Submitted on: 05/20/2018 Approved on: 08/07/2018 CASE REPORT

Intestinal malrotation: a diagnosis to consider in acute in newborns Antônio Augusto de Andrade Cunha Filho1, Paula Aragão Coimbra2, Adriana Cartafina Perez-Bóscollo3, Robson Azevedo Dutra4, Katariny Parreira de Oliveira Alves5

Keywords: Abstract Intestinal obstruction, is an anomaly of the , resulting from an embryonic defect during the phases of herniation, Acute abdomen, rotation, and fixation. The objective is to report a case of complex diagnostics and approach. The diagnosis was made . surgically in a patient presenting with hemodynamic instability, abdominal distension, signs of intestinal obstruction, and on abdominal X-ray, with suspected grade III necrotizing enterocolitis. During surgery, a resulting from poor intestinal rotation was found at a distance of 12 cm from the ileocecal valve. Hemodynamic instability and abdominal distension recurred, and another exploratory was required to correct new intestinal perforations. Therefore, early diagnosis with surgical correction before a volvulus appears is essential. Abdominal Doppler ultrasonography has been promising for early diagnosis.

1 Academic in Medicine - Federal University of the Triângulo Mineiro - Uberaba - Minas Gerais - Brazil 2 Resident in Pediatric Intensive Care - Federal University of the Triângulo Mineiro - Uberaba - Minas Gerais - Brazil 3 Associate Professor - Federal University of the Triângulo Mineiro - Uberaba - Minas Gerais - Brazil. 4 Adjunct Professor - Federal University of the Triângulo Mineiro - Uberaba - Minas Gerais - Brazil 5 Academic in Medicine - Federal University of the Triângulo Mineiro - Uberaba - Minas Gerais - Brazil

Correspondence to: Antônio Augusto de Andrade Cunha Filho. Universidade Federal do Triângulo Mineiro, Acadêmico de Medicina - Uberaba - Minas Gerais - Brasil. Rua: Frei Paulino, 30 - Nossa Sra. da Abadia, Uberaba - MG, CEP: 38025-180. E-mail: [email protected] / [email protected]

