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1.
Rev. Hosp. Ital. B. Aires (2004) ; 43(3): 147-149, sept. 2023. ilus
Article in Spanish | LILACS, UNISALUD, BINACIS | ID: biblio-1519047

ABSTRACT

Las malrotaciones por bandas de Ladd son un subtipo de anormalidades de la embriogénesis consistentes en prolongaciones fibrosas, producto de una fijación anómala del mesenterio. Se extienden desde el ciego mal rotado hacia el retroperitoneo, pudiendo producir compresión extrínseca del duodeno. En el 90% de los casos la presentación clínica tiene lugar dentro del primer año de vida como un cuadro agudo, en forma de oclusión duodenal o vólvulo de intestino delgado con la consecuente isquemia de este o hernia interna. En la edad adulta, las formas de presentación son menos específicas. Los métodos de referencia ("gold standard") utilizados para el diagnóstico son la seriada gastroduodenal y la tomografía computarizada. El tratamiento quirúrgico consiste en la cirugía de Ladd, cuyo abordaje convencional fue descripto en 1936 por William Ladd. Presentamos el caso de un paciente adulto con un cuadro oclusivo, causado por dicha anomalía, diagnosticado de forma oportuna y resuelto de manera segura por vía laparoscópica. (AU)


Ladd's band malrotations are a subtype of abnormalities of embryogenesis consisting of fibrous extensions, product of abnormal fixation of the mesentery, that goes from the poorly rotated cecum towards the retroperitoneum, which can cause extrinsic compression of the duodenum. In 90% of cases, the clinical presentation takes place within the first year of life, as an acute condition, like duodenal occlusion or small bowel volvulus with its consequent ischemia or internal hernia. In adulthood, the forms of presentation are less specific. The gold standard methods used for diagnosis are gastroduodenal series and computed tomography. Surgical treatment consists of Ladd's surgery, whose conventional approach was described in 1936 by William Ladd. We present ta case of an adult patient with an occlusive presentation, given by this anomaly, diagnosed in a timely manner and safely resolved by laparoscopic approach. (AU)


Subject(s)
Humans , Male , Adult , Young Adult , Digestive System Surgical Procedures/methods , Intestinal Volvulus/surgery , Digestive System Abnormalities/surgery , Intestinal Obstruction/surgery , Vomiting , Laparoscopy/methods , Intestinal Volvulus/diagnostic imaging , Digestive System Abnormalities/diagnostic imaging , Intestinal Obstruction/diagnostic imaging
2.
Philippine Journal of Surgical Specialties ; : 20-25, 2023.
Article in English | WPRIM | ID: wpr-984298

ABSTRACT

@#Ninety percent of cases of malrotation have shown signs and symptoms of intestinal obstruction by the first year of life. It is thus an often-overlooked etiology in adult patients. Evidence-based recommendations are also limited because of the paucity of cases. This paper discusses the two cases of malrotation from diagnosis to surgical management at a tertiary academic hospital. Both are previously well adult male patients with virgin abdomen who presented with vomiting and signs of intestinal obstruction. During medical decompression, CT scan with triple contrast clinched the diagnosis of malrotation for which Ladd's procedure was done, with no operative complications. The authors' experience and previous literature support early decompression, imaging, and surgery for all cases of malrotation regardless of severity of symptoms.

3.
Gac. méd. espirit ; 24(1): [8], abr. 2022.
Article in Spanish | LILACS | ID: biblio-1404895

ABSTRACT

RESUMEN Fundamento: La malrotación intestinal en una embarazada es infrecuente. La incidencia de la oclusión es baja y los vólvulos son más frecuentes en el tercer trimestre de la gestación. La poca especificidad de los síntomas hace más complejo el diagnóstico temprano y el tratamiento oportuno. La asociación de estas enfermedades se considera potencialmente letal para la madre y el niño. Objetivo: Describir el caso de una paciente con oclusión intestinal por vólvulo de intestino delgado y malrotación en el curso del embarazo. Reporte de caso: Paciente de 23 años, primigesta, con 24.4 semanas de gestación, con dolores abdominales previos; se recibió en la Unidad de Cuidados Intensivos por sospecha de abdomen agudo en el embarazo. Luego de estudios imagenológicos y de laboratorio se decidió su intervención quirúrgica con el diagnóstico de obstrucción intestinal. El diagnóstico intraoperatorio fue vólvulo de intestino delgado y malrotación intestinal tipo I, se le practicó desvolvulación y operación de Ladd. La evolución fue favorable y se conservó la gestación. Conclusiones: La malrotación intestinal complicada con una oclusión en el curso del embarazo es rara y el retraso del diagnóstico puede tener implicaciones desfavorables en la evolución de la madre y su futuro hijo. Las dificultades para el diagnóstico de esta enfermedad es preocupación de la comunidad científica y debe sospecharse en gestantes que presenten síntomas y signos de oclusión intestinal y alteraciones en los estudios imagenológicos.


