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Intestinal malrotation is a congenital rotational anomaly that occurs as a result of an arrest of normal rotation of the embryonic gut, said to occur in 1 in 6000 live births. Due to the abnormal caecal and appendix position, diagnosis of acute appendicitis becomes more challenging, thus leading to diagnostic and operative intervention delays. Our...
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Citations
... Midgut malrotation (MM) is largely a pediatric diagnosis, but initial detection can be made in adulthood [1,2]. It is a congenital anomaly that arises from incomplete rotation or abnormal position of the midgut during embryonic development [3]. ...
Background
Acute appendicitis is the most common cause of acute abdominal pain, requiring emergency surgery. Symptoms and signs of acute appendicitis usually occur in the right lower quadrant. However, approximately one-third of cases have pain unexcepted location due to its various anatomical locations. Acute appendicitis is a very rare cause of left lower quadrant pain; if it occurs, situs inversus (SI) and midgut malrotation (MM) are uncommon anatomic anomalies that complicate its diagnosis and management.
Clinical presentation
Here we present a 23-year-old Ethiopian male patient who presented with epigastric and left paraumbilical abdominal pain, fever, and vomiting of a day duration. On examination at admission, the patient had left lower quadrant tenderness. Later, with the help of imaging studies, the patient was diagnosed with left-side acute perforated appendicitis with intestinal nonrotation, and he was operated on and discharged improved after 6 days of hospital stay.
Conclusion
Physicians should be aware that acute appendicitis in patients with intestinal mal-rotation may be present with left-side abdominal pain. Although it is extremely rare, acute appendicitis should always be considered in the differential diagnosis of left-side abdominal pain. An increase in awareness of this anatomical variant is essential for physicians.
... Laparoscopic appendectomy was performed in 20 patients (27.4%); among these, in two cases appendectomy was combined with cholecystectomy [8,9]; in one case the extracorporeal appendectomy was performed [10]; in another case single port incision laparoscopic appendectomy was achieved [11]. There was one case of conversion to open surgery due to technical reason [12]. At last, in two cases surgery was not performed, but patients were treated conservatively with antibiotic therapy or radiologically guided drainage of abdominal collection [13,14]. ...
Introduction
Acute appendicitis is one of the most frequent intra-abdominal diseases requiring emergency surgical consult and treatment. The diagnosis of this condition is based on clinical features and radiologic findings. One-third of patients with acute appendicitis present unusual symptoms. There are several circumstances that may cause misdiagnosis and unclear prognostic prediction. Among these, situs viscerum inversus totalis and midgut malrotation can be challenging scenarios, leading to a delay in treatment, especially when these conditions are unknown. We decided to carry on a systematic review of published cases of acute appendicitis in the context of anatomical anomalies.
Methods
We used the MESH terms “appendicitis” AND “situs inversus” AND/OR “gut malrotation” to search for titles and abstracts. Inclusion criteria were patients with clinical and/or radiological diagnosis of acute appendicitis, with conservative or surgical management and with preoperative/intraoperative findings of situs viscerum inversus or gut malrotation. Additionally, previous reviews were examined. Exclusion criteria of the studies were insufficient patient clinical and demographic data.
Results
We included in this review 70 articles concerning 73 cases of acute appendicitis with anatomical anomaly. Patients were aged from 8 to 86 years (median: 27.0 years). 50 were male and 23 were female. 46 patients (63%) had situs viscerum inversus, 24 (33%) had midgut malrotation, 2 (2.7%) had Kartagener’s syndrome, one of them (1.4%) had an undetermined anomaly In 61 patients the anatomical anomaly was unknown previously (83.6%), while 16,4% already were aware of their condition.
Conclusion
Acute appendicitis can occur in association of rare anatomical anomalies and in these cases diagnosis can be challenging. Situs viscerum inversus and midgut malrotation should always be considered in the differential diagnosis of a patient with left lower quadrant pain, especially in younger population. Besides clinical features, it is fundamental to implement the diagnostic progress with radiological examination. Laparoscopic approach is useful to identify and treat acute surgical emergency and it is also a diagnostic tool and can be tailored in order to offer the best exposition of the operatory field for each single case.
