in newborns, while possible size reduction and resorption can be expected in most cases. Nonoperative management may also be recommended when signs of torsion are seen before birth and spontaneous asymptomatic involution of the cystic ovary is documented in early postnatal life. Accurate clinical and US follow-up is mandatory in all cases. Surgery must be reserved for symptomatic cases or when an alteration of the cystic structure is documented by US in a previously uncomplicated cyst.

Solitary Peutz-Jeghers Polyp in a Paediatric Patient

Hamartomatous polyps of Peutz-Jeghers are mostly found in patients affected by Peutz-Jeghers syndrome (PJS), but they can be rarely encountered in the general population. It is unclear whether a solitary Peutz-Jeghers polyp (PJP) is an incomplete form of PJS or a separate entity. We report a case of solitary PJP in a paediatric patient in whom the other features of PJS were absent. The patient underwent laparotomy due to small bowel intussusception secondary to an ileac polyp. Histological examination showed the characteristic features of PJP, but the patient did not fulfill the WHO criteria for PJS diagnosis (negative family history for PJS and absence of mucocutaneous pigmentation); moreover analysis of the STK11/LKB1 gene did not reveal any genomic abnormality. The clinical and investigative findings in our case suggest that the solitary PJP can be considered a different clinical entity from PJS.

Plexiform schwannoma of the esophagus in a child with neurofibromatosis type 2

Schwannoma is a benign neoplasia of the peripheral nerve sheath. Its localization in the gastrointestinal tract, and in particular in the esophagus, is very rare. According to the existing literature esophageal schwannoma has been reported so far only in adult patients. We report the case of an 11 year old patient with neurofibromatosis, type 2, who underwent surgical excision of a plexiform schwannoma of the esophagus.

The fetus with an abdominal wall defect: management and outcome

Despite prenatal diagnosis, maternal transport and early perinatal treatment in specialized hospitals, improvement in overall mortality has not been observed among newborns with abdominal wall defects (omphalocele and gastroschisis). A comparison has been made, for both anomalies, between 55 cases treated in the periods 1967-1979 and 30 treated in the periods 1980-1985. In this last group 14 prenatally detected cases were separated from cases discovered at birth. Recent advances in treatment have reduced mortality rates for both of these anomalies. This was especially true for gastroschisis; in omphalocele cases, associated abnormalities appeared to be an unavoidable limiting factor in survival. With prenatal diagnosis a surprising increase in mortality rate was observed among omphalocele cases detected before birth. A possible reason for this is the total number of antenatally diagnosed cases that, with maternal transport, are concentrated in centers where surgical facilities are available. Before introduction of antenatal diagnosis, most of these cases would never have been observed by the pediatric surgeon because of death prior to referral or treatment. Gastroschisis cases may benefit more from antenatal recognition and early treatment because multiple abnormalities or immaturity are not so important limiting factors in survival as in omphalocele cases. Reduced trauma and contamination of exposed viscera, immediate treatment and adequate supportive measures may significantly reduce mortality rate in gastroschisis cases. Operative delivery of fetuses with an abdominal wall defect is controversial. The risk of vaginal route delivery has been over emphasized. Intrapartum rupture of an omphalocelic sac was an extremely rare event before introduction of antenatal diagnosis.(ABSTRACT TRUNCATED AT 250 WORDS)

Malignant retroperitoneal paraganglioma: case report and review of the literature.

Paraganglioma is a rare neuroendocrine neoplasm observed in patients of all ages but it has not been characterized in children. The authors describe a retroperitoneal paraganglioma diagnosed by chance in an 11-year-old boy. Many aspects of retroperitoneal paraganglioma are still under investigation. The treatment of choice is radical resection. Surgery may be possible following chemotherapeutic debulking with cyclophosphamide, vincristine, and dacarbazine. 131I-MIBG radiotherapy has proved increasingly useful in reducing the pain associated with disseminated disease and also in facilitating surgical resection in cases that appear inoperable. Conventional radiotherapy is purely palliative and used to reduce the pain of bone metastases.

Prenatal diagnosis and management of some fetal intrathoracic abnormalities

Nine cases of fetal intrathoracic anomalies detected in utero and followed to birth are reviewed. There were 6 congenital diaphragmatic hernias (CDH), one congenital pleural effusion and two isolated cysts of the lung. All these conditions were potentially responsible for neonatal respiratory distress and received early intensive treatment after maternal transport and delivery had been arranged in a center with thoracic surgical facilities available. The risks of a delayed or missed diagnosis were thus avoided, especially for CDH. Despite intensive, traditional, respiratory support, started in the delivery room, mortality among prenatally detected cases of CDH was paradoxically high (83%), compared to mortality among 7 cases of CDH not detected in utero, referred in the same period to our Institution, and symptomatic within 6 h from birth (63%). With prenatal diagnosis the total number of CDH cases referred to a surgical center before birth increases. Many cases which would never have been treated in the past because of death before referral and treatment for severe pulmonary hypoplasia not compatible with life are thus observed and sometimes treated. Nevertheless, lung development continues to be a determining factor for survival even when intensive treatment at birth is available. Responsiveness to therapy is unpredictable before birth and proposed antenatal treatment is still far from being a realistic option. For the other three newborns, where a pleural effusion and pulmonary cysts were found, prenatal diagnosis helped to start appropriate treatment and to prevent neonatal hypoxia in two of them. In the third case, with an incommunicant, isolated pulmonary cyst, the outcome would have been favourable even without a prenatal diagnosis.