Fetal midgut volvulus is quite rare, and most cases are associated with abnormalities of intestinal rotation or fixation. We report a case of midgut volvulus without malrotation, associated with a meconium pellet, during the gestation period. This 2.79 kg, 33-wk infant was born via a spontaneous vaginal delivery caused by preterm labor. Prenatal ultrasound showed dilated bowel loops with the appearance of a ‘coffee bean sign’. This patient had an unusual presentation with a distended abdomen showing skin discoloration. An emergency laparotomy revealed a midgut volvulus and a twisted small bowel, caused by complicated meconium ileus. Such nonspecific prenatal radiological signs and a low index of suspicion of a volvulus during gestation might delay appropriate surgical management and result in ischemic necrosis of the bowel. Preterm labor, specific prenatal sonographic findings (for example, the coffee bean sign) and bluish discoloration of the abdominal wall could suggest intrauterine midgut volvulus requiring prompt surgical intervention.
Midgut volvulus; Coffee bean sign; Meconium ileus
Two of 15 children who survived neonatal meconium ileus had “meconium ileus equivalent.” They were treated with hydration, pancreatic enzyme therapy and antibiotics. One of these children died from pulmonary disease at the time of the bowel obstruction.
The survival rate of infants with meconium ileus is steadily improving because of prompt operative intervention, better preoperative and postoperative care and long-term treatment with enzyme supplements and antibiotics. Late intestinal obstruction due to adhesive bands, volvulus, intussusception or “meconium ileus equivalent” may occur in children previously treated for meconium ileus of infancy.
The omission of pancreatic enzyme supplementation and the occurrence of respiratory infections are frequently associated with “meconium ileus equivalent.”
In this series of patients four of the infants treated surgically for neonatal meconium ileus died in the early postoperative period.
The results of surgery for meconium ileus obstruction (37 cases) at Queen Elizabeth Hospital for Children, London, during the years 1953 to 1970 are presented. The surgical survival was 30% in the years 1953 to 1961, and 70% in the years 1962 to December 1970, inclusive. The long-term survival was 57%. Complications, such as small bowel atresia, gangrene, and meconium peritonitis, were associated with a higher mortality. The adoption of the Bishop Koop operation in 1962 coincided with the increase in the survival rate. An association between meconium ileus and hypertrophic pyloric stenosis was noted.
Various surgical methods are used to treat meconium ileus (MI), including resection with enterostomy (RES), primary anastomosis (RPA), and purse-string enterotomy with intra-operative lavage (PSI). The aim of this study is to discuss the surgical treatment of MI, based on our experience.
Patients and methods
Of the 41 MI patients treated at our institution between 1984 and 2007, 18 had simple MI and 23 had complex MI. These groups were analyzed according to treatment modality, concentrating on length of hospital stay, complications [peritonitis, septicemia, adhesive small bowel obstruction (ASBO), and malabsorption/diarrhea], need for additional surgical procedures, mortality.
Of the 18 patients with simple MI, 7 (39%) were successfully treated with diluted Gastrografin® enema. The remaining 11 patients were treated surgically: two underwent RPA, of whom one died; five had RES, of whom one developed ASBO; four underwent PSI, of whom two developed peritonitis. In the complex MI group, 14 patients underwent RPA, with peritonitis occurring in three (one died); nine underwent RES, of whom two developed ASBO.
In patients with simple MI, conservative treatment with diluted Gastrografin® enema is an effective initial treatment in our hands. In case of failure, RES is advisable. Patients with complex MI are candidates for RES. RPA and PSI seem to have higher complication rates.
Meconium ileus; Meconium peritonitis; Intestinal atresia; Cystic fibrosis; Diatrizoate; Gastrografin®
A case of giant meconium pseudocyst secondary to ileum volvulus perforation is presented. Conventional radiographic features of meconium peritonitis with secondary meconium pseudocyst formation are well described. Our case is unusual in comparison to other cases reported in the literature and needs to be reported because the meconium pseudocyst presented without the typical ultrasound features (calcifications, polyhydramnios and ascites) and was initially identified as an abdominal mass.
