Laparoscopic surgery for colon cancer with intestinal malrotation in adults: Two case reports and review of literatures in Japan

IntroductionIntestinal malrotation is a congenital anomaly, and its occurrence in adults is rare. Colon cancer with intestinal malrotation is far more rare. We herein report two cases of colon cancer with intestinal malrotation treated with laparoscopic surgery and reviewed the literatures in Japan.Presentation of casesCase 1 involved a 78-year-old man. Abdominal enhanced computed tomography (CT) showed that the tumor was located in the sigmoid colon. Intraoperatively, the cecum and ascending colon were located along the midline and the small intestine occupied the right side of the abdomen. The tumor was located in the cecum, and the patient was diagnosed with cecal cancer with intestinal malrotation. We performed laparoscopy-assisted ileocecal resection. Case 2 involved a 81-year-old man. Colonoscopy revealed a laterally spreading tumor in the cecum. Intraoperatively, the position of the small intestine and the ascending colon was similar to case 1, and Ladd’s band was found in front of the duodenum. Thus, we diagnosed the patient with a laterally spreading cecal tumor with intestinal malrotation and performed laparoscopy-assisted ileocecal resection.DiscussionA review of the literature revealed 49 cases of colon cancer with intestinal malrotation and laparoscopic surgery performed at 30.6%. If laparoscopic mesenteric excision for colon cancer with intestinal malrotation is unsafe because of the abnormalities of the artery, mesenteric excision should be performed outside the body.ConclusionIf the intestinal malrotation is diagnosed preoperatively, 3D-CT angiography should be used to reveal the vascular anatomic anomalies for safe performance of laparoscopic surgery.     

Malroatation of gut with superior mesenteric artery syndrome and multiple jejunal diverticula presenting as acute intestinal obstruction in 6th decade: A rare case report

INTRODUCTIONIntestinal malrotation is a disease of neonates and young children presenting as acute intestinal obstruction. Presentation of malrotation in elderly patients with intestinal obstruction is quite rare with only a few cases reported in literature. We report a case of intestinal malrotation presenting as acute obstruction in sixth decade.PRESENTATION OF CASEA 55 years old male presented to the emergency with features of acute intestinal obstruction. Imaging studies revealed intestinal malrotation. Exploratory laparotomy revealed malrotation with compression of 3rd part of duodenum and terminal ileum by superior mesenteric artery with multiple jejunal diverticula. Bypass procedures (duodenojejunostomy and ileo-colic anastomosis) with appendicectomy were done.DISCUSSIONMalrotation of gut is an anomaly usually presenting in neonatal period with complications such as midgut volvulus. Presentation in adult age is rare with most cases being asymptomatic. Ladd's procedure is the operation of choice with division of the Ladd's bands and appendicectomy being performed.CONCLUSIONSurgeons should keep a flexible approach in management of malrotation of gut presenting in late stages of life as more and more clinical variants to the presentations described in literature are being encountered.     

A rare presentation of midgut malrotation as an acute intestinal obstruction in an adult: Two case reports and literature review

INTRODUCTIONMidgut malrotation is a congenital anomaly presenting mainly in the childhood. Its presentation as an acute intestinal obstruction is extremely rare in adults usually recognized intra-operatively, therefore a high index of suspicion is always required when dealing with any case of acute intestinal obstruction.PRESENTATION OF CASEWe report two cases of young adults who presented with symptoms of acute intestinal obstruction and were diagnosed intra-operatively as cecal volvulus and paraduodenal hernia, respectively, caused by midgut malrotation. Post-operative CT scan confirmed these findings.DISCUSSIONMalrotation of the intestinal tract is a product of an aberrant embryology. The presentation of intestinal malrotation in adults is rare (0.2–0.5%). Contrast enhanced CT can show the abnormal anatomic location of a right sided small bowel, a left-sided colon and an abnormal relationship of the superior mesenteric vein (SMV) situated to the left of the superior mesenteric artery (SMA) instead of to the right.CONCLUSIONAnomalies like midgut malrotation can present as an operative surprise and awareness regarding these anomalies can help surgeons deal with these conditions.     

