Risk factors of necrotizing enterocolitis after surgery for intestinal atresia / 中国新生儿科杂志

Objective:To study the risk factors of necrotizing enterocolitis (NEC) after surgery for intestinal atresia.Method:From August 2013 to June 2020, children with intestinal atresia receiving surgery in our hospital were retrospectively reviewed. The patients were assigned into NEC group and non-NEC group according to the occurrence of postoperative NEC. Demographic data and clinical characteristics were summarized and the risk factors for postoperative NEC were analyzed using Logistic regression analysis method.Result:A total of 96 infants were enrolled and NEC occurred in 13 patients (13.5%) after surgery for intestinal atresia. Compared with the non-NEC group, the NEC group were diagnosed of intestinal atresia [4.0(1.5,6.0)d vs. 1.4(0,2.0)d, P<0.001] and received surgery [4.8(2.0,7.0)d vs. 3.1(1.0,4.0)d, P=0.034] at later ages. The incidences of complex intestinal atresia [76.9%(10/13) vs. 44.6%(37/83), P=0.030] and blood transfusion [46.2%(6/13) vs. 13.3%(11/83), P=0.007] in the NEC group were higher than the non-NEC group. Logistic regression analysis showed that the age of initial diagnosis of intestinal atresia ( OR=3.346, 95% CI 1.493~7.500, P=0.003), complex intestinal atresia ( OR=9.052, 95% CI 1.119~73.209, P=0.039) and blood transfusion ( OR=6.835, 95% CI 1.399~33.380, P=0.018) were independent risk factors for postoperative NEC. Conclusion:Patients with delayed diagnosis of intestinal atresia, complex intestinal atresia and blood transfusion within 48 hours after surgery should be monitored for the occurrence of postoperative NEC.

Diagnóstico prenatal de atresia duodenal en feto con Síndrome Down. A propósito de un caso / Prenatal diagnosis of duodenal atresia in fetus with Down syndrome. About a case

RESUMEN La atresia duodenal (AD) es una patología congénita infrecuente. Es el resultado de una falla en la recanalización del duodeno y se puede encontrar asociada a cromosomopatías como el Síndrome Down en el 30% de los casos. El síndrome de down se produce por una trisomía del par cromosómico 21 y es la aberración cromosómica más frecuente. Debido a la necesidad de contar con reportes de esta alteración presentamos el diagnóstico prenatal por ultrasonido y estudio citogenético de una paciente de 25 años de edad a la que a las 28 semanas de gestación se le detectó en abdomen fetal una imagen ecolúcida dual por debajo del estómago (Imagen en doble Burbuja) compatible con atresia duodenal. Por sospecha de asociación de este tipo de malformación con enfermedad cromosómica, se le realizó diagnóstico prenatal citogenético, cuyo resultado fue: 47,XX, +21, inv(9)(p11;q12), feto femenino positivo para una trisomía 21 e inversión pericéntrica del cromosoma 9. Luego del asesoramiento genético, la pareja optó por la interrupción excepcional de la gestación. Se confirmó por anatomía patológica la presencia de la atresia duodenal y rasgos dismórficos compatibles con el síndrome down.

ABSTRACT Duodenal atresia (AD) is a rare congenital pathology. It is the result of a failure in the recanalization of the duodenum and can be found associated with chromosomopathies such as Down syndrome in 30% of cases. Down syndrome is caused by a trisomy of the 21 chromosome pair and is the most frequent chromosomal aberration. Due to the need to have reports of this alteration, we present the prenatal diagnosis by ultrasound and a cytogenetic study of a 25-year-old patient who, at 28 weeks pregnancy, had a dual echolucent image below the fetal abdomen under the stomach (double bubble image) compatible with duodenal atresia. On the bases of the suspicion of the association type of malformation with chromosomal disease, a prenatal cytogenetic diagnosis was performed, resulting: 47, XX, +21, inv (9) (p11; q12), female fetus positive for trisomy 21 and pericentric inversion of chromosome 9. After genetic counseling, the couple opted for the exceptional pregnancy interruption. The presence of duodenal atresia and dysmorphic features compatible with down syndrome were confirmed by pathological anatomy.

