Successful Management of a Late Referred Giant Choledochal Cyst Type Ia in 8-Year-old Girl.

A choledochal cyst is a rare condition with a variable incidence and different modalities of presentation according to the context and the age of patients. Early diagnosis and complete resection are the only therapeutic options to prevent complications and malignancies. Type Ia, cystic dilatation of the entire extrahepatic duct, is the most observed, and a giant size (more than 10 cm in diameter) may rarely be reached. This patient group often has a long-lasting clinical history and a shared experience of uneasy access to specialist care. Huge dimensions are a complex challenge for complete resection, and the risk of permanent liver functional damage must be considered, as in the current case.

RésuméUn kyste cholédochal est une condition rare avec une incidence variable et différentes modalités de présentation selon le contexte et l'âge des patients. Le diagnostic précoce et la résection complète sont les seules options thérapeutiques pour prévenir les complications et les tumeurs malignes. Type IA, La dilatation kystique de l'ensemble du canal extrahépatique est la plus observée et une taille géante (plus de 10 cm de diamètre) peut rarement être atteinte. Ce groupe de patients a souvent des antécédents cliniques de longue durée et une expérience partagée d'accès mal à l'aise aux soins spécialisés. D'énormes dimensions sont Un défi complexe pour une résection complète et le risque de dommages fonctionnels du foie permanents doivent être pris en compte, comme dans le cas actuel.

The hidden burden of Pediatric urology in Sub-Saharan Africa: an analysis of hospital admission data from three East African Health Centres.

Specialist facilities for children are still unavailable in some Sub-Saharan African contexts. It is the case of pediatric urology, whose recent advances are still largely unshared. Prenatal diagnosis of urinary abnormalities (CAKUT) is largely unknown. Early recognition and referral of Undescended testis (UDT), Hypospadia, bladder exstrophy epispadias complex, ambiguous genitalia, stone disease, and tumours are uncommon in rural areas. Missed diagnosis is not uncommon and delayed management is associated with poor outcomes. We present a cross-sectional, descriptive study about the epidemiology of Pediatric urological admissions to three sub-Saharan East African Hospitals. All the urological cases between 0-18 years referred to three distinct East African Hospitals over 124 weeks were considered. Prevalence of different groups of diseases, age, and mode of presentation were reported. We found 351 cases (M/F 127/24) out of 2543 surgical referrals (13%). Seventy percent of cases were Hypospadias and UDT. Fifty percent of UDT were beyond 6, and most Hypospadias were between 4 and 7 yrs. CAKUT had a very low prevalence (4.84%), and about 50% of Wilms Tumours came too late to be resectable. In many African contexts, urology is still a tiny portion of the pediatric surgical workload compared to the 25% of European and American reports. There are also differences in the epidemiology of genitourinary conditions. A hidden burden of diseases may be presumed, remaining undiagnosed due to the shortage of specialist facilities.

Presentations of bladder exstrophy in a resource-limited setting and the role of Mainz II continent diversion for late referrals or failed primary closures: a multicentric report.

Primary closure techniques that have been updated and longterm follow-up for CBE (classic bladder exstrophy) may be out of reach for many patients living in resource-limited settings. Late referrals to medical care and primary closures that lack the necessary skills and facilities for comprehensive treatment are still common. Alternative and long-term surgical solutions may improve the lives of these unfortunate patients. During surgical outreach missions, patients with CBE, either non-operated or with a previous unsuccessful bladder closure, who were referred from vast under-resourced rural areas to three Eastern African hospitals, were studied. The following information is provided: mode of presentation, clinical history, diagnostic workout, management, and outcome. There were 25 cases (M/F ratio 17/8) ranging in age from two days to twenty years. Five of the seventeen patients who were not treated (35%) were under 120 days old and eligible for primary closure in a qualified tertiary center when one was available in the country. There were twelve late referred cases (ranging from 120 days to 20 years). Between the ages of ten months and twelve years, eight children arrived following a failed primary closure. In all of them, the bladder plate was too altered to allow closure. Following a preoperative diagnostic workout, a Mainz II continent internal diversion was proposed to fourteen patients with acceptable bowel control and postponed in the other three. Three cases were lost before treatment because parents refused the procedure. Twelve cases ranging in age from three to twenty years (mean seven years) were operated on. Eight people were followed for a total of 53.87 months (range: 36-120). Except for three people who complained of occasional night soiling, day and night continence were good. The average voiding frequency during the day was four and 1.3 at night. There was no evidence of a metabolic imbalance, urinary infection, or significant upper urinary tract dilatation. Two fatalities could not be linked to urinary diversion. Four patients were not followed up on. Due to the limited number of specialist surgical facilities, CBE late referral or failed closure is to be expected in a resource-limited context. In lieu of the primary closure, a continent internal diversion will be proposed and encouraged even at the level of a non-specialist hospital to improve the quality of life of these unfortunate patients. It is recommended that patients be warned about the procedure's potential long-term risks, which will necessitate a limited but regular follow-up.