Intestinal malrotation and volvulus in adult life

INTRODUCTIONMidgut volvulus due to intestinal malrotation is a rare cause of intestinal obstruction when occurring in adult life. This paper documents the difficulties in reaching an early diagnosis.PRESENTATION OF CASEWe describe the case of an 85-year-old man with non-specific abdominal complaints for 20 years, who presented with sudden onset central abdominal pain. An acute median laparotomy under general anaesthesia was performed during which a 360° clockwise rotation of the small bowel around the mesenteric pedicle of the superior mesenteric artery and vein was found.DISCUSSIONMalrotation is considered any deviation of the normal rotation of the midgut in embryological development, causing intermittent episodes of gastrointestinal obstruction or acute events of midgut volvulus. Although mainly a paediatric diagnosis, some cases do present in adult life. Radiologic investigations include: upper gastrointestinal contrast studies, Doppler sonography and a contrast enhanced CT of the abdomen. If a true malrotation is diagnosed or found by coincidence, a Ladd's procedure is advised, even if the patient is asymptomatic. There is no proven surgical strategy for preventing the recurrence of small bowel volvulus in case of malfixation of the midgut.CONCLUSIONNon-specific recurrent abdominal complaints in adults of any age should raise suspicion of the possibility of a midgut malrotation or malfixation with or without intermittent volvulus. This case highlights the importance of diagnosis in an early stage.     

A case of midgut volvulus related to adult intestinal malrotation found with weight loss after streptococcus infection: A case report and literature review

Introduction and importanceThe incidence of intestinal malrotation is 1 in 6000 births, and 90% of cases occur within the first year of life. Adult cases are rare, with a reported incidence of 0.2%–0.5% of all cases. The significance of reporting this case is to recognize that some adult-onset cases require surgery even in the absence of intestinal necrosis.Case presentationA 36-year-old man was infected with streptococcus and treated with antibiotics. He developed appetite loss and his weight decreased 12 kg in 4 months. His abdomen was flat and soft with no tenderness. A computed tomography scan showed that the horizontal duodenal leg was not anchored to the retroperitoneum. Rotation of the mesentery, which was wrapped around the superior mesenteric artery in a clockwise direction, was observed, suggesting midgut volvulus. We performed emergency surgery and Ladd’s procedure.Clinical discussionA previous study reported that the most common symptom in the chronic course of intestinal malrotation was abdominal pain in 41.2% of cases, and weight loss was observed in only 2.6% of patients. The high degree of intestinal adhesion suggests that repeated torsion and release and the development of collateral vessels may have contributed to the asymptomatic course.ConclusionAdult-onset intestinal malrotation should be considered as a differential diagnosis in the presence of weight loss and gastrointestinal symptoms. The timing of surgery is still controversial. In chronic cases, severe adhesion might be expected and laparoscopic surgery should be considered carefully.     

Post traumatic acquired multiple mesenteric defects

INTRODUCTIONInternal intestinal hernia has been defined as a bulging of the intestines through a normal or an abnormal peritoneal or mesenteric opening.¹ Internal hernias are a rare cause of small-bowel obstruction, with a reported incidence of 0.2–0.9%.²PRESENTATION OF CASEIn this report, the patient presented with multiple episodes of intestinal obstruction. High index of suspicion aided the appropriate management of this case. An abdominal CT revealed signs of small bowel obstruction. With negative signs and symptoms indicating adhesions, malignancy or inflammatory causes, mesenteric defect was suspected. When the patient underwent laparotomy, multiple mesenteric defects were found.DISCUSSIONIn the adult population, acquired mesenteric defects are more common than congenital defects. They can be caused by bowel surgery or abdominal trauma.¹¹ Patients with a history of blunt abdominal trauma may present with late complication caused by a missed diagnosis of an associated injury, such as bowel mesenteric injuries.In this case, the author describes a patient who developed multiple attacks of small bowel obstruction. He had no previous history of similar symptoms but did give a history of recent abdominal trauma managed conservatively. An abdominal CT was performed, and it showed signs of a mesenteric defect. In such a case, early operative intervention is essential to decrease morbidity and increase survival. ¹⁶CONCLUSIONThe diagnosis of post traumatic mesenteric injuries can be missed in conservatively managed trauma cases. For this reason, the decision of non-operative approach should be made following the exclusion of associated injuries.     

