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478
E Türk et al.
Venobiliary fistula related to umbilical venous catheter in a newborn Erdal Türk,1 Ramazan Soylar,2 Tulay Akca,2 Selim Serter3 and I˙rfan Karaca1 Departments of 1Pediatric Surgery, 2Neonatology Intensive Care Unit and 3Radiology, Faculty of Medicine, Izmir University, Izmir, Turkey Abstract
We present a case of venobiliary fistula due to umbilical venous catheter (UVC). UVC was inserted the day before surgery in a newborn who was scheduled for type IIIB jejunal atresia surgery. The UVC was superimposed on the liver. It was noted that the gastric drainage became chylous and increased to 790 and then 1977 mL daily. I.v. contrast tomography with 650 mL contrast showed that the opaque substance was dispersed around the catheter and a venobiliary fistula formed, with the administered fluid accumulating in the duodenum. Rapid improvement was seen in the clinical picture after the UVC was removed. Venobiliary fistula may develop in patients with UVC that is not placed appropriately, and can direct the fluid administered from the UVC to the gastrointestinal system through the choledochal duct. The importance of contrast computed tomography in the diagnosis of venobiliary fistula in the newborn is also emphasized.
Key words type IIIB jejunal atresia, umbilical vein catheterization, venobiliary fistula. Umbilical venous catheter (UVC) is preferred in many newborn intensive care units because it can be inserted quickly and manipulated easily without the need for anesthesia.1,2 In practice, it is often blindly inserted and the prevalence of placed incorrectly the first time is 20–37%.3 Many severe and fatal complications secondary to liver injury have previously been reported in the Englishlanguage literature but venobiliary fistula related to UVC in a newborn with proximal intestinal atresia has not previously been reported. We describe the case of a low-birthweight newborn who underwent surgery for type IIIB jejunal atresia and who had UVC, but suffered iatrogenic liver parenchyme damage and biliary duct fistula, with drainage through the nasogastric catheter of a large amount of parenteral nutrition (PN) fluid. The importance of computed tomography in the diagnosis is also emphasized.
Case report A girl born at 35 weeks weighing 2036 g from the first pregnancy of a 27-year-old mother was referred to hospital shortly after birth with a diagnosis of intestinal atresia. The first physical examination indicated epigastric distension. A double-bubble appearance on direct radiography and dilated proximal bowel loops with minimal bile duct dilatation on abdominal ultrasonography were observed. Bile drained from the inserted nasogastric tube. UVC (Argyle™ polyurethane umbilical vessel catheter, single lumen, 5 Fr/Ch [1.7 mm] × 15″ [38.1 cm]; Covidien AG, Mansfield, MA 02048, USA) was placed the day before the surgery because the patient was low birthweight, and it was considered that she would receive long-term parenteral treatment. The catheter end was superimposed on the liver on the abdominal anteroposterior radiograph (Fig. 1). No intervention was performed, however, because the UVC functioned effectively, and blood was received with Correspondence: Erdal Turk, MD, Yeni Girne Bulvarı 1825 Sok., No:12, Kars¸ıyaka, I˙zmir 35530, Turkey. Email: [email protected] Received 20 June 2014; revised 26 August 2014; accepted 30 September 2014. doi: 10.1111/ped.12608
© 2015 Japan Pediatric Society
Fig. 1 Preoperative correct placement of the umbilical venous catheter towards the hepatic hilum (black arrow).
Venobiliary fistula due to UVC
479
b
a
Venous-biliary fistula
Ductus choledochus Ductus choledochus Duodenum
Umbilical catheter Duodenum
Fig. 2 Intense hypodense appearance indicating the extravasation of contrast material at the right lobe of the liver and the opacified choledochal duct and dilated duodenum thought to be involved, in (a) coronal plane thick slab maximum intensity projection, and (b) volume-rendered imaging.
