Multicentric experience on double dartos flap protection in tubularized incised plate urethroplasty for distal and midpenile hypospadias.

PURPOSE: We report a multicenter experience using double dartos flap to protect the neourethra in TIP urethroplasty for distal and midpenile hypospadias. METHODS: A total of 394 patients underwent tubularized incised plate urethroplasty for primary distal and midpenile hypospadias using double dartos flap protection by ten pediatric surgeons and urologists at five different institutions. RESULTS: Tubularized incised plate urethroplasty protected by a double dartos flap was simple to perform and flaps were easy to obtain. Complications occurred in 23 patients (5.83%): fistulas 1.01% (4 cases), stenosis 0.25% (1 case), mild stenosis 2.53% (10 cases), dehiscence of ventral cutis 0.50% (2 cases) and penile torsion 1.26% (5 cases). All fistulae had a spontaneous resolution. CONCLUSION: Double dartos flap to protect tubularized incised plate urethroplasty is safe with a low complication rate. The neourethra is covered entirely with a double layer of vascularized tissue and the double coverage appears a good choice for preventing urethrocutaneous fistula formation.

An unusual case of asymptomatic appendicular cutting foreign body.

Foreign body ingestion is a common problem in pediatric age. Foreign bodies in the lumen of appendix is a well known event. In this condition perforation or appendicitis may occur even for blunt or sharp objects. For these reasons, in case of stasis of foreign body in the lumen of appendix a prophylactic appendectomy is recommended. A case of asymptomatic ingested cutting foreign body in the appendix is presented and the approach is discussed according to the literature data.

Surgical VLS Therapy of Oesophageal Achalasia in Pediatric Age: Four Case Reports.

The Authors present a retrospective review of their record of cases, characterized by 4 cases of achalasia in which it was performed a Heller myotomy with front fundoplication (Thall) in laparoscopic approach in the period from 2012 to 2019. In paediatric achalasia, the laparoscopic Heller myotomy seems to be the best treatment because of its multiple advantages offered by the minimally invasive technique. First of all, thanks to the video-technique, which allows a complete and extended myotomy, the accuracy of this operation is maximized; moreover, the post-operative pain is widely reduced, thanks to the minimal dissection and traction of the tissues; finally, but not negligible, this approach ensures a better aesthetic result than the classic open technique. With regard to the front fundoplication, the Authors suggest that it is mandatory because, even if it extends the operating time, it ensures a natural protection to the myotomy herniated mucosa and avoids gastro-oesophageal reflux, which often occurs after the surgical correction, thus obliging to perform a reoperation.

Complications of the "Nuss Procedure" In Pectus Excavatum.

During the pediatric age range, one the most frequent deformities of the chest wall are Pectus Excavatum (PE). Currently the treatment of choice for PE is surgical intervention following the Nuss procedure. In this study, we present a description of the complications associated with surgical treatment of PE with the Nuss technique, in patients with symmetrical or asymmetrical deformity, in different stages of disease severity. The study was conducted in collaboration with the Pediatric Hospital "Istituto G. Gaslini" of Genoa. We analyzed a cohort of 402 patients (334 males and 68 females), who underwent corrective surgery between 2005 and 2018. Within this group of patients, we observed 82 cases with complications (20.39%), 20 of which were intraoperative (4.98%) and 62 post-operative (15.42%). For the evaluation of complications, this group was arbitrarily divided into patients with symmetric and asymmetric Pectus and in patients with mild, moderate and severe Pectus using Haller's index. Although a small group of patients presented complications, overall results from data analysis show that the Nuss technique represents the preferred surgical procedure for the treatment of PE, in agreement with literature. Furthermore, in our results show no correlation between asymmetry or severity of PE with complication related to the surgery.

Papillary Thyroid Carcinoma in Paediatric Age.

In children, differentiated thyroid carcinoma is a rare condition. Early diagnosis is not always easy, because of the lack of clinical symptoms, but it has a pivotal role in performing a correct therapeutic process. The study describes three cases of papillary thyroid carcinoma. None of the three patients had a positive familiarity or exposure to risk factors. In two cases, the tumor occurred as a non-injurious swelling in the anterior cervical region, in the other case it occurred with a latero-cervical lymphadenopathy that had been persistent for a year. In the first two patients we made a certain diagnosis by the needle aspiration of the thyroid nodule; in the other case the diagnosis was made by surgical exeresis and histological analysis of the lymph nodes. We also performed blood chemistry and hormonal tests, neck ultrasound, chest x-ray. The three children underwent total thyroidectomy and two of them also underwent right-sided cervical lymph node exeresis because there was the presence of metastasis. In our experience, the best therapeutic strategy for children with differentiated thyroid carcinoma is the total thyroidectomy, followed or not by latero-cervical lymph node exeresis and radioiodiotherapy. The removal of the whole gland reduces the risk of relapse.

