Mercedes Romano

Spermatogenesis and cryptorchidism

Cryptorchidism represents the most common endocrine disease in boys, with infertility more frequently observed in bilateral forms. It is also known that undescended testes, if untreated, lead to an increased risk of testicular tumors, usually seminomas, arising from mutant germ cells. In normal testes, germ cell development is an active process starting in the first months of life when the neonatal gonocytes transform into adult dark (AD) spermatogonia. These cells are now thought to be the stem cells useful to support spermatogenesis. Several researches suggest that AD spermatogonia form between 3 and 9 months of age. Not all the neonatal gonocytes transform into AD spermatogonia; indeed, the residual gonocytes undergo involution by apoptosis. In the undescended testes, these transformations are inhibited leading to a deficient pool of stem cells for post pubertal spermatogenesis. Early surgical intervention in infancy may allow the normal development of stem cells for spermatogenesis. Moreover, it is very interesting to note that intra-tubular carcinoma in situ in the second and third decades have enzymatic markers similar to neonatal gonocytes suggesting that these cells fail transformation into AD spermatogonia and likely generate testicular cancer (TC) in cryptorchid men. Orchidopexy between 6 and 12 months of age is recommended to maximize the future fertility potential and decrease the TC risk in adulthood.

Diagnosis of Hirschsprung’s Disease: an age-related approach in children below or above one year

Aim  The aim of this study was to evaluate the effectiveness of a differential diagnostic approach to Hirshchsprung’s Disease (HD) on the basis of age.

Transumbilical Laparoscopic-Assisted Appendectomy in the Treatment of Acute Uncomplicated Appendicitis in Children

Transumbilical laparoscopic-assisted appendectomy (TULAA) is increasingly being performed worldwide. The authors report their experience in the treatment of acute uncomplicated appendicitis in children with TULAA. From January 2008 to December 2012 all types of acute appendicitis were divided, according to the clinical and ultrasonographic findings, into complicated (appendiceal mass/abscess, diffuse peritonitis) and uncomplicated. Complicated appendicitis was treated by open appendectomy (OA). All patients with the suspicion of uncomplicated appendicitis were offered TULAA by all surgeons of the team. Conversion to open or laparoscopic appendectomy (LA) was performed in case of impossibility to complete TULAA, depending on the choice of surgeon. The histopathologic examination of appendix was always performed. 444 children (252 males) with acute appendicitis were treated. The mean age was 9.2 years (range, 2 to 14 years). Primary OA was performed in 144 cases. In 300 patients a transumbilical laparoscopic-assisted approach was performed. TULAA was completed in 252 patients. Conversion to OA was performed in 45 patients and to LA in 3. Conversion was related to the impossibility to adequately expose the appendix in 47 patients and bleeding in 1. The mean operative time for TULAA was 42 minutes. Histopathologic examination of the appendix removed by TULAA showed a phlegmonous/gangrenous type in 92.8% of cases. Among the 252 TULAA there were 11 cases of umbilical wound infection. TULAA is a feasible and effective procedure for uncomplicated appendicitis in children. It combines the advantages of open and laparoscopic technique (low operative time, low complications rate, and excellent cosmetic results).

Clinical and radiological findings for early diagnosis of Herlyn–Werner–Wunderlich syndrome in pediatric age: experience of a single center

Abstract Objective: The authors present their experience in the management of pediatric patients with Herlyn–Werner–Wunderlich syndrome (HWWS) considering clinical classification and anatomical characteristics of the malformation. Methods: All the data of the patient presented at our Pediatric Surgery Unit from February 2010 to August 2015 were collected. According to the type of malformations, patients were divided in 3 groups: A (completely obstructed hemivagina), B (incompletely obstructed hemivagina), and C (communication between the duplicated cervices). Results: Six patients were treated in the study period. The mean age was 9 years (2 months–15 years). According to the characteristics of the HWWS, we had 5 patients in group A, 1 in group B, and none in group C. One-stage surgical treatment was performed in all cases of complete obstruction, but in one case a second look was necessary for a better resection of the septum. At a mean follow-up of 18 months all patients were symptoms free. Conclusions: Prognosis of this malformation is good in case of early diagnosis and treatment. We suggest that when a renal agenesia is diagnosed, the patient needs an ultrasonographic follow-up of the contralateral kidney but also of the genital tract to find each minimal abnormalities, furthermore, a MRI scanning before the onset of menstruation can be necessary.

