A novel therapy for idiopathic urethritis in children: Local steroid injection.

Idiopathic urethritis (IU) is difficult to manage and there is no standarized therapy. The technique of local steroid injection (LSI) for the treatment of IU in children and the results of the patients undergoing LSI from 2020 to 2021 in a single center are presented. Seven patients with IU underwent LSI. An internal urethrotomy was also performed in two patients with stricture. Complete resolution of symptoms and signs occurred in six patients. The remaining patient did not achieve total remission but did substantially improve symptoms. LSI seems to be an effective alternative for treatment of IU in children.

Is Being Overweight a Risk Factor for Torsion of the Appendix Testis in Children?: A Paired Case-Control Study.

OBJECTIVE: This study aimed to determine whether being overweight during childhood is a risk factor for torsion of the appendix testis (TAT). METHODS: We conducted a paired case-control study with all boys surgically treated for TAT, paired by age and sex with patients who were seen in the emergency department for a reason other than acute scrotum. Age and weight were registered, and weight percentile (WP) by age was calculated and compared between groups. Weight percentile was classified into 4 groups (<25, 25-50, 50-75, >75), and a conditional logistic regression analysis was performed to establish the risk of TAT related to the WP. RESULTS: A total of 980 patients were diagnosed as having TAT in our institution, of which 118 patients (12%) were surgically treated and randomly paired with 118 controls. Mean age was the same in both groups (11.2 years, P = 0.908). Patients with TAT had a higher median of WP (79; interquartile range, 49-94) than the control group (49; interquartile range, 20-79; P < 0.0001). For each WP point increase, risk of TAT raised by 2.2% (odds ratio [OR], 1.022; 95% confidence interval [CI], 1.011-1.032; P < 0.001). Weight percentile >75 increased the risk of TAT: 6-fold compared with WP <25 (OR, 5.9; 95% CI, 2.5-14; P < 0.001), 4-fold compared with WP of 25 to 50 (OR, 4.3; 95% CI, 1.8-10.5; P = 0.001), and 3-fold compared with WP of 50 to 75 (OR, 3.3; 95% CI, 1.5-7.6; P = 0.004). CONCLUSIONS: Overweight increases the risk of being operated on because of TAT. Because obesity is a rising problem, a greater incidence of TAT remains to be proved.

Translation and cultural adaptation of the pectus excavatum evaluation questionnaire to Spanish and Catalan.

PURPOSE: Pectus excavatum (PE) is the most common congenital chest wall deformity. It can have a negative effect in exercise tolerance. However, cosmetic features are the most frequent concerns in these patients. The PE evaluation questionnaire (PEEQ) is a patient reported outcome (PRO) tool to measure the physical and psychosocial quality-of-life changes after surgical repair of PE. No specific tool has been developed in our languages to evaluate PRO in PE patients. Our aim is to translate and culturally adapt the PEEQ to Spanish and Catalan. METHODS: Guidelines for translation of PRO were followed. The PEEQ, consisting of 34 items, was translated from English to Spanish and to Catalan. Three forward translations and one back translation were performed for each language. Cognitive debriefing interviews were developed. RESULTS: The reconciliation of the forward translations revealed a 14.7% of inconsistencies for each language. The Spanish back translation showed a 64.7% of disagreement with the source, the Catalan 58.8%. Changes in each reconciled version were made to amend the diverting items. Cognitive debriefing: Catalan version: 15 participants, 10 males, 5 patients had been operated. 12 patients showed difficulties for understanding 4 of the items. Spanish version: 17 participants, 11 males, 5 had been operated. 13 patients showed difficulties for understanding 4 of the items. We made modifications of the problematic items, in order to make them easier to understand for our patients. We tested the last version in a new group of patients. Catalan: 7 patients, 5 males. One patient showed difficulties for understanding item 11, so we added a further clarification of this item. Spanish: 7 patients, all males. There were any difficulties for understanding. CONCLUSION: After a thorough process of translation and cultural adaptation, we reached a Catalan and a Spanish version of PEEQ. This work constitutes the first step to reach a specific PE PRO tool in our languages. However, it needs to be validated, with a higher number of patients, before being widely used in a clinical setting.

