Long-term outcome for children undergoing open hepatico-jejunostomy for choledochal malformations: a 43-year single-center experience.

PURPOSE: To report on our 43-year single-center experience with children operated on for Choledochal Malformations (CMs), focusing on long-term results and Quality of life (QoL). MATERIALS AND METHODS: All consecutive pediatric patients with CMs who underwent surgical treatment at our center between October 1980 and December 2022 were enrolled in this retrospective study. We focused on long-term postoperative complications (POCs), considered to be complications arising at least 5 years after surgery. We analyzed QoL status once patients reached adulthood, comparing the results with a control group of the same age and sex. RESULTS: One hundred and thirteen patients underwent open excision of CMs with a Roux-en-Y hepaticojejunostomy (HJ). The median follow-up was 8.95 years (IQR: 3.74-24.41). Major long-term POCs occurred in six patients (8.9%), with a median presentation of 11 years after surgery. The oldest patient is currently 51. No cases of biliary malignancy were detected. The QoL of our patients was comparable with the control group. CONCLUSION: Our experience suggests that open complete excision of CMs with HJ achieves excellent results in terms of long-term postoperative outcomes. However, since the most severe complications can occur many years after surgery, international cooperation is advisable to define a precise transitional care follow-up protocol.

Extrarenal testicular Wilms' tumor in a 3-year-old child.

We report an extremely rare case of extrarenal testicular Wilms' tumor in a 3-year-old boy with intrabdominal undescended left testis. The patient was admitted because of pain and vomiting, with evidence of a huge abdominal mass. At surgery a large tumor arising from the intrabdominal testis was found. Histology showed the classical triphasic Wilms' tumor elements: epithelial, mesenchymal and blastemal areas. Extrarenal Wilms' tumors account for only 3% of all Wilms' tumors and just -100 cases have been reported in literature. Testicular origin is anecdotic. We present histomorphological, histogenetic, clinical, diagnostic, prognostic and therapeutic features of this rare tumor.

[Cryptorchidism: medium- and long-term follow-up].

AIM: Cryptorchidism represents the most frequent male genital anomaly in paediatric population and may potentially interfere with fertility and determine neoplastic testicular diseases. We wanted to evaluate the correlation between age at orchiopexy and follicle-stimulating hormone (FSH), luteinizing hormone (LH) and testosterone levels in adulthood, determining the long-term complications of surgical treatment. METHODS: Fifty-seven patients (mean age 19 years, range 18-27) surgically treated for cryptorchidism in pediatric age were included in a medium and long-term follow-up (10-19 years). We divided this population into four groups: A) monolateral cryptorchidism operated on before 36 months of age (15); B) monolateral cryptorchidism operated on over 36 months (32); C) bilateral cryptorchidism operated on before 36 months (5); and D) bilateral cryptorchidism operated on over 36 months (5). All patients underwent andrological examination, testosterone, FSH and LH dosage, measurement of testicular volume and spermiogram. RESULTS: Significant different FSH levels were found between group A and C and between A and D (P<0.01), while groups A and D presented also different mean testicular volume (P<0.01). In addition group D showed an abnormal morphology of spermiogram. The main complications found in follow-up were hydrocele (17,5%), varicocele (8,7%) and epididymal cysts (3.6%). CONCLUSION: Monolateral cryptorchidism is associated with normal fertility when treated early (group A). Subjects in Group D, on the contrary, have a rise of FSH, a reduction of testicular volume and semen abnormalities. The long-term follow-up of these patients can also detect associated.

Intravaginal testicular torsion in newborns. To fix or not to fix the contralateral testis?

Scrotal swelling suggesting testicular torsion is a rare urological emergency which requires a clinical urgent evaluation and most of the times must be managed surgically. In newborns it can occur in the postnatal period, usually within the twenty-eighth day of life, or more frequently in utero, during the descent of the testis into the scrotum. Usually its poor fixedness allows the testis an abnormal mobility inside the scrotum, configuring the framework of extravaginal torsion. On the contrary during the perinatal period a twist that takes place inside the tunica vaginalis, known as intravaginal torsion, is extremely uncommon and only few cases are well documented in the literature. Authors present a rare case of intravaginal testicular torsion occurred in perinatal period. In this situation only the early surgical exploration of the scrotum may allow the rescue of the gonad, although in rare cases. Timing of surgical treatment and need for contralateral testicular fixation remain controversial. However since the anatomical defect of the tunica vaginalis can be bilateral the surgical fixation even of the contralateral testis is important, now or later, in order to prevent any future torsion of this gonad. The authors also present a brief review of recent literature on the subject.

Surgical treatment of toraco-abdominal teratoma diagnosed in utero, approached with mini-invasive technique: a case report.

Thoracic tumours of childhood arise either in the mediastinum, or from the chest wall and rarely from the lung parenchyma. Mediastinal teratoma occur in the anterior mediastinum and are usually very large at presentation; initial treatment is under discussion, but it appears that surgical excision and histological examination are the treatments of choice and it will determine whether the lesion is benign or malignant. The authors report a case of thoracic-abdominal teratoma, diagnosed in utero, and treated successfully with thoracoscopy and laparoscopy. No complication was described in short follow up, and the child was discharged in day 8th postoperative day. Histology showed mature teratoma. Follow-up at one year: no evidence of recurrence. Due to the rarity of multifocal teratoma in newborns, author believe that mini-invasive technique was useful to stage histological the mass, giving the opportunity to plans a second stage in treatment of the patient. Day of hospitalization resulted shorter in both surgical approaches, and discomfort less for the thoracic approach. This staging must be anyway planned, in the way of prenatal counselling and in the perinatal period, with the team involved in the procedure, that include anaesthetists, oncology staff, obstetricians, neonatologists and pediatric surgeons. It is mandatory in cases like the one reported that a multisciplinary team manage the patients and expert surgeons, especially in minimal invasive surgery, make the adequate plans.

Heterogeneity of dermal OKT6+ cells in inflammatory and neoplastic skin diseases.

This immunopathologic study of both normal and pathologic skin specimens (contact dermatitis [CD], lichen planus [LP], cutaneous T cells lymphoma [CTCL], and histiocytosis X [HX]) allowed as to differentiate four types of dermal OKT6+ cells: (1) cells with the same morphologic features as epidermal Langerhans cells (LCs), rarely found in either normal or pathologic dermis; (2) cells structurally similar to LCs but lacking Birbeck granules (BGs), found mainly in CD and LP; (3) larger cells rich in cytoplasmic organelles, only 5% of which contained BGs. They were especially common CTCL; and (4) cells typical of HX.