Anatomical variations of the superior mesenteric artery and its clinical and surgical implications in humans / Variações anatômicas da artéria mesentérica superior e suas implicações clínicas e cirúrgicas em humanos

ABSTRACT Introduction: Superior mesenteric artery (SMA) usually arises from the abdominal aorta, just below the celiac trunk and it supplies the midgut-derived embryonic structures. Anatomical variations in this vessel contribute to problems in the formation and/or absorption of this part of the intestine and its absence has been recognized as the cause of congenital duodenojejunal atresia. Objective: To analyze SMA anatomical variations in humans and the possible associated clinical and surgical implications. Methods: This is a systematic review of papers indexed in PubMed, SciELO, Springerlink, Science Direct, Lilacs, and Latindex databases. The search was performed by two independent reviewers between September and December 2018. Original studies involving SMA variations in humans were included. SMA presence/absence, level, place of origin and its terminal branches were considered. Results: At the end of the search, 18 studies were selected, characterized as for the sample, method to evaluate the anatomical structure and main results. The most common type of variation was when SMA originated from the right hepatic artery (6.13%). Two studies (11.11%) evidenced the inferior mesenteric artery originating from the SMA, whereas other two (11.11%) found the SMA sharing the same origin of the celiac trunk. Conclusion: SMA variations are not uncommon findings and their reports evidenced through the scientific literature demonstrate a great role for the development of important clinical conditions, making knowledge about this subject relevant to surgeons and professionals working in this area.

RESUMO Introdução: A artéria mesentérica superior (AMS), normalmente, tem sua origem a partir da aorta abdominal, um pouco abaixo do tronco celíaco e é responsável pela irrigação das estruturas derivadas, embrionariamente, do intestino médio. Variações anatômicas nesse vaso contribui para defeitos na formação e/ou absorção dessa parte do intestino e a sua ausência tem sido reconhecida como a causa da atresia duodenojejunal congênita. Objetivo: Analisar as variações anatômicas dela em humanos e as possíveis implicações clínicas e cirúrgicas associadas. Métodos: Trata-se de uma revisão sistemática de artigos indexados nas bases de dados PubMed, SciELO, Springerlink, Scienc Direct, Lilacs e Latindex. A busca ocorreu por dois revisores independentes entre setembro e dezembro de 2018. Foram incluídos artigos originais envolvendo as variações da AMS em humanos. Considerou-se para este estudo a presença/ausência da AMS, o nível, local de origem e seus ramos terminais. Resultados: Ao final da busca foram selecionados 18 artigos, caracterizados quanto à amostra, método para avaliar a estrutura anatômica e principais resultados. O tipo de variação mais comum foi aquele cuja AMS se originou da artéria hepática direita (6,13%). Dois estudos (11,11%) evidenciaram a artéria mesentérica inferior originando-se a partir da AMS, enquanto outros dois (11,11%) constataram ser ela compartilhada na mesma origem do tronco celíaco. Conclusão: Variações na AMS não são achados incomuns e seus relatos evidenciados através da literatura científica demonstram grande importância para o desenvolvimento de condições clínicas importantes, tornando o conhecimento sobre esse assunto relevante para os cirurgiões e profissionais atuantes nesta área.

Audit of neonatal congenital anomalies required surgical intervention at tertiary care centre

Background: Nearly 10% of neonatal deaths are due to congenital malformations requiring surgical intervention. Hence our aim is to study spectrum and outcome of the different neonatal congenital anomalies requiring surgical intervention.Methods: This prospective study was conducted over a period of 2 years. 130 cases which required surgical intervention in neonatal period were included in the study. Plain x-ray abdomen was done in all the cases of our study. Ultrasound scan was done in all the cases to rule out renal and other anomalies. Contrast radiography was also performed in selected cases. All cases underwent their respective operations depending upon the diagnoses. Complication and mortalities during hospital stay were noted.Results: During the study period total 130 neonates underwent surgical intervention. Out of 130 cases 5(3.84%) neonates had trachea-esophageal fistula, 2(1.53%) had pure esophageal atresia, 5(3.84%) had duodenal atresia, 9(6.92%) had jejunal atresia, 14(10.76%) had ileal atresia, 3 had meconium ileus(2.30%), 9(6.92%) had malrotation, 15(11.5%) had HD and 5(3.84%) had Meckel's diverticulum, 10(7.69%) had Hypertrophic Pyloric Stenosis, 2(1.53%) had gastroschisis, 3(2.30%) had omphalocele, 16(12.30%) had anorectal malformation, 4(3.07%) had Patent Vitello Intestinal Duct, 3(2.30%) had persistent patent urachus, 4(3.07%) had congenital diaphragmatic hernia, 1(0.76%) had Congenital Lobar Emphysema, 4(3.07%) had Neural Tube Defects, 8(6.15%) had Inguinal Hernia, 6(4.61%) had Posterior Urethral Valve and 2(1.53%) had Pelvi-ureteric Junction Obstruction. There were 85 males and 45 females (M: F-2:1). Septicaemia (40%) was most common complication, 21 (16.15%) cases had mortality.Conclusions: There is lack of awareness regarding dog bite and its management among the rural population.

