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Previous studies in developing Xenopus and zebrafish reported that the phosphate transporter slc20a1a is expressed in pronephric kidneys. The recent identification of SLC20A1 as a monoallelic candidate gene for cloacal exstrophy further suggests its involvement in the urinary tract and urorectal development. However, little is known of the functional role of SLC20A1 in urinary tract development. Here, we investigated this using morpholino oligonucleotide knockdown of the zebrafish ortholog slc20a1a. This caused kidney cysts and malformations of the cloaca. Moreover, in morphants we demonstrated dysfunctional voiding and hindgut opening defects mimicking imperforate anus in human cloacal exstrophy. Furthermore, we performed immunohistochemistry of an unaffected 6-week-old human embryo and detected SLC20A1 in the urinary tract and the abdominal midline, structures implicated in the pathogenesis of cloacal exstrophy. Additionally, we resequenced SLC20A1 in 690 individuals with bladder exstrophy-epispadias complex (BEEC) including 84 individuals with cloacal exstrophy. We identified two additional monoallelic de novo variants. One was identified in a case-parent trio with classic bladder exstrophy, and one additional novel de novo variant was detected in an affected mother who transmitted this variant to her affected son. To study the potential cellular impact of SLC20A1 variants, we expressed them in HEK293 cells. Here, phosphate transport was not compromised, suggesting that it is not a disease mechanism. However, there was a tendency for lower levels of cleaved caspase-3, perhaps implicating apoptosis pathways in the disease. Our results suggest SLC20A1 is involved in urinary tract and urorectal development and implicate SLC20A1 as a disease-gene for BEEC.

Bladder exstrophy epispadias complex (BEEC) is a rare congenital malformation affecting both male and female with an incidence of 1/35,000 livebirths. The malformation involves the bladder, urethra, external genitalia and pelvis. The newborn presents with a lower abdominal defect, through which an open bladder template herniates. The malformation requires a staged surgical treatment but even after successful repair patients can experience psychosexual difficulties throughout their lives.This project investigates the presence or absence of a depressive disorder with sadness, emptiness, irritability, accompanied by somatic and cognitive changes which can significantly impact on the overall performance of the individual. The degree of alexithymia, a difficulty in verbalizing emotions, as well as overall quality of life of affected people is examined by means of several tests and specific interviews, created during the study. Aim of the study To evaluate the presence of alexithymia, depressive traits, difficulties in sexual assertiveness and/or in sex-lives and overall quality of life in people with BEEC compared with a group of unaffected peers. Materials and methods This case-control observational study included a group of BEEC cases compared with a group of control subjects unaffected by any urogenital malformation. Inclusion criteria for BEEC group were: proved diagnosis of BEEC; age> 16 years; sex M and F; compilation of informed consent and privacy forms. Inclusion criteria for control cases were: absence of any urogenital malformation; age> 16; sex M and F; compilation of informed consent and privacy forms. The following questionnaires were administered to fifty cases and fifty controls: the Beck Depression Interventory (BDI-II), the Toronto Alexithymia Scale (TAS-20), the Quality of Life, (SF-36), The Sexual Awareness Questionnaire (SAQ) and the Sexual History Form (SHF). The draft of a structured interview was administered to five cases. Data were analyzed by open source Rstudio and Kruskal-Wallis test. Results There were no differences between cases and controls as far as the presence of alexithymia and depressive traits are concerned. The QoL cases scored lower in physical activities and general health compared to controls; sex life experiences of the case group significantly differ from controls in terms of sexual monitoring (SAQ subscale). Women in the BEEC group had significantly lower scores in the Sexual History Form compared to controls. Conclusion Our findings demonstrate that the emotional burden suffered by the female affected population exceed that of the male population, therefore female would need an implementation of interventions to improve their sexual experiences. Further research on psycolosexual aspects and gender differences are needed to validate these data.