Porcine dermal patches as a risk factor for recurrence after congenital diaphragmatic hernia repair.

PURPOSE: Recurrence of congenital diaphragmatic hernia (CDH) is a treatment-related morbidity which can be preventable. There is no consensus about the ideal material for diaphragmatic substitution. The aim of our study is to identify if the use of porcine dermis patches increases the risk of CDH recurrence. METHODS: Retrospective review of medical records of CDH patients treated between 2013 and 2017 in our center was carried out. Demographic, clinical and surgical variables were collected. Regression analysis was performed to identify which factors increase the risk of recurrence. RESULTS: 50 patients entered the study. 94% of the patients had a left CDH, mean observed/expected lung-to-head ratio was 46%. 17 patients underwent a primary closure, the rest a patch closure: 25 Gore-Tex® and 8 porcine dermis patches were used. Seven patients presented recurrence (14%). Median follow-up time was 3.5 years (1.2-6.2). Univariate analysis revealed that the use of a porcine dermis patch (75%) increased the risk of recurrence compared with Gore-Tex® patch (4%) and primary closure (0%) p < 0.001 (HR 58.7; IC 95%: 6.9-501.2; p < 0.001). CONCLUSION: The main risk factor for CDH recurrence is the use of a porcine dermis patch. We do not recommend the use of these patches for CDH repair.

Effects of a Paediatric Antimicrobial Stewardship Program on Antimicrobial Use and Quality of Prescriptions in Patients with Appendix-Related Intraabdominal Infections.

The effectiveness of antimicrobial stewardship programs (ASP) in reducing antimicrobial use (AU) in children has been proved. Many interventions have been described suitable for different institution sizes, priorities, and patients, with surgical wards being one of the areas that may benefit the most. We aimed to describe the results on AU and length of stay (LOS) in a pre-post study during the three years before (2014-2016) and the three years after (2017-2019) implementation of an ASP based on postprescription review with feedback in children and adolescents admitted for appendix-related intraabdominal infections (AR-IAI) in a European Referral Paediatric University Hospital. In the postintervention period, the quality of prescriptions (QP) was also evaluated. Overall, 2021 AR-IAIs admissions were included. Global AU, measured both as days of therapy/100 patient days (DOT/100PD) and length of therapy (LOT), and global LOS remained unchanged in the postintervention period. Phlegmonous appendicitis LOS (p = 0.003) and LOT (p < 0.001) significantly decreased, but not those of other AR-IAI diagnoses. The use of piperacillin-tazobactam decreased by 96% (p = 0.044), with no rebound in the use of other Gram-negative broad-spectrum antimicrobials. A quasisignificant (p = 0.052) increase in QP was observed upon ASP implementation. Readmission and case fatality rates remained stable. ASP interventions were safe, and they reduced LOS and LOT of phlegmonous appendicitis and the use of selected broad-spectrum antimicrobials, while increasing QP in children with AR-IAI.

La atención primaria del paciente con el antecedente de una malformación digestiva, defectos de pared abdominal o diafragmáticos / The primary care of a patient with a history of a gastrointestinal malformation and abdominal wall or diaphragmatic defects

La supervivencia de los pacientes con antecedentes de malformaciones congénitas complejas ha aumentado en las últimas décadas. El pediatra de atención primaria debe conocer los problemas más habituales que pueden presentar este grupo de pacientes. Además, puede ofrecer una visión global que, a menudo, se pierde en las consultas especializada. En este trabajo se recogen algunas de las malformaciones congénitas digestivas y respiratorias más habituales, como atresia de esófago, defectos de pared abdominal, malformación anorrectal y enfermedad de Hirschsprung, y hernia diafragmática congénita. Se señalan los problemas de mayor interés para el pediatra, haciendo hincapié en las complicaciones a largo plazo y en todas las dimensiones de la persona