A study on the prognosis of different surgical procedures for severe jejunoileal atresia / 中国新生儿科杂志

Objective To compare the prognosis of different surgical procedures and to find the relatively safe and effective treatment for severe jejunoileal atresia(sJA).Method From January 2007 to June 2018,children with sJA receiving different surgical procedures in our hospital were retrospectively reviewed.Their clinical data were analyzed,including the survival rate,complication rate,unplanned re-operation rate and postoperative nutritional status.Result A total of 130 patients were enrolled in this study.According to the different types of surgical procedures,the patients were assigned into primary anastomosis group (58 cases,44.6％),Mikulicz double barrel ileostomy group (17 cases,13.1％) and Bishop-Koop anastomosis group (55 cases,42.3％).The overall mortality rate was 6.2％ (8/130).No significant differences existed in mortality rates among the three groups (P＞0.05).The incidences of gastrointestinal complications in primary anastomosis group (70.6％,12/17) and Mikulicz group (70.6％,12/17) were both higher Bishop-Koop group (34.5％,19/55),the differences were statistically significant (P＜0.05).The unplanned re-operation rates were 34.5％ (20/58) in the primary anastomosis group and 17.6％ (3/17) in the Mikulicz group,both higher than the Bishop-Koop group (3.6％,2/55),the differences were also statistically significant (P＜0.05).Multivariate analysis showed that the risk of complications in the primary anastomosis group (OR=3.434,95％CI 1.392～8.471) and Mikulicz group (OR=5.933,95％CI 1.467～23.991) were higher than the Bishop-Koop group.The risk of unplanned re-operation in the primary anastomosis group was 12.422 times as the Bishop-Koop group (95％CI 2.535～60.877).No significant differences existed between the Mikulicz group and the Bishop-Koop group in the risk of unplanned re-operation (P＞0.05).The weight for age (Z-score) in the Bishop-Koop group (-1.4,95％CI-2.0～-0.8) at the stoma closure time was better than the Mikulicz group (-3.2,95％CI-4.4～-2.0),the difference was statistically significant (P＜0.01).Conclusion Bishop-Koop anastomosis has lower complication rate and lower unplanned re-operation rate in the treatment of sJA.The nutritional status of children who received Bishop-Koop anastomosis is better than Mikulicz double barrel ileostomy at the stoma closure time.Bishop-Koop anastomosis is relatively safe and effective for sJA patients.

Colonic atresia in a newborn

ABSTRACT Introduction: Colonic atresia is the least common type of intestinal atresia; however, it must be suspected in patients with partial or complete intestinal obstruction, failure to pass meconium, vomit and abdominal distension. Good prognosis has been described in patients with timely treatment. Case report: This paper reports the case of a newborn patient presenting with vomit, abdominal distension, failure to pass meconium and a rapid progressive clinical deterioration. A colonic atresia was found during exploratory laparotomy, which required a temporary colostomy due to the discrepancy of the proximal and distal calibers. Subsequently, colonic anastomosis was performed using a protective colostomy that was finally closed. This patient had a good post-operative recovery. Conclusion: Colonic atresia must be considered as an important cause of distal intestinal obstruction in pediatric patients and, therefore, it should always be suspected. Ruling out other associated abnormalities is also recommended, as well as performing a rectal biopsy for Hirschsprung's disease to avoid complications.

