Pediatr Surg Int (2014) 30:579–586 DOI 10.1007/s00383-014-3504-z
ORIGINAL ARTICLE
Early prediction of complex midgut volvulus in neonates and infants Ilias Kanellos-Becker • Robert Bergholz • Konrad Reinshagen • Michael Boettcher
Accepted: 2 April 2014 / Published online: 23 April 2014 Ó Springer-Verlag Berlin Heidelberg 2014
Abstract Introduction Prognosis of midgut volvulus in neonates and infants younger than 1 year remains poor, as diagnostic findings may not be apparent until gut infarction had occurred. To characterize factors that help to predict complex midgut volvulus early was aim of this study. Methods Institutionally approved retrospective analysis of all children younger than 1 year treated for midgut volvulus at the author’s center from January 2002 to December 2011. Medical history, symptoms, laboratory and radiologic findings as well as sequelae of midgut volvulus were evaluated. Results In 10 years, 37 children fulfilled the inclusion criteria. Of these, 43 % developed complications, and mortality rate was 16 %. In 30 % of the patients, the only clinical sign was a sudden worsening of the general condition and abdominal distension (complex 19 % vs. simple 38 %). In one child with simple midgut volvulus, all clinical, laboratory and radiologic signs were negative. CART analysis identified a base excess below -1.70 and preterm birth (\36 weeks) as the best discriminators of complex and simple midgut volvulus. A score [1pt (comprised of these two factors) was found in all children with complex and in 14 % of simple midgut volvulus (p \ 0.001). A positive score ([1pt) offers a sensitivity of 100 % (81.7–100 %), specificity of 85.7 % (71.8–85.7 %), a PPV of 84.2 % (68.8–84.2 %) and NPV 100 % (83.8–100 %). Discussion The study shows that midgut volvulus has a substantial morbidity and mortality. Unfortunately, not all I. Kanellos-Becker R. Bergholz K. Reinshagen M. Boettcher (&) Department of Pediatric Surgery, University Hospital Hamburg-Eppendorf and Altona Children’s Hospital, Martinistrasse 54, 20246 Hamburg, Germany e-mail: [email protected]
affected children get picked up by history, laboratory and imaging. However, the proposed score helps to identify subject with increased risk of complications. It has the potential to facilitate and accelerate diagnosis of complex midgut volvulus; ultimately, it might help to reduce morbidity and mortality. Keywords Midgut volvulus Neonates Infants Predictors Complex
Background/Introduction High priority must be placed on early recognition of midgut volvulus to prevent ischemic loss of extensive bowel from strangulation. Severe bowel loss and intestinal failure result in long periods of hospitalization and possibly prolonged total parenteral nutrition. This is associated with substantial morbidity, which includes repeated episodes of septicemia, derangement of liver function and frequent admission to the hospital [1]. Intestinal and/or liver transplantation may be required. The quality of life of these patients and their family can be poor [2]. It is essential to identify midgut volvulus as early as possible to avoid subsequent morbidity and mortality [2]. About 75 % of all midgut volvulus cases occur within the first year of life, the majority of which are in the first month [3, 4]. It is associated with vomiting, abdominal distention and occasionally bloody stools. Bilious emesis in the full-term neonate should be considered as midgut volvulus until proven otherwise [3]. However, only 30 % of neonates with midgut volvulus present with initial bilious vomiting and 15 % with abdominal distention [3]. In most cases, laboratory findings and clinical signs are not apparent until gut infarction occurs [5, 6].
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580
Management of midgut volvulus is mainly surgical and includes the reduction in the twisted intestinal segments and division of peritoneal bands obstructing the duodenum (Ladd’s procedure) in patients with malrotation. To limit bowel resection, intestine of doubtful viability may be left in situ and a second look laparotomy or -scopy may be performed after 24–48 h in hope of recovery of the compromised tissue [7]. Recently, thrombolysis using tissue-type plasminogen after derotation of midgut volvulus to improve intestinal blood supply has been reported to be promising [2]. Yet despite all efforts, prognosis has not vastly improved in last decades. Morbidity and mortality remain high, especially in the newborn period [8]. The aim of this study was to characterize factors that help to predict midgut volvulus early in children younger than 1 year. Moreover, to identify signs, those warrant timely surgical exploration, as they might result in complications.