We describe the case of a 29-year-old Caucasian woman in her third trimester of pregnancy, in which an abdominal mass was detected in the fetus. The newborn was diagnosed in the early neonatal period with meconium pseudocyst secondary to ileum volvulus perforation.
The prenatal appearance of a meconium pseudocyst can be complemented by other signs of bowel obstruction (if present) such as polyhydramnios and fetal bowel dilatation. This is an original case report of interest to all clinicians in the perinatology and fetal ultrasound field. We consider that the utility of this case is the recognition that a meconium pseudocyst might appear without the typical ultrasound features and should be considered as a differential diagnosis when an echogenic intra-abdominal cyst is seen.
A mass of inspissated meconium in the distal colon or rectum is a relatively common cause of neonatal intestinal obstruction. The meconium-plug syndrome is unrelated to cystic fibrosis and meconium ileus. The clinical picture is frequently indistinguishable, without contrast study of the colon, from other forms of mechanical intestinal obstruction requiring laparotomy. A barium enema examination is almost always diagnostic, and use of this procedure usually results in dislodgement and passage of the plug.
No single cause for the excessive viscosity and tenaciousness of the obstructive meconium mass has been identified. Previous reports have generally emphasized the normal ganglion-cell content of the colon in affected patients.
Two infants are described who fulfilled all criteria for this syndrome but who were not rendered asymptomatic, as normally anticipated, by removal of the plug. Subsequent studies revealed the presence of Hirschsprung's disease in both patients. This diagnosis should be considered when an infant with meconium-plug obstruction of the colon fails to follow the usual satisfactory clinical course after the plug has been passed.
Volvulus of the intestine has recently been observed in three patients with idiopathic steatorrhea in relapse. Two patients gave a history of intermittent abdominal pain, distension and obstipation. Radiographic studies during these attacks revealed obstruction at the level of the sigmoid colon. Reduction under proctoscopic control was achieved in one instance, spontaneous resolution occurring in the other. The third patient presented as a surgical emergency and underwent operative reduction of a small intestinal volvulus. Persistence of diarrhea and weight loss postoperatively led to further investigation and a diagnosis of idiopathic steatorrhea. In all cases, treatment resulted in clinical remission with a coincident disappearance of obstructive intestinal symptoms. The pathogenesis of volvulus in sprue is poorly understood. Atonicity and dilatation of the bowel and stretching of the mesentery likely represent important factors. The symptoms of recurrent abdominal pain and distension in idiopathic steatorrhea necessitate an increased awareness of intestinal volvulus as a complication of this disease.
Eleven cases of neonatal intestinal obstruction associated with a white ileal meconium plug are described; 6 of these presented with complications--such as, giant meconium pseudocyst, perforation, volvulus, or atresia. Most of these complications are presumed to have arisen during the intrauterine period. Only one patient could be relieved of the ileal meconium plug by enemas. The condition of ileal meconium plug is not as benign as a meconium plug in the rectum or distal colon. A plea is made to restrict the name meconium plug syndrome to cases in which the meconium plug is white and chalky and the consequent intestinal obstruction can be relieved by enemas, without evidence of intestinal dysfunction in later life.
Five neonates with uncomplicated meconium ileus were successfully managed by laparotomy and T tube ileostomy. This method seems to offer advantages over other surgical techniques used in the management of uncomplicated meconium ileus that fails to respond to decompression by Gastrografin enema.
Acute sigmoid volvulus is typically caused by an excessively mobile and redundant segment of colon with a stretched mesenteric pedicle. When this segment twists on its pedicle, the result can be obstruction, ischemia and perforation. A healthy, 18-year-old Caucasian woman presented to the emergency department complaining of cramping abdominal pain, distention, constipation and obstipation for the last 72 h, accompanied by nausea, vomiting and abdominal tenderness. The patient had tympanitic percussion tones and no bowel sounds. She was diagnosed with acute sigmoid volvulus. Although urgent resective surgery seems to be the appropriate treatment for those who present with acute abdominal pain, intestinal perforation or ischemic necrosis of the intestinal mucosa, the first therapeutic choice for clinically stable patients in good general condition is considered, by many institutions, to be endoscopic decompression. Controversy exists on the decision of the time, the type of definitive treatment, the strategy and the most appropriate surgical technique, especially for teenagers for whom sigmoid resection can be avoided.