Santulli Enterostomy: A Considerable Method for Patients Who Require Proximal Enterostomy

PURPOSE: Santulli enterostomy has been used for various surgical abdominal conditions that require temporary diversion of bowel during a neonatal period. The aim of this study was to report clinical outcomes of Santulli enterostomy and to evaluate its usefulness. METHODS: Between January 2000 and December 2016, 40 neonates who underwent Santulli enterostomy were enrolled; Santulli enterostomies were performed for 25 patients without previous laparotomy (primary Santulli group) and 15 patients with previous laparotomy (secondary Santulli group). RESULTS: Small bowel atresia is the first common indication of Santulli enterostomy (22/40, 55.0%), and luminal discrepancy between proximal and distal bowel was the most common determinant factor of Santulli enterostomy (17/40, 42.5%). The median age at surgery and mean birth weight were 2 days and 2,480 g respectively in the primary group, and 71 days, 2,340 g respectively in the secondary group. Operation time was significantly longer in the secondary group than the primary group (156±48 minutes vs. 224±95 minutes, p=0.019), and there was no difference in the time taken to initiation of oral feeding between the two groups. Santulli enterostomy closure was performed at median 65 days after Santulli enterostomy for primary group and 70 days for secondary group. Six complications (15.0%) were found after Santulli enterostomy, and nine complications (24.3%) after Santulli enterostomy closure (p=0.302). The incidence of complications was significantly higher in secondary group than in primary group (4.5% vs. 53.3%, p=0.001), and the reoperation rate was also significantly higher in the secondary group (4.5% vs. 46.7%, p=0.004). CONCLUSION: Santulli enterostomy could be applied as a temporary enterostomy in neonatal patients with various surgical abdominal diseases. Considering the high complication rate after secondary Santulli enterostomy closure, decision making on the timing of enterostomy closure should be done with caution.

Analysis of diagnosis and treatment for intestinal atresia in 147 newborns / 中国新生儿科杂志

Objective To summarize the pathology of congenital intestinal atresia,the incidence and prenatal diagnosis rate of different types,and to analyze the location and type of intestinal atresia as well as the factors that affect the mortality of various types of intestinal atresia.Method We retrospectively analyzed the clinical data of 147 children with congenital intestinal atresia from January 2013 to March 2016,including gender,gestational age,parity,prenatal diagnosis or not,delivery methods,hospital admission,surgical methods,findings during surgery,combined malformations,complications and prognosis.They were analyzed statistically.Result A total of 147 cases,including 69 males and 78 females were enrolled.There were 40 premature infants and 107 full term cases.Twins were found in 3 cases.Hospital admission age range from 1 hour to 62 days;admission weight range from 1 480 g to 4 200 g;32 cases were diagnosed before birth.2 cases were abandoned before surgery because of trisomy 21.Postoperatively,the occlusion sites was confirmed as following:67 cases (46.2％) in ileum,43 cases (29.7％) in jejunum,26 cases (17.9％) in duodenum,and 9 cases (6.2％) in colon.The pathological types were as following:type Ⅰ 42 cases (29.0％),type Ⅱ 8 cases (5.5％),type Ⅲa 65 cases (44.8％),type Ⅲb 15 cases (10.3％) and type Ⅳ 15 cases (10.3％).22 cases (14.9％) were died because of refusal of treatment:7 cases were due to short bowel syndrome and meconium peritonitis,6 cases were due to postoperative chronic pseudo-obstruction,and 5 cases had anastomotic leakage requiring reoperation.1 case had postoperative enterocolitis and gave up treatment,1 case had anastomotic leak and sever systemic post-surgery infection and gave up further treatment,and 2 cases gave up because of 21-trisomy syndrome.Conclusion The operation plan of intestinal atresia should be based on the location and type of the blockade;the location and complications of the blockade (pseudo-obstruction,short bowel syndrome,and anastomotic leakage) are important factors affecting the treatment and prognosis.

