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Malrotation leading to massive chylous ascites is rare. A 9-year-old girl was investigated for slowly increasing abdominal distension under a year. She had no vomiting, weight loss, or pain, but was bothered in social situations. Medical investigations, including ultrasound and computed tomography scans, revealed massive ascites. Laparocentesis yielded milk-colored fluid, confirmed as lymph through laboratory analysis. A complete blood count, liver function and hematologic parameters, chyle cytology, bacterial cultures, and polymerase chain reaction for tuberculosis were all within normal limits.She was referred to a tertiary center for vascular anomalies. A dynamic contrast-enhanced magnetic resonance lymphangiography showed normal lymphatic anatomy without leakage or flow obstruction. A whole-body magnetic resonance imaging revealed a central mesenteric rotation.She was referred to a tertiary center for pediatric surgery, where a laparoscopic Ladd's procedure was performed using a new 5 mm pediatric sealing device, along with an appendectomy using a 5 mm stapler. To derotate the bowel, fenestrations were created in compartments containing a substantial amount of chyle and ascites, resulting in the drainage of 2.4 L of fluid. She was discharged the day after surgery and has been in good health for 1 year. We present a video illustrating the Ladd's procedure steps in this patient.

Intestinal malrotation is a congenital disorder affecting midgut fixation and causing intestinal malfunction. Severe clinical manifestations are mainly detected in childhood. In adult-life, diagnosis is usually incidental or associated with nonspecific abdominal pain attacks progressing to volvulation and ischemia on intestinal segments supplied by superior mesenteric artery, threatening life´s patients. We present our experience in two patients with a confirmed diagnosis of intestinal malrotation in order to increase knowledge concerning this pathology, poorly defined in asymptomatic patients. A review of the most relevant clinical aspects has been carried out based on its embryological nature and current therapeutic trends. Importance of including this condition in the differential diagnosis of recurrent abdominal pain is highlighted when it is associated with intestinal transit disorders, and the need for new treatment strategies that reduce risk of recurrence and provide quality of life., La malrotación intestinal es un proceso congénito que afecta a la fijación final del tracto intestinal generando malfunción intestinal. Sus manifestaciones clínicas graves son más frecuentes en la edad pediátrica. Su diagnóstico en el adulto suele ser casual o asociado a episodios no específicos de dolor abdominal que podrían evolucionar a volvulación y fenómenos isquémicos sobre segmentos intestinales dependientes de la arteria mesentérica superior, llegando a comprometer la vida del paciente. Exponemos nuestra experiencia en dos pacientes con diagnóstico confirmado de malrotación intestinal a fin de incrementar el conocimiento en esta patología, poco definida en pacientes asintomáticos. Se ha realizado una revisión de los aspectos clínicos más relevantes a partir de su naturaleza embriológica y las tendencias terapéuticas actuales. Enfatizamos la importancia de incluir este cuadro en el diagnóstico diferencial del dolor abdominal recurrente acompañado de alteraciones del tránsito intestinal, y la necesidad de nuevas estrategias de tratamiento que reduzcan el riesgo de recidiva y aporten calidad de vida.

Introduction: Adult intestinal malrotation with midgut volvulus is rare and most often diagnosed on abdominal imaging. Once the diagnosis is made, prompt surgical intervention is necessary. A finding suggestive of malrotation with midgut volvulus on abdominal imaging is the “whirlpool” sign where the superior mesenteric vein and superior mesenteric artery twist at the root of the abdominal mesentery. This sign was once thought to be pathognomonic, but recent studies have shown that it can be seen in asymptomatic patients.Case Report: A 20-year-old female presented to our emergency department with diffuse abdominal pain. Computed tomography demonstrated the “whirlpool” sign with a concern for malrotation with midgut volvulus. Surgical consultation was obtained and the patient was rushed to the operating room for an exploratory laparotomy. Normal mesenteric attachments were seen and no significant pathology was identified during the laparotomy. The patient was eventually diagnosed with gastritis and discharged in stable condition.Conclusion: Emergency physicians and surgeons alike should be cautious in confirming malrotation with midgut volvulus solely due to the “whirlpool” sign on abdominal imaging. Premature diagnostic closure can lead to unnecessary procedures and interventions for patients as in the case we report here.

