A rare case of intestinal malrotation in pregnancy resulting in hemicolectomy postnatally

Summary

Introduction: Small and large bowel obstruction secondary to volvulus on the background of intestinal malrotation is a very rare occurrence, especially in pregnancy. This can be associated with significant feto-maternal morbidity and mortality. Case report: We report a case of a pregnant lady who developed symptoms of subacute intestinal obstruction during the 2nd trimester of pregnancy and was subsequently dia­gnosed with intestinal malrotation on imaging. Though she had symptoms of abdominal pain and constipation persisting for 9 long weeks during pregnancy, there was no evidence of definite intestinal obstruction or volvulus on her abdominal magnetic resonance imaging. She underwent a caesarean section at 34 weeks of pregnancy due to worsening abdominal pain. Postnatally, she was dia­gnosed with midgut volvulus on a computer tomography scan, leading to obstruction of both small and large bowels and needed an emergency laparotomy and right hemicolectomy. Conclusion: The case highlights the importance of timely dia­gnosis and prompt management of intestinal obstruction in pregnancy with a multidisciplinary team approach.

Keywords:

pregnancy – bowel obstruction – intestines – malrotation

Introduction

Intestinal malrotation, complicated by volvulus and intestinal obstruction has a very low prevalence in adults, but can have serious consequences in pregnancy. The dia­gnosis can be challenging as symptoms can mimic obstetric complications leading to delay in management.

We report a rare of case of symptomatic intestinal malrotation dia­gnosed during second trimester of pregnancy. The lady was managed conservatively throughout pregnancy, until she developed symptoms of volvulus and intestinal obstruction postnatally, requiring an emergency laparotomy and hemicolectomy. Timely dia­gnosis and appropriate decision on conservative or surgical management of intestinal obstruction is important to reduce the risk of maternal-fetal morbidity and mortality.

This case highlights the importance of a multidisciplinary team (MDT) approach in managing intestinal malrotation in pregnancy to achieve good maternal and fetal outcome. Rarity of these cases and lack of evidence-based consensus for management warrants an individualised care plan. The publication of each case report adds to the body of literature.

Case report

A 30-year-old lady, in her second pregnancy, presented to the hospital in the 25^th week of pregnancy with history of generalised abdominal pain, constipation for a couple of days and multiple episodes of vomiting. She was unable to tolerate oral intake. On examination, she had generalised abdominal tenderness and distention. The symphysis fundal height palpated appropriate for gestational age. She had no previous history of any abdominal surgery.

A surgical cause for acute abdomen was suspected and an urgent surgical opinion was requested. In view of symptoms of subacute intestinal obstruction, she was managed with intravenous fluids, analgesia, naso-gastric tube and was kept nothing by mouth. Abdominal magnetic resonance imaging (MRI) revealed presence of intestinal malrotation with small bowel predominantly on the right side and large bowel on the left side, with no overt signs of intestinal obstruction or volvulus. She was kept as an inpatient and managed conservatively under the joint care of obstetricians and general surgeons.

Following an MDT review, conservative management was continued. Soft diet was started gradually, as tolerated by the patient. Her symptoms improved and she had bowel movements once in three to four days with the help of laxatives. However, at 27 weeks of gestation, her abdominal pain started to worsen again, especially after food. A repeat MRI of the abdomen was performed, which showed no signs of intestinal obstruction. She was continued on conservative management and commenced on total parenteral nutrition with the involvement of a dietician. In view of ongoing symptoms of abdominal pain requiring strong analgesia, parenteral nutrition was continued. A repeat MRI at 31 weeks did not suggest any signs of intestinal obstruction either. Serial ultrasound growth scans were performed, which confirmed good fetal growth velocity. Fetal wellbeing was also ensured with daily cardiotocographs (CTG) starting from week 28 of pregnancy. Our perinatal mental health midwife offered continuous support to the lady to ensure her mental wellbeing during this prolonged hospitalisation.

In view of worsening abdominal pain, a shared decision was made for an elective caesarean section at 34 weeks with antenatal corticosteroid cover, in anticipation of improved bowel transit and improvement in bowel symptoms following delivery of the baby. A baby girl was delivered in good condition weighing 2,350 g following an uncomplicated caesarean section.

