Neonatal intestinal occlusion due to duodenal duplication in association with malformed gallbladder sludge

Duodenal duplications are rare observations which can be diagnosed during early pregnancy via US scan. In the neonate they are often cause for intestinal occlusions. Surgical treatment can be limited by the duplication's anatomical interrelationships with adjacent organs. Biliary sludge is an uncommon finding in the first year of life, and can readily regress spontaneously. The association between duodenal duplication and sludge has never been described in the literature in the neonatal period. Here, for the first time, we report on the case of a newborn infant with cystic duplication of the duodenum associated with sludge in a misshapen gallbladder.     

Giant cystic abdominal masses in children

In this pictorial essay the common and uncommon causes of large cystic and cyst-like abdominal masses in children are reviewed. We discuss and illustrate the following: mesenchymal hamartoma, choledochal cyst, hydrops of the gallbladder, congenital splenic cyst, pancreatic pseudocyst, pancreatic cystadenoma, hydronephrosis, multicystic dysplastic kidney, multilocular cystic nephroma, adrenal hemorrhage, mesenteric and omental cysts, gastrointestinal duplication cyst, meconium pseudocyst, ovarian cysts and cystic neoplasms, hematocolpos, urachal cysts, appendiceal abscess, abdominal and sacrococcygeal teratoma, and CSF pseudocyst. We also describe imaging features and clues to the diagnosis.     

Prospective study on changes in the donor gallbladder contraction function after left lateral lobe hepatectomy

This study aimed to evaluate the feasibility of donor gallbladder preservation in liver transplantation. Conventional removal of the donor gallbladder is applied in a majority of pediatric liver transplantation. A total of 42 donors who underwent gallbladder preservation in liver transplantation from October 2013 to December 2015 at the Beijing Friendship Hospital, China, were enrolled for the study. The changes in gallbladder volume and the gallbladder EF of donors before and after surgery were measured through ultrasound, and the changes in the donor gallbladder contraction function before and after surgery were evaluated to help verify the feasibility of gallbladder preservation in living donor left lateral lobe hepatectomy. The gallbladder emptying index dropped to 42.67% in 2 weeks after surgery and gradually increased with the length of recovery time, which could reach 69.14% in 3 months after surgery. At that time, 97.6% of the donors were considered to have recovered their gallbladder contraction function. The gallbladder contraction function at an early stage after gallbladder preservation in liver transplantation is not obviously improved, but it can recover to a normal level in 1 month after surgery, indicating that the gallbladder preservation in hepatectomy of living donor can effectively guarantee the gallbladder contraction function.     

Completely isolated alimentary tract duplication in a neonate

A rare case of a completely isolated, alimentary tract duplication cyst in a 27-day-old neonate is reported. The duplication cyst was detected on antenatal fetal ultrasound and magnetic resonance (MR) imaging at 27 weeks’ gestational age. At surgery, the duplication cyst was in a retroperitoneal site with no apparent communication between the cyst and any portion of the alimentary tract. On histopathological examination, the diagnosis was a gastric duplication cyst. The patient’s postoperative course was uneventful. There have been eight cases of completely isolated duplication reported in the literature, of which seven were detected during the prenatal or neonatal period. No previous report in the English literature has described the fetal MR imaging findings of this type of duplication cyst.     

Urethral duplication in males: our experience in ten cases

Urethral duplication is a rare congenital anomaly, affecting mainly boys. Clinical presentation varies because of the different anatomical patterns of this abnormality. We report our experience in ten males affected by urethral duplication. We retrospectively reviewed the records of ten males affected by urethral duplication. Mild cases of distal type I duplications as well as “Y-type” duplication associated to anorectal malformation were excluded. Evaluation included voiding cystourethrography, retrograde urethrography, intravenous urography and urethrocystoscopy. Mean age at diagnosis was 46.7 ± 32.3 months A blind ending duplicated urethra (type I) was present in three patients, two urethras originating from a common bladder neck (type II A2) in three, an “Y-type” duplication in three and a complete bladder with incomplete urethral duplication in one. Surgical management included excision of the duplicated urethra in four patients while a displacement of the ventral urethra (in “Y-type” duplication) in perineal-scrotal or scrotal position was performed in two patients as first stage of urethral reconstruction. Good cosmetical and functional results were achieved in all six treated boys while surgical management was not required in four. Urethral duplication is often associated with genito-urinary and gastro-intestinal abnormalities. Embryology is unclear and a lot of hypotheses have been proposed. We believe that the same embryological explanation cannot be applied to all subtypes of urethral duplication. Management must be evaluated for each case. The overall prognosis is good, in spite of the presence of other severe associate congenital anomalies.     

