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1.
Rev. colomb. obstet. ginecol ; 72(2): 202-209, Apr.-June 2021. graf
Article in Spanish | LILACS | ID: biblio-1289317

ABSTRACT

Resumen Objetivos: Reportar el caso de una paciente con diagnóstico prenatal de atresia de yeyuno y hacer una revisión de la literatura en torno al resultado y al diagnóstico de esta entidad, implementando el uso de métodos no convencionales: ecografía tridimensional (3D) o resonancia magnética fetal (RM). Materiales y métodos: Se reporta el caso de una gestante de 18 años, remitida a la Unidad de Medicina Materno-Fetal, Genética y Reproducción del Hospital Virgen del Rocío de Sevilla (España), por feto con imagen quística abdominal, peristáltica, compatible con atresia de yeyuno, la cual se confirmó con ecografía 3D modo HDlive. Se realizó una búsqueda bibliográfica en Medline/PubMed, LILACS y Google Scholar, restringiendo por tipo de idioma (inglés y español) y fecha de publicación (enero de 1995 a junio de 2020). Se incluyeron estudios primarios de reportes y series de caso, que abordaran el resultado y el diagnóstico prenatal de esta patología. Resultados: La búsqueda identificó 1.033 títulos, de los cuales cuatro estudios cumplieron con los criterios de inclusión, estos fueron reporte o series de casos. En total se reportaron doce fetos con diagnóstico prenatal de atresia de yeyuno detectada con métodos no convencionales. Una gestación finalizó con óbito a la semana 26 de gestación y en todos los demás casos, el diagnóstico se ratificó durante el periodo neonatal, requiriendo la resección del segmento comprometido. Dos neonatos desarrollaron síndrome de intestino corto como consecuencia de una resección intestinal amplia. El resto experimentó una evolución posoperatoria favorable. Conclusión: La literatura disponible en torno al diagnóstico prenatal de atresia de yeyuno utilizando métodos no convencionales es escasa y se limita a reporte o series de casos. La literatura revisada sugiere que, en presencia de dilatación intestinal, la ecografía 3D y la resonancia magnética fetal podrían tener cierta utilidad a la hora de caracterizar la porción atrésica y establecer el diagnóstico diferencial. Se requieren más estudios que evalúen la utilidad diagnóstica de estas dos alternativas.


Abstract Objectives: To report the case of a patient with a prenatal diagnosis of jejunal atresia and to review the literature regarding the results and prenatal diagnosis of this entity, implementing the use of non-conventional methods (3D ultrasound or magnetic resonance imaging). Material and methods: Report of a case of an 18-year-old pregnant woman referred to the Maternal-Fetal Medicine, Genetics and Reproduction Unit of the Virgen del Rocío Hospital in Seville due to fetus with abdominal peristaltic cystic image, consistent with jejunal atresia, confirmed with 3D HDLive mode ultrasound. A bibliographic search was carried out in Medline/PubMed, Google Scholar and LILACS, restricting by type of language (English and Spanish) and date of publication (January 1995 to June 2020). Primary studies of reports and case series relating to the outcome and prenatal diagnosis of this pathology were included. Results: The search identified 1,033 titles, of which four studies met the inclusion criteria, these being reports or case series. A total of twelve fetuses with a prenatal diagnosis of jejunal atresia detected with unconventional methods were reported. In all cases, the prenatal diagnosis was confirmed during the neonatal period, which required resection of the compromised segment; one of them died and two neonates developed short bowel syndrome because of a wide bowel resection. The postoperative course in the remaining cases was favorable. Conclusion: The available literature on the prenatal diagnosis of jejunal atresia using non-conventional methods is scarce and is limited to case reports or case series. The literature reviewed suggests that, in the presence of intestinal dilation, 3D ultrasound and magnetic resonance imaging could be of some use in characterizing the atretic portion and establishing the differential diagnosis. More studies are required to evaluate the diagnostic utility of these two alternatives.