ABSTRACT Background: Intestinal malrotation in a pregnant woman is uncommon. The incidence of occlusion is low and volvulus are more frequent in the third trimester of pregnancy. The poor symptom specificity makes early diagnosis and treatment more complex prompt. The association of these diseases is considered potentially lethal for mother and child. Objective: To describe the case of a patient with intestinal occlusion due to intestinal volvulus thin and malrotation in the course of pregnancy. Case report: 23-year-old patient, primigravida, with 24.4 weeks of gestation, with previous abdominal pains; She was admitted to the Intensive Care Unit on suspicion of acute abdomen in pregnancy. After imaging and laboratory studies, it was decided her surgical intervention with the diagnosis of intestinal obstruction. The diagnosis intraoperative was small intestine volvulus and type I intestinal malrotation, she underwent devolvulation and operation of Ladd. The evolution was favorable and the pregnancy was preserved. Conclusions: Intestinal malrotation complicated by an occlusion in the course of Pregnancy is rare and delayed diagnosis may have unfavorable implications for the evolution of the mother and her future child. Difficulties in diagnosing this disease is a concern of the scientific community and should be suspected in pregnant women who present symptoms and signs of intestinal obstruction and alterations in imaging studies.


Subject(s)
Pregnancy Complications/surgery , Intestinal Volvulus , Intestinal Obstruction
4.
J. coloproctol. (Rio J., Impr.) ; 41(3): 325-328, July-Sept. 2021. ilus
Article in English | LILACS | ID: biblio-1346427

ABSTRACT

Intestinal malrotation is a congenital anomaly caused by incomplete rotation or absence of rotation of the primitive intestine along the axis of the upper mesenteric artery during embryonic development. Embryonic development and its anatomical variations were described by Dott in 1923. Intestinal malrotation is a rare condition among adults - prevalent in a mere 0.0001% to 0.19% of the population -, and it may be associated with other anatomical deformities. It can be asymptomatic or manifest with varying intensity, from obstruction to necrosis of intestinal segments. In general, this abnormality is diagnosed in the first year of life; however, symptomsmay appear later in life,making diagnosis in adults difficult on account of non-specific symptoms. In the present study, we report a case of intestinal malrotation associated with chronic non-specific symptoms progressing to mesenteric angina. (AU)


Subject(s)
Humans , Female , Aged , Torsion Abnormality/complications , Torsion Abnormality/diagnostic imaging , Mesenteric Artery, Superior , Internal Hernia , Meckel Diverticulum/diagnosis
5.
Article in Spanish | LILACS, UY-BNMED, BNUY | ID: biblio-1248723

ABSTRACT

La malrotación intestinal es un espectro de malformaciones, que incluye una gran variedad de alteraciones en el proceso de rotación y fijación del intestino. La ausencia completa de rotación intestinal, uno de los tipos de malrotación intestinal más frecuentes, puede presentarse como hallazgo asintomático o manifestarse clínicamente por un vólvulo del intestino medio. Sin embargo, incluso entre aquellos que se presentan con vólvulo del intestino medio, la clínica puede ser muy diferente, según el grado de isquemia y eventual necrosis intestinal. Se presentan dos casos clínicos de malrotación intestinal con vólvulo del intestino medio con presentaciones, imágenes, tratamientos y evoluciones muy disímiles. Se analizan los mismos a la luz de una revisión bibliográfica relevante al tema tratado, se sacan aprendizajes del manejo realizado y la evolución que presentaron, y se enfatizan los elementos de mayor jerarquía para optimizar el manejo de estos pacientes.