Acute sigmoid volvulus; Conservative treatment
We report a case of ileal atresia with meconium peritonitis evaluated by fetal MRI. Prenatal ultrasounds in the third trimester initially demonstrated a cystic abdominal mass that resolved with development of dilated bowel loops. Fetal MRI at 32 weeks gestation identified a perihepatic collection with several dilated small bowel loops and normal sized meconium filled rectosigmoid consistent with distal bowel perforation and loculated meconium peritonitis. Following delivery, the infant presented with bowel obstruction. Contrast enema revealed a normal sized rectosigmoid with small ascending and transverse colon. A distal ileal atresia type IIIa was documented at surgery.
Ileal atresia; meconium peritonitis; fetal abdominal masses; fetal MRI
Dilated fetal bowel is a sonographic finding that is associated to meconium ileus, a feature of cystic fibrosis (CF). Prenatal diagnosis of CF is possible through analysis of the cystic fibrosis transmembrane regulator gene mutations.
A male infant is described, who was referred to our Prenatal Diagnosis Center a 17th week of gestation with a dilated bowel loop on a prenatal scan. Amniocentesis was performed at 23rd week gestation and a homozygous F508del mutation was found. He was born at 38 weeks gestation, after an otherwise unremarkable pregnancy, and admitted to Neonatal Intensive Care Unit. He showed progressive abdominal distension without stools and was transferred to another Hospital to surgery. A total occlusion of terminal ileum with meconium and a microcolon were found, and resection of 8 cm of ileum and an ileostomy were performed.
The characteristic sonographic finding of a dilated bowel is an indication to search for CF mutations.
An oral intestinal lavage solution has been successfully used in the treatment of six patients with chronic distal intestinal obstruction syndrome (previously referred to as meconium ileus equivalent) complicating cystic fibrosis and a further case of recurrent small bowel obstruction. The patient with recurrent obstruction is unusual in having no evidence of pancreatic maldigestion, which previously has been considered a prerequisite for the syndrome.
Meconium abnormalities are characterized by a wide spectrum of severity, from the meconium plug syndrome to the complicated meconium ileus associated with cystic fibrosis. Meconium Related Ileus in absence of Cystic Fibrosis includes a combination of highly viscid meconium and poor intestinal motility, low grade obstruction, benign systemic and abdominal examination, distended loops without air fluid levels. Associated risk factors are severe prematurity and low birth weight, Caesarean delivery, Maternal MgSO4 therapy, maternal diabetes. In the last 20 yrs a new specific type of these meconium related obstructions has been described in premature neonates with low birth weight. Its incidence has shown to increase while its management continues to be challenging and controversial for the risk of complicated obstruction and perforation.
Materials and methods
Among 55 newborns admitted between 1992-2008 with Meconium Related Ileus as final diagnosis, data about Low Birth Weight infants (LBW < 1500 g) were extracted and compared to those of patients ≥ 1500 g. Hischsprung's Diseases and Cystic Fibrosis were excluded by rectal biopsy and genetic probe before discharge. A softening enema with Gastrografin was the first option whenever overt perforation was not present. Temporary stoma or trans appendiceal bowel irrigation were elected after unsuccessful enema while prompt surgical exploration was performed in perforated cases. NEC was excluded in all operated cases. Data collected were perinatal history and neonatal clinical data, radiological signs, clinical course and complications, management and outcome.