Laparoscopic resection of sigmoid colon cancer with intestinal malrotation: A case report

IntroductionIntestinal malrotation is a congenital abnormality which occurs due to a failure of the normal 270° rotation of the midgut. The non-rotation type is usually asymptomatic and discovered incidentally on imaging studies. Intestinal malrotation accompanied by colon cancer is extremely rare.Presentation of caseA 53-year-old male presented with postprandial abdominal discomfort. Colonoscopy showed a 14 mm polyp in the sigmoid colon and endoscopic polypectomy was performed. Pathological evaluation revealed an adenocarcinoma invading the submucosa more than 1000 μm with positive vertical and horizontal margins. A contrast enhanced computed tomography scan showed an anatomic variant of the ileocolic and inferior mesenteric arteries originating from a common channel branching from the abdominal aorta. Laparoscopic sigmoid colon resection was performed. The patient did well post operatively.DiscussionThe usual trocar placement for laparoscopic left side colectomy was used, and we found no difficulties intraoperatively. To secure safe ligation, the divisions of the common channel branching from the abdominal aorta were exposed as in a usual D3 dissection, and the inferior mesenteric artery was ligated after confirmation of the bifurcation of the ileocolic and inferior mesenteric artery.ConclusionTo the best of our knowledge, this is the first report of laparoscopic resection of a sigmoid colon cancer with intestinal malrotation. It was performed without difficulty using the usual trocar placement, with appropriate attention to the variant in vascular anatomy.     

Laparoscopic surgery for appendiceal cancer with intestinal malrotation in an adult: A case report

IntroductionIntestinal malrotation is a congenital anatomical anomaly resulting from abnormal midgut rotation. Many cases occur during childhood and present with intestinal obstruction and midgut volvulus. Intestinal malrotation rarely occurs in adults and is found incidentally because it is asymptomatic. We herein report a case of intestinal malrotation, and colorectal cancer operated laparoscopically.Presentation of caseA 78-year-old man presented to our Department of Surgical Gastroenterology with fecal occult blood. There were no abnormal findings in the physical examination. Colonoscopy revealed a type 3 tumor in the cecum. Contrast-enhanced computed tomography revealed that the tumor was located in the appendix along the midline of the abdomen. The small intestine and colon occupied the right and left sides of the abdominal cavity, respectively. The diagnosis was appendiceal cancer with nonrotation-type intestinal malrotation. A laparoscopy-assisted ileocecal resection was performed. During surgery, the right-side colon was not fixed to the retroperitoneum, and the right-side colon could be extracted out of the abdominal cavity through the umbilical wound with only adhesive dissection, and mesenteric and lymph node dissection can be performed outside the body. The postoperative course was uneventful.DiscussionAppendiceal cancer with intestinal malrotation is managed with laparoscopic surgery because this method is safe and minimally invasive.ConclusionThe laparoscopic approach may be safer and less invasive than laparotomy, and extracorporeal lymph node dissection is safe and reliable for patients with intestinal malrotation.     

Long-term outcomes of ultrashort bowel syndrome due to malrotation with midgut volvulus managed at an interdisciplinary pediatric intestinal rehabilitation center

PurposeThe purpose of this study was to describe long-term outcomes of pediatric-onset ultrashort bowel syndrome owing to midgut volvulus managed at an interdisciplinary intestinal rehabilitation center.MethodsPatients with a history of malrotation and pediatric-onset midgut volvulus causing extensive bowel loss (< 20% residual small bowel length expected for postconception age) and treated between 2010 and 2017 were reviewed. Data are expressed as median (IQR).ResultsTwenty-three patients had midgut volvulus at age 1 (0–21) day leading to 9 (8–12) percent predicted residual bowel length. Eight (35%) had gastroschisis. Follow-up was 8.5 (6.6–12.2) years from volvulus. Five (22%) patients underwent intestinal/multivisceral transplantation, and all achieved enteral autonomy. Eighteen (78%) patients remained transplant-free, 7 of whom achieved enteral autonomy after 718 (682–1030) days of parenteral nutrition. Transplant-free enteral autonomy was achieved by 0/6 patients with gastroschisis, compared to 7/12 without gastroschisis (p = 0.04). For the overall group, 18 (78%) patients had small bowel bacterial overgrowth, and 7 manifested symptomatic D-lactic acidosis. We observed 2 mortalities, one awaiting transplant and one 4 years following transplantation.ConclusionMidgut volvulus owing to malrotation with extensive bowel loss is associated with favorable long-term survival. Transplant-free enteral autonomy may be feasible, particularly in the absence of gastroschisis.Type of studyPrognosis study.Level of evidenceIIb, retrospective cohort study.     