Survival of patients with congenital malformation has improved over the last decades. Primary care paediatricians must be aware of the most common problems that this group of patients suffers. More importantly, paediatricians can offer a holistic view that is often lost in specialised consultation. This article is focused on common congenital malformation, such as oesophageal atresia, abdominal wall defects, anorectal malformation and Hirschsprung disease, and congenital diaphragmatic hernia. The main problems are shown, with special emphasis on long-term complications and all the dimensions of the individual

[The primary care of a patient with a history of a gastrointestinal malformation and abdominal wall or diaphragmatic defects]. / La atención primaria del paciente con el antecedente de una malformación digestiva, defectos de pared abdominal o diafragmáticos.

Survival of patients with congenital malformation has improved over the last decades. Primary care paediatricians must be aware of the most common problems that this group of patients suffers. More importantly, paediatricians can offer a holistic view that is often lost in specialised consultation. This article is focused on common congenital malformation, such as oesophageal atresia, abdominal wall defects, anorectal malformation and Hirschsprung disease, and congenital diaphragmatic hernia. The main problems are shown, with special emphasis on long-term complications and all the dimensions of the individual.

Poland Sequence: Retrospective Analysis of 66 Cases.

INTRODUCTION AND OBJECTIVES: The literature reports many variations of Poland syndrome, which is a congenital disorder and a rare condition. The associated features are extremely variable, including alterations in the ipsilateral thorax and upper extremity and other malformations. This is used to designate the condition as Poland sequence (PS) because a sequence is a primary defect with a secondary cascade of structural changes. It is generally accepted that it is characterized as pathognomonic by absence/aplasia of the sternocostal aspect of the pectoralis major muscle of one side. Because it is considered a rare disease, and the incidence is low, we aimed to determine the features in all patients diagnosed at our institution to analyze the patterns and outcomes. We also revised the diagnosis according to the new classification proposed by Romanini et al to facilitate future treatments as well as to propose an improvement in the assessment and initial behavior when PS is suspected. METHODS: We performed a retrospective study using our database from 1988. We identified 66 patients with a clinical diagnosis of PS in the Pediatric Plastic Surgery Unit of Hospital Sant Joan de Deu, Barcelona. We analyzed the medical charts, records, photographs, and imaging information to gather all the epidemiological information and clinical history, as well as the treatments received to analyze our experience with these patients. We used the TBN classification of Romanini et al, adding an S if there was upper extremity involvement (TBNS).We also conducted a systematic review consulting the following databases: PubMed, Cochrane Plus, Scopus, Web of Science, and Europe PMC. RESULTS: Among the 66 patients, 27 were men, and 39 were women. The right side was affected in 51 patients (77.3%) (19 men and 32 women). In a total of 37 patients (56.1%; 16 men and 21 women), there was no upper extremity involvement. We found 40 cases with other associations, with scoliosis being the most common. Before 2007, the team performed surgery in 1 male patient and 4 female patients; after 2007, the actual team has performed reconstruction in 8 male patients and 16 female patients. In the female patients, the average volume of breast implants was 252.5 cc, and the average volume for fat grafting was 103.5 cc on the side affected with PS. There is a wide range of chest-breast clinical involvement, which was why we decided to readdress all the imaging and clinical history to classify the patients using a new classification system to learn how to optimally treat these cases in the future. In female patients, we used Romanini's TBN classification and added an S for upper extremity involvement; the T1B1N1S0 was the most frequently observed classification. Owing to the sample size and the disparity in sex distribution, we also reassessed publications in PubMed, Cochrane Plus, Scopus, Web of Science, and Europe PMC with more than 5 cases; we found that there were a total of 1600 patients with PS in the literature and that the distribution was almost 50% for men and women, including the patients in our study (611 women, 561 men). CONCLUSIONS: We propose to include the presence of upper extremity involvement in Romanini's TBN classification (TBNS) for optimal management of these patients and use a standard number and profile for image documentation presurgery and postsurgery. We found that in the literature the ratio of men and women with PS was not 2 to 3:1, but 1:1. The most frequent phenotype of PS was T1B1N1S0 (hypoplasia or aplasia of the pectoralis muscles and soft tissue/breast hypoplasia/nipple-areolar complex with dislocation of <2 cm / absence of upper extremity involvement). There was no relationship between the side involved and patients' sex for the severity of the deformity and between the severity of the thoracic involvement and ipsilateral upper extremity association. In the literature, there are several plastic reconstruction methods available ranging from standard implants to microsurgery techniques. The selected procedure depends on the phenotype, patients' needs and expectations, availability of surgery in the hospital, and surgeons' criteria; thus, surgery has to be personalized while providing a match related to the risk/benefit, taking into account the degree of functional impairment. Since the author started her practice in the hospital in 2007, the timing for procedures has been proposed at the end of adolescence with 2 consecutive appointments without changes in the breast-thorax measures. The procedure performed has included implant use with lipofilling, yielding no major complications and a high rate of satisfaction during a maximum of 10 years of follow-up.