Gallbladder pseudopolyp formation following percutaneous liver biopsy

Following an unsuccessful liver biopsy in a 16-year-old girl in which only bile was obtained, a sonographic (US) study was performed. The findings were those of free fluid just adjacent to the gallbladder as well as a small intraluminal protrusion of the gallbladder wall. The US features of the gallbladder finding were consistent with a polyp. Gallbladder polyps are very rare in children, however, sporadic case reports do exist. An US study 24 h after the invasive procedure revealed complete regression of the free fluid; the gallbladder wall protrusion has disappeared. We suggest that the tip of the biopsy needle had penetrated the gallbladder wall and caused a bile leak. The needle penetration caused elevation of the inner wall layer at the entrance site, which was indistinguishable from the sonographic findings of a gallbladder polyp [1-4]     

Adenoma of the gallbladder associated with a gallstone in a child

We describe a rare case of adenoma of the gallbladder associated with a gallstone in a 9-year-old girl who was successfully treated by cholecystectomy. The diagnosis, which was suspected on repeated ultrasonographic examinations of the gallbladder, was confirmed at surgery and histologic examination. To our knowledge, this is the third documented case of adenoma of the gallbladder and the first case associated with a gallstone in childhood. Correspondence to: S. Y. Kim     

The size and contractility of the gallbladder in infants

The size of the gallbladder and the common bile duct as well as the contractility of the gallbladder were measured in infants under the age of 1 year. A total of 43 healthy infants were studied at their routine visit to the well-baby clinic at the ages of 6 weeks, 4 months, 8 months or 12 months. Gallbladder size was measured before and after a test meal. Fasting gallbladder size increased with the age of the infants. However, when the size was corrected for the infant's weight, no age-related variation was found. The contraction index (CI) was determined as a percentage decrement of planimetric gallbladder size from the initial size. The mean CI declined with age from 75% (±27%) mean (±SD)) at the age of 6 weeks to 24% (±28%) at the age of 12 months. Overall CI varied from –44% to 100%. An increase in gallbladder size after the test meal was observed in 4 infants. Gallbladder volumes were also calculated, but two-dimensional measurements were regarded as more accurate in infants. A large variation was found in the size as well as in the contractility of the gallbladder in infants less than 1 year of age.     

Implications of intestinal metaplasia of the gallbladder in children with pancreaticobiliary maljunction

Purpose  

Pancreaticobiliary maljunction (PBM) is associated with an increased frequency of gallbladder malignancy. Intestinal metaplasia is often observed in gallbladder disease and is a risk factor for gallbladder carcinoma in adults. The hyperplasia—dysplasia—carcinoma progression is one of the possible mechanisms involved in biliary carcinogenesis. In this study, we evaluate the gallbladders of children with PBM for intestinal metaplasia and other histological changes.     

Real-time sonography for screening of gallbladder dysfunction in children with type 1 diabetes mellitus

The aim of this study was to evaluate the occurrence of gallbladder dysfunction in children with type 1 diabetes mellitus using real-time ultrasonography. The study population consisted of 20 diabetic children (11 male, 9 female; age 11.7+/-2.8 years; diabetes duration 0.5-7 years) with clinically negative neuropathy findings and 15 healthy controls (11 male, 4 female; age 10.5+/-3.7 years). Three-dimensional measurements of the gallbladder were made before and 15, 30, 45, 60 min after intake of diet chocolate. Gallbladder volumes were calculated by the ellipsoid formula. Fasting gallbladder volume of diabetic children (16.9+/-9.5 ml) was significantly greater than that of the controls (10.6+/-5.3 ml; p=0.017). Ejection fraction and maximal contraction showed no significant difference between the two groups. Diabetic patients with multiple microvascular complications had diminished gallbladder motility. There was a negative correlation between BMI and maximal contraction (p<0.05). Nerve conduction velocity was diminished in 45% of the diabetic patients. In conclusion, gallbladder function is preserved in pediatric type 1 diabetic patients with a disease duration less then 10 years, but dilated gallbladder at rest may be an early sign of gastrointestinal autonomic neuropathy and a risk factor for gallstone formation.     

