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Mesenteric hernia in VLBW infants

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Mesenteric hernia causing bowel obstruction in very low-birthweight infants Katsuya Hirata,1 Hisayoshi Kawahara,2 Nobuko Shiono,1 Masahiro Nishihara,1 Akio Kubota,2 Masahiro Nakayama3 and Hiroyuki Kitajima1 Departments of 1Neonatal Medicine, 2Pediatric Surgery and 3Clinical Laboratory Medicine and Anatomic Pathology, Osaka Medical Center and Research Institute for Maternal and Child Health, Izumi, Osaka, Japan Abstract

Internal hernia through a mesenteric defect, called mesenteric hernia, is an uncommon cause of acute intestinal obstruction in newborns. Strangulated mesenteric hernia results in intestinal necrosis or perforation and progressive deterioration with fatal outcome, especially when it occurs in low-birthweight infants. We report two very lowbirthweight (VLBW) infants, who presented with acute intestinal obstruction related to mesenteric defect. The initial diagnosis was meconium obstruction in those cases, which is a common cause of bowel obstruction occurring in VLBW infants. Correct diagnosis of mesenteric hernia was difficult in these cases because of rapid deterioration and non-specific radiological findings. Awareness of the possibility of rare mesenteric hernia causing acute intestinal obstruction and surgical intervention in an appropriate timeframe are important for rescuing VLBW infants with such organic abnormalities.

Key words internal hernia, meconium obstruction, mesenteric hernia, premature infant, very low-birthweight infant.

Very low-birthweight (VLBW) infants occasionally present with functional ileus, such as meconium obstruction and intestinal motor dysfunction related to immaturity.1 Intestinal obstruction caused by functional or organic mechanisms is difficult to diagnose in this type of patient. Acute intestinal obstruction caused by internal hernia, such as a herniation of a viscus through a mesenteric defect, is a rare condition, but may result in serious intestinal damage, especially when it occurs in VLBW infants. We describe intestinal obstruction caused by mesenteric hernia that occurred in VLBW infants.

drainage was performed effectively on day 9 of age, he died of septic shock on day 22 of age, which was not directly caused by mesenteric hernia. Autopsy showed herniation of most of the small intestine through a 1.5 cm defect of the mesentery located at the end of the ileum. The strangulated segment of the intestine circumscribed by the defect had ischemic color change, but no characteristic pathological finding such as intravascular microthrombus, ulcer, or erosion of mucosa were detected. Perforation of the intestine was found at 5 cm proximal to the ileocolic valve close to the defect. Ileal perforation bordering the defect was considered attributable to mesenteric hernia (Fig. 1).

Case reports Case 1

A 548 g male infant was born at 22 weeks 5 days of gestation by vaginal delivery. His Apgar scores were 1 and 7 at 1 and 5 min, respectively. He was intubated, given surfactant, and placed on mechanical ventilation in the delivery room. Feeding intolerance with bilious emesis and remarkable abdominal distension were observed on day 9 of age. The patient’s condition acutely deteriorated, leading to shock, and laboratory data were as follows: white blood cell count, 33 200/mm3; platelet count, 7.5 × 104/ mm3; and metabolic acidosis, pH 7.188 and base excess −11.8. Plain abdominal radiograph showed free air in the abdominal cavity, suggesting perforative peritonitis. Although urgent Correspondence: Katsuya Hirata, MD, Department of Neonatal Medicine, Osaka Medical Center and Research Institute for Maternal and Child Health, 840 Murodo-cho, Izumi, Osaka 594-1101, Japan. Email: [email protected] Received 26 October 2013; revised 8 April 2014; accepted 30 April 2014. doi: 10.1111/ped.12409

Case 2

A 1084 g male infant was born at 28 weeks 4 days of gestation by vaginal delivery at home and was transferred to the neonatal intensive care unit immediately after resuscitation. He was treated with surfactant replacement and mechanical ventilation. Coarctation of the aorta and a large ventricular septal defect were diagnosed with an initial echocardiogram. Prostaglandin E1 was used to keep the ductus arteriosus patent, and palliative bilateral pulmonary artery banding was performed on day 10 of age. Contrast enema using sixfold diluted gastrografin was performed because of delayed meconium excretion and advanced abdominal distension on day 10 of age, which effectively relieved abdominal symptoms. His course was uneventful after re-introduction of enteral feeding on day 12 of age until the recurrence of feeding intolerance and abdominal distension on day 31 of age. His condition acutely deteriorated, with elevated C-reactive protein at 1.04 × 105 μg/L and marked metabolic acidosis with a pH of 6.982, and base excess of −19.9. Abdominal X-ray showed pneumatosis intestinalis in the ascending colon (Fig. 2a). Urgent laparotomy was performed on day 31 of age with the suspicion of © 2015 Japan Pediatric Society