Clinicopathological features of congential intestinal solitary fibromatosis: 2 cases report and literature review / 临床与实验病理学杂志

Purpose To study the clinicopathological features of congenital solitary intestinal fibromatosis (SIF).Methods Clinical,radiological and pathological features of 2 cases of SIF were analyzed along with a literature review.Results Two cases of SIF with one girl and one boy.Both presented with vomiting and abdominal distention at the birth day.A plain abdominal radiograph revealed small bowl obstruction and pneumoperitoneum.Jejunum atresia and perforation were detected at surgery.Microscopically,spindle tumor cells diffusely infiltrated in the whole intestinal wall,tumor cells were monomorphic with bland looking and rare mitosis was reviewed.Immunohistochemically,vimentin was positive while muscular markers as SMA,desmin and Myogenin were all negative.And S-100,CD117,CD34,ALK and β-catenin were not expressed.ETV6 split gene was not detected in one of the patients by FISH.Two patients of SIF had no tumor recurrence with follow-up till now.Conclusion Congenital SIF is a rare and unique intestinal tumor in neonate,and it should be differentiated from congenital fibrosarcoma and myofibroma.SIF appears a very good prognosis after segmental resection.

Intestinal Obstruction in Pediatric Age Group: A Clinico- Pathological Study.

Background: Intestinal obstruction is one of the commonest surgical emergencies seen in pediatric patients. In last few decades, the scenario of diagnosis and management of pediatric intestinal obstruction has been changed due to better understanding of pathophysiology, improvement in diagnostic methods, availability of better antibiotics, safer pediatric anesthesia and advanced pre and post operative intensive care. Objectives: To study the epidemiology, clinical features and outcome of intestinal obstruction in paediatric patients. Methods: The present prospective study was conducted in department of General Surgery, Index Medical College, Indore in paediatric patients admitted with clinical features and diagnosis of intestinal obstruction between July 2014 to June 2016 (2 years). Results: Majority of cases of intestinal obstruction in our study were in age group of 0-1 years (70.2%), followed by the age group of 1-5 years (20.2%) and more than 5 years (9.6%). Conclusion: Majority of patients (70.2%) were of less than one year age, and male to female ratio was found to be 3.5:1. Congenial causes of intestinal obstruction were more common (61.7%) than the acquired causes. Intussusception (18.1%) was the commonest cause of intestinal obstruction in this series.

Hirschsprung disease and hepatoblastoma: case report of a rare association / Doença de Hirschsprung e hepatoblastoma: relato de caso de uma associação rara

ABSTRACT CONTEXT: Hirschsprung disease is a developmental disorder of the enteric nervous system that is characterized by absence of ganglion cells in the distal intestine, and it occurs in approximately 1 in every 500,000 live births. Hepatoblastoma is a malignant liver neoplasm that usually occurs in children aged 6 months to 3 years, with a prevalence of 0.54 cases per 100,000. CASE REPORT: A boy diagnosed with intestinal atresia in the first week of life progressed to a diagnosis of comorbid Hirschsprung disease. Congenital cataracts and sensorineural deafness were diagnosed. A liver mass developed and was subsequently confirmed to be a hepatoblastoma, which was treated by means of surgical resection of 70% of the liver volume and neoadjuvant chemotherapy (ifosfamide, cisplatin and doxorubicin). CONCLUSION: It is known that Hirschsprung disease may be associated with syndromes predisposing towards cancer, and that hepatoblastoma may also be associated with certain congenital syndromes. However, co-occurrence of hepatoblastoma and Hirschsprung disease has not been previously described. We have reported a case of a male patient born with ileal atresia, Hirschsprung disease and bilateral congenital cataract who was later diagnosed with hepatoblastoma.

RESUMO CONTEXTO: A doença de Hirschsprung é uma desordem do desenvolvimento do sistema nervoso entérico, que é caracterizada pela ausência de células ganglionares no intestino distal, ocorrendo em cerca de 1 a cada 500.000 nascimentos. O hepatoblastoma é uma neoplasia maligna do fígado que geralmente ocorre em crianças de 6 meses a 3 anos, com prevalência de 0,54 casos por 100.000. RELATO DE CASO: Um menino com diagnóstico de atresia intestinal na primeira semana de vida evoluiu com diagnóstico concomitante de doença de Hirschsprung. Catarata congênita e surdez neurossensorial foram diagnosticadas. Surgiu lesão hepática com posterior confirmação de hepatoblastoma, tratado com ressecção cirúrgica de 70% do volume hepático e quimioterapia neoadjuvante (ifosfamida, cisplatina e doxorubicina). CONCLUSÃO: Sabe-se que a doença de Hirschsprung pode estar associada a síndromes de predisposição ao câncer, da mesma forma que o hepatoblastoma já foi correlacionado a certas síndromes congênitas malformativas. No entanto, até o momento, a associação de hepatoblastoma com a doença de Hirschsprung não foi descrita. Relatamos o caso de um menino que nasceu com atresia ileal, doença de Hirschsprung, catarata congênita bilateral e com posterior diagnóstico de hepatoblastoma.