Promoting major pediatric surgical care in a low-income country: a 4-year experience in Eritrea.

BACKGROUND: Access to pediatric surgical care in many sub-Saharan African countries is strongly limited by lack of medical facilities, adequate transport system, and trained medical and nursing manpower. The mortality rate for major congenital abnormalities remains extremely elevated in this area of the world. Strong efforts have been spent during the past decades to elevate the level of pediatric surgery standards in these countries through cooperation programs acting through periodical medical missions or supporting local medical teaching institutions. This is a report of a partnership between an Italian Medical Institution and the Eritrean Ministry of Health with the goal to improve pediatric surgical standard of care in the country. METHODS: During the past 4 years, teams composed of two pediatric surgeons, a pediatric anesthetist, and a pediatric nurse regularly visited the Orotta Medical and Surgical Referral Hospital of Asmara (Eritrea) to offer modern surgical treatment to children with major congenital abnormalities and to offer academic training to local medical and nursing staff. The team worked in local health structures. A total of 714 patients have been visited and 430 surgical procedures have been performed during 35 weeks of clinical work. Among them were 32 anorectal malformations, 11 Hirschsprung's disease cases, 8 bladder extrophies, and many other major surgical problems, such as congenital intestinal obstructions, obstructive uropathies, and solid tumors. RESULTS: The standard of care has been based on the principle of researching sustainable solutions. Surgical options and timing of each procedure have been decided to reduce hospitalization and the recourse to temporary stomas, indwelling catheterization, and prolonged intravenous feeding. Posterior sagittal anorectoplasties (PSARP) and posterior vagino-anorectoplasty (PSVARP) were performed for anorectal malformations, introducing minimal technical variants to reduce the need for postoperative nursing. Endorectal pull-through of functional stoma was the treatment of choice for patients with Hirschsprung's disease because frozen sections were not possible. Eight late-referred bladder extrophy cases were all managed by internal diversion (Mainz II pouch). Solid abdominal tumors always came to observation weeks or months after the first symptoms appeared. No CT scan was available and indications of surgery were based on clinical symptoms only. Only 11 of 18 cases were resectable, and only 5 of them with favorable histology survived, 2 after adjuvant therapy abroad. A large number of hypospadias were observed at the mean age of 4.5 years. Failures of previous attempts at correction were frequently found. The postoperative complications rate progressively decreased with the use of dripping stents to avoid the risk of accidental catheter removal or kinking. CONCLUSIONS: On the basis of our experience, major pediatric surgery in many under-resourced areas of sub-Saharan Africa can be developed, taking care to adapt surgical options to local conditions. Late referral of many congenital abnormalities, the impact of local culture, difficulties to establish regular follow-up, and shortage of facilities and medical devices must always be kept in mind before transferring modern protocols of management. Strong efforts have been devoted to train local medical and nursing staff to establish pediatric surgical manpower to cope with a still largely unanswered demand of care in this area of Africa.

Pitfalls and technical errors in the first approach to neonates with anorectal malformations in a non-specialist context: can we do any better? A review from three Eastern African Centres.

