Segmental Absence Of Intestinal Musculature Research Articles

Outcomes of endoscopic submucosal dissection for esophageal cancer with segmental absence of intestinal musculature

Perforation during esophageal endoscopic submucosal dissection (ESD) typically results from electrical damage. However, there are cases in which perforation occurs because of segmental absence of intestinal musculature (SAIM) without iatrogenic muscular injury. We investigated the occurrence rate and clinical course of SAIM during esophageal ESD. We conducted a retrospective review of esophageal ESDs performed between 2013 and 2019 at 10 centers in Japan. Five of 1708 (0.29%) patients received ESD for esophageal cancer and had SAIM. The median muscular defect size was 20mm. All lesions were resected without discontinuation. After resection, 3 patients were closed with Endoloop. Four patients had mediastinal emphysema. All patients were managed conservatively. SAIM is a very rare condition that is usually only diagnosed during ESD. Physicians performing esophageal ESD should be aware of SAIM. When SAIM is detected, the ESD technique should be modified to prevent full-thickness perforation.

A case of sigmoid colon perforation due to segmental absence of intestinal musculature (SAIM) accompanied by vascular Ehlers–Danlos syndrome: a case report

BackgroundSegmental absence of intestinal musculature (SAIM) is a partial defect of the intrinsic muscular layer of the intestinal tract. In this report, we describe a case of perforation of the sigmoid colon due to SAIM accompanied by vascular Ehlers–Danlos syndrome (vEDS), which was successfully treated by surgical therapy.Case presentationA male in his 30 s was being followed up for vEDS diagnosed by genetic testing. He had undergone two major vascular surgeries, abdominal aortic artery revascularization and thoracic endovascular aortic repair for a residual dissection and enlarging abdominal aortic aneurysm. On postoperative day 11, the patient developed perforation of the sigmoid colon for which intraperitoneal lavage and drainage, Hartmann surgery, and transverse colostomy were performed. Histological findings showed no disturbance of blood flow or diverticulum but did show a defect in the intrinsic muscular layer around the perforation site, leading to the pathological diagnosis of SAIM and associated perforation of the sigmoid colon. Postoperatively, the patient had no complications and was discharged on postoperative day 18. The patient is being followed as an outpatient and has experienced no relapse.ConclusionsBoth SAIM and vEDS, which may be related diseases, are associated with the presence of tissue fragility and have a high potential to cause intestinal perforation Caution should be exercised during surveillance in patients with constipation and examinations that cause increased intestinal pressure.

Colostomy fistula caused by segmental absence of intestinal musculature: a case report

BackgroundSegmental Absence of Intestinal Musculature (SAIM) is a rare condition associated with intestinal obstruction and perforation. Colostomy fistula as a presentation of SAIM and their association with anastomotic failure have not been previously reported. This case report aimed to raise awareness of this unique manifestation and its potential implications.Case presentationA 58-year-old male with a history of type 2 diabetes, hypertension, and lumbar hernia presented with diarrhea. Lower gastrointestinal endoscopy revealed a tumor in the rectum, for which he was diagnosed with a well-differentiated adenocarcinoma. The patient underwent a laparoscopic Hartmann operation. After the operation, an entero-entero-fistula was identified at the sigmoid colostomy site. Subsequently, laparoscopic reconstruction of the colostomy was performed, and the patient had a favorable postoperative course without complications. Histopathological examination confirmed the localized absence of the muscularis propria in the resected colon, with fibrosis and nearby ganglion cells.ConclusionsThis case highlights the rarity of a colostomy fistula as a manifestation of SAIM and emphasizes the need to consider SAIM in the differential diagnosis for such cases. The presence of SAIM-affected lesions poses a risk of anastomotic failure, underscoring the importance of assessing the risk of complications during future surgeries. Surgeons should be aware of the etiology and potential implications of SAIM to ensure appropriate management and minimize postoperative morbidity. Further studies are warranted to explore the underlying mechanisms and optimize surgical strategies for patients with SAIM and its associated complications. Increased awareness among clinicians is crucial for timely diagnoses and tailored interventions to improve patient outcomes.

A novel segmental absence of intestinal musculature with small intestinal stenosis: a case report