Enteric duplication cysts of the pancreas: a report of two cases and review of the literature

Enteric duplication cysts are rare congenital malformations that are most commonly diagnosed in children. Enteric duplications associated with the pancreas are especially uncommon, and may present with specific clinical findings such as severe pancreatitis. These cysts often pose unique surgical challenges. In addition, the diagnosis of pancreatic duplication cysts is often difficult, and may be confused with pancreatic pseudocysts or neoplasms. Herein we report two cases of pancreatic duplication cysts, and present a complete tabulation of all case reports of pancreatic-associated duplication cysts reported in the English literature. We conclude that pancreatic duplication cysts are a rare entity, most commonly found to occur in infants and children. We further find that although severe complications may arise as a result of their presentation and treatment, the rate of post-operative complications in patients between 3 and 21 years of age is extremely low, with the highest complication rate occurring in a bimodal distribution (<3-years and >21-years of age). Despite complications in the youngest and older patient populations, surgical excision remains the mainstay of therapy for pancreatic duplication cysts in all age groups.     

Gallbladder hypocontractility in infantile colic

We evaluated gallbladder contractility in 58 colicky infants and 57 age-matched controls. Gallbladder size was measured before and 1 h after feeding and the contraction index of the gallbladder was calculated. The results showed decreased contractility of the gallbladder in colicky infants: mean contraction index in colicky infants was 56% (95% confidence interval, 49–63%) and in controls 67% (61–73%). No difference was found in the fasting size of the gallbladder. Postprandial contraction was decreased in colicky infants examined in the evening (n = 14) compared with those examined in the morning (n = 44). Decreased contraction of the gallbladder was found in those colicky infants who presented colicky cry compared with colicky infants without symptoms at the time of examination. No structural abnormalities were found in the general abdominal survey. In conclusion, infants with colic have hypocontractility of the gallbladder. This indicates abnormal biliary tract physiology in association with infantile colic.     

Adenomyomatosis of the gallbladder resembling honeycomb in a child

Adenomyomatosis of the gallbladder is believed to be an uncommon pathologic condition of the gallbladder in childhood. Only three pediatric cases have been described in the literature up to now. Honeycomb gallbladder has been described in two adult patients; no patients have been reported in childhood until now. To the best of our knowledge, we report here the first case of adenomyomatosis of the gallbladder which resembled honeycomb, in a 9-year-old girl presented with recurrent abdominal pain. The diagnosis was made by ultrasound, and confirmed by magnetic resonance cholangiopancreatography and finally cholecystectomy. In conclusion, ultrasound scanning performed more generally in children presenting with recurrent abdominal pain might lead to accurate diagnosis of adenomyomotosis of the gallbladder during childhood.     

Pyloric duplication presenting with gastric outlet obstruction in the newborn period

Pyloric duplication is rare condition. A case of neonatal gastric outlet obstruction caused by a duplication of the pylorus is presented. This case, successfully treated surgically, illustrates that pyloric duplication should be considered in the differential diagnosis of neonatal gastric outlet obstruction. Offprint requests to: R. A. Superina, at the Department of Surgery     

Is renal ultrasound a reliable indicator of a nonobstructed duplication anomaly?

Twelve kidneys which had a non-obstructed duplication anomaly on excretory urography were studied sonographically. Ten of the twelve kidneys had a single central renal sinus echo complex; only two kidneys had a split sinus complex typical of duplication. Sonography is not a reliable technique to identify a nonobstructed duplication anomaly.     