INTRODUCTION: In sub-Saharan Africa, Anorectal malformations (ARM) are the most frequent cause of neonatal obstruction. Referral to a Pediatric Surgeon is frequently delayed. The first treatment is often delivered at not specialist level and mismanagement may result. AIM: To study ARM patients referred beyond neonatal period and managed at a non-specialist level. MATERIALS AND METHODS: One hundred and thirty patients were included (M/F ratio 63/67) among 144 admitted to three Eastern African Hospitals with Pediatric Surgical facilities. Demographics, type of anomaly, delay on referral, previous management, most commonly observed errors are reported. RESULTS: The Mean age at referral was 23 months (range five weeks - 23 years). Colostomy was the most frequent surgery (92 cases). Stomas often did not follow the recommended criteria. Ten per cent were not on the sigmoid, and 35% were not divided. "Loop" or "double-barrel" colostomies did not exclude the distal loop. Inverted (10,5%), prolapsed stomas (7,5%), short distal loop (16%) were observed. Twenty-four cases (26%) needed redo. Primary perineal exploration in eight patients resulted in incontinence. CONCLUSIONS: Investments on training practitioners, acting at District/Rural level, and closer links with tertiary centres are recommended to avoid ARM mismanagement and delayed referral to a Specialist.

The start-up phase of a non-specialist paediatric surgical service by outreach focused on capacity building at the Consolata Hospital Ikonda, Makete District, Tanzania.

age) are far lower than the recommended workforce size. Complex conditions require a significant increase in the number of paediatric surgeons. In contrast, children with minor diseases, living in rural areas, could be managed even at the district level by trained general surgeons. AIM: The aim of this study was to develop capacity for general paediatric surgical services in a district hospital by outreach with a focus on mentorship. METHODS: Capacity building priorities for non-specialist paediatric surgery were identified and addressed using evidence-based guidelines. Local general surgeons were involved in supervised clinical decision-making and in all surgical procedures. The visiting team provided daily meetings, weekly lectures, and on-job training. Electronic copies of recent surgical textbooks were provided together with video-conferencing distant specialist consultations. RESULTS: A total of 715 children were handled by the visiting team during the 27-week period. Four hundred and fifty diseases were diagnosed amongst 406 children. Awareness of paediatric surgical needs, improved management of most common conditions like congenital hernias, undescended testis, hypospadias and anorectal malformations needing temporary colostomy occurred. Local general surgeons were assisted in treating 358 cases of general paediatric surgical conditions. Updated early management protocols were introduced for more complex diseases needing referral to specialist centres like solid tumours and neonatal abnormalities. The visiting team operated major paediatric surgical cases at the Consolata Hospital Ikonda. LESSONS LEARNT: Surgical outreach and capacity building at the district hospital level could be a possible answer to the unmet paediatric surgical needs of children living in rural areas. Apprenticeship training for general surgeons may help to fill the gap provided that they are strongly motivated and supported on acquiring and implementing their paediatric surgical skills.

Challenges, constraints and failures that are related to the posterior sagittal anorectoplasty approach to anorectal malformations in a low-resource context: An experience from a sudanese tertiary referral centre.

BACKGROUND: Anorectal malformations (ARMs) in the sub-Saharan Africa are a common cause of neonatal referral for intestinal obstruction, and the posterior sagittal anorectoplasty (PSARP) approach is rapidly spreading. The small number of paediatric surgeons and the low-resource context limit children's access to care and constrain the quality of results. A retrospective, observational study has been done on a consecutive series of ARM cases admitted to a Sudanese tertiary paediatric surgical centre within the framework of a partnership between Italian and Sudanese academic institutions addressed to review and upgrade the standard of care of major congenital anomalies. MATERIALS AND METHODS: The authors collected 94 ARM cases in a 3 years' period. Conditions on referral, operative procedures, post-operative course and follow-up were recorded and examined. Their correlations with complications and outcome were analysed. RESULTS: The male/female ratio was 47/47. Eighty patients presented with an untreated ARM; 66 had a divided stoma and 14 had already a PSARP procedure, followed by a poor outcome or sequelae. In 25% of the cases, colostomy required re-doing. In 57 cases, a staged PSARP (primary or re-do) was done. Surgical-site infections occurred in nine patients. Some patients were lost to follow-up after preliminary colostomy. Post-operative dilatation programme suffered from the lack of systematic follow-up, and colostomy closure was possible in 46% of the cases due to problems in travelling and accessing hospital care. Anal stenosis was frequently observed among unfollowed patients. CONCLUSION: Despite PSARP's widespread adoption in Africa, the risk of complications and failures is high. Primary management is often inappropriate, and a high rate of colostomy-related complications is observed. Poverty and lack of transportation reduce attendance to follow-up, hampering the final results. Investments in healthcare facilities and retention of trained health providers are needed to improve the standard of care.