Risk Factors of Cholelithiasis Unrelated to Hematological Disorders in Pediatric Patients Undergoing Cholecystectomy

Background Pediatric cholelithiasis unrelated to hematological disorders is an increasing disease. We analyzed our experience in the surgical treatment of these cases to evaluate risk factors, clinical presentation, intervention and follow-up. Methods From January 2010 to December 2016, we retrospectively recorded all data (hematological study, familiarity, use of lithogenic drugs and parenteral nutrition) of cholecystectomies for cholelithiasis not related to hematological diseases. The body mass index (BMI) was calculated (obesity if > 25), medical treatment, surgery and follow-up were evaluated. All patients underwent ultrasound for diagnosis and major biliary tract assessment prior to surgery. All patients had a 1-year follow-up. Results There were twenty-four cases (eight males), with a median age of 11.2 years. Predisposing factors were familiarity in 19, use of lithogenic drugs in 5 and total parental nutrition (TPN) in 3. Median BMI was 19.8 kg/m2, with BMI > 25 kg/m2 in eight cases. Regarding the clinical presentation, 14 had acute pain in the right upper quadrant, 5 had cholecystitis and 5 had non-specific abdominal pain. The medical treatment lasted 6 months in all, except for five (three operated after 2 months and two after 12 months). Preoperative ultrasound did not show stones in the biliary tract. MRI was performed in three cases for suspected malformation of the biliary tract (negative). Laparoscopic cholecystectomy was performed in all cases: mean intervention time was 95 min. A case of postcolecystectomy syndrome was found. At follow-up, all were asymptomatic, except two (recurrent abdominal pain). Conclusion Main predisposing factors are familiarity and obesity. Preoperative ultrasound in our series replaced the intraoperative study of the biliary tract. Laparoscopic cholecystectomy is the gold standard.

A possible association between sports and isolated fallopian tube torsion in children and adolescent females.

Abstract Isolated fallopian tube torsion (IFTT) is a rare occurrence characterized by the rotation of the fallopian tube on its own axis, without twisting the ipsilateral ovary. Pre-operative diagnosis is difficult due to the lack of specific clinical symptoms and radiological signs. The etiology is to date unknown and only some pre-disposing factors have been reported. The history, clinical presentation, laboratory findings and surgical treatment of five patients aged 12–16 with IFTT were evaluated. All patients came to be examined for worsening abdominal pain, nausea or vomiting. 75% of them practiced sports involving sudden changes in body position. They all underwent blood tests, ultrasound and Magnetic Resonance and then laparoscopy for the persistence of acute symptoms. Laparoscopic salpingectomy was performed in three patients. Another two needed a conversion to open salpingectomy due to the presence of pelvic adhesions. During the follow-up period, every girl was fine. The cause of IFTT is undetermined. In this series, a close association between IFTT and sports causing rapid body movements was found. The possibility of IFTT should be considered in the differential diagnosis of acute abdominal pain in children or adolescent females, especially if they practice sports. Chinese abstract 单纯性输卵管扭转（IFTT）是指输卵管以其自身轴线旋转的一种罕见情况，不伴有同侧卵巢的扭转。由于缺乏特异性的临床症状和影像学征象，术前诊断困难。目前病因不明，只有一些高危因素被报道。本研究总结了5例12–16岁单纯性输卵管扭转患者的病史、临床表现、实验室检查及手术治疗情况。所有患者均因腹部疼痛加重、恶心或呕吐就诊。75%的患者参与了身体位置突然变化的体育运动。他们都进行了血液检测、超声和磁共振，然后因为持续的剧烈症状进行腹腔镜探查。其中3例患者行腹腔镜下输卵管切除术。另两例患者因盆腔粘连中转开腹行输卵管切除术。在随访期间，每一个女孩都很好。单纯性输卵管扭转原因不明。在这些病例中，我们发现：导致身体快速活动的体育运动与单纯性输卵管扭转有密切联系。对儿童或青春期女性急性腹痛鉴别诊断时应考虑单纯性输卵管扭转的可能，尤其是他们参与体育运动后。

One-trocar surgery

Si definisce “:one-trocar surgery” tutta la chirurgia mininvasiva (laparoscopica, retroperitoneoscopica, toracoscopica) che viene eseguita utilizzando un unico trocar e un’ottica operativa. La tecnica chirurgica puo essere completamente endoscopica o videoassistita. Questo approccio, descritto inizialmente per l’esecuzione dell’appendicectomia transombelicale videoassistita, prima nell’adulto e poi nel bambino, oggi trova numerose indicazioni in eta pediatrica, rappresentando, in taluni casi, la meno invasiva delle metodiche videochirurgiche. Inoltre, l’approccio videoassistito costituisce una felice sintesi dei vantaggi della mininvasivita e della chirurgia tradizionale.

Testicolo non palpabile

Il criptorchidismo e la mancata presenza di uno o di entrambi i testicoli nello scroto; l’incidenza e dell’ 1% a 1 anno di eta, del 3% nei nati a termine e del 33% nei prematuri. L’eziopatogenesi sembra essere multifattoriale per la possibile azione di diversi meccanismi: genetici, ormonali e meccanici. Circa il 20% dei pazienti criptorchidi ha un testicolo non palpabile; questo e tale perche agenesico, scomparso in utero (vanishing testis) o localizzato in addome a una distanza variabile dall’anello inguinale interno. La laparoscopia e la procedura piu efficace nella diagnostica del testicolo non palpabile e nella pianificazione chirurgica del testicolo intraddominale.