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Fig. 1 Photograph at autopsy in case 1, showing herniation of nearly the entire small intestine through a 1.5 cm defect of the mesentery located at the end of the ileum (○).

necrotizing enterocolitis. Operative findings showed that most of the ileum was herniated through a 3 cm mesenteric defect located close to the ileocolic valve (Fig. 2b). The intestinal tract from the end of the ileum to the ascending colon showed ischemic color change. Given that resection of the ascending colon was thought to be too invasive for his poor condition, a stoma was placed in the ileum. He died of feeding intolerance and liver dysfunction with portal hypertension after a long fasting period and repeated infection on day 127 of age.

Fig. 2 (a) Plain abdominal X-ray and (b) intraoperative photograph of patient 2. (a) Pneumatosis intestinalis in the ascending colon (▲); (b) most of the ileum was herniated through a 3 cm mesenteric defect located close to the ileocolic valve.

Discussion A major cause of intestinal obstruction in VLBW infants is functional obstruction, such as meconium obstruction: intestinal motor dysfunction primarily related to immaturity.1 In those infants, organic mechanisms, such as internal hernia or bowel atresia, are extremely uncommon causes of intestinal obstruction and only a few sporadic cases have been reported.2,3 The correct diagnosis of whether a functional or organic pathogenesis underlies intestinal obstruction is important, although such obstruction presents with undistinguishable symptoms in VLBW infants. Strangulated mesenteric hernia is a rare cause of intestinal obstruction in pediatric patients. The incidence of mesenteric hernia reported in a series of autopsies by Shaffner and Pennell was 0.2–0.9%.4 The exact pathogenesis of the mesenteric defect remains unclarified, although many hypotheses have been addressed, including regression of the dorsal mesentery, developmental enlargement of a hypovascular area, rapid lengthening of a segment of mesentery, and compression of the mesentery by the colon during fetal midgut herniation into the yolk sac.5 © 2015 Japan Pediatric Society

Although lethal intestinal herniation through congenital mesenteric defects may have a congenital etiology, most of the reported cases occurred in adulthood and the presentation in childhood is uncommon.2,5–9 Tang et al. reviewed 44 children with internal hernias, and showed that congenital internal hernias presented in two distinct age groups.6 While older children with internal hernias often present with non-specific abdominal pain, neonates and infants present acutely with abdominal distension and vomiting, usually bilious in nature. The current two patients presented acutely, and preoperative diagnosis was not made due to rarity, non-specific clinical presentation, and similarity of symptoms to meconium functional obstruction. To our knowledge, patient 1 is the smallest infant with mesenteric hernia to be reported. Fragile intestine of prematurity and existence of internal hernia may have increased the risk of perforation. In patient 2, the fact that an ischemic lesion was detected in the ascending colon, not in the herniated

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Mesenteric hernia in VLBW infants intestine, suggests that strangulation of the bowel itself is not the only cause of ischemic changes in internal hernias. Pressure of the herniated bowel loops and their mesentery compresses the vessels in the free margins of the defect and may cause ischemic changes. This pathological condition may be similar to that observed in the testicle at the time of the intestinal incarceration of inguinal hernia even when the intestine is not necrotic. The delay of diagnosis of organic intestinal obstruction leads to a fatal outcome in VLBW infants, although such infants also tend to develop functional ileus related to meconium plugs.1 We should consider the possibility of an internal hernia when VLBW infants present with acute intestinal obstruction. The usefulness of computed tomography of internal hernia has been reported,10 although whether it can be applied to VLBW infants is controversial. A suspicion of possible rare conditions causing intestinal obstruction and surgical intervention in appropriate timeframe are mandatory to rescue VLBW infants with a strangulated mesenteric hernia.

Acknowledgments The authors are grateful to all the children and parents who participated in this report. The authors also thank the medical staff working in the neonatal intensive care unit at Osaka Medical Center and Research Institute for Maternal and Child Health.