Improving standard of pediatric surgical care in a low resource setting: the key role of academic partnership.

BACKGROUND: An epidemiological transition is interesting Sub-Saharan Africa increasing the burden of non-communicable diseases most of which are of surgical interest. Local resources are far from meeting needs and, considering that 50% of the population is less than 14 years of age, Pediatric surgical coverage is specially affected. Efforts are made to improve standards of care and to increase the number of Pediatric surgeons through short-term specialist surgical Missions, facilities supported by humanitarian organization, academic Partnership, training abroad of local surgeons. This study is a half term report about three-years Partnership between the University of Chieti- Pescara, Italy and the University of Gezira, Sudan to upgrade standard of care at the Gezira National Centre for Pediatric Surgery (GNCPS) of Wad Medani. Four surgical Teams per year visited GNCPS. The Program was financed by the Italian Agency for Development Cooperation. METHODS: The state of local infrastructure, current standard of care, analysis of caseload, surgical activity and results are reported. Methods utilized to assess local needs and to develop Partnership activities are described. RESULTS: Main surgical task of the visiting Team were advancements in Colorectal procedures, Epispadias/Exstrophy Complex management and Hypospadias surgery (20% of major surgical procedures at the GNCPS). Intensive care facilities and staff to assist more complex cases (i.e. neonates) are still defective. Proctoring, training on the job of junior surgeons, anaesthetists and nurses, collaboration in educational programs, advisorship in hospital management, clinical governance, maintenance of infrastructure together with training opportunities in Italy were included by the Program. Despite on-going efforts, actions have not yet been followed by the expected results. More investments are needed on Healthcare infrastructures to increase health workers motivation and prevent brain drain. CONCLUSIONS: The key role that an Academic Partnership can play, acting through expatriated Teams working in the same constrained contest with the local workforce, must be emphasized. Besides clinical objectives, these types of Global Health Initiatives address improvement in management and clinical governance. The main obstacles to upgrade standard of care and level of surgery met by the Visiting Team are scarce investments on health infrastructure and a weak staff retention policy, reflecting in poor motivation and low performance.

Usefulness of large pleural flap for the treatment of children with recurrent tracheoesophageal fistula.

PURPOSE: Recurrent tracheoesophageal fistula (RTF) complicates 5-11% of cases of children treated at birth for esophageal atresia with inferior tracheoesophageal fistula (ITF), and it represents an important problem of respiratory morbility and mortality. Surgical correction of RTF is complex with high incidence of recurrence. The aim of this work is to demonstrate the usefulness of large vascularized pleural flap in the treatment of RTF and the prevention of recurrences. METHODS: From 2000 to 2007, four children (3 males and 1 female) referred to our hospital for respiratory symptoms secondary to RTF. Diagnosis of RTF was made by bronchoscopy and esophagogram with contrast medium. Operative repair involved resection of the fistula, suture of trachea and esophagus followed by interposition of large vascularized pleural flap (PF). RESULTS: There were no complications during surgical procedure and after 48 h, with easy respiratory weaning after 24 h. Hospital discharge ranged from 5 to 10 days. Long-term follow-up (range 18 months-5 years) demonstrated no evidence of recurrences. CONCLUSION: Large vascularized PF to closure RTF is a highly effective and physiologic method for preventing second recurrences.

Prenatal diagnosis of duodenal obstruction selects cases with a higher risk of maternal-foetal complications and demands in utero transfer to a tertiary centre.