Umbilical cord ulceration: An underdiagnosed entity

Umbilical cord ulceration is a rare condition presenting with sudden fetal bradycardia due to fetal hemorrhage and in most cases leading to intrauterine death. A strong association with intestinal atresia has been reported. Most cases present after 30 weeks of gestation, with preterm labor or rupture of membranes followed by sudden fetal bradycardia. We report two such cases of umbilical cord ulceration and review the available literature. One of the cases interestingly presented at 26 weeks, much earlier than what is reported in the world literature. In view of high perinatal mortality and morbidity, awareness of this condition is mandatory for timely and appropriate management to improve the fetal outcome.

Umbilical cord ulceration: An underdiagnosed entity

Umbilical cord ulceration is a rare condition presenting with sudden fetal bradycardia due to fetal hemorrhage and in most cases leading to intrauterine death. A strong association with intestinal atresia has been reported. Most cases present after 30 weeks of gestation, with preterm labor or rupture of membranes followed by sudden fetal bradycardia. We report two such cases of umbilical cord ulceration and review the available literature. One of the cases interestingly presented at 26 weeks, much earlier than what is reported in the world literature. In view of high perinatal mortality and morbidity, awareness of this condition is mandatory for timely and appropriate management to improve the fetal outcome.

Hipotiroidismo congénito y diafragma intraluminal no fenestrado. Rara asociación / Congenital Hypothyroidism and Intraluminal non-Fenestrated Diaphragm. Rare association

El hipotiroidismo congénito afecta a 1 de cada 3000 a 4000 neonatos y es una de las causas prevenibles de dificultades en el aprendizaje. Se presenta el caso de un recién nacido sexo masculino, que al nacer desarrolló síndrome de distréss respiratorio e intolerancia digestiva que progresó a la disfunción con distensión abdominal, además de signos dismórficos al examen físico. Se concluye el caso como hipotiroidismo congénito asociado a atresia duodenal por diafragma intraluminal no fenestrado. Se realizó intervención quirúrgica a las 46 horas de vida y en su posterior evolución presentó complicaciones mayores hasta fallecer. Se realizó una revisión actualizada sobre estas enfermedades en el período neonatal y se presentaron fotos del caso previo consentimiento familiar.

Congenital hypothyroidism affects 1 in 3000 to 4000 neonates and is one of the preventable causes of learning difficulties. The case is presented of a male newborn who developed, on his birth, respiratory distress syndrome and digestive intolerance, progressing to dysfunction with abdominal distension, together with dimorphic signs on medical examination. The case is concluded as congenital hypothyroidism associated with duodenal atresia due to intraluminal non-fenestrated diaphragm. Surgical intervention was made on the 46 hours of life and the patient presented complications on his further development, and finally deceased. An updated revision was made about these diseases in the neonatal period and pictures of the case were presented under parental permission.

An Isolated Tubular Intestinal Loop in a Neonate with Type II Intestinal Atresia / 대한주산의학회잡지

An isolated tubular intestinal loop (ITIL) means an anatomical or vascular communication with rest of the bowel loop and may provide an insight into the pathogenesis of intestinal atresia. We experienced a case of an ITIL identified in omentum of a 4-day-old neonate with type-II intestinal atresia. To our knowledge, this association has never been reported in the English literature. Omental wrapping of the incompletely resorbed ischemic bowel segment may explain this phenomenon in a case of congenital intestinal atresia.

Intestinal Atresia: A Four-Year Review of Cases in Ikeja-Lagos.