Intestinal malrotation is a spectrum of malformations that includes a great variety of alterations in the rotation and fixation process of the intestines. The total absence of intestinal rotation, one of the most frequents types of intestinal malrotation could present as an asymptomatic find or appear clinically as a midgut volvulus. However, even those that appear as midgut volvulus, could show quite different signs and symptoms according to the degree of ischemic insult and possible intestinal necrosis. Two clinical cases of intestinal malrotation with midgut volvulus with quite different presentations, images, treatments, and evolutions, are informed. The two cases are analyzed under a relevant bibliographic revision, knowledge is derived from the carried-out management and evolution, and elements for the future optimization of management are underlined.


A má rotação intestinal é um espectro de malformações, que inclui uma grande variedade de alterações no processo de rotação e fixação do intestino. A ausência completa de rotação intestinal, um dos tipos mais comuns de má rotação intestinal, pode se apresentar como um achado assintomático ou manifestar-se clinicamente como um volvo de intestino médio. Porém, mesmo entre aqueles que apresentam volvo de intestino médio, os sintomas podem ser muito diferentes, dependendo do grau de isquemia e eventual necrose intestinal. Dois casos clínicos de má rotação intestinal com volvo de intestino médio são apresentados com apresentações, imagens, tratamentos e evoluções muito diferentes. São analisados ​​à luz de uma revisão bibliográfica pertinente ao tema em questão, lições aprendidas com o manejo realizado e a evolução que apresentaram, e os elementos de maior hierarquia são enfatizados para otimizar o manejo desses pacientes.


Subject(s)
Humans , Male , Female , Infant, Newborn , Infant , Intestinal Volvulus/surgery , Intestinal Volvulus/diagnostic imaging , Treatment Outcome , Intestines/abnormalities
6.
Article | IMSEAR | ID: sea-213266

ABSTRACT

Intestinal malrotation, a congenital anomaly of the midgut, typically presents in pediatrics age group with the most feared complication of midgut volvulus and its catastrophic sequelae. Midgut volvulus secondary to intestinal malrotation is a rare presentation in adults more so in the elderly. Its rarity portends a diagnostic dilemma for both the surgeon and radiologist. We report a 65-year-old man admitted following a referral with a 12-hour history of acute abdomen with an initial diagnosis of acute pancreatitis but later had laparotomy with intraoperative findings of a midgut volvulus and gangrenous ileal segment secondary to intestinal malrotation. This case emphasizes the importance of early diagnosis to prevent the disastrous complication of this disease. The rarity of this condition portends a formidable diagnostic challenge in adults hence the awareness of its possibility and a high index of suspicion is crucial in diagnosis.

7.
Rev. Fac. Med. Hum ; 20(4): 720-726, Oct-Dic. 2020.
Article in English, Spanish | LILACS-Express | LILACS | ID: biblio-1141325

ABSTRACT

La malrotación intestinal es una anormalidad congénita de la rotación embriológica del intestino, se estima que más del 90% de pacientes con malrotación intestinal se presentará en los primeros 12 meses de vida. La presentación clínica en adultos se manifiesta de forma progresiva que ocurre generalmente durante el periodo postprandial, presentándose: vómitos intermitentes, dolor abdominal, pérdida de peso, diarrea crónica, peritonitis, intolerancia alimentaria, entre otros. El tratamiento dependerá de la presentación, ya sea aguda o crónica, requiriendo laparotomía de emergencia para determinar la causa. Se presenta un caso de abdomen agudo quirúrgico por apendicitis aguda en una paciente mujer de 27 años con malrotación intestinal, con presentación clínica inicial de dolor abdominal intenso, diarrea, puño percusión lumbar positiva, que finalmente llegó a una resolución adecuada y un buen pronóstico.


Intestinal malrotation is a congenital abnormality of the embryological rotation of the intestine, it is estimated that more than 90% of patients with intestinal malrotation will be presented in the first 12 months of life. The clinical presentation in adults is manifested in a progressive way that usually occurs during the postprandial period, presenting: intermittent vomiting, abdominal pain, weight loss, chronic diarrhea, peritonitis, food intolerance, among Another. Treatment will depend on presentation, either acute or chronic, requiring emergency laparotomy to determine the cause. There is a case of acute surgical abdomen for acute appendicitis in a 27-year-old patient with intestinal malrotation, with initial clinical presentation of severe abdominal pain, diarrhea, positive lumbar percussion fist, which finally reached a Proper resolution and a good prognosis.