On the second day after the caesarean section, the lady developed severe abdominal pain. A computed tomography (CT) scan of her abdomen (Fig. 1) showed features of intestinal malrotation with intestinal obstruction at two separate points; mid transverse colon and distal ileum. This was associated with early signs of bowel ischaemia.

An emergency laparotomy was performed. Intraoperatively, congenital fibrous bands were noted extending from the root of the mesentery, going across the small bowel and encircling the mid-transverse colon, resulting in both small and large bowel obstruction (Fig. 2). Caecum, ascending colon and proximal transverse colon were grossly distended with early ischemic changes secondary to volvulus around the fibrous bands. The fibrous bands were divided, small bowel was dissected from the bands and released, relieving the small bowel obstruction. In view of ischemic changes to the proximal large bowel, right hemicolectomy was performed with en bloc removal of the caecum, ascending colon and proximal transverse colon. End ileostomy was performed. She received care in the intensive unit postoperatively, recovered well and was discharged 5 days later.

A postnatal review was performed at 8 weeks. A smooth recovery and an uneventful postpartum period were noted. Contraceptive advice was discussed with her and further mental health support was offered. She was reviewed by the general surgery team, and is awaiting reversal of the stoma.

Discussion

Intestinal malrotation is a congenital anomaly resulting from incomplete rotation of intestines around the axis of the superior mesenteric artery during fetal development. It affects 1 in 500 live births [1]. The vast majority of complications associated with intestinal malrotation present in the 1^st month of life [2]. Adult presentation is very rare accounting for only 0.2–0.5% of cases, of which only 15% present with midgut volvulus [3]. Most adult cases of intestinal malrotation remain undia­gnosed as these are usually asymptomatic. Intestinal malrotation can cause chronic, non-specific abdominal pain that is usually missed for months to years or can present with an acute abdomen if associated with volvulus and obstruction [4]. Hence, a few cases may be incidentally dia­gnosed in adulthood during radiological investigations or operative interventions for acute bowel obstruction [2,5].

Volvulus occurs when a loop of intestine twists around itself and the mesentery that supplies it, causing bowel obstruction and ischaemia. Symptoms include abdominal pain, distension, vomiting and constipation [6]. Though it is a very rare condition, the incidence is more common during pregnancy than in the general population. It has been reported to be responsible for up to 25% of acute bowel obstructions in pregnant women and 3–5% in non-pregnant patients [7,8]. Midgut volvulus typically presents in the second and third trimesters of pregnancy. This phenomenon may be explained by displacement of the bowels by a rapidly enlarging gravid uterus. Secondly, relaxin release during pregnancy increases tissue pliability. Both of these factors may thus predispose to occurrence of midgut volvulus in already susceptible individuals such as those with congenital malrotation or adhesions [9,10].

Complications arising from intestinal obstruction and volvulus can be life threatening. The mortality rate of midgut volvulus in pregnancy is significantly higher (3–15%) with respect to the general population [10]. A recently published article reviewed 11 cases of intestinal malrotation in pregnancy, nine of which were complicated by volvulus and two of these cases resulted in maternal death secondary to intestinal ischemia, peritonitis, and sepsis [11].

Pregnancy can often mask the symptoms of intestinal obstruction and delay the dia­gnosis, as symptoms of constipation commonly occur in pregnancy. Dia­g­­- nosis during pregnancy is often delayed due to difficulties with imaging in the presence of a gravid uterus and concerns regarding radiation exposure to the fetus [8]. Equally, the rarity of this condition in pregnancy makes it clinically challenging to dia­gnose it.

In patients with subacute intestinal obstruction without any features of peritonitis, intestinal ischemia and sepsis, symptomatic relief and supportive treatment such as gastrointestinal decompression, parenteral nutrition and antispasmodic medications can be continued. We managed our patient conservatively from 25 till 34 weeks of gestation, as there was no MRI evidence of intestinal obstruction or volvulus. Expectant management with serial fetal growth monitoring resulted in good maternal-fetal outcome avoiding extreme prematurity.