Introduction: Intestinal atresia is one of the most common causes of neonatal intestinal obstruction worldwide. The pattern of presentation and management in our institution is reviewed. The objective of the study was to evaluate the pattern of intestinal atresias and stenoses in newborns who presented to our unit. Materials and Methods: A retrospective study of patients with intestinal atresias and stenoses who presented between September 2004 and November 2008. The clinical presentation, diagnoses, operative management, post operative care and outcome were obtained from the case notes. Results: Thirty cases were seen in that period, M:F; 1:1.5. Eleven (36.7%) were duodenal pathologies and 19 (63.3%) were jejunoileal. The main presenting symptom was bilious vomiting. Overall mortality is 40.9%. Prematurity and delayed presentations are the major contributing factors to mortality. Conclusion: Improvement of health care facilities as well as public health education to seek early intervention will improve outcome. Maternal awareness of this condition would lead to prompt seeking of treatment for paediatric patients.

Estenosis congénita de íleon en un lactante de 5 meses de edad con síndrome de obstrucción intestinal / Congenital stenosis of the ileum in a 5-month-old infant with intestinal obstruction syndrome

Introducción. La atresia intestinal se refiere a la obstrucción completa de la luz del intestino y la estenosis al bloqueo parcial de ésta. Ocurre un caso por cada 4,000 a 5,000 nacimientos y no existen diferencias de presentación en cuanto al sexo. Cuando la obstrucción es incompleta los signos como vómito, la distensión abdominal y el estreñimiento pueden aparecer poco tiempo después del nacimiento o retrasarse de forma indeterminada. No se presentan grandes dificultades para diagnosticar la atresia intestinal; en cambio es más complicado diagnosticar la estenosis. El síndrome obstructivo del lactante obliga a descartar la estenosis congénita intestinal. Se han presentado casos en edades más avanzadas aunque esto es raro. Caso clínico. Se presenta el caso de un paciente femenino de 5 meses de edad con un cuadro clínico de obstrucción intestinal que fue manejado quirúrgicamente; se encontró una estenosis congénita de íleon. La paciente evolucionó sin complicaciones. Conclusiones. El síndrome obstructivo del lactante por estenosis intestinal es una entidad rara que no suele sospecharse de primera instancia; sin embargo, debe descartarse al realizar el protocolo de estudio para obstrucción intestinal.

Background. Intestinal atresia refers to complete obstruction or partial stenosis of the intestinal lumen. The prevalence is 1/4000-5000 births, without gender predilection. When the blockage is incomplete, signs such as vomiting, abdominal distension, and constipation may occur shortly after birth or delayed in an unspecifed manner. In intestinal atresia, the challenge may not be significant; however, stenosis may pose considerable diffculties. Obstructive syndrome must be ruled out in infant congenital intestinal stenosis. Even more rare cases have occurred during later ages. Case Report. We report on a 5-month-old female infant with clinical symptoms of intestinal obstruction, which was managed surgically. Surgical finding was a congenital stricture of the ileum. The patient recovered without complications. Conclusions. Obstructive syndrome in infant intestinal stenosis is a rare entity, which is usually not first suspected, but it should be ruled out as a study protocol for intestinal obstruction.

Atresia duodenal con fístula esofagopleural: reporte de caso / Esophago-pleural fistula with duodenal atresia: case report

Las fístulas esofagopleurales en el recién nacido son malformaciones raras, cuya etiología aún se desconoce, y cuya asociación a la atresia duodenal está pobremente documentada. Se reporta en el presente artículo el caso de un recién nacido con múltiples malformaciones congénitas, en quien se diagnosticó una fístula esofagopleural asociada a atresia duodenal. Además, se revisa brevemente la literatura disponible, con énfasis en la posible fisiopatología, así como en las posibles manifestaciones clínicas y radiológicas de este tipo de fístulas.

Esophago-pleural fistulas have been poorly understood and only a few cases of its association with duodenal atresia have been described. This case report describes a newborn with multiple congenital malformations; who exhibits poor clinical evolution with hydroneumothorax and an esophago-pleural fistula with duodenal atresia. In this particular case, we review the possible physiopathology, clinical and radiological manifestations of this rare type of fistulas.