An unusual presentation of congenital intestinal malrotation in a nonagenarian

IntroductionAppendicitis is a common surgical pathology with many clinical presentations. Diagnosis can be challenging if there is an alteration to the normal position of anatomical structures and their relationships.Case presentationIn this case, we report an unusual presentation of congenital intestinal malrotation in a nonagenarian presenting with generalized abdominal pain and lactic acidosis found to have perforated appendicitis. The patient was admitted to the Hospitalist service for abdominal pain and misdiagnosed with bowel obstruction secondary to previous “operation”. After further review of imaging malrotation with internal hernia was suggested. The patient was taken emergently to the operating room and found to have perforated and gangrenous appendix in the midabdomen. An appendectomy was performed and patient was ultimately discharged home in good condition on postoperative day four.DiscussionMalrotation of the intestines is a developmental variant that occurs when normal fetal rotation of the intestines around the superior mesenteric artery and their peritoneal fixation fails to occur. Presentation typically occurs during infancy and diagnosis in an elderly patient is exceedingly rare. Operative intervention is often required as it is commonly associated with lactic acidosis or peritonitis.ConclusionsMalrotation can go undiagnosed well into elderly life and can present with unusual signs and symptoms. CT is very helpful in aiding the diagnosis but itself can be misleading.     

Duodenojejunal junction web masquerading as Wilkie’s syndrome: Report of a case

Wilkie’s syndrome (superior mesenteric artery syndrome) is a rare cause of obstruction to the third part of duodenum due to compression between the superior mesenteric artery and the abdominal aorta. Pathologies like malignant growth in the mesenteric root, the presence of a lymph nodal mass compressing the terminal duodenum, dissecting aortic aneurysm, and intestinal malrotation may mimic the condition, but are not true etiologies of the syndrome. A duodenojejunal web causing narrowing of the duodenojejunal junction and mimicking Wilkie’s syndrome has not been described before in the literature. We herein report a case of gastroduodenal obstruction due to a web in the duodenojejunal junction in a young female patient, which closely mimicked Wilkie’s syndrome but was finally diagnosed postoperatively. We highlight the first case of its kind in an adult and discuss the challenges in both the diagnosis and management.     

Laparoscopic treatment of mucinous adenocarcinoma of jejunum associated with celiac disease. Case report

Mucinous adenocarcinoma of the small bowel is very rare, and only few cases have been described in the literature. Association of this tumor with celiac disease has never been published. The authors report a unique case of jejunal mucinous adenocarcinoma in which a concomitant celiac disease has been histologically recognized. The difficult diagnosis, the role of laparoscopic surgery and the relationship between small bowel tumors and celiac disease are discussed. A 49-year-old man presented with recurrent melena, nausea, vomiting and anemia. A stenosis of the jejunum was documented by means of CT scan and video capsule enteroscopy. A laparoscopy was scheduled. A tumor, found in the first jejunal loop, was removed by laparoscopic surgery. Histopathology revealed a rare mucinous adenocarcinoma associated with epithelial changes secondary to celiac disease. Although small bowel tumors are rare entity, in patients with celiac disease complaining of symptoms related to altered intestinal transit or occult bleeding, an appropriate work-up should be planned for diagnosis. Mucinous type intestinal adenocarcinoma, even if never published before, could be observed. Laparoscopic surgery is often essential for the diagnosis and treatment.     

Newborn and toddler intestinal obstruction owing to congenital mesenteric defects

Transmesenteric hernia is a rare cause of intestinal obstruction most commonly affecting the small bowel. The mesenteric defect is usually 2 to 3 cm in diameter. The authors describe 2 cases of young pediatric patients presenting with bowel obstruction resulting from a congenital mesenteric defect. The initial patient had a 30-cm-wide congenital defect in the ileal mesentery through which the sigmoid colon and some loops of small bowel had herniated. The second patient is a newborn infant who presented with symptoms and radiographic evidence of proximal bowel obstruction initially thought to be resulting from malrotation with midgut volvulus but was found at surgical exploration to have a small defect in the ileal mesentery.     