aspiration. The patient was taken to surgery on the third day of life with a preliminary diagnosis of intestinal atresia. Type IIIB jejunal atresia (apple peel deformity) with a 1:10 difference in diameter between the proximal and distal segments was found on exploration, and primary anastomosis with tapering was performed. There was approximately 2–30 mL drainage until the fifth postoperative day, but the drainage amount increased after the sixth day to 790 mL and then 1977 mL daily. Peripheral i.v. access and UVC were in use at this time. The drainage volume was 350 mL in 4 h on the next day, and macroscopic examination of the fluid showed a murky sample with chylous appearance similar to PN fluid, while biochemistry indicated high glucose, triglyceride and protein. Abdominal ultrasonography showed heterogeneous echogenicity in the liver and a mass appearance ending around the catheter tip. Abdominal tomography after administering diluted contrast matter through the catheter showed an intense hypodense area, indicating extravasation of the contrast substance, in particular at sector 7–8 of the liver right lobe, and also opacification of the biliary tract, choledochal duct and dilated duodenum (Fig. 2). We removed the UVC immediately and the drainage from the nasogastric catheter decreased dramatically to 10 mL per day on the third day. The heterogenous hepatic masses in the liver were seen to have become smaller on the ultrasonographs obtained in the first and third weeks and to have disappeared completely in the third month.
Discussion Hypertonic PN, dopamine or materials with high pH that are infused through a malpositioned UVC are suggested as the most likely reasons for local injury, liver necrosis and abscess formation.4,5 Even when placed appropriately, accidental withdrawal or
dislodgement of the UVC during later manipulation and the changes occurring around the abdomen following rapid weight gain in babies with low birthweight can result in dislodgement of the UVC.5 Malpositioned UVC can lead to separation of the liver capsule, vascular damage in the parenchyma or damage in the biliary system, as in the present case.5,6 Diagnosis of this fatal UVC complication first requires suspicion of the condition by the clinician, and the non-invasive diagnostic method of ultrasonography can be used next. In unresolved cases, contrast abdominal tomography can be used to demonstrate the closed system created and make a definite diagnosis, as shown in Figure 2 in the present case. The placement of the UVC can be confirmed on ultrasonography and echocardiography, but electrocardiography has also recently been used for this purpose.3,7 The clinical signs in complications related to hepatic parenchymal injury are reported to appear between 4 h and 9 days, with a mean of 4 days.1,4,5 The UVC was reported to be under the diaphragm and superimposed on the liver in all of these patients.2,5,6,8 UVC localization was performed on direct radiography in the present case, and we saw that its end was under the diaphragm and superimposed on the liver parenchyma. The fact, however, that the fluid administered through the UVC passed easily, and blood was drawn with aspiration, influenced our decision to continue the treatment with UVC. Dilatation can be seen in the bile ducts due to the decreased drainage in the closed-loop system in approximately one-third of the babies with intestinal atresia.9 Reid reported the presence of dilatation in the common biliary tract in cases with congenital duodenal obstruction for the first time in 1973,10 and Bailey et al. reported on the relationship between duodenal atresia and choledochal cyst in 1993.11 The intraparenchymal bile duct dilatation in the present patient may have facilitated the creation of the © 2015 Japan Pediatric Society
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E Türk et al.
venobiliary shunt. We believe that the reflux of the fluid that had accumulated in the duodenum and proximal jejunum into the stomach may be facilitated because of the edema in the anastomosis line, and the yet ineffective intestines, and that all the given fluid was received through the nasogastric UVC, not some of it, as in the case reported by Mogbo and Wang.6 We also believe that the damaged area in the liver provided a natural drainage path without increasing in size, by creating a closed area within the liver, and also allowed the fluid to accumulate freely in the abdomen without causing perforation, thereby preventing temperature elevation, disturbed general state and abdominal distension. Although a sudden increase in abdominal distension in patients with UVC is highly suggestive of hepatic UVC complication, it should be kept in mind that the fluid given through the UVC in patients who develop venobiliary fistula, as in the present case, may pass to the gastrointestinal system through the choledochal duct, especially in babies with low birthweight and intestinal atresia.