Subject(s)
Humans , Female , Pregnancy , Intestinal Atresia , Jejunum/diagnostic imaging , Prenatal Diagnosis , Ultrasonography
3.
Journal of Zhejiang University. Medical sciences ; (6): 487-492, 2019.
Article in Chinese | WPRIM | ID: wpr-819057

ABSTRACT

OBJECTIVE@#To summarize the clinical characteristics and treatment of type Ⅲ-b congenital intestinal atresia (CIA).@*METHODS@#The clinical data of 12 type Ⅲ-b CIA treated in the Children's Hospital of Zhejiang University School of Medicine from January 2015 to December 2017 were analyzed retrospectively.@*RESULTS@#Of the 12 patients diagnosed as type Ⅲ-b CIA in operation, treatment was refused during operation by their parents in 2 cases. For one child, only the proximal intestine was partly resected in the first operation, dilatation and dysplasia of the duodenum was diagnosed and total duodenum was resected and sutured in the second operation, as the child had postoperative intestinal obstruction. For one child, due to the long distal normal intestine, distal apple-peel like intestine was partly resected without mesenteric reformation. For the rest 8 children total duodenum resection and mesenteric reformation were performed. During the postoperative follow-up, one case was early rejected for further treatment by parents, one case died from complex congenital heart disease, 5 cases had the complication of short bowel syndrome. All 8 survival children received parenteral nutrition support after operation, 5 of whom received parenteral nutrition support for more than 42 days, and they were followed up for 1-3 years after discharge. The short-time efficacy was satisfactory.@*CONCLUSIONS@#For children with type Ⅲ-b CIA, the distal apple-peel like intestine should be preserved as much as possible, the mesenteric reformation should be performed and the proximal dilated bowel should be partly resected and sutured. Postoperative nutritional support and early intestinal rehabilitation contribute to the compensation for rest intestines.


Subject(s)
Child , Humans , Intestinal Atresia , General Surgery , Therapeutics , Intestines , General Surgery , Parenteral Nutrition , Retrospective Studies , Short Bowel Syndrome , Treatment Outcome
4.
Article in English | LILACS, COLNAL | ID: biblio-989570

ABSTRACT

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.


Subject(s)
Humans , Intestinal Atresia , Colostomy , Colon
5.
Journal of the Korean Association of Pediatric Surgeons ; : 20-25, 2018.
Article in Korean | WPRIM | ID: wpr-740662

ABSTRACT

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.


Subject(s)
Humans , Infant, Newborn , Birth Weight , Decision Making , Enterocolitis, Necrotizing , Enterostomy , Incidence , Intestinal Atresia , Laparotomy , Methods , Peritonitis , Phenobarbital , Reoperation
7.
Chinese Journal of Gastrointestinal Surgery ; (12): 535-539, 2017.
Article in Chinese | WPRIM | ID: wpr-317592