The goal of this update regarding the diagnosis and care of persons with disorders of sex development (DSDs) is to address changes in the clinical approach since the 2005 Consensus Conference, since knowledge and viewpoints change. An effort was made to include representatives from a broad perspective including support and advocacy groups. The goal of patient care is focused upon the best possible quality of life (QoL). The field of DSD is continuously developing. An update on the clinical evaluation of infants and older individuals with ambiguous genitalia including perceptions regarding male or female assignment is discussed. Topics include biochemical and genetic assessment, the risk of germ cell tumor development, approaches to psychosocial and psychosexual well-being and an update on support groups. Open and on-going communication with patients and parents must involve full disclosure, with the recognition that, while DSD conditions are life-long, enhancement of the best possible outcome improves QoL. The evolution of diagnosis and care continues, while it is still impossible to predict gender development in an individual case with certainty. Such decisions and decisions regarding surgery during infancy that alters external genital anatomy or removes germ cells continue to carry risk. (C) 2016 S. Karger AG, Basel

L'identità di genere è una dimensione dell'identità sessuale e si riferisce alla persistente concezione personale di sé come maschio o femmina. Lo sviluppo delle identità di genere non è sempre conforme al sesso biologico, quindi anche in tenera età alcuni bambini non si identificano con il genere assegnato alla nascita (varianza di genere). In alcuni casi questa condizione può essere accompagnata da disagio (disforia di genere). I pediatri sono spesso il primo contatto che questi bambini / adolescenti hanno con il sistema sanitario, quindi dovrebbero essere in grado di rispondere alle domande delle famiglie e quando individuano un'eventuale disforia di genere dovrebbero affrontare la questione semplicemente, ricercando gli eventuali problemi psicosociali associati.

The bladder extrophy and epispadias is a rare disease that affects 1 / 40,000 live births. Children present at birth a malformation of varying degrees of their in genitals with consequent problems of incontinence and genital organ dysfunction. The treatment of this condition is purely surgical and aims, in part, to achieve urinary continence in some cases, in a natural way. However most cases need to use intermittent catheterization. The second objective is to achieve an aesthetic and functional reconstruction of the external genital tract. In this context of informing and supporting those born with this rare condition the idea to create a story to be used in an educational context, came up. This is to educate on disability and rare diseases. It also represents a very useful means for going into schools where there are children with urogenital conditions, because this teaches the class about diversity. Through the story, and the use of drawings we want to tell a story about a person and not about the disease and to see the person first as an individual and only then the condition they have.

il complesso estrofla vescicale-epispadia rappresenta un gruppo di malformazioni genito-urinarie che vanno dall’epispadia all’Estrofia Vescicale (EV) classica, all’estrofia cloacale. Rappresenta una delle condi zioni più difficili in urologia pediatrica. Oltre ai più o meno numerosi e complessi interventi chirurgici ne cessati per correggere le gravi malformazioni di vescica e genitali, potranno insorgere nel tempo problemi secondari relativi alla funzione renale, alla continenza urinaria e fecale, alla fertifità, allo sviluppo psicoso ciale e psicosessuale. E' auspicabile quindi che il paziente estrofico sia seguito, dalla nascita alla pubertà, in Centri dedicati alla cura di questa rara patologia.

In the appendix of the recent publication by Lee et al. entitled 'Global disorders of sex development update since 2006: perceptions, approach and care' [Horm Res Paediatr 2016;85:158–180, DOI: 10.1159/000442975], Massimo Di Grazia, Psychologist, is incorrectly mentioned to be from Cosenga, Italy. The correct city is Trieste, Italy.

Bladder exstrophy-epispadias complex (BEEC) represents a spectrum of genitourinary malformations concerning male and female sexes. It needs different surgical operations to obtain, primarily, secure abdominal wall closure, achieving urinary continence with preservation of renal function, and, finally, adequate cosmetic and functional genital reconstruction. The psychological feature and the sexual aspects of this pathology have important effects on the patient’s and their family’s past. So, the multidisciplinary approach, which involves psychologist and surgeon upfront, permits: to completely answer patients’ and relatives’ questions, to give emotional and sexual education, emphasizing the expression and the verbalization of the emotions, and to give people concerned useful tools to face the BEEC in a more conscious way. By treating the pathology in a multidimensional way, it is possible to accompany the patients and their families along the developmental steps, optimizing therapeutic intervention benefits.