Acknowledgment
2
3
4
5
6 7
8
9
The authors declare no conflict of interest. 10
References 1 Egyepong J, Jain A, Chow P, Godambe S. Parenteral nutrition – ascites with acute renal failure as a complication from an umbilical
11
venous catheter in an extremely low birth weight infant. BMJ Case Rep. 2011; 2011: bcr0220113813. Haase R, Hein M, Thale V, Vilser C, Merkel N. [Umbilical venous catheters – analysis of malpositioning over a 10-year period]. Z. Geburtshilfe Neonatol. 2011; 215: 18–22. Ades A, Sable C, Cummings S et al. Echocardiographic evaluation of umbilical venous catheter placement. J. Perinatol. 2003; 23: 24–8. Lim-Dunham JE, Vade A, Capitano HN, Muraskas J. Characteristic sonographic findings of hepatic erosion by umbilical vein catheters. J. Ultrasound Med. 2007; 26: 661–6. Yigiter M, Arda IS, Hicsonmez A. Hepatic laceration because of malpositioning of the umbilical vein catheter: Case report and literature review. J. Pediatr. Surg. 2008; 43: E39–41. Mogbo KI, Wang DC. Biliary venous fistula from umbilical catheter placement. Pediatr. Radiol. 1997; 27: 333–5. Tsui BC, Richards GJ, Van Aerde J. Umbilical vein catheterization under electrocardiogram guidance. Paediatr. Anaesth. 2005; 15: 297–300. Hermansen MC, Hermansen MG. Intravascular catheter complications in the neonatal intensive care unit. Clin. Perinatol. 2005; 32: 141–56, vii. McHugh K, Daneman A. Multiple gastrointestinal atresias: Sonography of associated biliary abnormalities. Pediatr. Radiol. 1991; 21: 355–7. Reid IS. Biliary tract abnormalities associated with duodenal atresia. Arch. Dis. Child. 1973; 48: 952–7. Bailey PV, Tracy TF Jr, Connors RH et al. Congenital duodenal obstruction: A 32-year review. J. Pediatr. Surg. 1993; 28: 92–5.
Bullous pemphigoid after allogeneic hematopoietic stem cell transplantation Keisuke Kato,1 Kazutoshi Koike,1 Chie Kobayashi,1,† Shigeruko Iijima,2 Takashi Hashimoto3,4 and Masahiro Tsuchida1 Division of Pediatric Hematology and Oncology, Ibaraki Children’s Hospital, 2Department of Dermatology, Mito Saiseikai General Hospital, Mito, 3Department of Dermatology, Kurume University School of Medicine and 4Kurume University Institute of Cutaneous Cell Biology, Kurume, Japan
1
Abstract
Bullous pemphigoid (BP) is an autoimmune skin disorder characterized by subepidermal blisters due to deposit of autoantibody against dermal basement membrane protein. It has been reported that BP can occur after allogeneic hematopoietic stem cell transplantation (HSCT). We describe a patient with BP having autoantibody against BP180 after unrelated-donor HSCT against T lymphoblastic leukemia. The patient was treated with steroid leading to complete resolution of BP, but T lymphoblastic leukemia progressed rapidly after steroid hormone treatment. Given that immunosuppressant may reduce graft-versus-tumor effect, immunomodulatory agents such as nicotinamide and tetracycline, erythromycin, and immunoglobulin may be appropriate as soon as typical blister lesions are seen after HSCT.
Key words bullous pemphigoid, hematopoietic stem cell transplantation, T-cell acute lymphoblastic leukemia.
Correspondence: Keisuke Kato, MD, Division of Pediatric Hematology and Oncology, Ibaraki Children’s Hospital, Futaba-dai, 3-3-1, Mito, Ibaraki 311-4145, Japan. Email: k-kato@ibaraki -kodomo.com † Present address: Department of Pediatrics, Institute of Clinical Medicine, University of Tsukuba, Tsukuba, Japan. Received 11 June 2014; revised 16 September 2014; accepted 30 September 2014. doi: 10.1111/ped.12561
© 2015 Japan Pediatric Society
Bullous pemphigoid (BP) is an autoimmune blistering disease.1 The skin lesion is characterized by tight blisters, erosions, and crusts over the entire surface of the body, including the oral mucosa. Histology indicates subepidermal blisters with inflammatory infiltration of eosinophils and lymphocytes in the dermis.1 In immunofluorescence (IF) staining, the junction between the dermis and epidermis has a linear deposition of immunoglobulin G (IgG) and complement C3.1 Indirect IF
478
E Türk et al.