ABSTRACT

<p><b>OBJECTIVE</b>To evaluate the efficacy of bowel plication combined with early enteral nutrition (EEN) in the enhanced recovery after surgery(ERAS) of jejunal atresia (JA) neonates.</p><p><b>METHODS</b>Between January 2005 and January 2014, 58 neonates with JA underwent surgical treatment in Children's Hospital of Nanjing Medical University. Their clinical data, including operation procedures, ages, birth weight, concomitant diseases, age at surgery, hospital stay, total parenteral nutrition (TPN), postoperative intestinal function recovery (the time to the first oral feeding and the time to oral feeding volume reaching 150 ml·kg·d), complications and reoperation, were retrospectively analyzed.</p><p><b>RESULTS</b>According to the surgical procedures, the 58 neonates were divided into three groups: control group(18 cases, undergoing atretic segments resection and primary anastomosis), bowel plication group(19 cases, undergoing bowel plication after atretic segments resection and primary anastomosis) and bowel plication combined with EEN group (21 cases, undergoing bowel plication combined with EEN). No significant differences of ages, birth weight, age at operation, and concomitant diseases were found among 3 groups (all P>0.05). The time of hospital stay, the time to the first oral feeding, the time to oral feeding volume reaching 150 ml·kg·d, and the time of TPN in bowel plication group were significantly shorter than those of control group [(19.3±4.4) d vs. (22.7±3.1) d, t=2.696, P=0.011; (9.8±3.3) d vs. (12.5±3.0) d, t=2.630, P=0.013; (18.5±4.1) d vs. (21.5±2.5) d, t=2.726, P=0.011; (13.1±2.9) d vs. (15.0±2.3) d, t=2.219, P=0.033]. However, above parameters of bowel plication combined with EEN group were significantly shorter than those of bowel plication group [(15.3±3.5) d vs. (19.3±4.4), t=4.120, P=0.003; (7.7±2.2) d vs. (9.8±3.3) d, t=2.428, P=0.020; (14.8±2.5) d vs. (18.5±4.1) d, t=3.752, P=0.001; (9.5±3.0) vs. (13.1±2.9) d, t=4.370, P=0.000].</p><p><b>CONCLUSION</b>The bowel plication combined with EEN contributes to the early use of intestinal function, shorten the time to the first oral feeding, and reduces the use of TPN, which can improve the recovery of jejunal atresia neonates.</p>


Subject(s)
Humans , Infant, Newborn , Anastomosis, Surgical , Comparative Effectiveness Research , Defecation , Digestive System Surgical Procedures , Methods , Enteral Nutrition , Methods , Intestinal Atresia , Rehabilitation , General Surgery , Jejunum , Congenital Abnormalities , General Surgery , Length of Stay , Parenteral Nutrition, Total , Postoperative Period , Retrospective Studies
8.
São Paulo med. j ; 134(2): 171-175, Mar.-Apr. 2016. tab, graf
Article in English | LILACS | ID: lil-782927

ABSTRACT

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.


Subject(s)
Humans , Male , Infant, Newborn , Hepatoblastoma/complications , Hirschsprung Disease/complications , Intestinal Atresia/complications , Cataract/congenital , Hepatoblastoma/diagnostic imaging , Hirschsprung Disease/diagnostic imaging , Intestinal Atresia/diagnosis
9.
Chinese Journal of Gastrointestinal Surgery ; (12): 1154-1159, 2016.
Article in Chinese | WPRIM | ID: wpr-323515

ABSTRACT

<p><b>OBJECTIVE</b>To explore the feasibility and safety of Bishop-Koop stoma procedure in the treatment of neonates with refractory congenital intestinal atresia.</p><p><b>METHODS</b>Clinical and follow-up data of 25 neonates with refractory congenital intestinal atresia undergoing Bishop-Koop stoma procedure in our center from January 2011 to December 2014 were retrospectively analyzed. Of 25 neonates, 13 (52%) were male, 12(48%) were female, the birth weight was 1600-3800 g (mean 2920 g), the age of admission was 10 hours to 20 days, and the age of operation was 1-58 d (mean 7 d). Diameter ratio of proximal atresia intestine to distal atresia intestine was all greater than 4. Eleven cases(44%) were high jejunal atresia, 3 cases(12%) type III( b, 7 cases(28%) type IIII(, 14 cases(56%) were identified as complex meconium peritonitis, and 3 cases (12%) received reoperation.</p><p><b>RESULTS</b>All the cases completed their Bishop-Koop stoma operations successfully with median operative time of 3 (1.2-4.5) hours and median intra-operative blood loss of 3.5(1-18) ml. The postoperative complication rate was 20%(5/25), including 3 cases of cholestasis, 1 case of ileus, and 1 case of neonatal necrotizing enterocolitis with septicemia who died 6 days after operation resulting in the mortality of 4%. Besides, 1 case gave up treatment because of economic reason. For the rest 23 neonates, the median first feeding time was 11 days and mean time was 11(5 to 20) days; the median time of postoperative total parenteral nutrition (TPN) was 15 days and mean time was 21 (5 to 68) days; the median hospital stay was 33 days and mean hospital stay was 25(12 to 81) days, respectively. Two-stage stoma closure operations were performed in all the 23 cases afterwards and no postoperative associated complications were found. When discharge after Bishop-Koop stoma operations, Z score of body weight was normal in 3 cases(13.0%) and lower than normal in 20 cases(87.0%), while in hospitalization for stoma closure, Z score of body weight was normal in 19 cases(82.6%) and lower than normal in 4 cases (17.4%). Of 23 cases, serum albumin level was normal in 9 cases(39.1%) before operation, in 3 cases (13.0%) when discharge and in 22 cases(95.7%) in hospitalization for stoma closure.</p><p><b>CONCLUSION</b>Bishop-Koop stoma procedure is safe and feasible in the treatment of neonates with refractory congenital intestinal atresia, and can obviously improve the nutritional status.</p>