Introduction: Adult intestinal malrotation with midgut volvulus is rare and most often diagnosed on abdominal imaging. Once the diagnosis is made, prompt surgical intervention is necessary. A finding suggestive of malrotation with midgut volvulus on abdominal imaging is the “whirlpool” sign where the superior mesenteric vein and superior mesenteric artery twist at the root of the abdominal mesentery. This sign was once thought to be pathognomonic, but recent studies have shown that it can be seen in asymptomatic patients.Case Report: A 20-year-old female presented to our emergency department with diffuse abdominal pain. Computed tomography demonstrated the “whirlpool” sign with a concern for malrotation with midgut volvulus. Surgical consultation was obtained and the patient was rushed to the operating room for an exploratory laparotomy. Normal mesenteric attachments were seen and no significant pathology was identified during the laparotomy. The patient was eventually diagnosed with gastritis and discharged in stable condition.Conclusion: Emergency physicians and surgeons alike should be cautious in confirming malrotation with midgut volvulus solely due to the “whirlpool” sign on abdominal imaging. Premature diagnostic closure can lead to unnecessary procedures and interventions for patients as in the case we report here.

The dilemma of acute non-traumatic abdominal pathologies in the pediatric population depends on the age of the patients and symptoms. Surgical etiologies in patients younger than 2 years of age include intussusception, pyloric stenosis, malrotation and midgut volvulus. In older patients, considerations become closer to differential etiologies in adults including acute appendicitis. Ultrasound and fluoroscopic examinations remain the mainstay for diagnosis that may even be therapeutic such as contrast/air enemas in intussusception reduction. There is an increasing role for CT in appendicitis and renal colic especially in assessing complications; however, it is less favored because it incurs radiation to the patient. This article summarizes the imaging approach to pediatric patients with acute abdominal pathologies presenting to the emergency department, and how imaging is instrumental in guiding diagnosis and treatment, with emphasis on radiation safety, in the context of providing typical imaging findings of each pathology in this pictorial review.

We report a novel patient with the phenotypic characteristics of Schaaf-Yang syndrome. In addition, the patient has a severe chronic digestive malfunction, rendering him dependent on intermittent enteral supplementation. To our knowledge, this is the first report of Schaaf-Yang syndrome associated with severe chronic digestive malfunction manifesting with both a malrotation and signs of a chronic intestinal pseudo-obstruction.

Congenital intestinal malrotation is mainly detected in childhood and caused by incomplete rotation and fixation of the intestines providing the prerequisites for life-threatening volvulus of the midgut. The objective of this study was to evaluate a large cohort of adult patients with intestinal malrotation. Thirty-nine patients, 15-67 years, were diagnosed and admitted to a university setting with congenital intestinal malrotation 2002-2013. The patients were divided into three age groups for stratified evaluation. Medical charts were scrutinized, and clinical outcome of surgery was reviewed. Twelve patients presented as emergency cases, whereas 27 were admitted as elective cases. Diagnosis was established in 33 patients who underwent radiological investigation and in the remaining 6 during surgery. A Ladd's operation was performed in 31 symptomatic patients; a conservative strategy was chosen in eight cases. Volvulus was more common in the younger age group. Twenty-six surgically treated patients were available for telephone interview, 1-12 years after surgery. All patients, except one, regarded their general condition improved to a high degree (n = 18) or with some reservation (n = 7). Twelve patients suffered remaining abdominal pain of a chronic and diffuse character. Due to recurrence of malrotation six patients were reoperated. Symptomatic malrotation occurs in both children and the adult population. Improved awareness and an accurately performed CT scan can reveal the malformation and enable surgical treatment. A Ladd's procedure relieved most patients from their severe complaints even when a history of several years of suffering existed.