Venobiliary fistula related to umbilical venous catheter in a newborn Erdal Türk,1 Ramazan Soylar,2 Tulay Akca,2 Selim Serter3 and I˙rfan Karaca1 Departments of 1Pediatric Surgery, 2Neonatology Intensive Care Unit and 3Radiology, Faculty of Medicine, Izmir University, Izmir, Turkey Abstract
We present a case of venobiliary fistula due to umbilical venous catheter (UVC). UVC was inserted the day before surgery in a newborn who was scheduled for type IIIB jejunal atresia surgery. The UVC was superimposed on the liver. It was noted that the gastric drainage became chylous and increased to 790 and then 1977 mL daily. I.v. contrast tomography with 650 mL contrast showed that the opaque substance was dispersed around the catheter and a venobiliary fistula formed, with the administered fluid accumulating in the duodenum. Rapid improvement was seen in the clinical picture after the UVC was removed. Venobiliary fistula may develop in patients with UVC that is not placed appropriately, and can direct the fluid administered from the UVC to the gastrointestinal system through the choledochal duct. The importance of contrast computed tomography in the diagnosis of venobiliary fistula in the newborn is also emphasized.
Key words type IIIB jejunal atresia, umbilical vein catheterization, venobiliary fistula. Umbilical venous catheter (UVC) is preferred in many newborn intensive care units because it can be inserted quickly and manipulated easily without the need for anesthesia.1,2 In practice, it is often blindly inserted and the prevalence of placed incorrectly the first time is 20–37%.3 Many severe and fatal complications secondary to liver injury have previously been reported in the Englishlanguage literature but venobiliary fistula related to UVC in a newborn with proximal intestinal atresia has not previously been reported. We describe the case of a low-birthweight newborn who underwent surgery for type IIIB jejunal atresia and who had UVC, but suffered iatrogenic liver parenchyme damage and biliary duct fistula, with drainage through the nasogastric catheter of a large amount of parenteral nutrition (PN) fluid. The importance of computed tomography in the diagnosis is also emphasized.
Case report A girl born at 35 weeks weighing 2036 g from the first pregnancy of a 27-year-old mother was referred to hospital shortly after birth with a diagnosis of intestinal atresia. The first physical examination indicated epigastric distension. A double-bubble appearance on direct radiography and dilated proximal bowel loops with minimal bile duct dilatation on abdominal ultrasonography were observed. Bile drained from the inserted nasogastric tube. UVC (Argyle™ polyurethane umbilical vessel catheter, single lumen, 5 Fr/Ch [1.7 mm] × 15″ [38.1 cm]; Covidien AG, Mansfield, MA 02048, USA) was placed the day before the surgery because the patient was low birthweight, and it was considered that she would receive long-term parenteral treatment. The catheter end was superimposed on the liver on the abdominal anteroposterior radiograph (Fig. 1). No intervention was performed, however, because the UVC functioned effectively, and blood was received with Correspondence: Erdal Turk, MD, Yeni Girne Bulvarı 1825 Sok., No:12, Kars¸ıyaka, I˙zmir 35530, Turkey. Email: [email protected] Received 20 June 2014; revised 26 August 2014; accepted 30 September 2014. doi: 10.1111/ped.12608
© 2015 Japan Pediatric Society
Fig. 1 Preoperative correct placement of the umbilical venous catheter towards the hepatic hilum (black arrow).
Venobiliary fistula due to UVC
479
b
a
Venous-biliary fistula
Ductus choledochus Ductus choledochus Duodenum
Umbilical catheter Duodenum
Fig. 2 Intense hypodense appearance indicating the extravasation of contrast material at the right lobe of the liver and the opacified choledochal duct and dilated duodenum thought to be involved, in (a) coronal plane thick slab maximum intensity projection, and (b) volume-rendered imaging.
