Arlettaz Mieth, R

Bode, P K

Moehrlen, U

Ramos y Muñoz, G

ZORA

Unnecessary laparotomy due to radiological misinterpretationSWISS SOCIETY OF NEONATOLOGYJUNE 20112Ramos y Muñoz G, Moehrlen U, Bode P, Arlettaz Mieth R, Clinic of Neonatology (RMG, AMR), University Hospital Zurich, Division of Surgery (MU), University Children‘s Hospital Zurich, Division of Pathology (BP), University Hospital Zurich© Swiss Society of Neonatology, Thomas M Berger, Webmaster   3A male infant was born in a level I hospital at 40 2/7 weeks of gestation by caesarean section due to patho-logical cardiotocogram. The mother was a 36-year-old G4/P4. The family history was unremarkable. The three older children (29, 18 and 13 years of age) were re-portedly healthy. The pregnancy had been uneventful, except for non-insulin-dependent gestational diabetes. The newborn had an umbilical arterial cord-pH of 7.24, base excess of -4 mmol/L and adapted well with an Apgar score of 9, 9 and 10 at 1, 5, and 10 minutes respectively.The initial postnatal course was uneventful with passage of meconium and normal breast feeding. However, at the age of 24 hours, passage of hard stool was obser-ved and the baby developed abdominal distension and had one episode of bilious vomiting. He was transfer-red to the NICU at the University Hospital in Zurich for further investigations. On admission at the age of 36 hours, the baby appea red well and was afebrile. Physical examination revealed a tensely distended abdomen with reduced intestinal pe-ristalsis. Livid-reddish periumbilical discoloration and a putrid smell from the umbilicus were observed. The baby had mild tachypnea without further signs of respi-ratory distress and without episodes of apneas. Blood pressure was normal. Blood gas showed normal values, lactate was slightly elevated at 3.5 mmol/l, C-reactive protein was 8 mg/L and leukocyte count was normal. CASE REPORT4At that point, the leading diagnoses were Hirschsprung‘s disease or meconium ileus. Shortly thereafter, however, a conventional ap abdominal radiograph was perfor-med and revealed dilated bowel loops, the presence of stool, and intestinal pneumatosis indicative of necroti-zing enterocolitis (NEC) (Fig. 1). The X-ray was not seen by a radiologist, but it was interpreted by both surgeons and neonatologists.After consulting with the pediatric surgeons, it was decided to treat this presumed case of NEC conserva-tively. Oral feedings were discontinued, a nasogastric tube was inserted and parenteral nutrition was started. After obtaining blood cultures, antibiotic therapy with amoxicillin/clavulanic acid and gentamycin was started. The clinical course over the next few hours was stable. There were no stools, no rectal bleeding, and only one episode of bilious vomiting. Six hours later, follow-up abdominal x-rays were obtained (Fig. 2, 3). On these images, the diagnosis of NEC became less likely as the air bubbles appeared to be mixed with stool. The baby became more uncomfortable with increasing abdominal distension and was transferred to the Univer-sity Children‘s Hospital of Zurich for an exploratory lapa-rotomy. Intraoperatively, there were markedly distended loops of bowel but no evidence of NEC. Hirschsprung‘s disease was now felt to be the likely diagnosis. No further surgical interventions were performed but the stool was intraoperatively mobilized by enemas. Later, a 5series of contrast studies of the small and large bowel showed a decreased recto-sigmoid diameter suggestive of Hirschsprung’s disease (Fig. 4).Following intra- and postoperative enemas, lar-ge amounts of stool were passed, followed by rapid clinical improvement. Enteral feeding with breast milk was started and well tolerated. Normal defecation was achieved by repeated dilatation of the anal canal with Hegar dilators. One week later, aganglionosis was de-monstrated on a colorectal mucosal biopsy confirming the diagnosis of Hirschsprung’s disease (Fig. 5). The baby was discharged home 2 weeks later. The final sur-gical resection of the abnormal recto-sigmoid section via pull-through procedure was successfully performed at the age of 6 weeks.6Fig. 1Abdominal X-ray at the age of 37 hours: possible intramural air in some bowel loops (asterisk). *  *  7Fig. 2Abdominal X-ray at the age of 43 hours: dilated loops of bowel and air bubbles which are not restricted to the bowel wall.A         8Fig. 3Lateral abdominal X-ray at the age of 43 hours without evidence of perforation. 