OBJECTIVES: It was the aim of this study to determine if prenatal diagnosis of congenital duodenal obstruction (CDO) selects high-risk pregnancies and demands special perinatal attention. METHODS: Medical records of 62 neonates with intrinsic CDO, admitted since 1981 in 2 institutions, were reviewed and divided into 2 groups: 39 cases, detected in utero by a prenatal ultrasonogram (group A), and 23 diagnosed at birth (group B). Prevalence of complete CDO, mean gestational age, mean birth weight, hydramnios, as well as maternal-foetal complications requiring emergency Caesarean section and associated with premature delivery were compared in the 2 groups. RESULTS: A complete CDO was found in 77% of patients in group A versus 48% of patients in group B (p < 0.02). Differences in mean gestational age and mean birth weight in the 2 groups were non-significant. Prenatally diagnosed patients presented a higher prevalence of hydramnios, maternal-foetal complications and premature delivery. CONCLUSIONS: Prenatal diagnosis selects patients with complete CDO and hydramnios. These pregnancies present a high incidence of maternal-foetal complications, which may require an emergency Caesarean section, and are frequently associated with premature delivery. These aspects must be considered in prenatal counselling. In utero transfer to a tertiary centre for delivery and appropriate perinatal care should be recommended.

Guidelines on pediatric day surgery of the Italian Societies of Pediatric Surgery (SICP) and Pediatric Anesthesiology (SARNePI).

The Italian Society of Pediatric Surgery (SICP) together with The Italian Society of Pediatric Anesthesia (SARNePI) through a systematic analysis of the scientific literature, followed by a consensus conference held in Perugia on 2015, have produced some evidence based guidelines on the feasibility of day surgery in relation to different pediatric surgical procedures. The main aspects of the pre-operative assessment, appropriacy of operations and discharge are reported.

The Start-Up of the first Hematopoietic Stem Cell Transplantation Center in the Iraqi Kurdistan: a Capacity-Building Cooperative Project by the Hiwa Cancer Hospital, Sulaymaniyah, and the Italian Agency for Development Cooperation: an Innovative Approach.

We describe the entire process leading to the start-up of a hematopoietic stem cell transplantation center at the Hiwa Cancer Hospital, in the city of Sulaymaniyah, Kurdistan Iraqi Region. This capacity building project was funded by the Italian Development Cooperation Agency and implemented with the support of the volunteer work of Italian professionals, either physicians, nurses, biologists and technicians. The intervention started in April 2016, was based exclusively on training and coaching on site, that represent a significant innovative approach, and led to a first autologous transplant in June 2016 and to the first allogeneic transplant in October. At the time of reporting, 9 months from the initiation of the project, 18 patients have been transplanted, 15 with an autologous and 3 with an allogeneic graft. The center at the HCH represents the first transplantation center in Kurdistan and the second in wide Iraq. We conclude that international development cooperation may play an important role also in the field of high-technology medicine, and contribute to improved local centers capabilities through country to country scientific exchanges. The methodology to realize this project is innovative, since HSCT experts are brought as volunteers to the center(s) to be started, while traditionally it is the opposite, i.e. the local professionals to be trained are brought to the specialized center(s).

Fetal intra-abdominal cysts: accuracy and predictive value of prenatal ultrasound.

OBJECTIVE: The finding of a fetal intra-abdominal cyst is relatively common and it can be due to a wide variety of clinical conditions. The aims of this study were to determine the accuracy of the prenatal ultrasound (US) in identifying the etiology of fetal intra-abdominal cysts and to describe the neonatal outcomes. METHODS: All cases of fetal intra-abdominal cystic lesion referred to our center between 2004 and 2012 were reviewed. Cysts of urinary system origin were excluded. Prenatal and postnatal data were collected. Our cohort was divided into subgroups according to the prenatal suspected origin of the lesion (ovarian, mesenteric, gastro-intestinal and other locations). For each subgroup, sensitivity, specificity, positive predictive value, false-positive rate and accuracy of fetal US were calculated. RESULTS: In total 47 fetuses (10/37 M/F) were identified. The mean gestational age at the time of diagnosis was 33 ± 4.9 weeks. Our cohort comprised of 25 ovarian cysts (10 simplex and 15 complex), 3 GI duplication, 6 mesenteric lesions, 4 meconium pseudocyst and 9 lesions of other origin. Surgery was performed in 38 cases (81%) at birth. The prenatally established diagnosis was exactly concordant with post-natal findings in 34 cases (72.3%). Sensitivity, specificity, positive predictive value and false-positive rate of US were 88.1%, 95.5%, 71% and 5.4%, respectively. Long-term outcome was good in almost 90% of the cases. CONCLUSIONS: The etiology of fetal intra-abdominal cysts can be prenatally diagnosed in about 70% cases. Despite the high risk of surgery at birth, the long-term outcome was good in most of the cases.