Laparoscopic Repair of Paediatric Indirect Inguinal Hernia: Modified Flip Flap Technique.

In paediatric age, indirect inguinal hernia represents more than 95% of the hernial disease. It is a congenital type, in contrast with adulthood in which acquired forms are more frequently found (1). The laparoscopic correction of indirect inguinal hernia is one of the most common surgeries performed in paediatric age. In recent years, various techniques have been introduced for the videolaparoscopic correction of this disease. The aim of this study is to provide an assessment of the efficacy and safety of the execution of a modified Flip-Flap technique, using tissue glue for filling of Peritoneal-vaginal duct (DPV), performed in order to ensure greater suture tightness and reduce the incidence of postoperative hydrocele. author1, author2, author 2, author1, author1, author 2 The Authors present a retrospective review of their record of cases, considering a total of 187 patients aged between 18 months and 14 years. For the correction of the hernial defect, the modified VLS Flip-Flap technique was carried out. The evaluation of safety, efficacy, operating time, relapse rate and development of short-term complications (such as postoperative hydrocele, scrotal hematoma or ecchymosis, atrophy or iatrogenic testicle ascension) was considered in a mean follow-up of 6 months. The Authors suggest that this variant of the peritoneal Flip-Flap technique is simple to perform; its safety, reproducibility and effectiveness is proven and has a percentage of relapses and complications overlapping with the "open" approach and superior to other laparoscopic techniques.

Minimally Invasive Treatment of Urolithiasis in Children: Evaluation of the Use of Flexible Ureterorenoscopy and Laser Lithotripsy.

Urolithiasis is a multifactorial disease; in recent years, its incidence has gradually increased in pediatric age. Among the factors involved in urolithiasis pathophysiology, urinary tract anomalies and metabolic diseases are the most relevant, although ethnicity and environmental factors may have an important role. The advances in technology and miniaturization of endoscopic devices have permitted the use of Retrograde Intrarenal Surgery (RIRS) to treat kidney and ureteral stones. Nowadays, flexible ureterorenoscopy and laser lithotripsy, which are techniques that have been applied in the management of adult upper urinary tract disorders, are also used in children as a minimally invasive treatment of urolithiasis with encouraging, effective and safe results. The Authors report a retrospective review of their record of cases considering 21 pediatric urolithiasis treatment procedures performed between October 2017 and April 2019 in a total of 17 patients (10 males and 7 females). Six procedures involved the use of the flexible ureterorenoscope (FURS) while in 15 procedures the application of the laser fiber was used (FURSL). A case of laser lithotripsy for bladder stone was included. The average age of patients was 10.5 years (2-18 years). The renal pelvis dilatation pretreatment was evaluated in post-operative follow-up. From the evaluation of the sample in analysis, the use of RIRS has good results in the treatment of paediatric urolithiasis, emerging as a valid option in the management of the paediatric population in terms of efficacy and safety, with an improvement in patient outcomes.

Genetic deficiency of factor VII and hemorrhagic diathesis. A case report and literature review.

FVII deficiency is a rather rare inherited hemocoagulation disorder that predisposes to hemorrhagic events, especially from mucous membranes, that are not predictable and severe as in hemophilia A. This defect produces prolonged prothrombin time (PT), reduced activity of FVII and normal activated partial thromboplastin time (aPTT). We report the case of a 43-year-old obese woman with severe deficiency of factor VII (FVII), probably genetic in nature, and meno-metrorrhagia associated with multiple fibromas of uterus. Our patient had no history of bleeding in infancy and young age, and in the past, before the disease was diagnosed, underwent major surgery operations (thyroidectomy and caesarian section) without hemorrhage. Patient's relatives with mild heterozygous deficiency of FVII (the father, a brother, a sister, a sister's daughter and the patient's son) did not show any bleeding tendency. This case report is discussed in the light of literature data ((source: Medline from 1964 to 1996). The different forms of congenital (isolated or combined with other clotting disorders) and acquired FVII deficiency, with the appropriate therapies, are reviewed. The clinician must consider FVII deficiency in cases of recurrent bleeding, and this disease, even if rather rare, should not be underestimated in clinical practice because it is potentially fatal.