8.
Rev. argent. cir ; 112(3): 325-328, jun. 2020. graf
Article in Spanish | LILACS | ID: biblio-1279746

ABSTRACT

RESUMEN La recurrencia de las hernias diafragmáticas congénitas, luego de reparadas quirúrgicamente, es de baja frecuencia. Se presenta el caso de un paciente masculino de 22 años con hernia diafragmática congénita reparada inicialmente al segundo día de vida, el cual presenta disnea grado 1 y enfermedad por reflujo gastroesofágico. Se evidencia por medio de imagen una hernia diafragmática izquierda, el saco herniario con contenido de bazo, colon, cola de páncreas y glándula suprarrenal izquierda. Se aborda de manera abdominal mediante incisión subcostal izquierda, se reduce el saco herniario, se coloca malla tipo en el defecto diafragmático y se realiza procedimiento de Ladd por la presencia de malrotación intestinal.


ABSTRACT Recurrence of congenital diaphragmatic hernia, after surgical repair, is infrequent. Here, we report the case of a 22-year-old male patient with a history of congenital diaphragmatic disease initally treated with surgery on the second day of life who presented class I dyspnea and gastroesophageal reflux. A left diaphragmatic hernia with a hernia sac containing the spleen, colon, tail of pancreas and left adrenal gland. The patient underwent surgical repair via a left subcostal incision; the hernia sac was reduced, the diaphragmatic defect was repaired with a mesh and the Ladd procedure was performed due to the diagnosis of intestinal malrotation.


Subject(s)
Humans , Male , Adult , Young Adult , Hernias, Diaphragmatic, Congenital/surgery , Radiography, Thoracic , Tomography, X-Ray Computed , Plastic Surgery Procedures , Hernias, Diaphragmatic, Congenital/diagnosis , Laparotomy
9.
The Malaysian Journal of Pathology ; : 477-481, 2020.
Article in English | WPRIM | ID: wpr-876044

ABSTRACT

@#Gastrointestinal pathology leading to the death in paediatric age group is uncommon. The diseases that encountered were mostly intestinal obstruction, peritonitis and gastrointestinal bleeding. Due to the severe symptoms, most of the patients presented to hospital in time and were treated appropriately. However, with the presence of contributing factors, certain gastrointestinal pathology can progress rapidly leading to the death. We report a rare case of intestinal volvulus in a 3 years old girl where the deceased presented with one day short history of vomiting before her demise. The contributing factors were bronchopneumonia sepsis and underlying intestinal malrotation identified via post-mortem examination.

10.
Article | IMSEAR | ID: sea-211851

ABSTRACT

Midgut malrotation is an incomplete rotation of the intestine which occurs during foetal development and usually presents in the neonatal period. The bowel is not fixed adequately and is thus held by a precariously narrow-based mesentery. The incidence of malrotation has been estimated at 1 in 6000 live births, it is rare for malrotation to present in adulthood. Author report a 40-year-old man presented to emergency department with generalized abdominal pain. His symptoms began 2 days before admission. The patient really weak and his mental state was somnolence. His abdomen was slightly extended, and intestinal peristalsis was obscure. Abdominal X-rays revealed dilatation of gastric, coiled spring appearance without free air and step ladder pattern. The patient then underwent laparotomy exploration. Upon entering the abdomen, ileum was noted to completely mobilized and founded volvulus segment, a clockwise twisting three times, about 30 cm proximal from ICJ, and soon author released it. The ligament of Treitz misplaced, there was in the right lower quadrant, close by ICJ. Then about 110 cm segment of ileum necrotic, the rest of the small bowel was normal, author found internal hernia in the right lower quadrant and there was Ladd's band. Clinicians should be aware of this serious cause of abdominal pain. The diagnosis of malrotation in adulthood is often delayed. Complete resolution of acute obstruction or chronic abdominal pain is the result of a high index of suspicion for malrotation, appropriate diagnostic studies, and aggressive treatment.