La Sindrome di Klinefleter (SK) fu descritta per la prima volta nel 1942 da Harry F. Klinefelter, Reifensteined Albright come una patologia endocrina “caratterizzata da un quadro clinico caratterizato da: testicoli fibrosi di ridotta dimensione, ginecomastia bilaterale, ipogonadismo iporgonadotropo primitivo maschile congenito con scarso funzionamento delle gonadi e ridotta quantità di gonadotropine, alterato metabolismo degli steroidi sessuali secondario alla scarsa concentrazione ematica di androgeni” (1). La SK ha una prevalenza di 1/660-1000 maschi nati vivi ma ancora oggi ben i 2/3 dei casi non vengono diagnosticati. La causa etiologica è stata scoperta nel 1959 da Jacobs e Strong, i quali hanno dimostrato un cromosoma X soprannumerario rispetto al cariotipo normale 46,XY (2). L’80% dei casi ha cariotipo 47,XXY, mentre la quota rimanente dei pazienti presenta aneuploidie più numerose come 48,XXXY, 49,XXXXY, caratterizzate da forme cliniche più gravi (3). Oltre ai segni clinici caratteristici,sono presenti problemi di natura psico-comportamentale (4), difficoltà di concentrazione, di comunicazione, disturbi della memoria uditiva e visiva a breve termine (MBT), scarsa socializzazione, lentezza nell’esecuzione dei movimenti. A livello intellettivo i soggetti non presentano ritardo mentale, ma piuttosto dimostrano un certo ritardo del linguaggio e organizzativo presentando un quoziente intellettivo (QI) di circa 10 punti inferiore rispetto alla media della popolazione generale (5-7). Alcune ricerche scientifiche descrivono i ragazzi affetti dalla SK, come timidi, immaturi e riservati, con problematiche nella realizzazione di relazioni sociali profonde e soddisfacenti, una maggiore probabilità di soffrire di disturbi del comportamento e disturbi del tono dell’umore che si accentuano nella fase adolescenziale (8, 9). De Ronde et al. (10) hanno rilevato punteggi minori nelle funzioni fisiche, sociali e psicosociali rispetto alla popolazione generale, tenendo anche conto della loro difficoltà a verbalizzare le emozioni, esprimendo lo scarso senso di benessere come un problema fisico e non dovuto ad altre dimensioni relazionali e sociali. I soggetti affetti da SK sviluppano solitamente una pubertà spontanea vera e un discreto numero riesce ad assumere comportamenti sessuali normali. Alle volte i pazienti dimostrano una riduzione della libido con limitazione della loro attività sessuale a pratiche masturbatorie. Questa modalità di vivere la sessualità è legata alla timidezza, ai problemi di carattere psico-sociale, alla scarsa pulsione sessuale e allo sviluppo di un’erotismo prepubere. Tutti i lavori specifici in letteratura hanno riconosciuto un ruolo fisiologico del testosterone (T) nei vari aspetti della risposta sessuale a partire dal desiderio sessuale, l’eccitazione, l’orgasmo e l’eiaculazione, cercando di sincronizzare l’erezione al processo emotivo (11). Yoshida et al. (12) hanno rilevato che il 67.5% dei soggetti affetti dalla SK dimostra un disturbo della funzione sessuale, ma senza differenza significativa con la percentuale di disturbi rilevata nei soggetti di controllo. Corona et al. (13) hanno riportato la presenza di disfunzione erettile (DE) severa, e eiaculazione precoce ma dopo una regressione logistica hanno verificato l’associazione di tali disturbi con il desiderio sessuale ipoattivo per il sottostante stato di ipogonadismo. Nielsen et al. (14) hanno evidenziato una minore probabilità di ricorrere alla masturbazione durante l’adolescenza e ai rapporti sessuali nel matrimonio rispetto ad un gruppo di maschi della stessa età. Dopo i 25 anni, circa il 70% dei pazienti con SK manifesta un generale calo della libido e della potenza sessuale come risulta del deficit generalizzato di androgeni. La terapia si basa sulla somministrazione di T che aumenta la forza muscolare, la resistenza alla fatica, migliora l’umore, la capacità di concentrazione, migliora le relazioni interpersonali, aumenta la performance sessuale e previene le comorbidità consequenziali. Si ipotizza che tale terapia possa migliorare anche il desiderio sessuale ipoattivo visto che in parte esso risulta influenzato oltre che da condizioni relazionali e intrapsichiche, sicuramente da quelle ormonali.

L'Associazione Italiana Estrofia Vescicale-Epispadia Onlus è nata nel 2006, fortemente voluta da pazienti adulti e genitori di bambini affetti da estrofia vescicale. L'A.E.V. crea il contatto tra i pazienti e le famiglie e favorisce lo scambio di informazioni. L'obiettivo è la sensibilizzazione e il coinvolgimento di tutti gli operatori che si occupano del problema-estrofia, in modo tale che la patologia venga trattata in maniera completa.