Value of supranormal function on 99m Tc-mercaptoacetyltriglycine renal scan in paediatric patients with obstructive hydronephrosis.

OBJECTIVES: To study related factors and clinical significance of supranormal function in paediatric patients with pelvi-ureteric junction obstruction, and to predict which factors cause renal function overestimation. PATIENTS AND METHODS: Patients who underwent pyeloplasty from 2012 to 2017 were prospectively collected. Variables were compared between patients with and without supranormal function on 99m Tc-mercaptoacetyltriglycine renal scan (supranormal defined as differential renal function [DRF] ≥55%). Univariate, multivariate logistic and linear regressions analyses were performed. RESULTS: Of 100 patients, 18 were excluded because of comorbidities. Nine patients (11.5%) showed preoperative supranormal function. The preoperative anteroposterior pelvic diameter (APD; 24 mm vs 35 mm, P = 0.026) and the ratio between preoperative pelvic and kidney volumes (0.2 vs 0.6, P = 0.003) were higher in supranormal kidneys. For each unit increase in the preoperative ratio between pelvic and kidney volumes, the risk of supranormal function rose 3.23-times (95% confidence interval [CI] 1.051-9.955). A preoperative APD ≥30 mm was a reliable predictor of supranormal function (area under the curve 0.804, 95% CI 0.707-0.902), with 88.9% sensitivity. Patients with either preoperative supranormal function or preoperative APD ≥30 mm had a greater reduction in renal function after pyeloplasty. CONCLUSION: Supranormal function is related to large hydronephrosis where geometrical features are modified. A preoperative APD ≥30 mm is a reliable predictive factor of supranormal function. Preoperative renal function is overestimated either in supranormal patients or severe hydronephrotic kidneys. DRF should be interpreted with caution in kidneys with large hydronephrosis with or without supranormal function. Surgical indication should not entirely rely upon DRF.

Bilateral hydrosalpinx in patients with Hirschsprung's disease.

PURPOSE: Hirschsprung's disease (HD) is uncommon in females. There are very few reports on the patients' obstetric and gynecological outcome. Hydrosalpinx causes pain and infertility. It is rare in nonsexually active teenagers. It may be because of an intrinsic disease of the fallopian tubes or secondary to surgery. AIM: to describe the relationship between hydrosalpinx and HD or its surgical approach; to report the impact of bilateral hydrosalpinx on fertility in HD. METHODS: The records of all females with HD since 1980 were reviewed. Only patients who reached menarche were included. Prevalence of hydrosalpinx and hydrosalpinx-free survival were compared after abdominoperineal (A) or transanal (T) surgery. Treatment for hydrosalpinx was reviewed. RESULTS: Seventeen out of 27 patients had reached menarche (Group A: 13 patients; Group T: 4 patients). Five patients in group A and none in group T presented bilateral hydrosalpinx (p=0.261). There were no statistical differences in hydrosalpinx-free survival between groups (p=0.344). Hydrosalpinx treatment: two bilateral and one unilateral salpingectomy, one pyosalpinx evacuation and one untreated. Three patients had conception desire: one has children; two are on IVF program. CONCLUSION: An association between hydrosalpinx and HD was observed. The development of hydrosalpinx was not associated with surgical approach in our study. Females with HD should have a gynecological follow-up for the development of hydrosalpinx, which can impair fertility. LEVEL OF EVIDENCE: Level III, retrospective comparative study.