11.
Rev. colomb. cir ; 34(3): 287-291, 20190813. fig
Article in Spanish | COLNAL, LILACS | ID: biblio-1016116

ABSTRACT

La malrotación intestinal es una malformación congénita que afecta hasta al 1 % de la población. Aproximada-mente, el 90 % de los casos se presenta en la edad pediátrica y, rara vez, en la población adulta, lo que convierte a esta alteración en un reto para los profesionales sanitarios. Se presenta el caso de una paciente que se inició con un cuadro de obstrucción intestinal y abdomen agudo; se le diagnosticó malrotación intestinal, vólvulo y obstrucción por bridas, durante la laparotomía exploradora ur-gente. El conocimiento de condición patológica es imprescindible para poder brindarle un correcto tratamiento quirúrgico


The intestinal malrotation is a congenital malformation that affects up to 1% of the population. Approximately 90% of cases are diagnosed in the pediatric age and rarely in the adult population, which makes this pathology a challenge for health professionals.This is a case of a patient who presented with intestinal obstruction and acute abdomen that was diagnosed during the emergency exploratory laparotomy with intestinal malrotation, volvulus and obstruction. The knowledge of this pathology is essential to perform its correct surgical treatment


Subject(s)
Humans , Intestinal Volvulus , Congenital Abnormalities , Abdomen, Acute , Intestinal Obstruction
12.
Pediatric Gastroenterology, Hepatology & Nutrition ; : 447-452, 2019.
Article in English | WPRIM | ID: wpr-760872

ABSTRACT

PURPOSE: Few studies have reported non-acute long-term morbidity rates in children with intestinal malrotation. The aim of this study was to investigate the rate of constipation in children undergoing Ladd's procedure for isolated intestinal malrotation. METHODS: This retrospective study included children aged <15 years who underwent Ladd’s procedure for intestinal malrotation between 2001 and 2016. Demographics, presence of volvulus perioperatively, need for bowel resection, short term (<30 days) and long-term complications, including mortality were recorded. Constipation was defined as treatment with laxatives at 1-year follow-up. RESULTS: Of the 43 children included in the study, 49% were boys. The median age at surgery was 28 days (0–5, 293 days). Volvulus occurred in 26 children (60.5%), and bowel resection was required in 4 children (9.3%). Short-term complications categorized as grades II–V according to the Clavien-Dindo classification occurred in 13 children (30.2%). Of these, 5 children (11.6%) required re-operation. Constipation was observed in 9 children (23.7%) at the 1-year follow-up. No difference was observed in the rate of perioperative volvulus between children with and without constipation (44% vs. 65%, p=0.45). Excluding re-operations performed within 30 days after surgery, 3 children (6%) underwent surgery for intestinal obstruction during the study period. CONCLUSION: Many children undergoing Ladd's procedure require bowel management even at long-term follow-up, probably secondary to constipation. It is important to thoroughly evaluate bowel function at the time of follow-up to verify or exclude constipation, and if treatment of constipation is unsuccessful, these children require evaluation for dysmotility disorders and/or intestinal neuronal dysplasia.


Subject(s)
Child , Humans , Classification , Constipation , Demography , Follow-Up Studies , Intestinal Obstruction , Intestinal Volvulus , Laxatives , Mortality , Neurons , Retrospective Studies
13.
Rev. chil. cir ; 70(6): 551-556, dic. 2018. tab, graf, ilus
Article in Spanish | LILACS | ID: biblio-978029

ABSTRACT

Introducción: Las pruebas de diagnóstico por imagen se han convertido en una pieza crucial en el diagnóstico del abdomen agudo en la urgencia, sin embargo, la conducta médica deberá basarse en los hallazgos iconográficos contextualizados. El signo tomográfico del remolino constituye un paradigma por su asociación con el vólvulo intestinal. Objetivos: Registrar pacientes con diagnóstico de abdomen agudo en busca del "signo del remolino" en la tomografía computada e identificar quiénes requirieron intervención quirúrgica. Métodos: Estudio analítico de corte transversal evaluándose 115 tomografías computadas de abdomen agudo obstructivo en búsqueda del signo del remolino. Resultados: 15 tomografías fueron excluidas por tratarse de estudios solicitados en posoperatorios. El signo del remolino se presentó en el 5%. Los diagnósticos fueron de mal rotación intestinal (2%), vólvulo de intestino delgado (1%), vólvulo de sigma (1%) y enterocolitis (1%). Sólo el 60% de los pacientes requirieron cirugía. Discusión: La presencia del signo del remolino no siempre obedece a una resolución quirúrgica. No es patognomónico de una sola entidad puntual, pudiendo representar un hallazgo en el contexto de otra patología aguda, por lo que se debería reconocer sus variantes para instaurar el tratamiento adecuado, sea quirúrgico o médico.