Introduzione: l’Estrofia Vescicale è una malattia rara caratterizzata da uno sviluppo incompleto della vescica e dell’uretra con compromissione dello sviluppo fisiologico degli organi genitali sia maschili che femminili. La prevalenza di tale malformazione congenita varia da 1/25.000 a 1/40.000 nati vivi. La patologia colpisce maschi e femmine con un rapporto di 2:1. La causa etiologica non è ancora nota. La terapia è esclusivamente chirurgica. Obiettivo: vista la persistenza in età adulta di un’anomalia anatomica più o meno importante dell’apparato genitale di questi pazienti sia maschi che femmine, ci siamo prefissi di ricercare se questa anomalia influisca sul loro profilo idiografico, psicosessuale e socio affettivo. Materiale e Metodi: l’indagine ha utilizzato lo strumento di rilevazione SESAMO (Sexuality Evaluation Schedule Assessment Monitoring - Boccadoro, Perillo ‘96). Sono stati individuati due gruppi d’indagine: un gruppo costituito dai pazienti (casi) e un gruppo di soggetti non portatori della patologia (controlli). I soggetti sono stati invitati a rispondere alla versione maschile o femminile del questionario sia in forma cartacea che elettronica. I questionari sono stati elaborati dal software SESAMO-R. Risultati preliminari: dei 13 casi e 13 controlli contattati, hanno risposto 10 casi (5 M e 5 F) e 10 controlli (5 M e 5 F) con un’età compresa tra i 21 e i 43 anni. 13 hanno compilato la versione cartacea del questionario mentre gli altri 7 hanno compilato direttamente la versione elettronica. Il questionario di coppia è stato compilato da 5 controlli e 3 casi. La maggior parte dei soggetti affetti dalla patologia evidenzia un profilo ansioso- depressivo, un importante desiderio di modificazione del proprio organo sessuale, scarse esperienze sessuali e difficoltà d’interazione con l’altro sesso. Discussione: dai risultati esposti emerge la necessità di presa in carico dei pazienti affetti da estrofia vescicale mediante incontri di counseling sessuologico e educazione sessuale fin dall’adolescenza al fine di migliorare il loro sviluppo psicosessuale e la qualità della vita relazionale in rapporto alla patologia.

Gender identity does not always develop in line with biological sex. Gender dysphoria at young age implies a strong incongruence between gender identity and the assigned sex; the rejection of one\´s sexual attributes and the desire to belong to the opposite sex; and a significant clinical suffering or impaired individual functioning in life spheres. The purpose of this chapter is a narrative review of the literature available on puberty suppression therapy through GnRH analogues. Biological puberty provides intense suffering to the adolescent with gender dysphoria who does not recognize himself in his own body. These drugs suppress the production of endogenous gametes and sex hormones. Although the effects of therapy are reversible, and biological development resumes spontaneously once the medication is stopped, the administration of GnRH analogues at a young age has fueled a scientific debate on the matter of the ethics of pharmacological intervention with minors. In conclusion, the studies considered show that GnRH analogues do not have long-term harmful effects on the body; prevent the negative psychosocial consequences associated with gender dysphoria in adolescence (suicidal ideation and attempts, self-medication, prostitution, self-harm); improve the psychological functioning of young transsexuals; and are diagnostic tools that allow adolescents to buy time to explore their gender identities.

Highlights • Small-bowel volvulus could be a presentation of a cystic mesenteric LM. • Diagnosis could be intraoperative. • Complete excision including, in some selected cases, a bowel resection, should be the treatment of choice., Introduction An acute abdomen in the form of small-bowel volvulus could be a presentation of a lymphatic malformation in childhood. Case presentation A 5 year old male was admitted to our Institute for an acute abdomen. Clinical aspects and radiological images were not specific for a certain diagnosis. Laparotomy revealed a big soft mass, with a milky content, completely involving about 50 cm of ileus with a partial volvulus of the intestinal loop. A complete mass excision and also a bowel involved resection were performed. After a histological examination, a lymphatic malformation was diagnosed. Discussion The diagnosis of a mesenteric lymphatic malformation could be intraoperative and a complete resection should be the treatment of choice. Sometimes it could be necessary to perform an involved bowel tract resection in the case of volvolus with ischemia. Conclusions Paediatricians and surgeons should bare in mind that an intrabdominal lymphatic malformation may present as a nonspecific an acute abdomen caused by a bowel volvolus and diagnosis may not be so simple preoperatively.