Use of 3D Prototypes for Complex Surgical Oncologic Cases.

INTRODUCTION: Physical 3D models known by the industry as rapid prototyping involve the creation of a physical model from a 3D computer version. In recent years, there has been an increasing number of reports on the use of 3D models in medicine. Printing such 3D models with different materials integrating the many components of human anatomy is technically challenging. In this article, we report our technological developments along with our clinical implementation experience using high-fidelity 3D prototypes of tumors encasing major vessels in anatomically sensitive areas. METHODS: Three patients with tumors encasing major vessels that implied complex surgery were selected for surgical planning using 3D prototypes. 3D virtual models were obtained from routine CT and MRI images. The models, with all their anatomical relations, were created by an expert pediatric radiologist and a surgeon, image by image, along with a computerized-aided design engineer. RESULTS: Surgeons had the opportunity to practice on the model before the surgery. This allowed questions regarding surgical approach; feasibility and potential complications to be raised in advance of the actual procedure. All patients then successfully underwent surgery as planned. CONCLUSION: Having a tumor physically printed in its different main component parts with its anatomical relationships is technically feasible. Since a gross total resection is prognostic in a significant percentage of tumor types, refinements in planning may help achieve greater and safer resections therefore contributing to improve surgical management of complex tumors. In this early experience, 3D prototyping helped significantly in the many aspects of surgical oncology planning.

Thoracoscopic segmentectomy of methylene blue dyed intralobar sequestrations.

Surgical resection of congenital lung lesions has evolved with minimally invasive and parenchyma-preserving techniques. Although these lesions are usually small and their limits can be suspected by direct vision or palpation, there are no clear anatomic landmarks to enable a precise resection. This report presents a new technique that helps to define the limits of intralobar sequestrations, leading to a safe and anatomic thoracoscopic segmentectomy.

Airway and vascular maturation stimulated by tracheal occlusion do not correlate in the rabbit model of diaphragmatic hernia.

BACKGROUND: In animal models of congenital diaphragmatic hernia (CDH), tracheal occlusion (TO) has induced maturation of both airway spaces and vascular structures. Airway and vascular response to TO are assumed to occur in parallel. This study aims to describe and measure the relationship between airway and vascular maturation induced by TO. METHODS: A rabbit model of CDH on gestational day (GD) 23 and TO on GD 28 (term = GD 31) has been used. Two study groups have been defined: DH (diaphragmatic hernia) and TO (DH treated with TO). Animals were collected on GD 30 and blood flow data of the pulmonary artery (pulsatility index (PI) and fractional moving blood volume) were ultrasonographically measured. Lung morphometry consisted of measurements of radial alveolar count (RAC) and arterial muscular thickness. RESULTS: Animals in the DH group (n = 9) had the worst hemodynamic parameters; their lungs were hypoplastic and had the thickest arterial muscular layer. Animals in the TO group (n = 10) had all these effects reversed. There were no correlations among hemodynamic, airway, and vascular parameters, except for RAC and PI (r = -0.528, P = 0.043). CONCLUSION: Airway and vascular maturation after TO appear to be uncorrelated effects. TO could trigger several pathways that separately regulate airway and vascular responses.

Heminephroureterectomy for duplex kidney: laparoscopy versus open surgery.