aspiration. The patient was taken to surgery on the third day of life with a preliminary diagnosis of intestinal atresia. Type IIIB jejunal atresia (apple peel deformity) with a 1:10 difference in diameter between the proximal and distal segments was found on exploration, and primary anastomosis with tapering was performed. There was approximately 2–30 mL drainage until the fifth postoperative day, but the drainage amount increased after the sixth day to 790 mL and then 1977 mL daily. Peripheral i.v. access and UVC were in use at this time. The drainage volume was 350 mL in 4 h on the next day, and macroscopic examination of the fluid showed a murky sample with chylous appearance similar to PN fluid, while biochemistry indicated high glucose, triglyceride and protein. Abdominal ultrasonography showed heterogeneous echogenicity in the liver and a mass appearance ending around the catheter tip. Abdominal tomography after administering diluted contrast matter through the catheter showed an intense hypodense area, indicating extravasation of the contrast substance, in particular at sector 7–8 of the liver right lobe, and also opacification of the biliary tract, choledochal duct and dilated duodenum (Fig. 2). We removed the UVC immediately and the drainage from the nasogastric catheter decreased dramatically to 10 mL per day on the third day. The heterogenous hepatic masses in the liver were seen to have become smaller on the ultrasonographs obtained in the first and third weeks and to have disappeared completely in the third month.
Discussion Hypertonic PN, dopamine or materials with high pH that are infused through a malpositioned UVC are suggested as the most likely reasons for local injury, liver necrosis and abscess formation.4,5 Even when placed appropriately, accidental withdrawal or
dislodgement of the UVC during later manipulation and the changes occurring around the abdomen following rapid weight gain in babies with low birthweight can result in dislodgement of the UVC.5 Malpositioned UVC can lead to separation of the liver capsule, vascular damage in the parenchyma or damage in the biliary system, as in the present case.5,6 Diagnosis of this fatal UVC complication first requires suspicion of the condition by the clinician, and the non-invasive diagnostic method of ultrasonography can be used next. In unresolved cases, contrast abdominal tomography can be used to demonstrate the closed system created and make a definite diagnosis, as shown in Figure 2 in the present case. The placement of the UVC can be confirmed on ultrasonography and echocardiography, but electrocardiography has also recently been used for this purpose.3,7 The clinical signs in complications related to hepatic parenchymal injury are reported to appear between 4 h and 9 days, with a mean of 4 days.1,4,5 The UVC was reported to be under the diaphragm and superimposed on the liver in all of these patients.2,5,6,8 UVC localization was performed on direct radiography in the present case, and we saw that its end was under the diaphragm and superimposed on the liver parenchyma. The fact, however, that the fluid administered through the UVC passed easily, and blood was drawn with aspiration, influenced our decision to continue the treatment with UVC. Dilatation can be seen in the bile ducts due to the decreased drainage in the closed-loop system in approximately one-third of the babies with intestinal atresia.9 Reid reported the presence of dilatation in the common biliary tract in cases with congenital duodenal obstruction for the first time in 1973,10 and Bailey et al. reported on the relationship between duodenal atresia and choledochal cyst in 1993.11 The intraparenchymal bile duct dilatation in the present patient may have facilitated the creation of the © 2015 Japan Pediatric Society
bs_bs_banner
480
E Türk et al.
venobiliary shunt. We believe that the reflux of the fluid that had accumulated in the duodenum and proximal jejunum into the stomach may be facilitated because of the edema in the anastomosis line, and the yet ineffective intestines, and that all the given fluid was received through the nasogastric UVC, not some of it, as in the case reported by Mogbo and Wang.6 We also believe that the damaged area in the liver provided a natural drainage path without increasing in size, by creating a closed area within the liver, and also allowed the fluid to accumulate freely in the abdomen without causing perforation, thereby preventing temperature elevation, disturbed general state and abdominal distension. Although a sudden increase in abdominal distension in patients with UVC is highly suggestive of hepatic UVC complication, it should be kept in mind that the fluid given through the UVC in patients who develop venobiliary fistula, as in the present case, may pass to the gastrointestinal system through the choledochal duct, especially in babies with low birthweight and intestinal atresia.