Residência Pediátrica 2018;8(3):141-146. DOI: 10.25060/residpediatr-2018.v8n3-08

141 INTRODUCTION contrast enema may be ordered to visualize the , whose position near DJJ suggests IMR. Abdominal ultrasonography Intestinal malrotation (IMR) is an anomaly of the mid- has a lower sensitivity and specificity than the tests previously gut, resulting from an embryonic defect during the phases mentioned. In cases of neonates with a history of of herniation, rotation, and fixation. It may present as non- and peritonitis, laparotomy can be performed after initial -rotation, incomplete rotation, reverse rotation, or mesocolic measurements are taken for diagnosis and treatment, even 1 . without a radiological diagnosis.4,5 The condition affects 0.2%–1% of the population and is At diagnosis, patients generally need urgent surgical 2 symptomatic in 1/2500–6000 of cases. In 30%–60% of cases, treatment due to the high risk of the disease. However, the it is associated with other malformations and diseases such recommended treatment in asymptomatic patients remains as , Meckel’s diverticulum, intussusception, controversial.5 Surgical correction was described by Ladd in Hirschsprung’s disease, mesenteric cyst, anomalies of the ex- 1936. The procedure is based on counterclockwise correc- trahepatic biliary ducts, congenital heart disease, congenital tion of the rotation of the volvulus, dividing the Ladd bands, diaphragmatic hernia, and abdominal wall defects (omphalo- lengthening the mesenteric bundle, and positioning the small cele and ). intestine in the lower right quadrant and the Embryological development of the intestine is complex. in the upper left quadrant.6 Laparoscopic correction was Initially, there is intestinal herniation outside the abdominal described by Zee and Bax in 1995 and is similar to the open cavity, with one counterclockwise rotation (270°) in relation surgery.7 Laparoscopy has been used in stable patients, whe- to the axis of the superior mesenteric . At approximately reas the open technique is preferred when there are volvulus 12 weeks of gestation, the midgut returns to the abdominal and related complications.8-10 Cecal appendectomy, which is cavity and the –jejunal junction (DJJ) attaches to recommended by many authors for IMR treatment, is contro- the posterior wall of the abdomen, with its left side on the versial because many others contraindicate it.9 vertebral column and the Treitz ligament, while the cecum is 3 affixed to the lower right quadrant. IMR results from failure CASE REPORT in extracelomic intestinal rotation, often with DJJ located in the upper right quadrant and the cecum in the upper abdo- We present the case of a 22-year-old mother, gravida 2 men. This anomalous fixation results from adhesive bands in para 1. The firstborn was a healthy 5-year-old girl at the time the gallbladder, duodenum, and right abdominal wall. This of the second pregnancy. Prenatal consultations did not show produces a narrow mesenteric base, which is predisposed to complications, and obstetric ultrasonography results perfor- intestinal volvulus. Less often, the intestine makes one clo- med 5 days before delivery were normal. The grandfather ckwise rotation (90°), bringing the duodenum forward and the reported that the patient went to the emergency room on June colon backward, forming a tunnel which can partially obstruct 24, 2016, after her water broke at 26 weeks, and was diagno- the mesenteric vessels.4 sed with premature amniotic rupture. Tocolysis was initiated IMR classically manifests in newborns as vomiting and a bed for the newborn was requested in the neonatal bile. Volvulus occurs in the mesenteric base, causing the intensive care unit (NICU). Lack of fetal heartbeat was noted superior mesenteric vessels to twist. Continuous and intrauterine fetal death was diagnosed. Labor was then causes symptoms such as hematochezia, irritability, pain, and induced and the fetus was expelled at 11:00 am (6/26/2016). abdominal distension. It can progress to intestinal necrosis, According to the mother, the newborn had a gestational which manifests as abdominal erythema, signs of peritonitis, age of 26 weeks and weighed 915 g; the newborn had no hear- , and death. Older children may present chronic tbeat or fetal movements. Death was confirmed; the newborn volvulus, with abdominal pain (colic), abdominal distension was placed in a box, where he remained for 2 hours. However, with intermittent vomiting, diarrhea, gastrointestinal bleeding, the nursing team noticed respiratory movements and alerted and malnutrition.5 the medical team, which began resuscitation and intensive Prenatal diagnosis of isolated IMR is difficult and is care. After resuscitation, the newborn was transferred to the usually accomplished by ultrasonographic observation of pediatric emergency room of a tertiary university hospital, complications of the volvulus (intestinal distension, meco- where he was admitted at 7:00 pm (6/26/2016). nial peritonitis, and ascites). After birth, the key symptom is On admission to the emergency room, the newborn vomiting bile. In general, the first test that is performed is a was in poor overall condition, with cold and cyanotic extremi- simple X-ray of the abdomen, which shows signs of intestinal ties, oxygen saturation of 85% in ambient air, rhonchi and rales obstruction, which, in severe cases, is accompanied by intes- on auscultation, heart rate of 130 bpm, and heart murmur, and tinal pneumatosis, which suggests necrotizing enterocolitis. was administered oxygen by a hood. Orotracheal intubation Currently, the gold standard for IMR diagnosis is gastroin- (OTI) was performed, followed by aspiration and surfactant ad- testinal contrast to assess the position of DJJ. In ministration (200 mg/kg); capillary glycemia was checked (low) doubtful cases, serial contrast X-rays of the and and 2 mL of 10% glucose was prescribed. Antibiotic therapy

Residência Pediátrica 2018;8(3):141-146. 142 was initiated with ampicillin, sulbactam, and gentamycin for early neonatal sepsis, and dobutamine was initiated due to distributive shock. The child was transferred to the NICU. Serum test results on admission were AST = 488.5 U/L, ALT = 89.2 U/L, total bilirubin = 2.768 mg/dL, indirect = 2.201 mg/dL and direct = 0.567 mg/dL, CK = 443 U/L, CKMB = 426 U/L, hemoglobin = 12.00 g/dL, leukocyte count = 28250 cells/mm3 (2% myelocytes, 6% metamyelocytes, 17% rods, 66% segmented, 1% eosinophils, 0% basophils, 23% lymphocytes, and 10% monocytes), non-reactive se- rology (toxoplasmosis, rubella, syphilis, cytomegalovirus, HIV, and HTLV), negative bacterial blood culture, and slight bilateral reticulogranular infiltrate and normal abdomen on radiography of the thorax and abdomen Figure 1. Sub- sequently, echocardiography conducted on June 28, 2016, showed 1.8-mm PCA; transfontanellar ultrasonography on June 30, 2016, showed ventricular dilation and absence of bleeding.

Figure 2. Simple X-ray of the abdomen at day 8: pneumoperitoneum (note rugby-ball shape).