... Dessa maneira, torna-se importante o conhecimento a respeito da localização do apêndice vermiforme. Alguns sugerem que a apendicite em uma localização incomum pode vir acompanhada de sintomas e sinais atípicos, e é provável que seja diagnosticada erroneamente ou não diagnosticada, resultando em uma maior incidência de perfuração e complicações (Collins, 1963;Shekhar, Hendahewa, Premaratne, 2015). Outros autores, ainda destacam, que essa taxa de apendicite perfurada é maior em crianças do que em adultos, variando de 5 a 75% (Hung, Lin, Chen, 2012;van den Bogaard et al., 2016). ...
Esta obra constituiu-se a partir de um processo colaborativo entre professores, estudantes e pesquisadores que se destacaram e qualificaram as discussões neste espaço formativo. Resulta, também, de movimentos interinstitucionais e de ações de incentivo à pesquisa que congregam pesquisadores das mais diversas áreas do conhecimento anatômico de diferentes Instituições de Ensino Superior, seja públicas e privadas, de abrangência nacional e internacional. Tem como objetivo integrar ações interinstitucionais nacionais e internacionais com redes de pesquisa que tenham a finalidade de fomentar a formação continuada dos profissionais da área de saúde, por meio da produção e socialização de conhecimentos anatômicos nas diversas áreas dos saberes. Agradecemos aos autores pelo empenho, disponibilidade e dedicação para o desenvolvimento e conclusão dessa obra. Esperamos também que esta obra sirva de instrumento didático-pedagógico para estudantes, professores dos diversos níveis de ensino em seus trabalhos e demais interessados pela temática.
... Dessa maneira, torna-se importante o conhecimento a respeito da localização do apêndice vermiforme. Alguns sugerem que a apendicite em uma localização incomum pode vir acompanhada de sintomas e sinais atípicos, e é provável que seja diagnosticada erroneamente ou não diagnosticada, resultando em uma maior incidência de perfuração e complicações (Collins, 1963;Shekhar, Hendahewa, Premaratne, 2015). Outros autores, ainda destacam, que essa taxa de apendicite perfurada é maior em crianças do que em adultos, variando de 5 a 75% (Hung, Lin, Chen, 2012;van den Bogaard et al., 2016 ...
Esta obra constituiu-se a partir de um processo colaborativo entre professores, estudantes e pesquisadores que se destacaram e qualificaram as discussões neste espaço formativo. Resulta, também, de movimentos interinstitucionais e de ações de incentivo à pesquisa que congregam pesquisadores das mais diversas áreas do conhecimento anatômico de diferentes Instituições de Ensino Superior, seja públicas e privadas, de abrangência nacional e internacional. Tem como objetivo integrar ações interinstitucionais nacionais e internacionais com redes de pesquisa que tenham a finalidade de fomentar a formação continuada dos profissionais da área de saúde, por meio da produção e socialização de conhecimentos anatômicos nas diversas áreas dos saberes. Agradecemos aos autores pelo empenho, disponibilidade e dedicação para o desenvolvimento e conclusão dessa obra. Esperamos também que esta obra sirva de instrumento didático-pedagógico para estudantes, professores dos diversos níveis de ensino em seus trabalhos e demais interessados pela temática.
... As a developmental arrest, it could be in a subcecal position along with the caecum. Duplicate appendix [1] left sided appendix [2], horse shoe appendix [3], helical appendix [4], and lateral pouch appendix [5] are some of its reported anomalies. Knowledge of all possible variations of appendix could be useful in diagnostic and surgical procedures. ...
Vermiform appendix is a highly variable organ, which is an enigma to the surgeon in terms of its position, size and symptoms. Attachment of its tip to the surrounding organs is relatively rare. Here we present a rare variation of vermiform appendix. The appendix was 12 cm long and was in retrocecal and retrocolic position. Its tip was attached to the right lobe of the liver through a fibrous band which was 2 cm long. This variation might pose difficulties in appendicular resection, renal and hepatic surgeries.
... Various cases of appendicitis with an atypical location similar to our case have been described. 5,[8][9][10] In conclusion, diagnosing left-sided appendicitis is quite challenging for clinicians and surgeons leading to delayed intervention. It must be also kept in mind that appendicitis presents with a wide range of clinical signs and symptoms and left-sided appendicitis is one of the differentials of left lower abdominal pain. ...