Subject(s)
Female , Humans , Infant, Newborn , Male , Ileus , Intestinal Atresia , General Surgery , Length of Stay , Parenteral Nutrition, Total , Postoperative Complications , Reoperation , Retrospective Studies , Surgical Stomas
10.
Obstetrics & Gynecology Science ; : 388-392, 2016.
Article in English | WPRIM | ID: wpr-129972

ABSTRACT

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.


Subject(s)
Female , Pregnancy , Bradycardia , Hemorrhage , Intestinal Atresia , Membranes , Obstetric Labor, Premature , Perinatal Mortality , Rupture , Ulcer , Umbilical Cord
11.
Obstetrics & Gynecology Science ; : 388-392, 2016.
Article in English | WPRIM | ID: wpr-129957

ABSTRACT

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.


Subject(s)
Female , Pregnancy , Bradycardia , Hemorrhage , Intestinal Atresia , Membranes , Obstetric Labor, Premature , Perinatal Mortality , Rupture , Ulcer , Umbilical Cord
12.
Rev. cienc. salud (Bogotá) ; 13(2): 309-316, mayo-ago. 2015. tab
Article in Spanish | LILACS, COLNAL | ID: lil-767527

ABSTRACT

Presentación del caso: El objetivo de este artículo es presentar la experiencia exitosa del manejo multidisciplinario de una paciente con síndrome de intestino corto y falla intestinal con evolución a la adaptación intestinal. Se trata de una recién nacida prematura con atresia intestinal tipo IV, con múltiples atresias intestinales, quien evolucionó a la falla intestinal y requirió manejo con soporte nutricional parenteral prolongado, múltiples esquemas antibióticos, probióticos, multivitaminas, nutrición enteral con fórmula elemental, hasta lograr su adaptación intestinal y llevar a una dieta normal. La falla intestinal en estos pacientes es un reto para el equipo de salud, ya que no solo implica el manejo quirúrgico de su condición de base, si no del soporte nutricional, equilibrio hidroelectrolítico, disfunción hepática por colestasis, infecciones asociadas, etcétera. Discusión: El síndrome de intestino corto con evolución a la falla intestinal en niños es una condición, cuya prevalencia va en aumento en el ámbito mundial, gracias a los avances en el cuidado intensivo neonatal, cirugía neonatal y en el soporte nutricional de pacientes con condiciones como gastrosquisis, onfalocele y enterocolitis necrotizante. A pesar de las limitaciones del sistema de salud, es posible ofrecer un tratamiento multidisciplinario e integral para llevarlos hasta la adaptación intestinal.


