Survey
* Your assessment is very important for improving the workof artificial intelligence, which forms the content of this project
* Your assessment is very important for improving the workof artificial intelligence, which forms the content of this project
Bariatric surgery wikipedia , lookup
Surgical management of fecal incontinence wikipedia , lookup
Inflammatory bowel disease wikipedia , lookup
Gastrointestinal tract wikipedia , lookup
Gastric bypass surgery wikipedia , lookup
Colonoscopy wikipedia , lookup
INTESTINAL MALROTATION Dr.T.KIRAN KUMAR FINAL YEAR POST GRADUATE GENERAL SURGERY CONTENTS • • • • • • • Definition History Embryology Rotational disorders Presentation Diagnosis Management 3 DEFINITION Malrotation is a congenital abnormal position of the bowel within the peritoneal cavity and usually involves both the small and the large bowel. HISTORY Intestinal development –Earliest descriptions by Mall in 1898. Later expanded by Frazer and Robins in 1915. 1928- Dott translated preliminary embryologic observations into problems encountered clinically. 1932 ,Ladd described the evaluation and surgical treatment of malrotation. EMBRYOLOGY Development of midgut begins with the differentiation of the primitive intestinal tract 4th week of gestation Foregut Hindgut Midgut • Most accepted model of midgut maturation involves Herniation Rotation Retraction Fixation Disproportional growth and elongation of midgut (4th gestational week) Herniation into extraembryonic coelom 3 separate 90 degrees turns, all in counter clock wise direction around superior mesenteric artery . The first 90 degrees rotation outside the abdomen . 2nd 90 degrees during the return of the intestine into abdominal cavity(10th gestational week) . Duodenojejunal junction passes posterior to superior mesenteric artery Last rotation in the abdomen Primitive intestine has thus completed a 270 degrees counter clock wise rotation . Duodenojejunal junction becomes fixed in the left upper abdomen cecum is anchored in the right lower quadrant. ROTATIONAL DISORDERS Clinical disorders may arise when intestinal rotation fails to occur or is incomplete. Genetic mutations may predipose the host to malrotation. Mutations in the gene BCL6 resulting in absence of left sided expression of its transcript lead to reversed cardiac orientation ,defective ocular development and malrotation. NONROTATION : Failure of normal intestinal 270degrees counter clock wise rotation around Superior mesenteric artery. Duodenojejunal limb SIGNIFICANT RISKS Midgut volvolus Extrinsic duodenal obstruction cecocolic limb INCOMPLETE ROTATION Normal rotation has been arrested at or near 180 degrees. CECUM IN RIGHT UPPER ABDOMEN Obstructing peritoneal bands will present. REVERSE ROTATION PRESENTATION Presents mainly in childhood. Incidence: 1 in 6000 live births Small proportion of adults - Acute or chronic symptoms of intestinal obstruction. - Intermittent and recurrent abdominal pain 75% of patients- during the first month of life Another 15% with in 1 year. INCIDENCE Most adult diagnosis of Malrotation are made in asymptomatic patients ;either on imaging investigations for unrelated conditions or at operations for other pathology Incidence in adults is approximately 0.00001% to 0.19%. Incidental diagnosis is becoming increasingly common. The true diagnosis is fraught with immense difficulty because - Presents with non-specific symptoms . - Adult Surgeons usually have low index of suspicion. Associated anomalies Associated anomalies In percentage Intestinal atresia 5-26 Imperforate anus 0-9 Cardiac anamolies 7-13 Duodenal web 1-2 Meckels diverticulum 1-4 Hernia 0-7 Trisomy 21 3-10 PRESENTATION IN ADULTS: Presents in numerous ways and the symptoms are nonspecific. The clinical diagnosis in adolescents and adults is difficult because it is rarely considered on clinical grounds. Many patients remain asymptomatic and the diagnosis is discovered incidentally during investigations or laparotomy for other unrelated problems in adult life. Adults with a rotational abnormality of the gut usually present differently to paediatric patients. Two distinct patterns of adult presentations have been reported in the literature: Acute Chronic Chronic presentation is more common in adults. This is characterised by intermittent crampy abdominal pain, bloating, nausea and vomiting over several months or years. The symptoms may be highly nonspecific. However, the range of clinical presentations, underlines the need for a high index of suspicion of midgut malrotation, when investigating the cause of intermittent and varying abdominal symptomatology in a healthy adult . Diagnostic delays are common in this group of patients because of the nonspecific nature of the presentations. The pathophysiology of these chronic symptoms - o pressio effe t of Ladd s a ds. The other group of symptomatic adults typically present with symptoms of acute bowel obstruction. May or may not report a previous history of abdominal symptoms. occasionally have symptoms and signs of an impending abdominal catastrophe. Acute presentation may be due to