Pediatr Surg Int (2014) 30:579–586
became symptomatic. Recorded laboratory values included blood gas analysis (BGA), electrolytes, white blood count (WBC) and C-reactive protein (CRP) at various times. In all patients, intraoperative swabs for microbiology were taken. An experienced pediatric radiologist performed ultrasound studies and assessed features such as ‘‘whirlpool’’ sign [winding of the superior mesenteric vein (SMV) around the superior mesenteric artery (SMA)], obstruction and pneumatosis. Hanging X-rays were evaluated for gasless abdomen, air-fluid levels, gastric dilation and free air. On upper gastrointestinal contrast study, configuration of the duodenojejunal junction found below and to the right of the first lumbar pedicle in subjects with malrotation and the ‘‘spiral’’ sign (the twisted segment of small bowel has a characteristic corkscrew-like appearance) was assessed [9]. Subjects were divided into two groups—in complex (those with complications such as bowel resection, intestinal failure, prolonged parenteral nutrition and death) and simple midgut volvulus.
Materials and methods Statistics Study design An institutionally approved retrospective analysis of all patients’ explorative laparotomy for ‘‘midgut volvulus’’ at the Altona Children’s Hospital, University Medical Center Hamburg-Eppendorf, Germany, from January 2002 until December 2011 was performed. Methods Patients with a postoperative diagnosis of midgut volvulus were selected from the hospital database. Data were collected using patient charts, operating theater records, office notes as well as ICD-9 and CPT codes. Infants older than 1 year were excluded from the study. Data gathered for medical history included duration of symptoms, associated symptoms, previous episodes of symptoms, gestational week and birth weight, perinatal adaptation like APGAR score, umbilical cord pH, prenatal cardiotocography (CTG), known risk factors for volvulus (diaphragmatic hernia, omphalocele/gastroschisis, prune belly syndrome, intestinal atresia, meconium ileus, congenital heart disease, imperforate anus, annular pancreas, biliary atresia), operations, medication. Postoperatively time until feeding advancement, need for parental nutrition, time until discharge and death were documented. All participants had been examined physically by an attending of neonatology or pediatric surgery. Various aspects of the physical examination were recorded, including abdominal distension and guarding. Only laboratory features were included that were determined at admission or with 6 h after the subjects
123
Statistical analysis was performed using SAS 9.3 and SPSS 21.0. Differences between groups were calculated using Mann–Whitney test and Fisher’s exact test and are expressed by value as well as 95 % confidence intervals (CI) or means as well as standard deviation (SD). Univariate analysis was performed on preoperative variables comparing patients with complex to those without simple midgut volvulus. Continuous variables were partially converted to categorical variables. Finally, to identify the best predictors of midgut volvulus, regression tree analysis (CART) was performed. CART was chosen over multivariable regression analysis as it has the ability to utilize large numbers of predictor variables and offers non-reliance on the underlying distributions for statistical inference [10]. The level of significance was set at 0.05.