Short bowel syndrome with progression to intestinal failure in children is a condition whose prevalence is increasing worldwide, thanks to advances in neonatal intensive care, neonatal surgery, and nutritional support of patients with conditions such as gastroschisis, omphalocele and enterocolitis. Objective: To present the successful experience of multidisciplinary management of a patient with short bowel syndrome and intestinal failure with progression to intestinal adaptation. Clinical case: A newly born premature with intestinal atresia type IV with multiple intestinal atresia, which evolved to intestinal failure and required management with prolonged parenteral nutritional support, multiple antibiotic schemes, prebiotics, multivitamins, enteral nutrition with elemental formula, in order to achieve their intestinal adaptation and enabling a normal diet. The evolution of these patients' intestinal failure is a challenge for the health team, as it not only involves the surgical management of the patient's condition, but the basic nutritional support, the fluid and electrolyte balance, hepatic dysfunction due to cholestasis associated infections, etc. Conclusion: Despite the limitations of our health care system, it is possible to offer a multidisciplinary and integrated treatment to lead to intestinal adaptation.


Caso clínico: O objetivo é apresentar a experiência de sucesso do manejo multidisciplinar de uma paciente com síndrome de intestino curto e falha intestinal com evolução à adaptação intestinal. Se trata de uma recém nascida prematura com atresia intestinal tipo IV com múltiplas atresias intestinais que evolucionou à falha intestinal e requereu manejo com suporte nutricional parental prolongado, múltiplos esquemas antibióticos, probióticos, multivitaminas, nutrição enteral com fórmula elementar, até conseguir sua adaptação intestinal e levar a uma dieta normal.A falha intestinal nestes pacientes é um desafio para a equipe de saúde, devido a que não só implica o manejo cirúrgico de sua condição de base, mas também do suporte nutricional, equilíbrio hidroeletrolítico, disfunção hepática por colestase, infeções associadas, etc. Discussão: a síndrome de intestino curto com evolução à falha intestinal em crianças é uma condição cuja prevalência vai em aumento a nível mundial, devido aos avanços no cuidado intensivo neonatal, cirurgia neonatal e no suporte nutricional de pacientes com condições como gastrosquise, onfalocele e enterocolite. Apesar das limitações de nosso sistema de saúde, é possível oferecer um tratamento multidisciplinar e integral para leva-los até a adaptação intestinal.


Subject(s)
Humans , Female , Infant, Newborn , Infant , Short Bowel Syndrome , Therapeutics , Intensive Care, Neonatal , Intestinal Atresia
14.
Chinese Journal of Gastrointestinal Surgery ; (12): 801-803, 2015.
Article in Chinese | WPRIM | ID: wpr-260267

ABSTRACT

<p><b>OBJECTIVE</b>To investigate the safety and efficacy of endoscopic duodenal valvectomy in the treatment of congenital duodenal membranous stenosis.</p><p><b>METHODS</b>Clinical data of two children with congenital duodenal membranous stenosis undergoing endoscopic duodenal valvectomy in our institute within October 2014 were analyzed retrospectively. This procedure was performed with Microknife XL and CRE balloon catheter through porous channel in the 9 mm flexible endoscope.</p><p><b>RESULTS</b>The first case was a 2-year-old boy who received two endoscopic operations including duodenal diaphragm resection and duodenal dilatation because of incision retraction. The second case was a 19-month-old gril who received once endoscopic duodenal valvectomy. Duodenal obstruction of these two children was relieved after operation. Postoperative x-rays showed no perforation. They could play in the floor 6 hours after operation without any complains, drink water 12 hours, take liquid diets 2 days and half solid food 3 days after operation. During follow-up a month after operation, the body weight gained was 1.5 and 1.0 kg respectively, and the dietary components was significantly improved.</p><p><b>CONCLUSION</b>Endoscopic duodenal valvectomy is feasible and effective in the treatment of congenital duodenal membranous stenosis.</p>


Subject(s)
Child, Preschool , Female , Humans , Infant , Male , Constriction, Pathologic , Duodenal Diseases , Duodenoscopy , Intestinal Atresia , Intestine, Small , Congenital Abnormalities , Retrospective Studies
15.
Medical Journal of Islamic World Academy of Sciences. 2014; 22 (4): 165-167
in English | IMEMR | ID: emr-154146