[Familial Marfan's syndrome. A critical review and presentation of a clinical case]. / Sindrome di Marfan familiare. Revisione critica e presentazione di un caso clinico.

Aim of this paper is to describe and discuss, on the basis of an exhaustive review of literature, the case of a 14-year-old girl with familiar Marfan's syndrome. This disease is a generalized inherited disorder with involvement of connective tissue and symptoms affecting ocular, skeletal and cardio-vascular systems, usually diagnosed in young age and associated with a poor prognosis because of late severe aortic complications (dissection, dilation or aneurysms, regurgitation, etc.). The young patient sought to our medical attention because of severe leanness, delayed menarca and irregular menstruations; physical examination disclosed the typical "morphotype of Marfan" with long limbs, slenderness of hands and feet, severe kyphoscoliosis, narrow chest with "pectus excavatum", marked hyperextensible joints, and high arched palate with malocclusion. Echocardiography demonstrated a mild mitral valve prolapse. The 43-year-old patient's mother presented an undiagnosed Marfan's syndrome, despite typical morphotype, muscle-skeletal alterations and moderate dilation of ascending aorta. The maternal grand-mother deceased prematurely for cardiopathy of unknown origin. It is stressed that the disease should not be underestimated, because the early diagnosis is important for the patient's prognosis, allowing the early preventive surgical intervention for correcting aortic or valvular alterations. The echocardiography represents a sensitive and noninvasive mean, useful to manage the patients with proven or suspected aortic dilation. The subjects with Marfan's syndrome who exhibit rapid progression of aortic dilation, or an aortic diameter in excess of 50 mm, should be considered for an elective surgical intervention (at low mortality and suitable to increase remarkably the lifetime expectation). Women with syndrome of Marfan should be advised on the high cardio-vascular risk during pregnancy.

[Precordial discomfort and ECG changes of repolarization associated with hypomagnesemia in a young women following colectomy for diffuse colonic lipomatosis]. / Oppressione precordiale e turbe elettrocardiografiche della ripolarizzazione associati ad ipomagnesemia in giovane donna colectomizzata per lipomatosi diffusa del colon.

Aim of this paper is to describe and discuss, on the basis of the available literature, the case of a young woman, previously colectomized for diffuse lipomatosis of the colon, showing hypomagnesemia and symptomatic (precordial discomfort) changes of repolarization phase, detected by ECG, probably due to coronary spasm. This hypomagnesemia (1.4 mEq/1) was probably due to altered intestinal absorption of magnesium, linked to a short bowel syndrome. The ECG changes and the precordial symptom were completely reversed by a relative short treatment with magnesium per os, which increased the magnesium level to low borderline value (1.6 mEq/1). The observation of ECG changes with precordial discomfort, probably linked to hypomagnesemia, suggests the need for routinary magnesium determinations to detect deficiency of this electrolyte, with the scope of improving the diagnosis and the treatment of several symptoms, otherwise difficult to interpret.

[Protein S deficiency and thrombophilia: presentation of a clinical case and review of the literature]. / Deficit di proteina S e trombofilia: presentazione di un caso clinico e revisione della letteratura.

We report the case of a 22-year-old obese woman with severe protein S deficiency, probably genetic in nature, associated with recurrent venous thrombosis. Protein S deficiency is a rather rare disease: it may be an inherited, either homozygous (purpura fulminans at neonatal age), heterozygous, or acquired disorder. The thrombophilic state may be manifested as deep vein thrombosis or thrombophlebitis of the superficial veins with a high risk of pulmonary embolism in the young, and it is often exacerbated by pregnancy. In our case, the presenting event, bilateral deep venous (iliac-femoral) thrombosis complicated by disseminated intravascular coagulation, had occurred when the patient was 13 years old. We started long-term therapy with oral coagulants, i.e. warfarin even if the latter may cause skin necrosis ("warfarin dermatitis") in some patients with protein S deficiency. The clinician must consider protein S deficiency in cases of recurrent thrombosis, particularly in young patients: the importance of early implementation of long-term preventive therapy should not be underestimated.

[Diffuse symptomatic polypoid lipomatosis of the colon with hyperplasia of epiploic appendices: a case report]. / Diffusa e sintomatica lipomatosi polipoide del colon con iperplasia delle appendici epiploiche: descrizione di un caso clinico.