An unusual case of transverse mesocolic internal hernia with abnormality of both hands and high arched feet

IntroductionAn internal abdominal herniation is the protrusion of a viscus through a normal or abnormal mesenteric or peritoneal aperture. It is a rare cause of small bowel obstruction with a reported incidence of 0.2–0.9%. It can either be acquired through a trauma or surgical procedure or can be related to congenital peritoneal defects. Herniation through transverse mesocolon is very rare.Presentation of caseA case of acute intestinal obstruction due to internal herniation through a congenital rent in transverse mesocolon with rotation of gut approximately 180° around axis of the band. Patient also had bilateral hypoplastic thenar muscles with rudimentary 1st metacarpals and high arched feet. Reduction along with derotation of gut, with closure of the rent in transverse mesocolon and fixation of the caecum to lateral peritoneum was performed.DiscussionThe preoperative diagnosis of mesenteric defect is difficult because of wide range of acute abdominal symptoms, and there are no specific radiographic findings. CT is the most important diagnostic tool is, with 77% accuracy in such cases. Due to the risk of strangulation of the hernial contents, even small internal hernias are dangerous and may be lethal.ConclusionInternal hernia should be suspected in patients with signs and symptoms of intestinal obstruction, particularly in the absence of inflammatory intestinal diseases, external hernia or previous laparotomy. Surgical decision-making is on the basis of clinical findings of intestinal strangulation or ischemia, and emergency laparotomy should be performed without preoperative diagnosis of such a rare disease.     

Superior mesenteric artery syndrome: A diagnosis to be kept in mind (Case report and literature review)

IntroductionSuperior mesenteric artery (SMA) syndrome or what is called Wiklie’s syndrome is one of the rare causes of small bowel obstruction. Its exact incidence is not known. It is due to decrease in Aortomesenteric angle.Case presentationA thirty-Four-year old male patient presented to our accident and emergency (department) with 3 days history of epigastric pain, which was not radiating anywhere. It had no aggravating or relieving factors. Patient complained of repeated attack of vomiting as well. Contrast enhanced Computed tomography (CT) showed duodenal obstruction caused by superior mesenteric artery compression on 3rd part of duodenum.DiscussionSuperior mesenteric artery syndrome (SMA) is one of the rare causes of small bowel obstruction. Incidence of superior mesenteric artery syndrome reported in literature is ranging from 0.1 to 0.3%. The most common cause is significant weight loss which leads to loss of retroperitoneal fat. Treatment usually is conservative but surgical intervention should be considered if that failed.ConclusionSuperior mesenteric artery syndrome is a rare cause of intestinal obstruction but should be kept in mind. Persistent vomiting after history of weight loss should raise the suspicion of this diagnosis. Upper GI endoscopy may be necessary to exclude mechanical causes of duodenal obstruction. Contrast enhanced CT scan is useful in the diagnosis of superior mesenteric artery syndrome and can provide diagnostic information.     

A rare case of heterotopic pancreatitis and intestinal malrotation in a COVID-19 positive patient. COVID-19, causative or coincidence?

Introduction and importanceHeterotopic pancreas (HP) is defined as the presence of pancreatic tissue without anatomical and vascular continuity with the main body of the pancreas. HP typically remains asymptomatic, however complications such as acute pancreatitis can arise. Gastrointestinal involvement with coronavirus disease 2019 (COVID-19) is not uncommon and there are reported cases of associated pancreatitis.Case presentationA 31-year-old male presented to the Emergency department (ED) with a 3-day history of right iliac fossa pain. The patient was found to have COVID-19 and a planned laparoscopic appendectomy was later converted to a midline laparotomy when a mass close to the duodeno-jejunal (DJ) flexure was identified. Following a hand-sewn anastomosis the patient made a good post-operative recovery. Histology illustrated the presence of heterotopic pancreatic tissue within the small bowel with underlying fat necrosis typical of acute pancreatitis. Follow-up radiology supported the intraoperative finding of intestinal malrotation.Clinical discussionRarely the combined presence of intestinal malrotation and HP in patients has been noted. Heterotopic pancreatitis can present in a multitude of ways and it is a difficult diagnosis to make pre-operatively. Emerging literature documents the potential presentation of COVID-19 with acute pancreatitis. The expression of angiotensin-converting enzyme 2 (ACE2) receptors on the pancreas is believed to play a role.ConclusionThis is the first documented case of heterotopic pancreatitis with intestinal malrotation in a COVID-19 positive patient. We hypothesise that the COVID-19 infection contributed to the heterotopic pancreatitis.     

