Congenital duodenal obstruction due to a preduodenal portal vein
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《美国医学杂志》
Department of Pediatric Surgery, Maulana Azad Medical College, New Delhi, India
Abstract
Only about 80 cases of pre-duodenal portal vein (PDPV), a rare congenital anomaly of portal vein development that was first described by Knight in 1921, have been reported till date.[1] We report a neonate with duodenal obstruction caused by PDPV and briefly review the available literature.
Keywords: Neonatal with duodenal obstruction; PDPV
Congenital duodenal obstruction is a rare anomaly with incidence of about 1 in 10000. Most common cause of duodenal obstruction is duodenal atresia.[2] Other common causes of duodenal obstruction are annular pancreas, Ladd's bands and malrotation. PDPV is one of the rare causes of duodenal obstruction. In a large series of patients with congenital duodenal obstructions, PDPV was found in only 4% cases.[2] PDPV was not exactly the cause of duodenal obstruction in most of these; the obstruction was caused by associated malrotation or duodenal atresia. Extensive search of literature revealed that duodenal obstruction caused primarily by PDPV is extremely rare with only 10 cases reported in literature hitherto.[3] We report a neonate in whom the duodenal obstruction was caused primarily by PDPV.
Case report
A 7-day-old male neonate weighing 1.5 kg, born to gravida A para (G 4 P 1 ) mother at 35 weeks of gestation by cesarean section elsewhere, was referred for complaints of persistent non-bilious vomiting, upper abdominal distension and failure to pass meconium. Polyhydramnios was reported on the single antenatal ultrasonography that the mother underwent in her third trimester of pregnancy; no specific cause could be identified. At admission, the patient was dehydrated with tachycardia, feeble peripheral pulses and hypothermia. There was minimal upper abdominal distension. Gastric peristalsis was visible but no pyloric tumor could be palpated. Apex beat was shifted towards the right, but the trachea was placed centrally and air entry was equal bilaterally. Clinical diagnosis of pyloric or proximal duodenal obstruction with associated dextrocardia was made in this premature, low-birth weight neonate.
The patient was managed with intravenous fluids and antibiotics. Naso-gastric aspirates were nonbilious. Arterial blood gas analysis was suggestive of compensated metabolic acidosis. Blood urea and serum creatinine were raised; serum electrolytes were normal. Sepsis profile was positive. Blood culture was sent just after admission that was reported later as positive for Klebsiella species. Babygram showed single gas shadow of massively distended stomach that was suggestive of pyloric atresia and also confirmed dextrocardia Figure1. After stabilization, laparotomy was undertaken that revealed a prominent PDPV obstructing the first part of duodenum and a hugely dilated stomach Figure2. Semi-annular pancreas was also noted. Gall bladder and spleen were normal and there were no associated malformation of biliary system or polysplenia. Duodenostomy was performed anterior to portal vein. The baby required mechanical ventilatory support post-operatively. Unfortunately, the patient succumbed to sepsis on second post-operative day.