9Fig. 4B        Contrast study of the colon at the age of 9 days: nar-rowing in the region of the rectosigmoid.10Fig. 5Histopathology of Hirschsprung‘s disease: A) Affected segment: multiple nerve fibers in the lamina propria with increased acetylcholinesterase activity (arrows); B) Normal colon without increased acetylcholineste-rase activity (histochemical reaction for acetylcholi-nesterase on frozen sections); C) Affected segment: Absence of submucosal ganglion cells; D) Normal colon with normal distribution of ganglion cells in the submucosa (arrows) (histochemical reaction for nitric oxide synthase on frozen sections).A                             B                    C                             D                    11Hirschsprung‘s disease is caused by the absence of intramural ganglion cells in the lower digestive tract leading to a developmental disorder of the enteric nervous system. It is characterized by the interaction of multiple genes and manifests with low, sex-depen-dent penetrance and variability in the configuration of the aganglionic segment (1). In Hirschsprung‘s disease, the neural crest stem cells, which migrate along the intestine to establish the myenteric and submucous plexus, abort their migration during the embryonic development. This leads to a dysfunction of the organization of parasympathetic fibers, which grow into the distal colon. Gut overactivity with con-tinuous contraction of the bowel due to a sustained release of acetylcholine is the result (2).The incidence of Hirschsprung’s disease is reported to be about 1:5‘000 live births in both hemispheres (3). It com-monly presents in the newborn period sometimes with life-threatening bowel obstruction requiring surgery.The most prevalent gastrointestinal emergency in newborns is due to NEC which affects mostly extre-mely preterm infants. Without doubt, symptoms of Hirschsprung-associated enterocolitis (HAEC) and neonatal enterocolitis can be similar (4). Newborn infants with Hirschsprung’s disease presenting soon after birth are at greater risk of developing postope-rative HAEC. HAEC is associated with significant peri-operative morbidity and mortality. Several studies on DISCUSSIONA                             B                    12HAEC have been published, however, the pathogene-sis of this condition remains unclear (5).In the neonate, pneumatosis intestinalis is almost al-ways associated with NEC (6). Intestinal pneumatosis can be difficult to recognize on abdominal x-ray, or can be confounded with less relevant findings, like the presence of small gas bubbles within the stool filled bowel lumen, as was the case in our patient. Additio-nal investigation with abdominal ultrasound can help to detect intestinal perforation related to neonatal en-terocolitis. It has been shown that abdominal ultra-sound is superior to plain radiography in identifying portal venous gas and echogenic free fluid as early signs of intestinal perforation (7). Our patient developed signs and symptoms of ileus at the age of 24 hours. Although Hirschsprung‘s disease was felt to be the likely diagnosis based on history and clinical findings, radiological misinterpretation of intestinal pneumatosis lead to a diagnosis of NEC and explorative laparotomy. Early repetitive enemas would probably have led to rapid clinical amelioration and avoidance of surgery.See also COTM 03/2005: Intestinal perforation in Hirschsprung‘s disease.13REFERENCES     1. Tam PK, Garcia-Barceló M. Genetic basis of Hirschsprung’s  disease. Pediatr Surg Internat 2009;25:543-5582. Feichter S, Meier-Ruge WA, Bruder E. The histopathology of    gastrointestinal motility disorders in children. Semin Pediatr    Surg 2009;18:206-2113. Kenny SE, Tam PK, Garcia-Barceló M. Hirschsprung‘s disease.    Semin Pediatr Surg 2010;19:194-2004. Raboei EH. Necrotizing enterocolitis in full-term neonates:  is it aganglionosis? Eur J Pediatr Surg 2009;19:101-1045. Lacher M, Fitze G, Helmbrecht J, et al. Hirschsprung- associated enterocolitis develops independently of NOD2  variants. J Pediatr Surg. 2010; 45:1826-18316. Hsieh WS, Yang PH, Huang CS, Wang CR. Hirschsprung‘s    disease presenting with diffuse intestinal pneumatosis in a    neonate. Acta Paediatr Taiwan 2000;416:336-3387. Dilli D, Suna Oguz S, Erol R et al. Does abdominal sonography   provide additional information over abdominal plain  radiography for diagnosis of necrotizing enterocolitis in  neonates? Pediatr Surg Int 2011; 273:321-327ˇACKNOW-LEDGEMENTWe thank T. Schraner, MD, Department of Dia-gnostic Imaging, Children’s University Hospital, Zu-rich, for the images of the contrast study (Fig. 4).Swiss Society of Neonatologywww.neonet.chwebmaster@neonet.chCONTACTSUPPORTED BYconcept & design by mesch.ch

Unnecessary laparotomy due to radiological misinterpretation

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Unnecessary laparotomy due to radiological misinterpretation

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