On July 04, 2016, the patient developed hemodyna- mic instability and abdominal distension. Simple abdominal X-ray showed intestinal obstruction, pneumoperitoneum, and signs of grade III necrotizing enterocolitis Figure 2. Laparotomy was then indicated. The procedure revealed peritonitis with intestinal perforation associated with the midgut volvulus from IMR at a distance of 12 cm from the ileocecal valve and signs of ischemia, but no necrosis. Perforation suturing was formed along with cleaning of the abdominal cavity, excision of Ladd’s adhesions between the duodenum and transverse colon and the angle of the , appendectomy, repositioning of the bowel loops, and closing of the planes and the supraumbilical transverse incision on the right Figures 3 to 6. After recovery, the patient again developed hemo- dynamic instability and abdominal distension. Another simple X-ray of the abdomen showed pneumoperitoneum Figure 7; therefore, another exploratory laparotomy was performed (7/13/2016). This time, two perforations were revealed, one 20 cm from the duodenum near the site of Figure 1. Simple X-ray of the thorax and abdomen at admission. the lysis of adhesions from the first surgery and another

Residência Pediátrica 2018;8(3):141-146. 143 at the site of the previous suture, which was blocked by intestinal loops. There was also duodenal semi-occlusion by bilious residue. The upper perforation was sutured, and 5 cm of the loop at the site of the second perforation was sectioned with termino-terminal anastomosis. There were no signs of peritonitis. The infant developed distributive shock and coagu- lation disorder in the immediate postoperative period. He was diagnosed with cystic hygroma in the parietal region on transfontanellar ultrasonography (7/21/2016). An electro- cardiogram (7/26/2016) showed right chamber overload. A dilated eye exam (7/29/2016) showed avascular peripheral retina. The patient developed pulmonary pneumonia asso- ciated with broncodysplasia (8/01/2016). He also required blood transfusions due to anemia (8/04/2016). Elective extubation was attempted (8/11/2016), and the patient was kept on non-invasive ventilation (NIV), but he deve- loped apnea, hypothermia, and bradycardia, and OTI was again required. With a diagnosis of late sepsis and positive blood cultures for Serratia marcescens, the patient was treated for pneumonia (8/16/2016). Elective extubation was performed (8/24/2016) with the patient maintained on NIV, but reintubation was necessary on 8/25/2016 due to respiratory distress and chest X-ray showing total ate- lectasis of the right lung. The patient had hemodynamic instability requiring vasoactive drugs and treatment for sepsis with positive blood cultures for Staphylococcus haemolyticus. He was treated for bacterial conjunctivitis (9/04/2016, 9/19/2016, and 10/15/2016). A dilated eye exam (9/09/2016) sho- wed stage 2 retinopathy of prematurity, Zone III, which Figure 3. A - Patient state during anesthetic induction. Note the discrepancy was treated with laser photocoagulation (9/09/2016 between the size of the hands of the anesthesiologist and that of the newborn; and 9/23/2016). Elective extubation was performed B - Patient in the immediate postoperative period. again (9/12/2016), with worsening respiratory pattern (9/21/2016) and reintubation. The patient also underwent bilateral herniorrhaphy with orchidopexy (9/22/2016). He was definitively extubated (10/15/2016) and maintained a good respiratory pattern on NIV, despite respiratory stridor and chest X-ray showing atelectasis on the right lung. On 10/19/2016, he was transferred from the ICU to the ward in good general condition, breathing spontaneously in am- bient air, weighing 2310 g and using a nasogastric catheter, with good progression to oral diet. Echocardiography was conducted again (10/03/2016), with the following results: POF 2.5 mm, PCA 2.5 mm, and normal ejection fraction; electroence- phalography (11/08/2016) showed epileptiform activity in the right middle temporal region and diffuse disorganized pattern. The patient progressed uneventfully in the ward, the nasogastric catheter was removed (11/10/2016), and the infant was discharged (11/11/2016). At the time of this Figure 4. Intestinal perforation at a distance of 12 cm from the ileocecal valve with report, he is doing well and followed up at the outpatient . clinic after discharge from the ICU.

Residência Pediátrica 2018;8(3):141-146. 144 Figure 5. Illustration of the type of intestinal malrotation found according to the classification by Grosfeld (1992).

Figure 6. Photo of bowel loops after suturing the perforation in the terminal and reversing the volvulus resulting from intestinal malrotation.

COMMENTS IMR requires surgical correction before volvulus occurs, but diagnosis rarely occurs during this period. Abdominal Doppler ultrasonography permits locating the superior mesenteric vessels and checking the position of the vein in relation to the artery, which has shown promi- sing results for early diagnosis. Figure 7. Control simple X-ray of the abdomen at 8 days post-procedure.

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