Congenital intestinal malrotation is mainly detected in childhood and caused by incomplete rotation and fixation of the intestines providing the prerequisites for life-threatening volvulus of the midgut. The objective of this study was to evaluate a large cohort of adult patients with intestinal malrotation. Thirty-nine patients, 15-67 years, were diagnosed and admitted to a university setting with congenital intestinal malrotation 2002-2013. The patients were divided into three age groups for stratified evaluation. Medical charts were scrutinized, and clinical outcome of surgery was reviewed. Twelve patients presented as emergency cases, whereas 27 were admitted as elective cases. Diagnosis was established in 33 patients who underwent radiological investigation and in the remaining 6 during surgery. A Ladd's operation was performed in 31 symptomatic patients; a conservative strategy was chosen in eight cases. Volvulus was more common in the younger age group. Twenty-six surgically treated patients were available for telephone interview, 1-12 years after surgery. All patients, except one, regarded their general condition improved to a high degree (n = 18) or with some reservation (n = 7). Twelve patients suffered remaining abdominal pain of a chronic and diffuse character. Due to recurrence of malrotation six patients were reoperated. Symptomatic malrotation occurs in both children and the adult population. Improved awareness and an accurately performed CT scan can reveal the malformation and enable surgical treatment. A Ladd's procedure relieved most patients from their severe complaints even when a history of several years of suffering existed.

Congenital intestinal malrotation is mainly detected in childhood and caused by incomplete rotation and fixation of the intestines providing the prerequisites for life-threatening volvulus of the midgut. The objective of this study was to evaluate a large cohort of adult patients with intestinal malrotation. Thirty-nine patients, 15-67 years, were diagnosed and admitted to a university setting with congenital intestinal malrotation 2002-2013. The patients were divided into three age groups for stratified evaluation. Medical charts were scrutinized, and clinical outcome of surgery was reviewed. Twelve patients presented as emergency cases, whereas 27 were admitted as elective cases. Diagnosis was established in 33 patients who underwent radiological investigation and in the remaining 6 during surgery. A Ladd's operation was performed in 31 symptomatic patients; a conservative strategy was chosen in eight cases. Volvulus was more common in the younger age group. Twenty-six surgically treated patients were available for telephone interview, 1-12 years after surgery. All patients, except one, regarded their general condition improved to a high degree (n = 18) or with some reservation (n = 7). Twelve patients suffered remaining abdominal pain of a chronic and diffuse character. Due to recurrence of malrotation six patients were reoperated. Symptomatic malrotation occurs in both children and the adult population. Improved awareness and an accurately performed CT scan can reveal the malformation and enable surgical treatment. A Ladd's procedure relieved most patients from their severe complaints even when a history of several years of suffering existed.

Congenital intestinal malrotation is mainly detected in childhood and caused by incomplete rotation and fixation of the intestines providing the prerequisites for life-threatening volvulus of the midgut. The objective of this study was to evaluate a large cohort of adult patients with intestinal malrotation. Thirty-nine patients, 15-67 years, were diagnosed and admitted to a university setting with congenital intestinal malrotation 2002-2013. The patients were divided into three age groups for stratified evaluation. Medical charts were scrutinized, and clinical outcome of surgery was reviewed. Twelve patients presented as emergency cases, whereas 27 were admitted as elective cases. Diagnosis was established in 33 patients who underwent radiological investigation and in the remaining 6 during surgery. A Ladd's operation was performed in 31 symptomatic patients; a conservative strategy was chosen in eight cases. Volvulus was more common in the younger age group. Twenty-six surgically treated patients were available for telephone interview, 1-12 years after surgery. All patients, except one, regarded their general condition improved to a high degree (n = 18) or with some reservation (n = 7). Twelve patients suffered remaining abdominal pain of a chronic and diffuse character. Due to recurrence of malrotation six patients were reoperated. Symptomatic malrotation occurs in both children and the adult population. Improved awareness and an accurately performed CT scan can reveal the malformation and enable surgical treatment. A Ladd's procedure relieved most patients from their severe complaints even when a history of several years of suffering existed.