Introduction: The imaging test have become an important piece on the diagnosis of the acute abdomen in emergency, nevertheless medical conduct should be based on contextualized iconographic findings. The tomographic "whirlpool sign" establishes a surgical paradigm for its association with the bowel volvulus. Objetive: To register patients with diagnosis of acute abdomen looking for the "whirlpool sign" in the computed tomography and identify who needed surgical intervention. Methods: Analytical cross-sectional study. 115 tomographies of acute obstructive abdomen have being evaluated. Results: 15 tomographies were excluded. The prevalence of the whirlpool sign was 5%. Diagnoses were intestinal malrotation (2%), midgut volvulus (1%), sigmoid volvulus (1%) and enterocolitis (1%). Only 60% of the patients required surgery. Discussion: The presence of the "whirlpool sign" not always determines a surgical resolution. It is not a pathognomonic sign of a punctual entity; it could represent an ordinary finding in the context of several pathologies. To apply the correct treatment, surgical or medical, for these patients it was important to recognize "whirlpool sign" variants.


Subject(s)
Humans , Male , Female , Adult , Middle Aged , Aged , Aged, 80 and over , Tomography, X-Ray Computed , Intestinal Volvulus/diagnostic imaging , Abdomen, Acute/diagnostic imaging , Signs and Symptoms , Cross-Sectional Studies , Intestinal Volvulus/surgery
14.
Rev. Asoc. Méd. Argent ; 131(2): 27-30, jun. 2018. ilus, tab
Article in Spanish | LILACS | ID: biblio-973099

ABSTRACT

Existen múltiples patologías del abdomen que pueden ser diagnosticadas por parte del especialista en Diagnóstico por Imágenes; entre ellas, la malrotación intestinal es un hallazgo generalmente incidental. El médico imagenólogo debe ser capaz de reconocer los signos de la malrotación intestinal, al tratarse de una entidad patológica con complicaciones graves, como el vólvulo de intestinal. Para el diagnóstico, es clave su sospecha, así como las variantes de la normalidad que pueden conducir a un diagnóstico erróneo.


There are many diseases of the abdomen that can be diagnosed by the specialist in diagnostic imaging, including the intestinal malrotation is a finding usually incidental. The doctor specialist imaging must be able to recognize the signs of intestinal malrotation is a pathological entity, with serious complications, such as intestinal volvulus. For the diagnosis, it is the key to your suspicion, as well as the variants that can lead to a false diagnosis.


Subject(s)
Female , Humans , Adult , Intestine, Small/abnormalities , Intestine, Small/diagnostic imaging , Intestinal Volvulus/complications , Multidetector Computed Tomography , Diagnostic Imaging
15.
Int. j. morphol ; 34(2): 665-670, June 2016. ilus
Article in Spanish | LILACS | ID: lil-787052

ABSTRACT

La ausencia congénita de la vena porta hepática es una malformación en extremo rara, fue descrita por primera vez en 1793 por John Abernethy y a la fecha se han reportado sólo 101 casos. Afecta con mayor frecuencia a mujeres y determina que el drenaje venoso intestinal sea derivado hacia el territorio de las venas sistémicas. Es también conocida como derivación porto-sistémica extra hepática congénita (CEPS), por su sigla en inglés, y se asocia a otras alteraciones congénitas,incluyendo anomalías cardíacas, de las vías biliares, enfermedades metabólicas y retardo mental. En este trabajo presentamos el hallazgo de esta malformación en el cadáver de un niño de dos años de edad, donde la vena porta seguía un trayecto anómalo y se unía a la vena renal derecha. El confluente venoso "mesentérico-esplénico-renal" así formado presentaba un trayecto descendente, recibía a la vena gonadal derecha, y desembocando en la confluencia de las venas ilíacas comunes. Esto se asociaba a mal rotación intestinal, arteria hepática aberrante y a vena cava inferior izquierda, situación descrita sólo una vez en la literatura. El hallazgo de estas anomalías anatómicas en los cadáveres disecados con fines docentes en nuestro Departamento de Anatomía, tiene un valor formativo indiscutible para nuestros alumnos de pre y postgrado, quienes pueden valorar las implicancias de este conocimiento anatómico en la clínica diaria.