Acknowledgment
2
3
4
5
6 7
8
9
The authors declare no conflict of interest. 10
References 1 Egyepong J, Jain A, Chow P, Godambe S. Parenteral nutrition – ascites with acute renal failure as a complication from an umbilical
11
venous catheter in an extremely low birth weight infant. BMJ Case Rep. 2011; 2011: bcr0220113813. Haase R, Hein M, Thale V, Vilser C, Merkel N. [Umbilical venous catheters – analysis of malpositioning over a 10-year period]. Z. Geburtshilfe Neonatol. 2011; 215: 18–22. Ades A, Sable C, Cummings S et al. Echocardiographic evaluation of umbilical venous catheter placement. J. Perinatol. 2003; 23: 24–8. Lim-Dunham JE, Vade A, Capitano HN, Muraskas J. Characteristic sonographic findings of hepatic erosion by umbilical vein catheters. J. Ultrasound Med. 2007; 26: 661–6. Yigiter M, Arda IS, Hicsonmez A. Hepatic laceration because of malpositioning of the umbilical vein catheter: Case report and literature review. J. Pediatr. Surg. 2008; 43: E39–41. Mogbo KI, Wang DC. Biliary venous fistula from umbilical catheter placement. Pediatr. Radiol. 1997; 27: 333–5. Tsui BC, Richards GJ, Van Aerde J. Umbilical vein catheterization under electrocardiogram guidance. Paediatr. Anaesth. 2005; 15: 297–300. Hermansen MC, Hermansen MG. Intravascular catheter complications in the neonatal intensive care unit. Clin. Perinatol. 2005; 32: 141–56, vii. McHugh K, Daneman A. Multiple gastrointestinal atresias: Sonography of associated biliary abnormalities. Pediatr. Radiol. 1991; 21: 355–7. Reid IS. Biliary tract abnormalities associated with duodenal atresia. Arch. Dis. Child. 1973; 48: 952–7. Bailey PV, Tracy TF Jr, Connors RH et al. Congenital duodenal obstruction: A 32-year review. J. Pediatr. Surg. 1993; 28: 92–5.
Bullous pemphigoid after allogeneic hematopoietic stem cell transplantation Keisuke Kato,1 Kazutoshi Koike,1 Chie Kobayashi,1,† Shigeruko Iijima,2 Takashi Hashimoto3,4 and Masahiro Tsuchida1 Division of Pediatric Hematology and Oncology, Ibaraki Children’s Hospital, 2Department of Dermatology, Mito Saiseikai General Hospital, Mito, 3Department of Dermatology, Kurume University School of Medicine and 4Kurume University Institute of Cutaneous Cell Biology, Kurume, Japan
1
Abstract
Bullous pemphigoid (BP) is an autoimmune skin disorder characterized by subepidermal blisters due to deposit of autoantibody against dermal basement membrane protein. It has been reported that BP can occur after allogeneic hematopoietic stem cell transplantation (HSCT). We describe a patient with BP having autoantibody against BP180 after unrelated-donor HSCT against T lymphoblastic leukemia. The patient was treated with steroid leading to complete resolution of BP, but T lymphoblastic leukemia progressed rapidly after steroid hormone treatment. Given that immunosuppressant may reduce graft-versus-tumor effect, immunomodulatory agents such as nicotinamide and tetracycline, erythromycin, and immunoglobulin may be appropriate as soon as typical blister lesions are seen after HSCT.
Key words bullous pemphigoid, hematopoietic stem cell transplantation, T-cell acute lymphoblastic leukemia.
Correspondence: Keisuke Kato, MD, Division of Pediatric Hematology and Oncology, Ibaraki Children’s Hospital, Futaba-dai, 3-3-1, Mito, Ibaraki 311-4145, Japan. Email: k-kato@ibaraki -kodomo.com † Present address: Department of Pediatrics, Institute of Clinical Medicine, University of Tsukuba, Tsukuba, Japan. Received 11 June 2014; revised 16 September 2014; accepted 30 September 2014. doi: 10.1111/ped.12561
© 2015 Japan Pediatric Society
Bullous pemphigoid (BP) is an autoimmune blistering disease.1 The skin lesion is characterized by tight blisters, erosions, and crusts over the entire surface of the body, including the oral mucosa. Histology indicates subepidermal blisters with inflammatory infiltration of eosinophils and lymphocytes in the dermis.1 In immunofluorescence (IF) staining, the junction between the dermis and epidermis has a linear deposition of immunoglobulin G (IgG) and complement C3.1 Indirect IF