30 cases with BW ≥ 1500 g had an M/F ratio16/14, Mean B.W. 3052 g, Mean G.A. 37 w Caesarean section rate 40%. There were 10 meconium plug syndrome, 4 small left colon syndromes, and 16 meconium ileus without Cystic Fibrosis. Five cases were born at our institution (inborn) versus 25 referred after a mean of 2, 4 Days (1-7) after birth in another Hospital (outborn). They were managed, after a Gastrografin enema with 90% success rate, by 1 temporary Ileostomy and 2 trans appendiceal irrigation. 25 cases with BW< 1500 g (LBW) had M/F ratio 11/14, Mean B.W. 818 g, Mean G.A. 27 w, Caesarean section rate 70%, assisted ventilation 16/25. There were 8 inborn and 17 outborn. Gastrografin enema was successful in 6 out 8 inborn infants only, all referred within one week from birth. There were 12 perforations mainly among late referred LBW outborn.
Meconium Related Ileus without Cystic Fibrosis responds to conservative management and softening enema in most of mature infants. In LBW clinical course is initially benign but as any long standing bowel obstruction management may present particular challenges. Clinical and plain radiographic criteria are reliable for making diagnosis and testing for Cystic Fibrosis may not be indicated. Enema may be resolutive when performed in a proper environment. Perforated cases may be confused with NEC which is excluded by clinical history, no signs of sepsis, lab signs missing, abdominal signs missing, typical radiological signs missing. The higher complication rate is recorded among cases delivered and initially managed in Neonatal Units without co-located Surgical Facilities. Early diagnosis and aggressive medical therapy may lead to higher success rate and help avoiding surgical interventions. Surgical therapy in uncomplicated cases, unresponsive to medical management, should be minimally aggressive.
Volvulus of the small bowel, although rare, carries a high risk of strangulation and ischemic necrosis. It is usually caused by the rotation of a loop of small intestine around an adhesion band or stoma. We present a case of an anterior gastropexy band, giving rise to a small bowel volvulus, necessitating resection due to infarction. This band resulted from separation of the most distal anterior gastropexy suture from the anterior abdominal wall. The distensible nature of the stomach and its resultant post-prandial gain in weight produced tremendous shearing forces on the gastropexy sutures, and, as our case demonstrates, a greater number of gastropexy sutures does not protect against this complication.
Volvulus; Small bowel; Anterior gastropexy; Laparoscopy
Intestinal malrotation occurs when there is a disruption in the normal embryological development of the bowel. The majority of patients present with clinical features in childhood, though rarely a first presentation can take place in adulthood. Recurrent bowel obstruction in patients with previous abdominal operation for midgut malrotation is mostly due to adhesions but very few reported cases have been due to recurrent volvulus. We present the case of a 22-year-old gentleman who had laparotomy in childhood for small bowel volvulus and then presented with acute bowel obstruction. Preoperative computerised tomography scan showed small bowel obstruction and features in keeping with midgut malrotation. Emergency laparotomy findings confirmed midgut malrotation with absent appendix, abnormal location of caecum, ascending colon and small bowel. In addition, there were small bowel volvulus and a segment of terminal ileal stricture. Limited right hemicolectomy was performed with excellent postoperative recovery. This case is presented to illustrate a rare occurrence and raise an awareness of the possibility of dreadful recurrent volvulus even several years following an initial Ladd’s procedure for midgut malrotation. Therefore, one will need to exercise a high index of suspicion and this becomes very crucial in order to ensure prompt surgical intervention and thereby preventing an attendant bowel ischaemia with its associated high fatality.
Gut volvulus; Intestinal malrotation; Acute bowel obstruction; Computerised tomography scan; Laparotomy
The introduction of the Malone procedure has improved the outlook for children with severe faecal incontinence. Phosphate and saline enemas are administered through the exteriorized appendix in antegrade fashion to achieve evacuation and ensure cleanliness. The appendix functions as a non-refluxing catheterizable channel: If it is not available for use, a tubularized caecal flap is a safe alternative. We have constructed Malone stomas using the appendix in 20 patients and another seven patients have undergone the caecal flap modification. The mean age was 8.6 years. Eleven of the patients were boys and 16 (59%) were girls. Six children required dilatation or revision of their stomas for stenosis. One developed small bowel obstruction and another has stopped using the stoma. The results of the continence enemas were considered to be very good by the vast majority of patients and their carers. Our recent experience suggests that bisacodyl may be a valuable adjunct to the antegrade enemas of phosphate and saline. We believe that this procedure may be extended with benefit to adults with serious faecal incontinence in whom standard measures have failed.