Congenital absence of the hepatic portal vein is an extremely rare malformation that was first described by John Abernethy in 1793. Only 101 cases had been described in the literature until 2015 and most affected females. In this anomaly, also known as congenital extrahepatic porto-systemic shunt (CEPS), intestinal venous drainage is derived towards the territory of the systemic veins and could be associated with other birth defects, including heart and biliary tract anomalies, metabolic diseases, mental retardation. We present the case of a 2-year-old boy who died because of multifocal pneumonia and necropsy showed anatomical findings consistent with this malformation as an incidental finding. The portal vein followed an anomalous course and joined the right renal vein, forming the "mesenteric-splenic-renal" venous collector, which then received the right gonadal vein and ended at the confluence of the common iliac veins. In our case this anomaly was associated to intestinal malrotation, aberrant hepatic artery and persistent left inferior vena cava, situation described once in the literature. The finding of these anatomical abnormalities in cadavers has a great teaching value for our undergraduate and graduate students who are learning anatomy and they can also assess the associated clinical.


Subject(s)
Humans , Male , Child, Preschool , Intestines/abnormalities , Portal Vein/abnormalities , Vena Cava, Inferior/abnormalities , Cadaver
16.
Chinese Journal of Applied Clinical Pediatrics ; (24): 124-126, 2016.
Article in Chinese | WPRIM | ID: wpr-491522

ABSTRACT

Objective To screen the neonatal malrotation with PITX2 gene exon 2 and 5 gene mutation through the study on molecular genetics.Methods From January 201 2 to December 201 4,1 5 cases of neonatal malro-tation infants(experimental group)and 25 healthy newborn infants(healthy control group)were selected as the research subjects from the First Affiliated Hospital of Shihezi University Medical College.The experimental group included 1 5 ca-ses of volvulus,4 cases of volvulus with duodenal atresia and 3 cases of volvulus with jejunal atresia.The clinical fea-tures were recorded and 3 mL peripheral venous blood from each subject was collected.After ethylenediamine tetraacetic acid (EDTA)anticoagulation,genomic DNA was extracted.Polymerase chain reaction (PCR)was used to amplify the exon 2 and exon 5 of PITX2 gene,and the direct sequencing method was used to screen whether there were mutations in these 2 loci.Results According to the findings of the matching gene,PITX2 gene exon 2 and exon 5 mutations were not detected in 15 cases with intestinal malrotation of the experimental group and 25 healthy newborns in the healthy control group.Conclusions Polymorphisms is not detected in PITX2 gene exon 2 and exon 5 in small groups of newborn,but this does not exclude the possibility the gene caused newborns suffering from intestinal malrotation by other means.

17.
Chinese Journal of Digestive Surgery ; (12): 848-851, 2015.
Article in Chinese | WPRIM | ID: wpr-480787

ABSTRACT

Objective To explore the feasibility and effect of laparoscopic Ladd's surgery for the treatment of intestinal malrotation in infants.Methods The clinical data of 50 infants with intestinal malrotation who were admitted to the Wuhan Medical & Health Center for Women and Children from January 2011 to December 2013 were retrospectively analyzed.Of 50 infants, 27 infants receiving the open Ladd's surgery were allocated into the open surgery group and 23 infants receiving the laparoscopic Ladd's surgery were allocated into the laparoscopy group.The operation time, time to anal exsufflation, duration of hospital stay, postoperative incision infection and intestinal obstruction in the 2 groups were observed.All the patients were followed up by outpatient examination or telephone interview till March 2014.The measurement data with normal distribution were presented as x-± s and analyzed by the t test, and count data were analyzed using the chi-square test or Fisher exact probability.Results The operation time, time to anal exsufflation and duration of hospital stay were (69 ± 7)minutes, (41 ±9)hours and (10.4 ± 2.4)days in the open surgery group, which was significantly different from (92 ± 13)minutes, (28 ±5)hours and (6.4 ± 1.5) days in the laparoscopy group (t =6.21, 16.50, 6.34,P < 0.05).Two infants had incision infection and 1 infant had intestinal obstruction in the open surgery group.There was no patient with complications in the laparoscopy group.All the patients were followed up for the median time of 13 months (range, 6-24 months), with a good survival and no other symptoms.Conclusion Laparoscopic Ladd's surgery is safe and feasible compared with open surgery, and it could be used as a prior operation method for treatment of intestinal malrotation in infants.