BackgroundSegmental absence of intestinal musculature (SAIM) is a rare cause of intestinal obstruction and perforation due to partial or complete defects in the intestinal muscularis propria in neonates and is occasionally observed in adulthood.Case presentationThe first case of small intestinal stenosis derived from SAIM, which was difficult to differentiate from Crohn’s disease (CD), is reported. A 4-year-old girl presented with abdominal pain, anemia, and a positive fecal occult blood test. She was initially diagnosed with CD and started on treatment. Because her gastrointestinal symptoms persisted, her previous pediatricians tried to carry out capsule endoscopy, but it was not possible because the patency capsule was retained. Therefore, she was referred to our institute and re-evaluated. The patency capsule examination was repeated to re-evaluate small intestinal passage, but it stagnated again. Abdominal ultrasonography showed a poorly deformable intestinal tract that narrowed rapidly from the dilated segment and had a thin wall with an irregular laminar structure. In addition, unlike the typical ultrasonic CD findings, the power Doppler signal enhancement at the intestinal wall and “creeping fat sign” were not found. The patient was referred for laparoscopic observation to pediatric surgeons, who confirmed a prominently dilated intestinal tract 40 cm proximal to the ileocecal valve, which was resected. Histopathological findings showed longitudinal muscle hypoplasia of the resected, dilated intestinal tract and fat replacement of the muscle layer. At the stenosis site, the muscle layer was fibrotic and showed incomplete muscle arrangement. Because of these findings, she was diagnosed with SAIM. After the surgical treatment, no gastrointestinal symptoms relapsed, and the fecal occult blood test has remained negative for 2 years. Moreover, 8 months after surgery, double-balloon endoscopy showed no abnormalities, such as a longitudinal ulcer and cobblestone appearance.ConclusionsIn the present case, SAIM involved not only intestinal ileus and perforation, but also small intestinal stenosis. Although no other reports have demonstrated the usefulness of abdominal ultrasonography for the diagnosis of SAIM, the present report suggests that ultrasonography may be useful for differentiating SAIM from CD by close observation of the area around the small intestinal stenosis.

Microperforation due to segmental absence of intestinal musculature during endoscopic submucosal dissection forearlyesophageal cancer: a case study.

We report a case of a 75-year-old man found, during ESD for early esophageal cancer, with a segmental absence of intestinal musculature (SAIM) that caused a microperforation. A microperforation in the absence of muscle injury was encountered during an initial circumferential incision that was immediately closed with clips. The area of the SAIM was transparent, with portions of mediastinal organs seen through the submucosal layer, the movement of which was synchronized with respiration. The SAIM was subsequently completely closed prophylactically using clips. Our case shows that the rare existence of patients with SAIM should be considered when performing esophageal ESD, particularly if mediastinal organs are observed through a relatively transparent submucosal layer. The early detection of SAIM suggests this enabled us to avoid an esophageal perforation by dissecting the shallow submucosal layer. A microperforation during initial entry should alert experienced ESD endoscopists to the possibility of SAIM.

Segmental absence of intestinal musculature in an extremely low birthweight preterm infant

Abstract Background The segmental absence of intestinal musculature is a rare clinical entity, usually manifested in the neonatal period. It is more frequent in preterm infants, particularly in very low birthweight infants. Typically, there are intestinal perforation or intestinal obstruction symptoms. Case presentation The authors report a case of a 30-week-gestational age extremely low birthweight newborn who presented, on the fourth day of life, with a progressively acute abdomen and radiological findings suggestive of intestinal perforation. An emergency laparotomy with segmental ileal resection was performed; intestinal perforation was not confirmed. The histopathological examination of the resected distended bowel revealed an area of severe hypoplastic muscularis propria (with remaining layers intact). Conclusion Preoperative diagnosis of segmental absence of intestinal musculature is extremely difficult; its definitive diagnosis relies solely on the histopathological examination. The clinicians and pathologists should be aware of this rare condition, the treatment and prognosis of which differs from the more common necrotising enterocolitis.

Segmental absence of intestinal musculature with metachronous bowel perforations in an infant

Abstract Segmental absence of intestinal musculature is a rare condition. A female patient was born at 39 weeks gestational age with birth weight of 2,900 g. The patient was prenatally diagnosed as having segmental bowel distension in the fetal stage. She manifested bilious emesis with abdominal distension at day 1. Although excretion of viscous meconium was observed by gastrografin enema, gastrointestinal perforation developed. Emergency laparotomy and peritoneal drainage was required at that time and further laparotomy was performed on day 15. Multiple perforations were recognized discontinuously from the jejunum to the transverse colon, and jejunostomy was constructed. Additional bowel perforations occurred and re-exploration was required at day 43. We found newly formed small perforations in the proximal jejunum, ileum and the transverse colon and a tube jejunostomy and a colostomy were established. The patient required prolonged TPN management, which induced correlated cholestasis and liver failure, and died at day 143. Pathologic findings showed partial hypoplasia of the intrinsic muscle layer in the small intestine and diagnosed as segmental absence of intestinal musculature. Her disorder was unusual in its presentation, which included prenatal bowel dilatation, metachronous superimposed bowel perforation, and extensive discrete lesions from the jejunum to the transverse colon.

Segmental absence of intestinal musculature: A rare cause of intestinal obstruction in infants

Segmental absence of intestinal musculature (SAIM) is a rare entity of unknown cause. Presenting features vary from intestinal obstruction, necrotizing enterocolitis, volvulus, and intussusception to spontaneous intestinal perforations. In majority of the cases, SAIM is a histological diagnosis. Here, we present a case of a 1-month female baby who presented with fever, abdominal distension, and vomiting. Resected segment of intestine histologically showed focal segmental absence of the muscularis propria. To the best of our knowledge, fewer than 50 cases of SAIM have been reported in the literature so far.