ABSTRACT

We would like to present findings from six patients with duodenal atresia


Subject(s)
Humans , Male , Female , Intestinal Atresia , Infant, Newborn
16.
Pediatric Gastroenterology, Hepatology & Nutrition ; : 257-262, 2014.
Article in English | WPRIM | ID: wpr-195961

ABSTRACT

Severe combined immunodeficiency (SCID) is a life-threatening syndrome of recurrent infections and gastrointestinal alterations due to severe compromise of T cells and B cells. Clinically, most patients present symptoms before the age of 3 months and without intervention SCID usually results in severe infections and death by the age of 2 years. Its association with intestinal anomalies as multiple intestinal atresias (MIA) is rare and worsens the prognosis, resulting lethal. We describe the case of a four year-old boy with SCID-MIA. He presented at birth with meconium peritonitis, multiple ileal atresias and underwent several intestinal resections. A targeted Sanger sequencing revealed a homozygous 4-bp deletion (c.313DeltaTATC; p.Y105fs) in tetratricopeptide repeat domain 7A (TTC7A). He experienced surgical procedures including resection and stricturoplasty. Despite parenteral nutrition-associated liver disease, the patient is surviving at the time of writing the report. Precocious immune system assessment, scrutiny of TTC7A mutations and prompt surgical procedures are crucial in the management.


Subject(s)
Humans , Male , B-Lymphocytes , Immune System , Intestinal Atresia , Liver Diseases , Meconium , Parturition , Peritonitis , Prognosis , Severe Combined Immunodeficiency , T-Lymphocytes , Writing
17.
Chinese Journal of Gastrointestinal Surgery ; (12): 816-819, 2014.
Article in Chinese | WPRIM | ID: wpr-254409

ABSTRACT

<p><b>OBJECTIVE</b>To investigate the feasibility and efficacy of laparoscopic procedure in the diagnosis and treatment of congenital intestinal atresia and stenosis in neonates and infants.</p><p><b>METHODS</b>Between September 2009 and September 2013, 55 cases with intestinal atresia and stenosis underwent laparoscope-assisted procedures in our department. There were, 32 males and 23 females, Twelve cases were diagnosed as duodenal atresia and stenosis and 43 as intestinal atresia and stenosis. The age at hospitalization was 7 minutes to 7 months(mean 9.88 d). After the diagnosis by multiport or transumbilical single-site laparoscopic exploration, cases with duodenal atresia and stenosis and part of the cases with proximal jejunum were treated by laparoscopic operations to remove the septum and restore intestinal continuity. In other cases with intestinal atresia and stenosis, laparoscopic inspection was performed to make diagnosis and then the proximal and distal ends of the atresia were exteriorized through the umbilical port site for end-to-oblique anastomosis.</p><p><b>RESULTS</b>All the 55 cases underwent this minimally invasive approach, and no case was converted to open surgery. The operative time of laparoscopic procedure for duodenal atresia and stenosis (n=12) ranged from 80 to 145 min(mean, 110 min). During the follow-up of 3-34 months, one case had recurrent postoperative vomiting induced by giant duodenal expansion above anastomotic stoma and died the second day after operation. The operative time of laparoscopic surgery for intestinal atresia and stenosis (n=43) ranged from 35 to 70 min (mean 46 min). During the follow-up of 3-36 months, 3 cases complicated with meconium peritonitis had postoperative short bowel syndrome and died. One case died of intestinal perforation at 3 month postoperatively. One case died of intestinal adhesion at 7 month postoperatively. The rest of cases had favorable outcomes.</p><p><b>CONCLUSION</b>Laparoscopic surgery for the diagnosis and treatment of intestinal atresia and stenosis has advantages of small incision, less trauma, and rapid recovery.</p>


Subject(s)
Female , Humans , Infant , Infant, Newborn , Male , Follow-Up Studies , Intestinal Atresia , General Surgery , Laparoscopy , Methods , Retrospective Studies , Treatment Outcome
18.
Lima; s.n; 2013. 77 p. tab, graf.
Thesis in Spanish | LILACS, LIPECS | ID: biblio-1113352