Tracheomalacia in oesophageal atresia: morphological considerations by endoscopic and CT study.

OBJECTIVE: A Tracheomalacia complicates 11-33% of cases of Oesophageal Atresia with distal Tracheo-Oesophageal Fistula. The lesion generally involves only the thoracic segment of the trachea, and it has close anatomical relationships with the mediastinal structures, specially with the aortic arch. We therefore tried to define the most important morphotypes of tracheobronchial malacia by using dynamic fiberoptic bronchoscopy (DFB) and spiral multilayer computed tomography (CT). METHODS: Between 1999 and 2003 we studied 40 children from two different institutions who had been operated on at birth for oesophageal atresia. All patients were been submitted to DFB, and the positive cases underwent examination by CT with an iodinated contrast medium. CT angiographic images of great vessels and multiplanar and three-dimensional images of the airways (virtual broncoscopy and broncography) were obtained for morphological evaluation. RESULTS: Twenty-five patients (62%) tested positive for malacia using DBF and all were also confirmed by CT study. In 11 cases (46%), the malacia was located at the thoracic section of the trachea, which was occluded by compression of the aorto-innominate complex. A simple intrinsic tracheomalacia without any vascular compression was present in eight cases (33%), while in five cases (21%), the malacia was complex. CONCLUSIONS: A correct morphological analysis of the malformed segment permitted 'tailored surgery' for each individual patient, allowing us to take account of the type of malacia, its length, and the compressive action exercised by the mediastinal great vessels.

Ultrasound in newborns and children suffering from non-traumatic acute abdominal pain: imaging with clinical and surgical correlation.

The purpose of this article is to review ultrasonographic appearance of the most common causes of non-traumatic acute abdominal pain in pediatric patients and to understand the applications and limitations of this technique giving a practical approach showing different clinical cases. A pictorial review of cases was made presenting the most common causes of neonatal and pediatric non-traumatic acute abdominal pain; sonographic features are discussed. Ultrasound in conjunction with Color Doppler imaging is a valuable tool in the evaluation of neonatal and pediatric non-traumatic acute abdominal pain; causes of acute abdomen in children could vary depending on the ages of the children.

Antenatal ultrasonographic features associated with segmental small bowel dilatation: an unusual neonatal condition mimicking congenital small bowel obstruction.

Segmental dilatation of the intestine (SDI) is an unusual condition presenting during the neonatal period, with symptoms of obstruction. Late diagnosed cases are also reported. The clinical polymorphism, and the lack of specificity of radiological investigations, make diagnosis difficult. Prenatal detection of abdominal cystic lesions or bowel dilatation has occasionally been reported to be associated with SDI. We herein report two cases of SDI, with a prenatal ultrasonographic suspicion of intestinal abnormality. In both infants, a dilatation of the ileum was found at surgery, without any evident site of obstruction or abnormal histology. SDI must be taken into consideration when a prenatal alert of possible bowel obstruction is not followed by postnatal clinical signs.

Jejunoileal Atresia: Factors Affecting the Outcome and Long-term Sequelae.