Results In 10 years, 49 patients with isolated midgut volvulus were treated. Of these, 37 met the inclusion criteria (75.5 %). Patient’s details are summarized in Table 1. Among the children with midgut volvulus, 21 patients had no complications (simple) and 16 developed complications (complex: 15 bowel resections, 6 prolonged intestinal failures, 6 deaths). About 32 % of the subjects had an underlying disease that is associated with midgut volvulus (2 patients with CDH, 8 with intestinal atresia, stenosis or duplication, 2 with meconium ileus). Children of the complex group were delivered significantly more often via C-section (p = 0.013), born earlier
Pediatr Surg Int (2014) 30:579–586
581
Table 1 Features All (n = 37) Male Gestational week Preterm birth (\36 weeks)
Simple volvulus (n = 21)
Complex volvulus (n = 16)
*p
73 % (45 %)
76 % (44 %)
69 % (48 %)
0.62
34.7 (5.6)
37.3 (4.5)
31.2 (5.0)
0.02
43 % (50 %)
14 % (36 %)
81 % (40 %)
ORIGINAL ARTICLE
Early prediction of complex midgut volvulus in neonates and infants Ilias Kanellos-Becker • Robert Bergholz • Konrad Reinshagen • Michael Boettcher
Accepted: 2 April 2014 / Published online: 23 April 2014 Ó Springer-Verlag Berlin Heidelberg 2014
Abstract Introduction Prognosis of midgut volvulus in neonates and infants younger than 1 year remains poor, as diagnostic findings may not be apparent until gut infarction had occurred. To characterize factors that help to predict complex midgut volvulus early was aim of this study. Methods Institutionally approved retrospective analysis of all children younger than 1 year treated for midgut volvulus at the author’s center from January 2002 to December 2011. Medical history, symptoms, laboratory and radiologic findings as well as sequelae of midgut volvulus were evaluated. Results In 10 years, 37 children fulfilled the inclusion criteria. Of these, 43 % developed complications, and mortality rate was 16 %. In 30 % of the patients, the only clinical sign was a sudden worsening of the general condition and abdominal distension (complex 19 % vs. simple 38 %). In one child with simple midgut volvulus, all clinical, laboratory and radiologic signs were negative. CART analysis identified a base excess below -1.70 and preterm birth (\36 weeks) as the best discriminators of complex and simple midgut volvulus. A score [1pt (comprised of these two factors) was found in all children with complex and in 14 % of simple midgut volvulus (p \ 0.001). A positive score ([1pt) offers a sensitivity of 100 % (81.7–100 %), specificity of 85.7 % (71.8–85.7 %), a PPV of 84.2 % (68.8–84.2 %) and NPV 100 % (83.8–100 %). Discussion The study shows that midgut volvulus has a substantial morbidity and mortality. Unfortunately, not all I. Kanellos-Becker R. Bergholz K. Reinshagen M. Boettcher (&) Department of Pediatric Surgery, University Hospital Hamburg-Eppendorf and Altona Children’s Hospital, Martinistrasse 54, 20246 Hamburg, Germany e-mail: [email protected]
affected children get picked up by history, laboratory and imaging. However, the proposed score helps to identify subject with increased risk of complications. It has the potential to facilitate and accelerate diagnosis of complex midgut volvulus; ultimately, it might help to reduce morbidity and mortality. Keywords Midgut volvulus Neonates Infants Predictors Complex
Background/Introduction High priority must be placed on early recognition of midgut volvulus to prevent ischemic loss of extensive bowel from strangulation. Severe bowel loss and intestinal failure result in long periods of hospitalization and possibly prolonged total parenteral nutrition. This is associated with substantial morbidity, which includes repeated episodes of septicemia, derangement of liver function and frequent admission to the hospital [1]. Intestinal and/or liver transplantation may be required. The quality of life of these patients and their family can be poor [2]. It is essential to identify midgut volvulus as early as possible to avoid subsequent morbidity and mortality [2]. About 75 % of all midgut volvulus cases occur within the first year of life, the majority of which are in the first month [3, 4]. It is associated with vomiting, abdominal distention and occasionally bloody stools. Bilious emesis in the full-term neonate should be considered as midgut volvulus until proven otherwise [3]. However, only 30 % of neonates with midgut volvulus present with initial bilious vomiting and 15 % with abdominal distention [3]. In most cases, laboratory findings and clinical signs are not apparent until gut infarction occurs [5, 6].
123
580
Management of midgut volvulus is mainly surgical and includes the reduction in the twisted intestinal segments and division of peritoneal bands obstructing the duodenum (Ladd’s procedure) in patients with malrotation. To limit bowel resection, intestine of doubtful viability may be left in situ and a second look laparotomy or -scopy may be performed after 24–48 h in hope of recovery of the compromised tissue [7]. Recently, thrombolysis using tissue-type plasminogen after derotation of midgut volvulus to improve intestinal blood supply has been reported to be promising [2]. Yet despite all efforts, prognosis has not vastly improved in last decades. Morbidity and mortality remain high, especially in the newborn period [8]. The aim of this study was to characterize factors that help to predict midgut volvulus early in children younger than 1 year. Moreover, to identify signs, those warrant timely surgical exploration, as they might result in complications.