Gastric perforation in neonate has always been a catastrophe associated with high morbidity. The high mortality rate reflects to a certain extent the precariousness of the neonatal period, but the process evolves so rapidly that prompt diagnosis is necessary if improved survival rates are to be achieved. It is a mysterious entity regarding its cause, but three mechanisms; spontaneous perforation, trauma and ischemia are acceptable reasons for neonatal gastric perforation. Nonetheless, we present you here a rare case of malrotation with mid gut volvulus which presented with apparently spontaneous gastric perforation (1x1cm). Primary repair of the perforation with derotation of the volvulus was done. We question the spontaneity of neonatal gastric perforation and urge the need to look for the contributing cause.

Duodenal atresia is a common cause of neonatal obstruction. It is frequently associated with other malformations such as Down syndrome, cardiac anomalies, malrotation or annular pancreas. Double duodenal atresia is an exceptional malformation. There are only few publications on this subject and none are in association with “Cri du Chat” (Cat Cry) syndrome. We present a newborn, prenatally diagnosed with duodenal atresia and with “Cri du Chat” syndrome. The double duodenal atresia was actually of two different types (type I and type II), associated with malrotation. The second atresia was a peroperative finding at reintervention, five days later. We wish to share our experience in order to avoid unnecessary surgery and co-morbidities., SCOPUS: ar.j, info:eu-repo/semantics/published

Congenital intestinal malrotation is mainly detected in childhood and caused by incomplete rotation and fixation of the intestines providing the prerequisites for life-threatening volvulus of the midgut. The objective of this study was to evaluate a large cohort of adult patients with intestinal malrotation. Thirty-nine patients, 15-67 years, were diagnosed and admitted to a university setting with congenital intestinal malrotation 2002-2013. The patients were divided into three age groups for stratified evaluation. Medical charts were scrutinized, and clinical outcome of surgery was reviewed. Twelve patients presented as emergency cases, whereas 27 were admitted as elective cases. Diagnosis was established in 33 patients who underwent radiological investigation and in the remaining 6 during surgery. A Ladd's operation was performed in 31 symptomatic patients; a conservative strategy was chosen in eight cases. Volvulus was more common in the younger age group. Twenty-six surgically treated patients were available for telephone interview, 1-12 years after surgery. All patients, except one, regarded their general condition improved to a high degree (n = 18) or with some reservation (n = 7). Twelve patients suffered remaining abdominal pain of a chronic and diffuse character. Due to recurrence of malrotation six patients were reoperated. Symptomatic malrotation occurs in both children and the adult population. Improved awareness and an accurately performed CT scan can reveal the malformation and enable surgical treatment. A Ladd's procedure relieved most patients from their severe complaints even when a history of several years of suffering existed.

In a textbook published in 1882, His exhibits drawings devoted to intestine development. The last figure depicts a typical case of gut malrotation, but there is no comment about this in the text. It could be possible that this omission should be due to the fact that mechanisms of gut development were not totally elucidated at this time. © 2011 Elsevier Masson SAS., SCOPUS: ar.j, info:eu-repo/semantics/published

In a textbook published in 1882, His exhibits drawings devoted to intestine development. The last figure depicts a typical case of gut malrotation, but there is no comment about this in the text. It could be possible that this omission should be due to the fact that mechanisms of gut development were not totally elucidated at this time. © 2011 Elsevier Masson SAS., SCOPUS: ar.j, info:eu-repo/semantics/published