Discussion
Embryologically, PDPV results from persistence of a primitive vitelline vein rather than passing inferior and behind the pancreas on its cephalad path to liver, it crosses in front of the duodenum and pancreas. As many as 3/4th cases of PDPV have some features of faulty situs determination like polysplenia syndrome or heterotaxy syndrome.[3] As many as 5-10% patients of biliary atresia have been found to have PDPV at surgery; this association represents a more confined phenotypic result of faulty situs determination. Associations of PDPV with preduodenal common bile duct have also been reported[4]. Review of literature revealed the presence of 'isolated' symptomatic or asymptomatic PDPV in only 1/4th cases.[5]
Antenatal diagnosis of duodenal obstruction is evident by association of polyhydramnios with dilated fluid-filled stomach and duodenum appearing as double-bubble on ultrasonography.[6] PDPV as a cause of duodenal obstruction has never been picked up on antenatal ultra-sonography.[7] PDPV may lead either to complete or partial duodenal obstruction but it may even go unrecognized in childhood manifesting later in adult life. It may occasionally be an incidental finding. Only in about 10 cases duodenal compression could be confidently described as being due to a preduodenal portal vein.[3] When completely obstructed, characteristic presentation is onset of vomiting within a few hours of birth and intolerance of attempted feedings. Complete duodenal obstruction is evident postnataly on abdominal roentgenogram showing double-bubble appearance with no distal bowel gas shadow.[2] Partial obstruction, however, usually results in recurrent episodes of vomiting, failure to thrive, and aspiration over a period of months to years. Patients with symptoms of partial obstruction should undergo an upper gastrointestinal contrast study.[2] Some cases are not recognized until adult life and may present with gastric outlet obstruction later in life.[8] PDPV may be an incidental finding in an adult at the time of cholecystectomy.[9]
Pre-operative diagnosis of PDPV as a cause of duodenal obstruction is not mandatory since all cases of duodenal obstruction require laparotomy, but recognition of the entity is important during surgery, as PDPV might only be an incidental finding not producing obstruction. Surgical importance also lies in the fact that it can predispose intra-operative complications including iatrogenic hemorrhage from the abnormal vein, or damage to the biliary tract or the distended duodenum.
Although congenital duodenal obstruction is a relative surgical emergency, the patient should not be rushed to the operating room until the infant's hemodynamic and electrolyte status is corrected and the baby is stable. Because of high incidence of associated anomalies, a prompt system review should be carried out to detect other defects including evaluation for cardiac anomalies. Definitive treatment is duodenostomy anterior to portal vein.[2] Also, a thorough examination of the abdominal cavity including palpation of gall bladder and spleen should be done to rule out other associated anomalies.[2]
The question always asked by the parents is about probability of recurrence of the disease in the next offsprings. The sole animal model study in rats suggests autosomal recessive inheritance transmission of the PDPV.[10]
References
1. Mordehai J, Cohen Z, Kurzbart E, Mares AJ. Preduodenal portal vein causing duodenal obstruction associated with situs inversus, intestinal malrotation, and polysplenia: A case report. J Pediatr Surg 2002; 37: E5.
2. Grosfeld JL, Rescorla FJ. Duodenal atresia and stenosis: reassessment of treatment and outcome based on antenatal diagnosis, pathologic variance, and long-term follow-up. World J Surg 1993; 17: 301-309.
3. Georgacopulo P, Vigi V. Duodenal obstruction due to a preduodenal portal vein. J Pediatr Surg 1980; 15 : 339-340.
4. Yi SQ, Tanaka S, Tanaka A, Shimokawa T, Ru F, Nakatani T. An extremely rare inversion of the preduodenal portal vein and common bile duct associated with multiple malformations. Report of an adult cadaver case with a brief review of the literature. Anat Embryol (Berl) 2004; 208 : 87-96.
5. Tsuda Y, Nishimura K, Kawakami S, Kimura I, Nakano Y, Konishi J. Preduodenal portal vein and anomalous continuation of inferior vena cava: CT findings. J Comput Assist Tomogr 1991; 15 : 585-588.
6. Hancock BJ, Wiseman NE. Congenital duodenal obstruction: the impact of antenatal diagnosis. J Pediatr Surg 1989; 24 : 1027-1031.
7. Choi SO, Park WH. Preduodenal portal vein: A cause of prenatally diagnosed duodenal obstruction. J Pediatr Surg 1995; 30 : 1521-1522.
8. John AK, Gur U, Aluwihare A, Cade D. Preduodenal portal vein as a cause of duodenal obstruction in an adult. ANZ J Surg 2004; 74 : 1032-1033.
9. Papaziogas T, Papaziogas B, Paraskevas G, Lazaridis C, Patsas A. Preduodenal portal vein in the adult. Morphologie 2000; 84 : 33-36.