Appendicitis rarely presents with an left lower abdominal pain especially when the intestine is non-rotated or malrotated. Its diagnosis becomes quite troublesome to clinicians and delays prompt intervention. Non-rotation is the most common type of intestinal malrotation. Here, we present a case of a 40-year-old female with previously undiagnosed intestinal non-rotation with left lower abdominal pain and features of localised peritonitis. Abdominal ultrasonography and multidetector computerised tomography showed left-sided appendicitis with intestinal non-rotation. Diagnostic laparoscopy followed by explorative laparotomy and appendectomy was performed. Clinicians and surgeons are usually trained to diagnose and operate on right-sided appendix, thus, diagnosing and promptly intervening on left-sided appendicitis is quite challenging. Left-sided appendicitis must be kept in mind if a patient presents with left lower abdominal pain. Timely radiological scans like ultrasonography and computerised tomography scans help in prompt diagnosis in these cases.
Keywords:
appendicitis; case reports; peritonitis.
... The diagnosis become more challenging when it comes to left-sided acute appendicitis which is frequently associated with congenital abnormalities, most commonly situs inversus totalis, followed by intestinal malrotation. 3,4 Situs inversus totalis is a rare congenital condition which body organ was in an inverted position. 2 We report a rare case of left-sided acute appendicitis in a patient with situs inversus totalis. ...
... In situs inversus totalis, there is a complete transposition of all organs, where the partial situs inversus patients might have either abdominal or thoracic organs transposition. 2,4,5 Acute appendicitis is a common cause of lower abdominal pain in adults. 3 The initial evaluation should include thorough history taking, physical examination, laboratory tests and Alvarado score calculation. ...
Background: Acute appendicitis is an acute abdominal case with a high incidence and often requires emergency surgical intervention. Situs inversus totalis is a rare congenital abnormality in which the thoracic and abdominal organs are totally transposed. Left-sided acute appendicitis (LSAA) is common in patients with congenital abnormalities such as situs inversus totalis. Diagnosis of LSAA is even more challenging, especially in peripheral areas which can lead to misdiagnosis and complications such as perforation or abscess. Imaging modalities are usually required to confirm the diagnosis. Case Presentation: A 21 years old male patient came to the emergency department with chief complaint of severe abdominal pain, starting 3 days earlier, localized to the epigastric area and migrating to the left lower quadrant. The patient also demonstrates fever, nausea and vomiting. Physical examination revealed an increasing abdominal wall rigidity and tenderness in the left lower quadrant as well as rebound tenderness. Laboratory tests revealed leucocytosis and increased neutrophils count. The patient underwent 3-position abdominal radiograph after chest X-ray showed dextrocardia. Exploratory laparotomy was done in this patient, showing a perforated appendix and totally transposed abdominal organs. Conclusion: Imaging modalities such as ultrasonography and abdominal CT Scan can be helpful in determining the diagnosis of LSAA and reduce the likelihood of misdiagnosis and complication. The possibility of LSAA and situs inversus should be suspected in a patient presenting with left lower quadrant abdominal pain with proven dextrocardia from chest X-ray. Keyword: Left-sided acute appendicitis, situs inversus totalis, dextrocardia
... 4 Some authors suggest that appendicitis in an unusual location may be presented with atypical symptoms and signs and is likely to be misdiagnosed or undiagnosed, resulting in a higher incidence of perforation and complications. 5,6 The aim of our study is to investigate if there are differences in the clinical symptoms of appendicitis according to the appendiceal position in pediatric patients and to demonstrate that laparoscopic approach is safe and effective in any appendiceal location by comparing each location to another. ...
... Several series have reported that an abnormal location of the appendix is likely to have an atypical clinical presentation, resulting in a higher incidence of misdiagnoses and complications. 5,6 Other authors, however, reported that the appendiceal position does not alter the presentation of the appendicitis. 7,8 In our study, there were no statistically significant differences in the clinical symptoms and signs between the groups, except for the location of the abdominal pain. ...