INTRODUCTION: During fetal development, the hippocampal structures fold around the hippocampal sulcus into the temporal lobe. According to the literature, this inversion should be completed at gestation week (GW) 21. Thereafter, the hippocampal shape should resemble the adult shape. However, incomplete hippocampal inversion (IHI) is found in 19% of the common population. The aim of this study was to study fetal hippocampal development by examining neonates born preterm. METHODS: We analyzed cranial ultrasound examinations, performed as a part of the routine assessment of all preterm infants, over a 3-year period and excluded the infants with brain pathology. The final material consisted of 158 children born <35 GW. A rounded form (the ratio between the horizontal and vertical diameters of the hippocampal body <25 GW and >/=25 GW was statistically highly significant (p < 0.001). The frequency of bilateral IHI was highest in the youngest age group. In the other groups, the left-sided IHI was the most common. CONCLUSION: In about 50% of the neonates, hippocampal inversion is not completed up to GW 24; but from 25 GW onwards, the frequency and laterality of IHI is similar to that in the adult population.

Malrotation with associated volvulus is a potentially lethal event for a neonate. The gold standard for diagnosis is an upper gastrointestinal contrast study. However this can delay the diagnosis and the timing of surgical intervention. We present a novel abdominal radiographic sign; duodenal and gastric dilatation occurring in association with limited small bowel gas confined to the right lower quadrant of abdomen and the total absence of colonic air that is indicative of malrotation with associated volvulus. This allows for an earlier diagnosis and expeditious surgery.

We present a case in which misinterpretation of radiological images resulted in an incorrect diagnosis of malrotation. This case highlights the importance of adequate radiological review of images and of ensuring that the clinical picture matches the findings. Had this not been questioned in this case the baby could have undergone an unnecessary operation with potential mortality and morbidity.

Intestinal malrotation is a developmental anomaly affecting the position and peritoneal attachments of the small and large intestines during fetal life. Most often the diagnosis is established in the first year of life on the basis of abdominal pain and bile-stained vomiting secondary to bowel obstruction. The antenatal diagnosis can be suggested by identification of the complications such as bowel dilatation, ascites or meconium peritonitis. We describe two cases of isolated antenatal gastric malposition without any other associated anomaly that were confirmed after birth to be due to intestinal malrotation. We suggest that such an antenatal finding should alert the paediatrician to close clinical follow-up and prompt the diagnosis and surgical treatment in case of abdominal pain and/or bilious vomiting., Case Reports, Journal Article, SCOPUS: ar.j, info:eu-repo/semantics/published

Congenital intestinal malrotation is a developmental anomaly resulting from interruption of the physiological herniation and return to the abdominal cavity of the midgut during the 6th to 10th week of embryological development. Normal vascular and anatomic relationships used as landmarks during pancreaticoduodenectomy (PD) are altered in patients with congenital malrotation. We present 3 cases of PD in adults with congenital intestinal rotation disorders. Three adult patients with congenital rotational disorders required PD. Two of these patients had bilio-pancreatic tumors, and 1 cadaveric donor underwent total pancreatectomy during pancreas allograft procurement. All patients had arterial and venous anomalies around the celiac trunk and mesenteric vessels, respectively. The midgut and hindgut in each case were shifted toward opposite sides of the abdominal cavity. Modifications to the standard approach to PD were made, and outcomes were favorable in each case. Each patient showed anatomic abnormalities with the need for identifying vascular structures through their expected (or projected) course and location before parenchymal division or ligation of any vessel. This approach becomes crucial in cases of vascular anomalies, such as ones occurring in congenital malformations, and can be used in similar situations encountered during pancreaticoduodenectomy.