Due to worsening bowel symptoms a caesarean section was performed at 34 weeks. As symptoms continued to worsen even following delivery of the baby, intestinal obstruction was suspected clinically and this dia­gnosis was confirmed on CT scan. A midline laparotomy to explore the intestines could have been performed at the time of the caesarean section; however, at the time of the caesarean section there was no clinical suspicion or confirmed dia­gnosis of intestinal obstruction on imaging and the MDT felt that due to the risks involved with exploration, a midline incision was not indicated.

Since intestinal malrotation in pregnancy is very rare and there is no evidence-based consensus on management, each case requires an individualised care plan with MDT involvement. Obstet­ricians, general surgeons, radiologists, and dieticians, all have a vital role to play in the management of these patients to achieve a good maternal and fetal outcome.

Delay in dia­gnosis and management of intestinal obstruction can result in potentially life-threatening complications such as bowel perforation, peritonitis, sepsis and increased fetal and maternal morbidity and mortality [12]. Intestinal obstruction complicating pregnancy has been reported to result in maternal mortality of 6–20% and fetal mortality of 20–26% [10]. Hence, the basis of management of such cases lies in timely dia­gnosis of intestinal obstruction, early decision to discontinue conservative management and prompt surgical management to avoid serious complications.

The other important challenge in the management of intestinal obstruction during pregnancy lies in fulfilling the nutritional needs of a pregnant woman. Our patient could not tolerate oral intake and was on total parenteral nutrition for nine weeks during pregnancy. The parenteral nutrition needs alteration with advancing gestation in pregnancy and these patients need to be under the close monitoring of a dietician. With prolonged administration of parenteral nutrition, the possibility of developing metabolic complications such as electrolyte imbalance, micronutrient deficiencies, refeeding syndrome and line complications like thrombosis and phlebitis should always be considered [13]. Maternal undernutrition during pregnancy is also very likely to affect fetal growth and development [14]. Hence, it is important to monitor fetal growth with serial growth scans.

Another important but often ignored aspect in the management of these patients is the care of their mental health. Prolonged hospital admission during pregnancy, uncertainty over the dia­g­- nosis and outcome of the condition, concerns regarding the wellbeing of the baby, and complications associated with laparotomy and formation of a stoma, can all result in increased anxiety and depression and severely affect the quality of life in this group of patients [15]. It is vital for all healthcare providers involved to be supportive and mindful of the emotional and mental wellbeing of these patients. Our patient was under the care of our perinatal mental health midwife who provided invaluable support during her inpatient stay.

Conclusion

Intestinal obstruction secondary to midgut volvulus has a higher incidence in pregnancy than the general population. Clinical presentation of subacute intestinal obstruction can be mistaken for pregnancy-related symptoms.

Early dia­gnosis and appropriate management of intestinal obstruction is important to reduce the risk of maternal-fetal morbidity and mortality. Dia­gnosis of intestinal obstruction can be difficult in pregnancy due to challenges with clinical presentation and dia­gnostic imaging. Women with clinical suspicion of intestinal obstruction in pregnancy should have an urgent MRI to dia­gnose and determine the aetiology of intestinal obstruction.

Symptomatic intestinal malrotation can be managed conservatively if there is no evidence of intestinal obstruction or volvulus until delivery as demonstrated in our case. Lack of evidence-based consensus on management of malrotation and volvulus in pregnancy raises the importance of an MDT approach and individualised care plan.

Case comes from the Department of Obstetrics and Gynaecology, Barnsley Hospital NHS Foundation Trust, United Kingdom.

ORCID authors

S. Kaur 0000-0003-4694-2426

P. Cai 0000-0002-7879-6120

Submitted/Doručeno: 27. 12. 2022

Accepted/Přijato: 20. 2. 2023

Simar Kaur, MRCOG

Department of Obstetrics and Gynaecology

Barnsley Hospital NHS Foundation Trust

Gawber Road

S75 2EP Barnsley

United Kingdom

simar12@gmail.com, simar.kaur1@nhs.net