OBJECTIVE: To report our experience of laparoscopic heminephroureterectomy (Hnu) in pediatric patients with duplex anomalies, in comparison to open surgery. PATIENTS AND METHODS: Retrospective review of data from patients who underwent Hnu from 2005 to 2008 was performed. The patients were divided into two groups: laparoscopic (LHnu) and open surgery (OHnu). Laparoscopic surgery was performed by transperitoneal approach in majority of cases. Open surgery was performed by retroperitoneal approach in all cases. RESULTS: Group LHnu: nine patients (8 females, 1 male) with median age of 14 months (range 3-205). Transperitoneal approach was performed in eight patients. Mean operative time was 182 min (CI 95% 146-217). No conversion to open surgery was necessary and there were no complications. Mean hospital stay was 2.44 days (CI 95% 1.37-3.52). Group OHnu: eight patients (3 females, 5 males) underwent nine heminephrectomies at median age of 6.9 months (range 1-12). Mean operating time was 152 min (CI 95% 121-183). There were no complications and mean hospital stay was 4.38 (CI 95% 2.59-6.16) days. Statistical analysis showed no statistically significant difference (P>0.05) in operating time between groups while mean hospital stay was significant (P=0.021). CONCLUSION: The laparoscopic approach is feasible, safe, reduces hospital stay, does not increase operating time and has better cosmetic results. We believe this should be the first option for heminephrectomy.

Fifteen years of experience in the treatment of anorectal malformations.

AIM: To analyze our experience in the treatment of anorectal malformations (ARM) with the posterior sagittal anorectoplasty (PSARP), and our modifications through the last few years and the outcomes. MATERIALS AND METHODS: We reviewed 107 cases divided into two groups: Former (F: 1994-2003) and Recent (R: 2004-2008). Type of ARM, associated anomalies, management and complications were noted. A telephone questionnaire regarding continence outcome was addressed to the 74 cases older than 3 years. RESULTS: According to the type of ARM, there were 53 perineal fistulas, 2 anal stenoses, 11 no fistulas, 12 rectourethral fistulas (5 rectobulbar and 7 rectoprostatic fistulas), 22 vestibular fistulas, 1 rectovesical fistulas and 6 cloacas. A total of 47 patients presented with 73 associated malformations. As much as 45 colostomies were performed, including 5 perineal fístulas, with 6 of 7 vestibular fístulas in group F and only 8 of 15 in group R. We had 19 complications of PSARP. The most frequent one was rectal mucosa prolapse in 14 (12F and 2R) and 2 wound infections (F). Continence was good in 62, poor in 3 and fair in 5. Seven out of eight children with poor or fair continence had associated malformations. CONCLUSIONS: All perineal fístulas can be managed without colostomy. Vestibular fístulas can be safely treated without colostomy in otherwise healthy patients without severe malformations. Overall, continence is good, and fair/poor results are related to associated malformations. Cumulative experience helps avoid colostomies and reduce complication and reoperation rates.

Lung tissue blood perfusion changes induced by in utero tracheal occlusion in a rabbit model of congenital diaphragmatic hernia.

OBJECTIVE: To analyze the impact of in utero tracheal occlusion (TO) on lung tissue blood perfusion, as measured by fractional moving blood volume (FMBV) and conventional spectral Doppler, in a rabbit model of congenital diaphragmatic hernia (CDH). METHODS: In 50 fetal rabbits, a left CDH was surgically created at 23 days of gestational age (GA). At 28 days of GA, the surviving CDH fetuses were randomly assigned to undergo either TO (CDH+TO group) or a sham operation (CDH group). Twenty littermates, which were not operated on, served as internal normal controls. At 30 days of GA, lung perfusion estimated by FMBV and spectral Doppler of the proximal intrapulmonary artery were evaluated in the right lung during cesarean section. Doppler waveform analysis included the pulsatility index (PI), peak early diastolic reverse flow and peak systolic velocity. RESULTS: Eleven CDH fetuses, 9 CDH+TO and 20 controls were suitable for the study. CDH fetuses showed a significantly higher PI [8.0 (SD 1.8) vs. 5.22 (SD 1.1), p < 0.001] and lower FMBV [13.5% (SD 4.6) vs. 23.0% (SD 2.1), p < 0.001] than the controls. In contrast, CDH+TO fetuses had a significantly lower PI [5.8 (SD 2.3) vs. 8.0 (SD 1.8), p = 0.015] and higher FMBV [27.6% (SD 7.1) vs. 13.5% (SD 4.6), p < 0.001] than CDH fetuses, with values similar to the controls. Peak early diastolic reverse flow and peak systolic velocity showed nonsignificant differences among the study groups. The lung to body weight ratio at necropsy correlated positively with lung FMBV (r = 0.60, p < 0.001) and negatively with the pulmonary artery PI (r = -0.48, p < 0.01). CONCLUSION: Tracheal occlusion is consistently associated with increased lung tissue perfusion and decreased intrapulmonary impedance in a rabbit model of CDH.