... Appendicitis may mimic other acute abdominal diseases, so it should be considered in the differential diagnosis of diseases like mesenteric adenitis, urinary tract infection, Meckel's diverticulum, cholecystitis or gynecological pathology in females. 4,6 The rate of perforated appendicitis is higher in children than in adults and varies from 5 to 75%. 9,10 Different Clinical Decision Rules (CDR), like the Pediatric Appendicitis Score (PAS) and the Alvarado score, have been developed in order to improve the accuracy of appendicitis diagnosis in children and to prevent perforation of the appendix. ...
Objective:
To investigate how symptoms vary according to the appendiceal position in pediatric patients and to demonstrate that the laparoscopic approach is safe and effective in any appendiceal location by comparing each location to another.
Methods:
The medical records of 1,736 children aged 14 or younger who underwent laparoscopic appendectomy over a period of 14 years were analyzed retrospectively. Patients were divided according to the position of the appendiceal tip into four groups: anterior, pelvic, retrocecal and subhepatic. The Kruskal-Wallis and chi-square tests were used with the Bonferroni correction, with a significant p<0.05.
Results:
The appendiceal location was anterior in 1,366 cases, retrocecal in 248 cases, pelvic in 66 cases and subhepatic in 56 cases. There were no significant differences between the groups in terms of patient age and gender. Abdominal pain was the only symptom with statistically significant differences between the groups. The rate of perforated appendicitis was higher in the subhepatic and pelvic positions. Intraoperative complications and conversions were not statistically significant. Technical difficulties and operative time were higher in subhepatic position. The rate of postoperative complications was similar between the different locations, except for bowel obstruction, which was higher in pelvic appendicitis.
Conclusions:
The clinical symptoms of appendicitis hardly ever change with the position of the appendix. The laparoscopic approach is safe and effective, regardless the appendiceal location.
... The incidence of acute appendicitis is 1.33 per 1000 in males and 0.99 per 1000 in females. 2 Shifting abdominal pain from epigastric region to the right lower abdomen with tenderness on compression are the classic clinical manifestations and usually associated with fever and vomiting. The appendix is normally less than 6 mm in diameter. ...
... 3 Left sided appendicitis is rare and the possibilities include an abnormally enlarged right sided appendix with the tip at the left or appendicitis associated with intestinal malrotation or situs inversus totalis. 2 Embryonic development of the gastrointestinal tract starts at 4th week of gestation. The total of 270 degrees anticlockwise rotation of the mid gut occurs until 12th week of gestation before it is fixed to the mesentery and posterior abdominal wall.¹ ...
... The incidence of malrotation is 1 in 500 new born and 64-80 % of the neonates present within first month of life. 2 Upper gastrointestinal series is the investigation of choice for diagnosis of malrotation in children and demonstrates failure of the duodenaljejunal junction to cross the midline and lies below duodenal bulb. 5,6 Intestinal malrotation in adult is uncommon and occasionally noted as incidental finding on adult population. ...
A 28-yr-old male presented with sudden onset pain at the epigastric region followed by left lower abdominal pain. CT scan of the abdomen revealed reverse relation of the superior mesenteric artery and superior mesenteric vein with large bowel loops including the cecum on left side and small bowel loops on the right. An inflamed appendix arising from the base of left sided cecum was identified. The intra operative findings were consistent with CT features. This is a rare case of left sided acute appendicitis with intestinal malrotation in an adult patient. CT scan is diagnostic modality of choice and excludes other possible disease presenting with similar clinical features.
... In addition malrotation presenting as acute left sided appendicitis is even rare with few individual case reports described in the literature. 1,2 The unusual clinical presentation of left lower abdominal pain without bowel and urinary symptoms should arise the possibility of this rare clinical entity. Contrast enhanced CT-scan of the abdomen and pelvis is the diagnostic method of choice which reveals the anatomic details of intestinal malrotation, findings of acute appendiceal inflammation and its possible complications. ...
p>A 28-yr-old male presented with sudden onset pain at the epigastric region followed by left lower abdominal pain. CT scan of the abdomen revealed reverse relation of the superior mesenteric artery and superior mesenteric vein with large bowel loops including the cecum on the left side and small bowel loops on the right. An inflamed appendix arising from the base of left sided cecum was identified. The intraoperative findings were consistent with CT features. This is a rare case of left sided acute appendicitis with intestinal malrotation in an adult patient. CT scan is diagnostic modality of choice and excludes other possible disease presenting with similar clinical features.</p