ABSTRACT

Las malformaciones congénitas son trastornos en el desarrollo del cuerpo durante la gestación, causados por factores genéticos y/o ambientales. Alrededor de 2 a 5 por ciento de 600 000 niños de nuestro país, presenta alguna malformación congénita, Muchas de ellas son percibidas como muy poco frecuentes, aunque su magnitud se ha incrementado en forma importante al disminuir otras causas de enfermedades del recién nacido. Tal es el caso de la Atresia Intestinal que es la causa más frecuente de obstrucción intestinal en el recién nacido y requieren tratamientos quirúrgicos complejos en centros especializados a cargo de personal multidisciplinario. El Instituto Nacional de Salud del Niño, es centro de referencia de pacientes con esta patología, además cuenta con tecnología en UCIN para el manejo postoperatorio. Es por esta razón que se realizó el presente trabajo de investigación, el cual es un estudio descriptivo observacional, retrospectivo, en el que se "Describe el Manejo Quirúrgico de los Recién Nacidos con Atresia Intestinal en el Servicio de Cirugía del Instituto Nacional de Salud del Niño de enero del 2000 a diciembre del 2011", obteniéndose los siguientes datos: Ligero predominio del sexo femenino; son más frecuentes en Lima 38.8 por ciento; el diagnóstico clínico de atresia en 77.55 por ciento; la atresia más frecuente fue duodenal 38.8 por ciento; el tipo I más frecuente; los factores de riesgo asociados: prematuridad 24.49 por ciento, bajo peso al nacer 26.53 por ciento, malformaciones asociadas 20.4 por ciento y cromosomopatías 18.36 por ciento; las cardiopatías las más frecuentes en 42.37 por ciento; la cromosomopatía más frecuente fue el Síndrome de Down 47.37 por ciento asociada a atresia duodenal; la eliminación de meconio presente en 20.41 por ciento. la radiografía de abdomen y el tránsito intestinal dilucidaron el diagnostico en el 100 por ciento; los hallazgos intraoperatorios demostraron malformaciones congénitas concomitante en...


Congenital malformations are developmental disorders of the body during pregnancy, caused by genetic and/or environmental. Around 2-5 per cent of 600 000 children in our country, has any congenital malformation, Many of them are perceived as very rare, but its magnitude has increased significantly to reduce other causes of disease of the newborn. Such is the case of Intestinal atresia is the most common cause of intestinal obstruction in the newborn and require complex surgical treatment in specialized centers by multidisciplinary staff. The National Institute of Child Health, is a referral center for patients with this condition, also has technology for managing postoperative UCIN. It is for this reason that we undertook the present research work, which is a descriptive observational. retrospective, in which "Describes the Surgical Management of Newborns with Intestinal Atresia in the Department of Surgery of the National Institute of Health Child January 2000 to December 2011", with the following data: Slight female predominance; Are more common in Lima 38.8 per cent; Clinical diagnosis of atresia in 77.55 per cent; The most frequent was duodenal atresia 38.8 per cent; Type I most frequent: associated risk factors: 24.49 per cent prematurity, low birth weight, 26.53 per cent, 20.4 per cent associated malformations and chromosomal 18.36 per cent, the most common heart disease in 42.37 per cent, the most common chromosomal abnormality was the Syndrome of Down 47.37 per cent associated with duodenal atresia; Meconium eliminating present in 20.41 per cent; Of abdominal radiography and intestinal transit elucidated the diagnosis in 100 per cent; intraoperative findings showed concomitant congenital malformations 32.65 per cent; remodeling technique was performed Stranded in jejunoileal atresia, intestinal resection remains the most widely used technique in the proximal and distal stump Nixon: Primary anastomosis was performed in all cases of duodenal...