10. Nakajima H, Li L, Nomura T. Anomalous (preduodenal) portal vein: autosomal recessive mutation in AKR/J mice. Comp Med 2001; 51 : 22-25.(Pathak D, Sarin Yogesh Ku)
Abstract
Only about 80 cases of pre-duodenal portal vein (PDPV), a rare congenital anomaly of portal vein development that was first described by Knight in 1921, have been reported till date.[1] We report a neonate with duodenal obstruction caused by PDPV and briefly review the available literature.
Keywords: Neonatal with duodenal obstruction; PDPV
Congenital duodenal obstruction is a rare anomaly with incidence of about 1 in 10000. Most common cause of duodenal obstruction is duodenal atresia.[2] Other common causes of duodenal obstruction are annular pancreas, Ladd's bands and malrotation. PDPV is one of the rare causes of duodenal obstruction. In a large series of patients with congenital duodenal obstructions, PDPV was found in only 4% cases.[2] PDPV was not exactly the cause of duodenal obstruction in most of these; the obstruction was caused by associated malrotation or duodenal atresia. Extensive search of literature revealed that duodenal obstruction caused primarily by PDPV is extremely rare with only 10 cases reported in literature hitherto.[3] We report a neonate in whom the duodenal obstruction was caused primarily by PDPV.
Case report
A 7-day-old male neonate weighing 1.5 kg, born to gravida A para (G 4 P 1 ) mother at 35 weeks of gestation by cesarean section elsewhere, was referred for complaints of persistent non-bilious vomiting, upper abdominal distension and failure to pass meconium. Polyhydramnios was reported on the single antenatal ultrasonography that the mother underwent in her third trimester of pregnancy; no specific cause could be identified. At admission, the patient was dehydrated with tachycardia, feeble peripheral pulses and hypothermia. There was minimal upper abdominal distension. Gastric peristalsis was visible but no pyloric tumor could be palpated. Apex beat was shifted towards the right, but the trachea was placed centrally and air entry was equal bilaterally. Clinical diagnosis of pyloric or proximal duodenal obstruction with associated dextrocardia was made in this premature, low-birth weight neonate.
The patient was managed with intravenous fluids and antibiotics. Naso-gastric aspirates were nonbilious. Arterial blood gas analysis was suggestive of compensated metabolic acidosis. Blood urea and serum creatinine were raised; serum electrolytes were normal. Sepsis profile was positive. Blood culture was sent just after admission that was reported later as positive for Klebsiella species. Babygram showed single gas shadow of massively distended stomach that was suggestive of pyloric atresia and also confirmed dextrocardia Figure1. After stabilization, laparotomy was undertaken that revealed a prominent PDPV obstructing the first part of duodenum and a hugely dilated stomach Figure2. Semi-annular pancreas was also noted. Gall bladder and spleen were normal and there were no associated malformation of biliary system or polysplenia. Duodenostomy was performed anterior to portal vein. The baby required mechanical ventilatory support post-operatively. Unfortunately, the patient succumbed to sepsis on second post-operative day.