Relato raro de atresia jejunoileal em dois irmãos nascidos com três anos de diferença / Rare jejunoileal atresia in two singleton brothers born three years apart

A atresia do intestino delgado é malformação congênita, conhecida como causa comum de obstrução intestinal no período neonatal. A atresia duodenal familiar é extremamente rara e tem sido atribuída a aberrações cromossômicas e a consaguinidade, sugerindo herança autossômica recessiva. As anomalias intestinais distais, como a atresia jejunoileal,são malformações congênitas raras do intestino delgado e têm sido relacionadas a oclusões vasculares tanto no início quanto no final da gestação além de causas genéticas. A atresia jejunoileal familiar em gêmeos é de ocorrência extremamente rara e é relacionada a algumas drogas e tinturas que são instiladas durante a gestação para diagnósticopor amniocentese e tem sido descrita como causa da atresia jejunoileal em irmãos gêmeos. Autores de dois diferentes estudos descrevem que esta atresia é significantemente mais frequente em gêmeos que em irmãos nascidos separadamente. No presente artigo descrevemos dois casos extremamente raros em dois irmãos nascidos com três anosde diferença, ambos com atresia jejunoileal sem outras malformações associadas ou anomalias cromossômicas, que foram acompanhados pela equipe em 2004 e 2007, respectivamente. Apesar das investigações, não foram encontradas nenhuma razão para essa ocorrência. Contudo, os autores estão seguindo os dois meninos desde então e não detectaramalterações no desenvolvimento de nenhum deles que pudessem sugerir qualquer malformação associada.

Small intestinal atresia is a common congenital malformation and it is a well-known cause of intestinal obstruction in neonates. Familial occurrence of duodenal atresia is extremely rare and has been attributed to chromosomal aberrations and parental consanguinity suggesting autosomal recessive inheritance. Distal intestinal anomalies, such as jejunoileal atresia, are a rare congenital malformation of the small bowel and have been related to vascular occlusion in the earlier or later stages in pregnancy and genetic causes. Familial jejunoileal atresia in twins is an extremely rareoccurrence that is attributed to the use of some chemicals and other dyes instillated during diagnostic amniocentesis and has been described as a cause of jejunoileal atresia in twin-brothers. Authors of two different research institutes stated that jejunoileal atresia is significantly more frequent in twins than in singletons. In the present article we describean extremely rare occurrence in two singleton infants, who were born three years apart, with similar jejunoileal atresia with no other associated malformations or chromosomal anomalies, who were treated in 2004 and 2007, respectively. Despite investigation, we did not find any reason for this particular occurrence; however they will be closely followed inorder to detect any development alterations that could indicate an associated malformation.

El manejo de la atresia intestinal tipo IIIb sigue siendo un reto / The management of Type III intestinal atresia still remains being a challenge

La atresia intestinal constituye una de las causas más frecuentes de obstrucción intestinal en el recién nacido y es causada por catástrofes vasculares mesentéricas tardías durante el desarrollo embrionario; sin embargo, puede tener otras causas. La atresia de tipo IIIb es rara y su manejo es un reto para todo el equipo médico. Este tipo de atresia sigue siendo de difícil manejo y requiere un equipo completo interdisciplinario para obtener un buen resultado: en primer lugar, lograr la supervivencia y, posteriormente, lograr un crecimiento y un desarrollo normales. La técnica quirúrgica incluye una anastomosis término-terminal; en complicaciones como las del caso presentado, se recurre a las plastias en YV, y a la colocación de férula en el intestino con sonda fenestrada para proteger la anastomosis, con el fin de mantener descomprimido el intestino y permeable distalmente.

Intestinal atresia constitutes one of the most frequent causes of intestinal obstruction in the newborn and it is caused by delayed mesenteric vascular catastrophes during embryonic development; nevertheless, it may have other causes. Atresia type IIIb is rare and its management is a challenge for all of the members of the medical team. This type of atresia continues being of difficult management, and needs a complete multidisciplinary equipment in order to attain good results: firstly, to ensure the survival of the child and subsequently; to achieve normal growth and development. The surgical technique includes endto- end anastomoses and, in complications like those presented in this case, we place YV shaped plasty and making an intestinal splint with a fenestrated probe in order to protect the anastomosis, maintaining bowel decompressed and distally-permeable.