CONTEXT: Jejunoileal atresia (JIA) is a common abnormality. The outcome is conditioned by several variables. Nutritional problems, and long-term sequelae are described among those who survive. AIM: To correlate the type of JIA and its management to the outcome and long-term quality of life. SETTINGS AND DESIGN: Forty-three cases over a 17-year period (1992-2009). Perinatal data, management, and outcome were extracted from the clinical notes. The cases that had survived were contacted to get information about their present condition. MATERIALS AND METHODS: Morbidity and mortality were matched to maturity, birth weight, mode of diagnosis, type of JIA, associated anomalies, and management and duration of parenteral nutrition. Growth and quality of life in 34 cases were evaluated via a telephone interview at a minimum of one year from surgery. STATISTICAL ANALYSIS: Fisher test, Linear regression test, Kruskal-Wallis test, Dunn's comparison test. RESULTS: Male/Female ratio was 25/18 and median birth weight was 2.644 g. Prenatal diagnosis was recorded in 34%. Six patients (14%) had associated anomalies. Primary surgery was resection and anastomosis in 88% and temporary stoma in 12%. Length of the resected bowel ranged from 3 to 65 cm. Whenever multiple atresia was found, the bowel length was saved by multiple anastomosis. Three dehiscences and three adhesive obstructions required a reoperation. Two patients (4%) died due to a central catheter-related sepsis. Prenatal diagnosis did not influence the outcome and was associated with a higher rate of Cesarean deliveries. Interview, at a median of nine years, showed normal growth in 85%. One case of short bowel syndrome is still on parenteral support at 22 months. CONCLUSIONS: Preserving bowel length and reducing the recourse to stoma is the key to good outcome and growth. Sequelae are correlated with the type of atresia and length of residual bowel; however, they decrease their severity with time.

Preventing posterior sagittal anoplasty 'cripples' in areas with limited medical resources: a few modifications to surgical approach in anorectal malformations.

BACKGROUND: Anorectal malformations (ARM) are the most common neonatal emergencies in Sub-Saharan Africa countries. Late presentation, lack of pediatric facilities and trained paediatric surgeons influence the outcome of these patients. This study reports a 5-year of experience in the management of ARM at the Orotta Referral Hospital in Asmara (Eritrea) and proposes some modified surgical approaches to minimize the risk of complications and the length of hospital stay. MATERIALS AND METHODS: We reviewed the records of 38 patients with ARM observed between September 2006 and April 2011. Since 2009 a modification of original posterior sagittal anorectoplasty (PSARP) was introduced, consisting in a long rectal stump (3 cms) closed and left at the perineal level, to be trimmed after two weeks. This avoided mucous spillage on the wound and prevented contamination. Post-operative course and outcome were evalued in the two group of patients divided according the type of surgical technique (Group A: Standard PSARP; Group B: Modified PSARP). RESULTS: There were 21 boys and 17 girls aged 4 days to 9 years (median age 182 days). Of the 38 patients, 2 infants died before surgery and 3 refused preliminary colostomy. Previously confectioned colostomies often required revision or redoing due to severe prolapse or malposition. When possible, primary sigmoid colostomy was performed. There were 15 patients in Group A and 18 in Group B. Wound infection or disruption were recorded in 7 cases (46%) in Group A and in 2 (11%) in Group B. Late complications were related to anal stenosis, which required long term dilatations. Three cases needed a PSARP redo (2 in Group A, 1 Group B). CONCLUSIONS: We believe that our simple modification of original PSARP technique could be of help lowering post-operative complications rate and reducing hospital stay. Family compliance is mandatory for long-term surgical success. A relevant time must be spent in training to stoma care and post-operative anal dilatation.

Delivery in a tertiary Center with co-located surgical facilities makes the difference among neonates with prenatally diagnosed major abnormalities.

OBJECTIVE: Impact of prenatal diagnosis (PD) of major abnormalities on neonatal outcome is matter of debate. Unfortunately cases with and without PD may sometimes not be comparable. This is generally related to a lower maturity (GA) and weight (BW) secondary to a high rate of preterm cesarean sections (CSs) for clinical convenience. Present study tried to find out if in utero transfer to a Center with co-located surgical facilities reduces these potential risk factors. METHODS: 152 cases with prenatally detectable conditions were studied and divided according to PD; the following data were compared: GA, BW, obstetrical complications, associated malformations, mode and site of delivery, outcome. Cases with PD delivered in our Center (Inborn, IB) or transferred after birth (Outborn, OB) were compared. RESULTS: 61 cases had a PD (IB/OB ratio 34/27); GA and BW were lower respect to no-PD cases and a higher CS rate was found among OB cases, not justified by complicated pregnancies. No differences in outcome were observed. CONCLUSIONS: Elective preterm CS is still largely practiced for fetuses with PD of a major congenital anomaly in Centers without co-located surgical facilities either in the presumption of safer delivery or to facilitate postnatal transfer. This leads to a lower GA and BW and may spoil potential impact of PD on outcome. This may be avoided promoting prenatal transfer to a Center with co-located surgical facilities.