A Case of Segmental Absence of Intestinal Musculature Causing Colonic Perforation

A Case of Segmental Absence of Intestinal Musculature Causing Colonic Perforation

Segmental absence of intestinal musculature concerning a child with acute appendicitis

Segmental absence of intestinal musculature (SAIM) is a rare histopathologic entity with few described cases, most of which in neonates. Rare reports of pediatric cases have been made. Pathogenesis is still uncertain. This report aims to highlight a pathologic condition that substantially affects the clinical evolution of an abdominal post-operative state as well as the therapeutic approach. We present the case of a 4-year old child, previously healthy, with a 4-day long intestinal obstruction. The exploratory laparotomy demonstrated peritonitis from a necrosed and perforated acute appendicitis. Post-operatory intestinal perforation led to a second laparotomy, with resection of a perforated ileal segment and one with vascular compromise. Pathologic analysis showed segmental absence of intestinal musculature of the small bowel. On a third operative time, resection of the macroscopically injured bowel segments and high protective enterostomy were made. A three years follow-up shows a healthy, well-developed child, who has already undergone enterostomy closure with no intercurrences. Etiology remains to be defined, possibly related to congenital or ischemic acquired defects. It usually manifests in neonates, but we present a case of a 4-year old child. Treatment consists of resection of the injured bowel segments. • The SAIM may be congenital and may only manifest after a triggering event such as an acute illness. • This diagnosis should be kept in mind during the laparotomy in order to avoid misdiagnosis. • Complete resection of intestinal segment with SAIM is curative.

Segmental absence of intestinal musculature: A rare case report

Segmental absence of intestinal musculature (SAIM) is a rare cause of intestinal obstruction and/or perforation seen in neonates, and rarely in adults. We present a case of SAIM in a 48-year-old male, who presented with acute abdominal symptoms and was repeatedly explored because of recurrence of abdominal symptoms.

Segmental Absence of Intestinal Musculature –Presentation in a 10-Year-Old Boy with an Extensive Involved Segment

Segmental absence of the intestinal musculature is a rare cause of acute abdomen most likely associated with intestinal perforation and sepsis in neonates and adults. We present a case of a 10-year-old boy who developed acute abdomen and was treated with right hemicolectomy and partial resection of the ileum. The ileum showed a 20-cm-long stenotic segment showing prominent thinning of the intestinal wall. This case occurred in an older child, which appears to be rare when comparing the literature. The bowel defect was also the largest described to date, indicating the potential effect of an atonic segment leading to obstructive disease.

Segmental absence of intestinal musculature: an increasingly reported pathology

BackgroundSegmental absence of the intestinal musculature (SAIM) is a known but clinically rare entity involving partial or complete absence of the intestinal muscularis propria. Clinical presentation is consistent with peritonitis, and treatment involves an emergent laparotomy, bowel resection, and reanastamosis or possible ostomy creation. Diagnosis results from histopathologic evaluation of the resected intestinal specimen. Most of the publications are case reports. MethodsWe retrospectively reviewed all surgical pathology files at a tertiary pediatric hospital from 2003 to 2010, identifying children who were noted to have absence of intestinal musculature on pathology. Patients meeting criteria were reviewed in detail, and data regarding clinical presentation, diagnostic testing, radiologic findings, treatment, and outcome were recorded. ResultsFive patients were identified between 2003 and 2010 who received the diagnosis of SAIM. Patient age ranged from 1 to 99 days of life. All children were born preterm by cesarean section, had a mean birth weight of 828 ± 338 g, and were intubated after birth. ConclusionsThe 5 patients presented are reviewed and contrasted with previous cases presented in the literature. Theories of pathogenesis and classification are discussed, and the cases are labeled as primary versus secondary SAIM.

Segmental absence of intestinal musculature with intestinal atresia and ileal perforation

Sir,Intestinal obstruction is a common cause for emergency surgical intervention in the newborn infant. Intestinal atresia accounts for about one-third of all cases of neonatal intestinal obstructi...

A case of congenital segmental absence of intestinal musculature

A case of congenital segmental absence of intestinal musculature

Segmental absence of intestinal musculature: An unusual cause of intestinal obstruction in the neonate

An unusual cause of small bowel obstruction in the neonate is presented. This rare congenital abnormality consists of segmental absence of the intestinal musculature with intact serosa and mucosa. This report represents the second known case of this abnormality. The etiology of this condition is unknown, and the diagnosis is apparently dependent upon laparotomy, transillumination of the bowel, and microscopic examination of the intestinal wall.

Neonatal intestinal obstruction due to absence of intestinal musculature: A new entity

Summary A neonate with intestinal obstruction was found to have a segmental absence of intestinal musculature. This entity is not found in the literature and is dependent upon laparotomy for diagnosis.