Cecal volvulus is uncommon in pediatric patients and there are few reports of cecal volvulus with cerebral palsy. Here, we report the case of a 19-year-old male patient who presented with abdominal distension, a history of cerebral palsy, refractory epilepsy due to lissencephaly, and chronic constipation. An abdominal x-ray and computed tomography without contrast enhancement showed fixed dilated bowel intensity in the right lower abdomen. Despite decompression with gastric and rectal tube insertion, symptoms did not improve. The patient underwent an exploratory laparotomy that revealed cecal volvulus. Cecal volvulus usually occurs following intestinal malrotation or previous surgery. In this patient, however, intestinal distension accompanying mental disability and chronic constipation resulted in the development of cecal volvulus. We suggest that cecal and proximal large bowel volvulus should be considered in patients presenting with progressive abdominal distension combined with a history of neuro-developmental delay and constipation.
Cecum; Inteseinal volvulus; Lissencephaly
A 75-year old male presented to the emergency room with worsening abdominal pain and distension. Plain radiographs were suggestive of a large bowel obstruction due to volvulus. An attempt to detorse the volvulus and decompress the colon endoscopically failed, after which the patient was taken for an exploratory laparotomy. A transverse colon volvulus was found, and an extended right hemicolectomy and ileostomy was performed. We discuss the diagnosis and management of transverse colon volvulus and review the pertinent literature.
Chronic idiopathic intestinal pseudo-obstruction (CIIP) is a rare syndrome of ineffectual gut motility associated with clinical, endoscopic and radiological exclusion of mechanical causes, as well as evidence of air–fluid levels in distended bowel loops. A case of small bowel volvulus in a patient with an established diagnosis of CIIP is presented. The case is illustrated by images of operative findings and computed tomography scan reconstruction, showing the classical appearances of small bowel volvulus. The patient recovered well after surgery and is maintained on parenteral nutrition. CIIP is a heterogeneous disorder in which the primary aims of management are nutrition, pain control and the avoidance of unnecessary repeated laparotomies. However, even in the presence of an established diagnosis of CIIP, surgeons should be vigilant to the possibility that an operable mechanical obstruction may still occur.
Fetal intestinal volvulus is a rare life-threatening condition. Late diagnosis of volvulus contributes to high rate of morbidity and mortality. It has variable degrees of presentation and survival. Intrauterine volvulus may be complicated by intestinal atresia due to ischemic necrosis. To our knowledge, there are three reported cases of term fetal demise. We report a case of fetal intestinal volvulus with perinatal survival of the largest term infant described with this complication to date. The volvulus was associated with type 3A jejunal atresia and intestinal pathology was noted on prenatal ultrasound. The infant was born via urgent cesarean delivery at 376/7 weeks of gestation and underwent emergent exploratory laparotomy with resection of small bowel and primary end-to-end anastomosis. Intrauterine intestinal volvulus may be suspected on prenatal ultrasound but only definitively diagnosed postnatally. Signs of fetal distress and volvulus are rarely associated with reports of survival in the term fetus. We review reported cases of prenatally suspected volvulus in infants documented to survive past the neonatal period. As fetal volvulus and most intestinal atresias/stenoses manifest during the third trimester, we recommend that the limited fetal anatomical survey during growth ultrasounds at 32 to 36 weeks routinely include an assessment of the fetal bowel.
fetal intestinal volvulus; perinatal mortality; intussusception; intrauterine volvulus
Intestinal obstruction due to sigmoid volvulus during pregnancy is rare. The presenting signs/symptoms seen in these patients are the same as with non-pregnant patients. Fetal and maternal mortality rates are higher during pregnancy due to delays in diagnosis. We aimed to present four patients diagnosed with sigmoid volvulus during pregnancy and puerperium in our clinic. Diagnosis requires a high index of suspicion in a patient who presents with complaints of abdominal pain and evidence of bowel obstruction. Prompt intervention is necessary to minimize maternal and fetal morbidity and mortality.