Pediatr Surg Int (2014) 30:579–586
became symptomatic. Recorded laboratory values included blood gas analysis (BGA), electrolytes, white blood count (WBC) and C-reactive protein (CRP) at various times. In all patients, intraoperative swabs for microbiology were taken. An experienced pediatric radiologist performed ultrasound studies and assessed features such as ‘‘whirlpool’’ sign [winding of the superior mesenteric vein (SMV) around the superior mesenteric artery (SMA)], obstruction and pneumatosis. Hanging X-rays were evaluated for gasless abdomen, air-fluid levels, gastric dilation and free air. On upper gastrointestinal contrast study, configuration of the duodenojejunal junction found below and to the right of the first lumbar pedicle in subjects with malrotation and the ‘‘spiral’’ sign (the twisted segment of small bowel has a characteristic corkscrew-like appearance) was assessed [9]. Subjects were divided into two groups—in complex (those with complications such as bowel resection, intestinal failure, prolonged parenteral nutrition and death) and simple midgut volvulus.
Materials and methods Statistics Study design An institutionally approved retrospective analysis of all patients’ explorative laparotomy for ‘‘midgut volvulus’’ at the Altona Children’s Hospital, University Medical Center Hamburg-Eppendorf, Germany, from January 2002 until December 2011 was performed. Methods Patients with a postoperative diagnosis of midgut volvulus were selected from the hospital database. Data were collected using patient charts, operating theater records, office notes as well as ICD-9 and CPT codes. Infants older than 1 year were excluded from the study. Data gathered for medical history included duration of symptoms, associated symptoms, previous episodes of symptoms, gestational week and birth weight, perinatal adaptation like APGAR score, umbilical cord pH, prenatal cardiotocography (CTG), known risk factors for volvulus (diaphragmatic hernia, omphalocele/gastroschisis, prune belly syndrome, intestinal atresia, meconium ileus, congenital heart disease, imperforate anus, annular pancreas, biliary atresia), operations, medication. Postoperatively time until feeding advancement, need for parental nutrition, time until discharge and death were documented. All participants had been examined physically by an attending of neonatology or pediatric surgery. Various aspects of the physical examination were recorded, including abdominal distension and guarding. Only laboratory features were included that were determined at admission or with 6 h after the subjects
123
Statistical analysis was performed using SAS 9.3 and SPSS 21.0. Differences between groups were calculated using Mann–Whitney test and Fisher’s exact test and are expressed by value as well as 95 % confidence intervals (CI) or means as well as standard deviation (SD). Univariate analysis was performed on preoperative variables comparing patients with complex to those without simple midgut volvulus. Continuous variables were partially converted to categorical variables. Finally, to identify the best predictors of midgut volvulus, regression tree analysis (CART) was performed. CART was chosen over multivariable regression analysis as it has the ability to utilize large numbers of predictor variables and offers non-reliance on the underlying distributions for statistical inference [10]. The level of significance was set at 0.05.
Results In 10 years, 49 patients with isolated midgut volvulus were treated. Of these, 37 met the inclusion criteria (75.5 %). Patient’s details are summarized in Table 1. Among the children with midgut volvulus, 21 patients had no complications (simple) and 16 developed complications (complex: 15 bowel resections, 6 prolonged intestinal failures, 6 deaths). About 32 % of the subjects had an underlying disease that is associated with midgut volvulus (2 patients with CDH, 8 with intestinal atresia, stenosis or duplication, 2 with meconium ileus). Children of the complex group were delivered significantly more often via C-section (p = 0.013), born earlier
Pediatr Surg Int (2014) 30:579–586
581
Table 1 Features All (n = 37) Male Gestational week Preterm birth (\36 weeks)
Simple volvulus (n = 21)
Complex volvulus (n = 16)
*p
73 % (45 %)
76 % (44 %)
69 % (48 %)
0.62
34.7 (5.6)
37.3 (4.5)
31.2 (5.0)
0.02
43 % (50 %)
14 % (36 %)
81 % (40 %)