Is ultrasonography a good screening test for intestinal malrotation?

Background

Early diagnosis of malrotation can prevent fatal midgut volvulus. Abnormal orientation of the superior mesenteric artery (SMA) and vein (SMV) on ultrasonography (US) has been described in malrotation. We aimed to determine the accuracy of this technique.

Methods

All children undergoing both upper gastrointestinal series (UGI) and US for possible malrotation over a 3-year period were reviewed. Patients were excluded if US did not include SMV/SMA orientation or if the duodenojejunal flexure was not visualized on UGI.

Results

Of 211 eligible patients, UGI and US were both normal in 62% and both abnormal in 15%. Forty-four had abnormal US and normal UGI (false positive, 21%), and 5 patients had normal US and abnormal UGI (false negative, 2%). Of these 5, none were found to have a short mesenteric base, which put them at risk for volvulus. Among abnormal ultrasounds, inversion of SMV/SMA and a “whirlpool” sign were more predictive for malrotation and volvulus than anterior/posterior orientation.

Conclusions

Ultrasonography is a good screening tool that effectively rules out malrotation at risk for volvulus. Children with an abnormal ultrasound should have an UGI or go to the operating room, depending on clinical findings.     

Outcomes after the Ladd procedure in patients with heterotaxy syndrome, congenital heart disease, and intestinal malrotation

Purpose

Heterotaxy syndrome (HS) patients often present with congenital heart disease and intestinal malrotation. Controversy exists regarding the management of these patients. Risk of midgut volvulus, morbidity from elective operations, and overall prognosis must be weighed when considering a Ladd procedure on asymptomatic HS/intestinal malrotation patients.

Methods

This is a retrospective review comparing HS and non-heterotaxy syndrome (NHS) patients undergoing a Ladd procedure at Children's Hospital Boston (Mass) from January 1997 to September 2007.

Results

Thirty-one HS and 51 NHS patients were identified. After a Ladd procedure, HS patients remained in the hospital 12.9 days, had a 9.7% risk of small bowel obstruction, and a 9.7% in-hospital mortality. If allowed to develop abdominal symptoms, 27% of HS patients with intestinal malrotation had a midgut volvulus at surgery. Intra-HS group comparison demonstrated longer hospital stays in symptomatic patients (P = .01). Mortality was greater in the HS than NHS patients, but deaths were related to cardiac disease and not to the Ladd procedure.

Conclusions

Elective Ladd procedures are well tolerated by HS patients. Given the risk of midgut volvulus and in light of improved survival beyond infanthood, once identified, HS patients with asymptomatic malrotation should be offered a prophylactic Ladd procedure.     

A rare case of ileus caused by ileum endometriosis

IntroductionWe report our experience involving a rare case of ileum endometriosis complicated with small bowel obstruction.Presentation of case33 years old female patient, admitted to emergency service with abdominal pain, abdominal distension, and vomiting. Abdominal X-ray showed dilated small bowel loops. Computerized tomography scan showed dilated small intestine segments excepting last ileum loop, gastric distension, enlarged ovaries.Emergency laparotomy was performed, showing acute bowel obstruction due to a stenotic tumor placed on the terminal ileum, cecum tumors, multiple tumors in Douglas pouch, multiple mesenteric enlarged lymph nodes. Right colectomy is performed with an ileo-transverso stomy placed in right hypochondrium. Postoperative evolution without complication, patient discharged after 13-days hospitalization. After hormonal treatment, she returned for a second look and ileotransverso anastomosis.DiscussionGastrointestinal involvement of endometriosis has been found in 3%–37% of menstruating women. Ileum localization is very rare (1%–7%), causing intestinal obstruction 7%–23% of cases. Intraoperative differential diagnosis is difficult, predisposing at confusion with other types of tumors. In the absence of fast microscopic exam, the tumor was considered malignant and imposed a right hemicolectomy.ConclusionIntestinal obstruction due to ileum endometriosis is a rare condition, however, it should always be considered in the differential diagnosis in women of reproductive age.