18.
Annals of Coloproctology ; : 110-113, 2015.
Article in English | WPRIM | ID: wpr-23355

ABSTRACT

A 44-year-old man had been suffering from nausea, vomiting and watery diarrhea for 5 days and was then admitted to Dankook University Hospital. He had suffered from several episodes of mild symptoms, including abdominal distension, loss of appetite, easy satiety, nausea, vomiting, and diarrhea throughout his lifetime, but most episodes had been ignored by him or physicians. An upper gastrointestinal series and a computed tomography scan revealed an intestinal malrotation with a volvulus. In order to untwist the small bowel in a counterclockwise direction to about 180 degrees, we had to perform not only a dissection of Ladd's band, but also a dissection of other adhesions between the mesocolon and the mesenteric vessel trunk. Surgical intervention needs to be performed for an old intestinal malrotation with any symptoms because the structural or morphological change proceeds as time passes, which is caused by fibrosis due to tension being repetitively applied to Ladd's band, leading to its contraction. Furthermore, a severe contraction may even lead to a fixed partial volvulus.


Subject(s)
Adult , Humans , Appetite , Diarrhea , Fibrosis , Intestinal Volvulus , Mesocolon , Nausea , Vomiting
19.
Chinese Journal of Ultrasonography ; (12): 243-246, 2014.
Article in Chinese | WPRIM | ID: wpr-671756

ABSTRACT

Objective To evaluate the value of ultrasound in the diagnosis of congenital intestinal malrotation and midgut vovulus.Methods The findings of ultrasound of 161 children with clinically suspected intestinal malrotation were analysed retrospectively,and were compared with the results of the operation and/or the upper gastrointestinal contrast study.Results The positive sign of ultrasound for congenital intestinal malrotation was that the superior mesenteric vein(SMV) being located at the left of the superior mesentery artery(SMA),or being directly anterior to the SMA,and the positive sign for midgut vovulus was clockwise whirlpool sign (clockwise rotation of the small bowel,its mesentery and the SMV around the SMA).The sensitivity of ultrasound for congenital intestinal malrotation was 82%,and the specificity was 94%.Those for midgut vovulus were 94% and 94% respectively.Conclusions Special sign as the superior mesenteric vein(SMV) being located at the left of the superior mesentery artery(SMA) strongly indicate congenital intestinal malrotatione,and the clockwise whirlpool sign means great possibility of midgut vovulus.

20.
Clinical Endoscopy ; : 201-204, 2014.
Article in English | WPRIM | ID: wpr-8103

ABSTRACT

Most infants with repaired gastroschisis develop normally and remain in good health. About 10% of patients with gastroschisis have other malformations. We report a case of choledocholithiasis and intestinal malrotation in an adolescent with repaired gastroschisis. A 17-year-old girl presented with fever, jaundice, and abdominal pain. She had undergone an operation to repair gastroschisis at birth. Physical examination revealed icteric sclera, a tight abdominal wall, and a longitudinal surgical scar at the midline. An abdominal computed tomography scan revealed a round calcifying lesion near the pancreas and a midline-positioned liver and gallbladder. Absence of the retroperitoneal duodenum and the anterior and left-sided position of the superior mesenteric vein compared with the superior mesenteric artery were observed. Results of abarium examination revealed intestinal malrotation. Endoscopic retrograde cholangiopancreatography revealed diffuse dilatation of the biliary trees and a malpositioned gallbladder. A single stone was removed by using a basket. The clinical symptoms improved after the patient underwent endoscopic retrograde cholangiopancreatography.


Subject(s)
Adolescent , Female , Humans , Infant , Abdominal Pain , Abdominal Wall , Cholangiopancreatography, Endoscopic Retrograde , Choledocholithiasis , Cicatrix , Dilatation , Duodenum , Fever , Gallbladder , Gastroschisis , Jaundice , Liver , Mesenteric Artery, Superior , Mesenteric Veins , Pancreas , Parturition , Physical Examination , Sclera
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