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References 1 Garza-Cox S, Keeney SE, Angel CA, Thompson LL, Swischuk LE. Meconium obstruction in the very low birth weight premature infant. Pediatrics 2004; 114: 285–90. 2 Malit M, Burjonrappa S. Congenital mesenteric defect: Description of a rare cause of distal intestinal obstruction in a neonate. Int. J. Surg. Case Rep. 2012; 3: 121–3. 3 Oretti C, Bussani R, Janes A, Demarini S. Multiple segmental absence of intestinal musculature presenting as spontaneous isolated perforation in an extremely low-birth-weight infant. J. Pediatr. Surg. 2010; 45: E25–7. 4 Shaffner Lde S, Pennell TC. Congenital internal hernia. Surg. Clin. North Am. 1971; 51: 1355–9. 5 Page MP, Ricca RL, Resnick AS, Puder M, Fishman SJ. Newborn and toddler intestinal obstruction owing to congenital mesenteric defects. J. Pediatr. Surg. 2008; 43: 755–8. 6 Tang V, Daneman A, Navarro OM, Miller SF, Gerstle JT. Internal hernias in children: Spectrum of clinical and imaging findings. Pediatr. Radiol. 2011; 41: 1559–68. 7 Ming YC, Chao HC, Luo CC. Congenital mesenteric hernia causing intestinal obstruction in children. Eur. J. Pediatr. 2007; 166: 1045–7. 8 Garignon C, Paparel P, Liloku R, Lansiaux S, Basset T. Mesenteric hernia: A rare cause of intestinal obstruction in children. J. Pediatr. Surg. 2002; 37: 1493–4. 9 Fujita A, Takaya J, Takada K et al. Transmesenteric hernia: Report of two patients with diagnostic emphasis on plain abdominal X-ray findings. Eur. J. Pediatr. 2003; 162: 147–9. 10 Blachar A, Federle MP, Dodson SF. Internal hernia: Clinical and imaging findings in 17 patients with emphasis on CT criteria. Radiology 2001; 218: 68–74.

Pott’s puffy tumor in a 12-year-old boy Gülhadiye Avcu,1 Nursen Belet,1 Senem Cengel Kurnaz,2 Arzu Karli1 and Gülnar Sensoy1 Pediatric Infectious Disease and 2Otorhinolaryngology, Ondokuz Mayis University, Samsun, Turkey

1

Abstract

Pott’s puffy tumor (PPT) is a rare complication of sinusitis characterized by subperiosteal abscess and osteomyelitis of the frontal bone. Early diagnosis and treatment is vital before it causes intracranial complications such as subdural empyema or brain abscess. Herein we describe the case of a 12-year-old patient who developed preseptal cellulitis and PPT, and was successfully treated with abscess drainage, sinus surgery and long-term antibiotic therapy.

Key words frontal osteomyelitis, Pott’s puffy tumor, sinusitis.

Pott’s puffy tumor (PPT) is defined as subperiosteal abscess accompanied by osteomyelitis after frontal sinusitis.1 PPT was first described by Sir Percivall Pott in 1775. It may develop after a complication of acute or chronic sinusitis, trauma or insect bite rarely.1 The most common symptoms are headache, periorbital edema, fever, vomiting, lethargy and swelling in the Correspondence: Gülhadiye Avcu, MD, Pediatric Infectious Disease, Ondokuz Mayıs University, Kurupelit/Samsun, Samsun, Turkey. Email: [email protected] Received 26 February 2014; revised 28 April 2014; accepted 30 April 2014. doi: 10.1111/ped.12440

frontal region.2 PPT is more common in adolescents but it can also be seen in elderly people.2 PPT is a risk factor for intracranial complications such as subdural empyema and brain abscess, and intracranial complications are more common in children than adults. Intracranial complications have been reported in 60–85% of all PPT cases.3 Early diagnosis and treatment are vital for the prevention of serious complications. In this paper, we describe the case of a 12-year-old male patient who was hospitalized with a pre-diagnosis of preseptal cellulitis, which was then changed to a diagnosis of PPT on follow up, to emphasize the importance of PPT, a rare complication of sinusitis, in differential diagnosis. © 2015 Japan Pediatric Society

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Mesenteric hernia causing bowel obstruction in very low-birthweight infants.

Internal hernia through a mesenteric defect, called mesenteric hernia, is an uncommon cause of acute intestinal obstruction in newborns. Strangulated ...
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