46,XY ovotesticular disorder of sex development is extremely rare and indicates the presence of both testis and ovary in the same patient. Gender assignment in newborns represents a therapeutic challenge. We describe and comment on our multidisciplinary approach, ten years after the Chicago consensus meeting on disorder of sex development.

Previously genome-wide association methods in patients with classic bladder exstrophy (CBE) found association with ISL1, a master control gene expressed in pericloacal mesenchyme. This study sought to further explore the genetics in a larger set of patients following-up on the most promising genomic regions previously reported. Genotypes of 12 markers obtained from 268 CBE patients of Australian, British, German Italian, Spanish and Swedish origin and 1,354 ethnically matched controls and from 92 CBE case-parent trios from North America were analysed. Only marker rs6874700 at the ISL1 locus showed association (p = 2.22 × 10−08). A meta-analysis of rs6874700 of our previous and present study showed a p value of 9.2 × 10−19. Developmental biology models were used to clarify the location of ISL1 activity in the forming urinary tract. Genetic lineage analysis of Isl1-expressing cells by the lineage tracer mouse model showed Isl1-expressing cells in the urinary tract of mouse embryos at E10.5 and distributed in the bladder at E15.5. Expression of isl1 in zebrafish larvae staged 48 hpf was detected in a small region of the developing pronephros. Our study supports ISL1 as a major susceptibility gene for CBE and as a regulator of urinary tract development.

Summary Introduction Bladder-exstrophy-epispadias complex (BEEC) represents a spectrum of urogenital step-wise malformations: epispadias, complete exstrophy, and cloacal exstrophy. Psychosexual development in adolescent patients with BEEC may become especially problematic. At present, there are few contributions in the literature investigating the validity of psychosexual treatment in order to tackle this particularly emotional and personal development phase. Objective The study aimed at verifying the efficacy of an intervention methodology for psychosexual support of a group of adolescents with BEEC. The main goal of the intervention program was to educate the adolescents and re-frame how they see themselves or feel about themselves, especially in relation to BEEC. In particular it was predicted that the program could: (1) improve the perception of pleasure concerning the body, particularly regarding the genital area, giving proper and specific information on pleasure, masturbation and medical history of BEEC; and (2) elicit a more relational-affective perspective on sexuality. Study design 13 adolescent patients took part in the 1-year program. The effects of the intervention program were verified through a test-retest methodology using Sexuality Evaluation Schedule Assessment Monitoring (SESAMO). Results The results showed that participants changed their attitude in several psychosexual areas, more specifically: psycho-environmental situations, body experience, areas of pleasure, medical and sexual history, and motivation and conflict areas ( Summary Table ). Discussion This study demonstrated, for the first time, that a targeted program may significantly improve the psycho-sexual condition of adolescents with BEEC. In particular, this research showed that adolescents need to be able to discuss and tackle topics of a psychological and sexual nature, as well as receive understandable answers that can be put into practice in their everyday lives. The study had several methodological limitations, especially owing to the limited number of participants, the absence of a follow-up period of a few months after the intervention, and the overall exploratory nature of the program. Conclusion This intervention methodology may be considered a first attempt at improving the self-esteem of adolescents with BEEC, by contrasting forms of psychological difficulties in order to improve the quality of life of these young people. Table . Areas Median percentile rank-pre pre-intervention Median percentile rank-post postintervention M F M F Related psycho-environmental situations 84* 89* 37 37 Body experience 98* 98* 46 38 Areas of pleasure 90* 90* 37 37 Medical and sexual history 99* 98* 40 40 Motivation and conflict 95* 95* 31 31

Contains fulltext : 172059.pdf (Publisher’s version ) (Open Access) BACKGROUND: The bladder exstrophy-epispadias complex (BEEC) represents the severe end of the congenital uro-rectal malformation spectrum. Initial studies have implicated rare copy number variations (CNVs), including recurrent duplications of chromosomal region 22q11.21, in BEEC etiology. METHODS: To detect further CNVs, array analysis was performed in 169 BEEC patients. Prior to inclusion, 22q11.21 duplications were excluded using multiplex ligation-dependent probe amplification. RESULTS: Following the application of stringent filter criteria, seven rare CNVs were identified: n = 4, not present in 1307 in-house controls; n = 3, frequency of