Volvulus of the midgut or ileocaecum -. Internal herniation aused y Ladd s a ds -. Identified when affected by other common abdominal diseases Clinical features of malrotation in neonates: • Bilious vomiting Cardinal • sign • • Other • signs Late malrotation must be the presumed diagnosis until proven otherwise Abdominal pain , Distension, Hypovolemic shock. • Hematemesis, • Melena from progressive mucosal ischemia signs Finally • Mesenteric vascular compromise rapidly leads to peritonitis , sepsis shock and death DIAGNOSIS Ultrasound scan USS Plain abdominal radigraphy Computed tomography scan CT IDENTIFIED USING Mesentric arteriography Magnetic resonance imaging scan MRI CONVENTIONAL PLAIN RADIOGRAPHY • Neither sensitive nor specific . • Right-sided jejunal markings and the absence of a stool-filled colon in the right lower quadrant may be suggestive, leading to further investigation. ABDOMINAL COLOUR DOPPLER USS • Reveal malposition of the SMA, raising the suspicion of gut malrotation with or without the abnormal location of the hollow viscus . • Reported gold standard for diagnosis of gut UPPER GASTROINTESTINAL CONTRAST STUDY malrotation particularly in the paediatric age group • Shows duodenum and dudenojujenal flexure located to the right of spine • Shows abnormally located ileocaecum and right colon CONTRAST ENEMA WITH UGI STUDY • Findings may be non specific • Normal study does not exclude the possibility of gut malrotation MALROTATION • Previously used but now rarely indicated MESENTRIC ANGIOGRAPHY • Shows the abnormal relationship between and detect the patency of mesentric vasculature • Increasingly used COMPUTED TOMOGRAPHY • Now considered the investigation of choice • Accuracy is 80% CT and MRI scans show - SMV to be in an anomalous position; posterior and to the left of the SMA. - The abnormal anatomical arrangements of the midgut with the duodenum not crossing the spine. - hirlpool appeara e (first described by Fisher) - Internal herniation secondary to Ladd s a ds. The role of mesenteric angiogrphy has been superseded by the CT scan which has the overall advantage in detecting the -Abnormal location of the midgut . - Reversed mesenteric anatomical relationship . - Other intra-abdominal anomalies associated with malrotation . MANAGEMENT Symptomatic midgut malrotation - requires surgical intervention. Management of asymptomatic patients is more controversial. Choi et al reviewed 177 patients over a 35-year period. - Found that asymptomatic patients had a low risk of intestinal volvulus. - Elective surgery is not necessary. - Requires close follow-up. However, it is increasingly argued that all suitable patients with intestinal malrotation should undergo surgical correction regardless of age . Several case series have recommended that - It is impossible to predict which patients will develop catastrophic complications - So ele ti e Ladd s pro edure should e perfor ed i all patients with intestinal malrotation. - The potential risks of the procedure need to be weighed against the benefits. The surgical management of intestinal malrotation was first described by William Ladd in 1936 and this remains the mainstay of treatment. Six key elements in operative correction of malrotation 1. Entry into abdominal cavity and evisceration(open) 2. Counterclock wise detorsion of the bowel 3. Division of ladd s bands 4. Broadening of small intestine mesentry 5. Incidental appendicectomy 6. Placement of small bowel along the right lateral gutter and colon along the left lateral gutter. The origi al Ladd s pro edure as des ri ed for the paediatric population group and the full components of this procedure may not be offered in the adult group. Complications after surgery Recurrent volvolus is low Post operative intussception has been noted in 3.1% of all patients who underwent ladd procedure 10% of patients may develop an adhesive small bowel obstruction requiring laporotomy after the procedure Laparoscopic procedure There are recent reports of the use of the laparoscopic approach in the surgical treatment of intestinal malrotation. The technique appears to be safe and effective when performed by experienced laparoscopic surgeons, especially in the absence of volvulus . First proposed by van der zee s i 1995. Four port technique provides adequate visualisation First goal is to determine if malrotation is actually present and thus confirm preoperative imaging. Detorsion. Expeditious dissection may progress by identifying 2nd portion of duodenum and excising restricting peritoneal bands. Next, reduction of volvolus if present , mobilisation of duodenum , jejunum and incision of anterior mesenteric leaflet. Lastly appendicectomy. Comparision of laporoscopic and open procedures In a review of patients undergoing open and laparoscopic ladd procedure ,comparative results were favorable for the laparoscopic group for Resumption of oral intake (1.8 days vs 2.7 days) Shorter hospitalisation (4 days vs 6.1 days) Requirements for iv narcotics on postoperative day . Operative time (194 min vs 143 min) Conversion to an open operation occurred in 25%.