We describe here an autopsy case presenting bilateral cryptorchidism, left pelvic kidney and intestinal malrotation, evoking a "reverse rotation" with hypoplasia of the cecum. Furthermore, the colon showed alternate bands of stenosis and dilatation. This association has not yet been described, and is discussed in the light of the embryological events of kidney, gut and gonad development. Two hypotheses can explain gut stenoses (vascular consequences of the malrotation and type Hirschprung aganglionic segments)., Case Reports, Journal Article, SCOPUS: ar.j, info:eu-repo/semantics/published

We describe here an autopsy case presenting bilateral cryptorchidism, left pelvic kidney and intestinal malrotation, evoking a "reverse rotation" with hypoplasia of the cecum. Furthermore, the colon showed alternate bands of stenosis and dilatation. This association has not yet been described, and is discussed in the light of the embryological events of kidney, gut and gonad development. Two hypotheses can explain gut stenoses (vascular consequences of the malrotation and type Hirschprung aganglionic segments)., Case Reports, Journal Article, SCOPUS: ar.j, info:eu-repo/semantics/published

The purpose of this study was to evaluate the diagnostic value of the “whirlpool sign” detected at color Doppler sonography in adults complaining of acute abdominal pain. During a 2-year period, the whirlpool sign was systematically looked for with color Doppler sonography in all patients admitted in the emergency room for acute abdominal pain. The whirlpool sign was observed in 13 patients: 2 cases of surgically proven primary volvulus of the small bowel, 10 cases of proven or suspected small bowel obstruction secondary to adhesions, and 1 case of acute leukemia with enlarged mesenteric lymph nodes. In the first 12 cases, the whirlpool sign was associated with radiologic (N=9) and sonographic (N=12) signs of small bowel obstruction. In the last case, no sign of obstruction was observed. In patients who had the whirlpool sign and who underwent operations (N=6), primary or secondary volvulus was present in 3 cases but was absent in the other 3 cases. The whirlpool configuration detected with color Doppler sonography has to be considered as a sign of limited value, because it may be observed in various conditions, including primary or secondary volvulus, postsurgical adhesions without volvulus, and acute abdominal diseases without obstruction.

type:text, This is a case report of a neonate with impending gastric rupture due to malrotation with gastric outlet obstruction. A preoperative plain abdominal X-ray showed expansion of the gastric bubble. A barium meal demonstrated an unusual bulging of the gastric wall extending from the fundus to the body of the stomach on the greater curvature side and malrotation of the duodenal loop. At operation a malrotation with volvulus and a rupture of the seromuscular layer with bulging of the mucosa in the stomach was found. We think that this unusual bulging of the gastric wall in the barium meal is an important radiological sign of impending gastric rupture.

This is a case report of a neonate with impending gastric rupture due to malrotation with gastric outlet obstruction. A preoperative plain abdominal X-ray showed expansion of the gastric bubble. A barium meal demonstrated an unusual bulging of the gastric wall extending from the fundus to the body of the stomach on the greater curvature side and malrotation of the duodenal loop. At operation a malrotation with volvulus and a rupture of the seromuscular layer with bulging of the mucosa in the stomach was found. We think that this unusual bulging of the gastric wall in the barium meal is an important radiological sign of impending gastric rupture.

This is a case report of a neonate with impending gastric rupture due to malrotation with gastric outlet obstruction. A preoperative plain abdominal X-ray showed expansion of the gastric bubble. A barium meal demonstrated an unusual bulging of the gastric wall extending from the fundus to the body of the stomach on the greater curvature side and malrotation of the duodenal loop. At operation a malrotation with volvulus and a rupture of the seromuscular layer with bulging of the mucosa in the stomach was found. We think that this unusual bulging of the gastric wall in the barium meal is an important radiological sign of impending gastric rupture.

This is a case report of a neonate with impending gastric rupture due to malrotation with gastric outlet obstruction. A preoperative plain abdominal X-ray showed expansion of the gastric bubble. A barium meal demonstrated an unusual bulging of the gastric wall extending from the fundus to the body of the stomach on the greater curvature side and malrotation of the duodenal loop. At operation a malrotation with volvulus and a rupture of the seromuscular layer with bulging of the mucosa in the stomach was found. We think that this unusual bulging of the gastric wall in the barium meal is an important radiological sign of impending gastric rupture.