Median raphe cysts of the perineum in children.

Median raphe cysts of the perineum are uncommon congenital lesions of the male genitalia. They can be found all the way from the distal penis and scrotum toward the perineum in a midline position. They are considered as congenital alterations in embryologic development. A case of a 6 year-old boy is presented. Review of the literature relevant to children was made regarding the embryologic, diagnostic, and treatment aspects. We believe it is important that adult and pediatric urologists recognize these lesions and their management to provide the appropriate information to the parents.

Sclerosing cholangitis secondary to hepatic artery ligation after abdominal trauma.

Several causes have been postulated as responsible for secondary sclerosing cholangitis (SSC), mainly in adults, and, although in very different situations, ischaemia seems to be one of the most important factors. The term 'ischaemic cholangitis' has been used as a collective label for all these ischaemia-induced bile duct lesions. The biliary epithelium is dependent on arterial blood flow, unlike the hepatic parenchyma, which receives a dual blood supply from the hepatic artery and the portal vein. This makes the biliary epithelium very susceptible to changes in arterial blood flow. We present one adolescent patient who developed SSC after abdominal trauma with hepatectomy and ligation of the right hepatic artery. Different factors could have helped in the development of SSC in our patient (septicaemia, bile duct destruction, cholecystectomy) but right hepatic artery ligation seems to be the most important aetiological factor in the development of secondary ischaemic cholangitis.

Feminització quirúrgica. Lògica o obligada? / Feminización quirúrgica. ¿lógica u obligada? / Surgical feminization. Logical or obligatory?

Pacient de 13 anys d’edat, de sexe cromosòmic 46 XY i de sexe social femení, etiquetada com a síndrome de feminització testicular que va acudir a la consulta per realitzar la feminització dels seus genitals externs. En no coincidir les dades anamnèsiques amb l’exploració física i els exàmens complementaris, aprofitant la intervenció feminitzant es va fer un cultiu de fibroblastes de pell genital i un estudi del DNA de limfòcits de sang perifèrica que van posar de manifest la presència de dues mutacions que expliquen el dèficit de l’enzim 5 reductasa per mutació en el seu gen (AU)

Paciente de 13 años de edad, de sexo cromosómico 46 XY y de sexo social femenino, etiquetada de síndrome de feminización testicular que acudió a la consulta para realizar la feminización de sus genitales externos. Al no coincidir los datos anamnésicos con la exploración física y los exámenes complementarios, en el curso de la intervención feminizante se hizo un cultivo de fibroblastos de piel genital y un estudio del DNA de linfocitos de sangre perifé- rica que pusieron de manifiesto la presencia de dos mutaciones que explican el déficit del enzima 5 reductasa por mutación en su gen (AU)

A 13 year-old patient was referred to undergo surgical feminization of her external genitalia. The patient had male chromosomal sex (46 XY) and female social sex, and had been diagnosed with testicular feminization syndrome. Since physical examination and laboratory evaluation did not fit with the clinical history, a biopsy of genital skin was performed and fibroblasts were isolated for culture, and peripheral blood lymphocytes were analyzed. These studies demonstrated the presence of 2 mutations resulting in lack of 5 reductase (AU)

Congenital epigastric evisceration: a case report.

A rare case of abdominal wall defect at the epigastric midline is presented. This newborn boy had only the greater omentum eviscerated, and no other abnormalities could be detected. This case does not seem to relate to the abdominal wall defects reported so far in the literature. This singular case contributes to enlarge the spectrum of congenital defects of the abdominal wall.