Large-bowel volvulus is a rare cause of bowel obstruction in the industrialized world. We analyzed the presentation and outcome of 49 patients at the Princess Alexandra Hospital, Brisbane, Australia, who received a diagnosis of colonic volvulus from 1991 to 2001.
A retrospective chart study was carried out.
Twenty-nine patients had sigmoid volvulus (59%), 19 patients had cecal volvulus (39%) and 1 patient had a transverse colon volvulus (2%). The diagnosis of sigmoid volvulus was made accurately on plain abdominal radiography or contrast enema in 90% of cases (n = 26), compared with only 42% of cases (n = 8) of cecal volvulus. Twenty-two patients with sigmoid volvulus were treated initially with endoscopic decompression. The success rate was 64% (n = 14). There was a high early recurrence rate of sigmoid volvulus for those treated by endoscopic decompression alone (43%) during a mean period of 32 days. Of the 14 patients with cecal volvulus who were treated with right hemicolectomy, 12 had primary anastomosis and 2 had end ileostomy with mucous fistula formation. There was no anastomotic leak following right hemicolectomy with primary anastomosis, even though 6 of these patients had an ischemic cecum.
Endoscopic decompression of the sigmoid volvulus was safe and effective as an initial treatment but has a high early recurrence rate. Any patient who is fit enough to undergo operation should have a definitive procedure during the same admission to avoid recurrence. Cecal volvulus is associated with a higher incidence of gangrene and is treated effectively by right hemicolectomy with or without anastomosis. The need for swift operative intervention is emphasized.
Meconium ileus at birth, distal intestinal obstruction syndrome (DIOS), and constipation are an interrelated group of intestinal obstruction syndromes with a variable severity of obstruction that occurs in cystic fibrosis patients. Long-term follow-up studies show that today meconium ileus is not a risk factor for impaired nutritional status, pulmonary function, or survival. DIOS and constipation are frequently seen in cystic fibrosis patients, especially later in life; genetic, dietary, and other associations have been explored. Diagnosis of DIOS is based on suggestive symptoms, with a right lower quadrant mass confirmed on abdominal radiography, whereas symptoms of constipation are milder and of longer standing. In DIOS, early aggressive laxative treatment with oral laxatives (polyethylene glycol) or intestinal lavage with balanced osmotic electrolyte solution and rehydration is required, which now makes the need for surgical interventions rare. Constipation can generally be well controlled with polyethylene glycol maintenance treatment.
Abdominal radiography; Anthropometric variables; Body mass index; Coefficient of fat absorption; Constipation; Cystic fibrosis transmembrane regulator; Cystic fibrosis; Distal intestinal obstruction syndrome; Fat malabsorption; Height; Nutritional status; Meconium ileus; Meconium ileus equivalent; Modifier genes; Pulmonary function; Steatorrhea; Spirometry; Survival; Weight
Internal hernias are a rare cause of bowel obstruction in the neonate and present with bilious vomiting. Newborns may be at risk of loss of significant length of bowel if this rare condition is not considered in the differential diagnosis of bilious emesis.
PRESENTATION OF CASE
We report a case of a twin with an internal hernia through a defect in the ileal mesentery who presented with neonatal bowel obstruction. The patient had a microcolon on the contrast enema suggesting that the likely etiology was an intra-uterine event most likely a vascular accident that prevented satisfactory meconium passage into the colon.
An internal hernia is rarely considered in the differential diagnosis of distal bowel obstruction in a neonate with a microcolon. Congenital trans-mesenteric hernias constitute only 5–10% of internal hernias. True diagnosis of trans-mesenteric hernias is difficult due to lack of specific radiology or laboratory findings to confirm the suspicion.
When clinical and radiological findings are not classical, rare possibilities such as an internal hernia must be considered in the differential diagnosis, to avoid catastrophic bowel loss.
Internal hernia; Congenital mesenteric defect; Neonatal bowel obstruction