Subject(s)
Male , Female , Humans , Infant, Newborn , Intestinal Atresia/surgery , Infant, Newborn, Diseases , Observational Study , Retrospective Studies , Cross-Sectional Studies
19.
Chinese Journal of Gastrointestinal Surgery ; (12): 44-47, 2013.
Article in Chinese | WPRIM | ID: wpr-314861

ABSTRACT

<p><b>OBJECTIVE</b>To summarize the experiences and advantages of laparoendoscopic single-site (LESS) surgery for neonatal intestinal atresia and stenosis.</p><p><b>METHODS</b>Twenty patients of neonatal intestinal atresia and stenosis were treated with LESS procedure in Huai'an Women and Children's Hospital of Jiangsu Province between October 2010 and April 2012. The clinical data were retrospectively analyzed.</p><p><b>RESULTS</b>Among these patients, 13 were male, 7 were female. Age at admission ranged from 10 min to 1 d. Four cases were premature, and 3 were born with low birth weight (<2500 g). One was diagnosed with duodenal atresia, 1 with duodenal stenosis, 9 with jejunal atresia, 2 with jejunal stenosis, and 7 with ileal atresia. Laparoscopic exploration was performed in all the cases by transumbilical procedure, the proximal and distal ends were exteriorized from the umbilical port site for anastomosis. Twenty neonates with intestinal atresia and stenosis were performed using this new minimally invasive approach, with no cases converted to open operation or standard laparoscopy. The operative time was 35-60 (mean, 40) min. The intraoperative bleeding was 3-5 ml. Two cases were given up treatment by their parents on the second postoperative day. For the other 18 patients, oral intake started on postoperative day 5-10 (mean, 7), and discharged from hospital on the postoperative day 10-20 (mean, 13). The follow up ranged from 1 to 11 months, during which 1 case died, 3 cases were managed with conservative treatment for diarrhea or malnutrition. The other 14 cases grew up healthily.</p><p><b>CONCLUSION</b>The technique of LESS in the treatment of neonatal atresia and stenosis is simple and the outcomes are satisfactory.</p>


Subject(s)
Female , Humans , Infant, Newborn , Male , Constriction, Pathologic , General Surgery , Intestinal Atresia , General Surgery , Laparoscopy , Methods , Retrospective Studies
20.
JSP-Journal of Surgery Pakistan International. 2013; 18 (4): 182-185
in English | IMEMR | ID: emr-161924

ABSTRACT

To find out causes, modes of presentations and surgical outcome in neonates with duodenal obstruction. Descriptive case series. Department of Paediatric Surgery National Institute of Child Health [NICH] Karachi, from September 2011 to August 2013. All neonates presenting with duodenal obstruction were included. Age at presentation, symptoms and signs, associated anomalies, treatment provided and outcome were recorded. Investigations done included CBC, serum electrolytes, urea / creatinine, x-ray abdomen and echocardiography. Surgical procedures were tailored according to the cause of obstruction. Data was recorded on a performa. Analysis was done using SPSS version 17. A total of 17 patients [males 13, females 4] were managed. Age ranged from 1 day to 20 day [mean=4 day]. Weight of the babies ranged from 1.5 kg to 3.0 kg [mean=2.24 kg]. One patient was delivered via cesarean section. The causes of obstruction were duodenal atresia [n=8], annular pancreas with duodenal atresia [n=4], annular pancreas with duodenal atresia and malrotation [n=3] and only malrotation [n=2]. Double duodenal atresia was present in one patient. Associated imperforate anus was found in five cases, multiple atresias of gut and jejunal atresia in one case each. Three patients had Down syndrome. All patients were operated. Two patients had anastomotic leak. A total of seven patients survived. Those who expired had duodenal atresia [n=5], annular pancreas group [n=4] and malrotation [n=1]. Sepsis was the major contributor to the mortality. Late presentation with low birth weight were the salient observations. Multiple associated gut related surgical anomalies added to operative stress. High mortality [59%] was of concern and sepsis remained the major contributor


Subject(s)
Humans , Male , Female , Infant, Newborn , Intestinal Atresia , Pancreas/abnormalities , Pancreatic Diseases , Intestinal Volvulus
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