Discussion
Embryologically, PDPV results from persistence of a primitive vitelline vein rather than passing inferior and behind the pancreas on its cephalad path to liver, it crosses in front of the duodenum and pancreas. As many as 3/4th cases of PDPV have some features of faulty situs determination like polysplenia syndrome or heterotaxy syndrome.[3] As many as 5-10% patients of biliary atresia have been found to have PDPV at surgery; this association represents a more confined phenotypic result of faulty situs determination. Associations of PDPV with preduodenal common bile duct have also been reported[4]. Review of literature revealed the presence of 'isolated' symptomatic or asymptomatic PDPV in only 1/4th cases.[5]
Antenatal diagnosis of duodenal obstruction is evident by association of polyhydramnios with dilated fluid-filled stomach and duodenum appearing as double-bubble on ultrasonography.[6] PDPV as a cause of duodenal obstruction has never been picked up on antenatal ultra-sonography.[7] PDPV may lead either to complete or partial duodenal obstruction but it may even go unrecognized in childhood manifesting later in adult life. It may occasionally be an incidental finding. Only in about 10 cases duodenal compression could be confidently described as being due to a preduodenal portal vein.[3] When completely obstructed, characteristic presentation is onset of vomiting within a few hours of birth and intolerance of attempted feedings. Complete duodenal obstruction is evident postnataly on abdominal roentgenogram showing double-bubble appearance with no distal bowel gas shadow.[2] Partial obstruction, however, usually results in recurrent episodes of vomiting, failure to thrive, and aspiration over a period of months to years. Patients with symptoms of partial obstruction should undergo an upper gastrointestinal contrast study.[2] Some cases are not recognized until adult life and may present with gastric outlet obstruction later in life.[8] PDPV may be an incidental finding in an adult at the time of cholecystectomy.[9]
Pre-operative diagnosis of PDPV as a cause of duodenal obstruction is not mandatory since all cases of duodenal obstruction require laparotomy, but recognition of the entity is important during surgery, as PDPV might only be an incidental finding not producing obstruction. Surgical importance also lies in the fact that it can predispose intra-operative complications including iatrogenic hemorrhage from the abnormal vein, or damage to the biliary tract or the distended duodenum.
Although congenital duodenal obstruction is a relative surgical emergency, the patient should not be rushed to the operating room until the infant's hemodynamic and electrolyte status is corrected and the baby is stable. Because of high incidence of associated anomalies, a prompt system review should be carried out to detect other defects including evaluation for cardiac anomalies. Definitive treatment is duodenostomy anterior to portal vein.[2] Also, a thorough examination of the abdominal cavity including palpation of gall bladder and spleen should be done to rule out other associated anomalies.[2]
The question always asked by the parents is about probability of recurrence of the disease in the next offsprings. The sole animal model study in rats suggests autosomal recessive inheritance transmission of the PDPV.[10]
References
1. Mordehai J, Cohen Z, Kurzbart E, Mares AJ. Preduodenal portal vein causing duodenal obstruction associated with situs inversus, intestinal malrotation, and polysplenia: A case report. J Pediatr Surg 2002; 37: E5.
2. Grosfeld JL, Rescorla FJ. Duodenal atresia and stenosis: reassessment of treatment and outcome based on antenatal diagnosis, pathologic variance, and long-term follow-up. World J Surg 1993; 17: 301-309.
3. Georgacopulo P, Vigi V. Duodenal obstruction due to a preduodenal portal vein. J Pediatr Surg 1980; 15 : 339-340.
4. Yi SQ, Tanaka S, Tanaka A, Shimokawa T, Ru F, Nakatani T. An extremely rare inversion of the preduodenal portal vein and common bile duct associated with multiple malformations. Report of an adult cadaver case with a brief review of the literature. Anat Embryol (Berl) 2004; 208 : 87-96.
5. Tsuda Y, Nishimura K, Kawakami S, Kimura I, Nakano Y, Konishi J. Preduodenal portal vein and anomalous continuation of inferior vena cava: CT findings. J Comput Assist Tomogr 1991; 15 : 585-588.
6. Hancock BJ, Wiseman NE. Congenital duodenal obstruction: the impact of antenatal diagnosis. J Pediatr Surg 1989; 24 : 1027-1031.
7. Choi SO, Park WH. Preduodenal portal vein: A cause of prenatally diagnosed duodenal obstruction. J Pediatr Surg 1995; 30 : 1521-1522.
8. John AK, Gur U, Aluwihare A, Cade D. Preduodenal portal vein as a cause of duodenal obstruction in an adult. ANZ J Surg 2004; 74 : 1032-1033.
9. Papaziogas T, Papaziogas B, Paraskevas G, Lazaridis C, Patsas A. Preduodenal portal vein in the adult. Morphologie 2000; 84 : 33-36.
10. Nakajima H, Li L, Nomura T. Anomalous (preduodenal) portal vein: autosomal recessive mutation in AKR/J mice. Comp Med 2001; 51 : 22-25.(Pathak D, Sarin Yogesh Ku)