Diffuse and symptomatic lipomatosis of the colon, particularly when associated with hyperplasia of the appendices epiploicae, is a rare disease. We report here the case of a lean, 26-year-old woman who had polypoid lipomatosis of the colon with submucosal and subserous expression (involving the mesentery and peritoneum) associated with extreme hyperplasia of the appendices epiploicae. Her disease led to a recurrent subocclusive syndrome which required total colectomy. We discuss the case and review the literature on this subject. The particular characteristics of this case and early onset of disease would suggest that our patient was affected by a congenital lipomatous syndrome linked to mesenchymal dysplasia. Long-term monitoring will enable prompt intervention in the case of reformation of lipomatous tissue.

[Day surgery and one day surgery in pediatric surgery: personal experience]. / "Day surgery" e "one day surgery" in chirurgia pediatrica: esperienze personali.

The Authors report their experience with 738 children observed and operated for minor diseases in the period January 1989-June 1993. The children were hospitalized for day surgery or one day surgery. After some surgical, anaesthesiological, and postoperative pathophysiological considerations, and on the basis of their results (reduced local and general postoperative complications), the Authors confirm the benefit of this type of organization in terms of reduced nosocomial infections, reduced psychological traumas and increased socio-economic advantages.

[Surgery of hyperthyroidism. Personal experience]. / Gli ipertiroidismi di interesse chirurgico. Esperienze personali.

The Authors report their experience in the management of surgical hyperthyroidism, evaluating the different clinical pictures and the possibility of non surgical treatment. Advantages and disadvantages of both hemithyroidectomy and total thyroidectomy, are also described. The Authors conclude affirming their preference for techniques such as total hemithyroidectomy or thyroidectomy in some types of hyperthyroidism.

[Intestinal invagination in childhood: etiopathogenetic evaluations and details of surgical technique]. / L'invaginazione intestinale in età pediatrica: valutazioni etiopatogenetiche ed accorgimenti di tecnica operatoria.

Intussusception in childhood, especially the idiopathic type, is herein considered. The Authors evaluate some etiopathological aspects on the basis of their experience in 34 patients, and affirm the need of an early and correct diagnosis. They also consider therapeutical management and surgical approach, evaluating local and general conditions of the patient as well as time passed since the onset of symptoms. Finally the importance of an accurate diagnostical examination during the quiescent period and some details of the surgical technique are stressed.

[Intestinal invagination in adults: 3 case reports]. / L'invaginazione intestinale nell'adulto: osservazioni su tre casi clinici.

The Authors report their experience in the surgical management of three adults affected by intussusception. The different symptoms and different diagnostic approach, compared to those of childhood, induced the Authors to some evaluations on pathophysiology of intussusception in adults. On the basis of the different pathogenesis some differential criteria between the two forms are stressed, finally suggesting a systematic surgical approach in adults.

[Primary biliary peritonitis without perforation in childhood: etiopathogenetic evaluations and 2 case reports]. / Peritoniti biliari primitive senza perforazione in età pediatrica: valutazioni etiopatogenetiche ed osservazioni su due casi clinici.

The Authors review biliary peritonitis. After some evaluations on etiopathogenesis, biliary peritonitis without perforation in childhood is discussed. The rarity of this form is considered as well as etiopathological hypothesis, difficulties of diagnosis and surgical management. Two cases of primary biliary peritonitis without perforation observed and operated in two children are reported.

[An update on parathyroid surgery]. / Attualità in tema di chirurgia delle paratiroidi.

The operative management of patients with hyperparathyroidism is controversial. High rates of persistent hypercalcemia and postoperative hypoparathyroidism are seen in multiple hyperplasia and bilateral neck exploration. Patients undergoing unilateral neck exploration with removal of a single parathyroid adenoma have a rapid clearance of PTH which declines within hours after surgery. There is a sensitive immunometric assay (IRMA) for the intact molecule which demonstrates a decline in 15 minutes during surgery. Intraoperative measurement of PTH may be complementary to surgical skill and histopathologic information and may modulate extension of neck exploration.

Scrotal dog bite: unusual case and review of pediatric literature.

Animal bites to human external genitalia are rare. Only a few cases of scrotal dog bite in children have been reported. We present an additional specific case of a scrotal dog bite in a child because the lesion and its repair have not been previously reported in published pediatric studies. A traumatic resection of the right testicular vas deferens was repaired by microsurgical vasoepididymal anastomosis. A review of the published data was also performed to analyze the management of scrotal dog bite lesions.

Fetus in fetu: report of an additional, well-developed case.

An 8-month-old boy presenting with a fast-growing abdominal mass was operated upon to remove the tumor, which was confirmed to be a well-formed fetus in fetu. The authors describe the clinical, radiologic, and surgical findings and review the literature.