Summary Introduction The bladder exstrophy–epispadias complex (BEEC) represents a spectrum of malformations that affect the anatomical and functional structure of the urogenital system. The parents of patients affected by this condition are subject to particularly stressful situations, such as worrying about their child's health, long hospital stays, concerns about the health and constant need for personal care for their children, that can profoundly compromise the quality of family life. Objective The objective of this explorative qualitative study is to evaluate the social situation and the psychological strategies implemented by the mothers of children between 6 and 10 years of age who are affected by BEEC. Study design Fourteen mothers of children aged 6–10 years and affected by BEEC (9 boys and 5 girls) were interviewed. Data on the mothers' experiences were collected through semi-structured interviews (Table). Results The qualitative analysis of the interviews showed that participants described experiences that were characterised by emotions such as fear and anger. Each mother had implemented a different and, sometimes, dysfunctional strategy in order to cope with the complex situation of the son/daughter. The aspects that most clearly emerged from mothers' descriptions were (1) the traumatic situation at the birth of the baby, (2) the sense of embarrassment concerning the pathological condition as the child was growing and the consequent sense of isolation of the mother, and (3) the fluctuation of feelings towards the multidisciplinary staff, which was sometimes seen as an important source of help and some other times as too destabilising and not helpful at all. Discussion The study provided some insight into the psychological and social conditions experienced by mothers of children with BEEC, which could serve as a basis for developing multidisciplinary teams with greater awareness about families living with this condition and better timing in addressing their needs. Conclusions Mothers of children with BEEC show emotional and social difficulties. This is a crucial aspect to consider when planning a multidisciplinary approach to the treatment/therapy, especially considering that children examined in this study are approaching adolescence. Table . Semi-structured interview topic. Number of questions 1 Impact of the diagnosis 4 2 Relationship with the medical team 2 3 Relationship with the partner 4 4 Social life: relationship with colleagues at work, relatives and friends 4

AIM: to assess, by a questionnaire, the level of children’s satisfaction relating to three informative cards, including an original nursery rhyme, administered to the patient before a medical procedure. MATERIALS AND METHODS: We created three types of specific informative cards, relating to three medical topics, with the double role function: informing the child before the medical procedure and distracti ng him/her through an original nursery rhyme read aloud by the authors . To assess the level of children's satisfaction relating to the cards, we administered to the patients a satisfaction questionnaire. RESULTS: According to children's satisfaction, the cards resulted funny and useful. CONCLUSION: The cards can be considered a feasible and applicable original funny technique of information and distraction for surgical children.

In questa ricerca si affronta l’aspetto della sessualita/affettivita di persone che presentano una disabilita intellettiva (DI). Per affrontare questi aspetti e stata considerata la prospettiva degli operatori delle comunita che hanno in carico la persona con disabilita intellettiva. La tipologia delle comunita coinvolte nella ricerca presentano vari assetti organizzativi ed un grado di eterogeneita delle patologie di disabilita intellettiva degli utenti assistiti. Lo strumento utilizzato per l’indagine condotta sugli operatori e stato il questionario SMRAI. La ricerca ha rilevato una carenza nella formazione specifica dell’educazione affettiva/sessuale, ma anche nella capacita di affrontare tutte le dinamiche degli atteggiamenti personali che mettono gli operatori in confronto con i propri valori e la propria cultura, arricchendo il tutto con delle riflessioni di bioetica su questo tema.

Highlights • There are no clear guidelines in the treatment of a perforated appendicitis associated with localized abscess. • Our team’s therapeutic choice, also with presence of coprolith, is the initial conservative case management followed by a routine interval appendectomy. • Interval appendectomy should be performed not later than 4 months after discharge., Introduction There are no clear guidelines in the treatment of a perforated appendicitis associated with periappendiceal abscess without generalized peritonitis. Presentation of cases We retrospectively studied six examples of treated children in order to discuss the reasons of our team’s therapeutic approach. Some children were treated with a conservative antibiotic therapy to solve acute abdomen pain, planning a routine interval appendectomy after some months. Others, instead, underwent an immediate appendectomy. Discussion By examining these examples we wanted to highlight how the first approach may be associated with shorter surgery time, fewer overall hospital days, faster refeeding and minor complications. Conclusion Our team’s therapeutic choice, in the case of a perforated appendicitis with an abscess and coprolith is an initial conservative case management followed by a routine interval appendectomy performed not later than 4 months after discharge.