Mediastinal enteric duplication cyst containing aberrant pancreas

Objective : The purpose of the study is to report a unique association of clinical and pathological findings in a neonate. Foregut enteric duplication cysts-rare developmental anomalies that are associated with midline vertebral fusion anomalies.Methods : We had a neonate with foregut duplication cyst who presented at birth with respiratory distress. The child also had associated communicating hydrocephalus. The patient underwent excision of the duplication cyst alongwith a ventriculo-peritoneal shunt.Result : The excised specimen revealed a duplication cyst lined by aberrant pancreatic tissue.Conclusion : The present case demonstrates histologically the presence of both pancreatic and gastric tissue.     

Congenital diaphragmatic hernia with a pure duplication of chromosome 1q: report of the first surviving case

Congenital diaphragmatic hernia (CDH) occurs in multiple malformation syndromes and associations, and has been associated with cytogenetic aberrations on almost every chromosome arm. However, CDH with a duplication of chromosome 1q is very rare in the literature, and all previously reported cases with detailed clinical courses died soon after birth. We present the first surviving case of CDH with a duplication of 1q12–q23, who had arthrogryposis multiplex congenita and hypertrophic cardiomyopathy. CDH patients with a proximal duplication of chromosome 1q may have a chance for survival, and CDH with a duplication of chromosome 1q is not necessarily a lethal association.     

DEFECT IN BILE ACID CONCENTRATING ABILITY OF THE GALLBLADDER IN CONGENITAL CHLORIDE DIARRHOEA

Abstract. Bakkeren, J., Monnens, L. and van Os, C. (Departments of Paediatrics and Physiology, University of Nijmegen, Nijmegen, The Netherlands). Defect in bile acid concentrating ability of the gallbladder in congenital chloride diarrhoea. Acta Paediatr Scand, 70:43, 1981.–Congenital chloride diarrhoea is assumed to be caused by a defect in the coupled NaCl influx mechanism in the ileum. As a similar coupled NaCl transport mechanism has been postulated in the gallbladder, the concentrating ability of the gallbladder was studied in a patient with congenital chloride diarrhoea. Bile acid concentrations were measured in the duodenal fluid before and after stimulation of gallbladder contraction by cholecysto-kinin. In the chloride-diarrhoea patient no increase in bile acid concentration was established after cholecystokinin injection, in contrast to a pronounced increase in three control children, suggesting that the absorption of salt and water by the gallbladder may be disturbed in the patient. The results support the postulated similarity of the NaCl transport mechanisms in the ileum and gallbladder. In congenital chloride diarrhoea one defect in a NaCl transport protein could explain the disturbances in electrolyte absorption.     

Gallbladder contraction in familial dysautonomia

The impaired function of the autonomic nervous system in patients with familial dysautonomia (FD) is frequently associated with gastrointestinal dysfunction. This study sought to determine whether gallbladder contraction is also affected in these patients. Nine consecutive patients with FD were assessed by real-time ultrasonography for gallbladder volume and calculated percentage of gallbladder contraction before and 45-60 min after a 50 g fatty meal, and compared with nine healthy control patients, matched for age and gender. Gallbladder volume before the fatty meal was found to be larger in healthy controls than in FD patients (25.4 ± 9.5 and 15.4 ± 7.4 ml³, respectively; p = 0.024). No significant difference was demonstrated between the groups after the fatty meal (controls: 12.1 ± 6.0; FD: 9.0 ± 6.6 ml, respectively) and the calculated percentage of gallbladder contraction was similar in both groups (controls: 53.4 ± 16.6; FD: 44.8 ± 18.8%). These results indicate that gallbladder function in FD patients does not differ from the general population and preventive measures for gallstone formation are not required.     

Surgical treatment of penile duplication

IntroductionPenile duplication is a rare anomaly with an incidence of 1 in 5,500,000. It is normally associated with other malformations, such as double bladder, presence of the cloaca, imperforate anus, duplication of the rectosigmoid and vertebral deformities.Presented here is the surgical technique used to resolve a rare case of complete penile duplication in a 5-year-old child, without any other malformation.Patient and MethodsA male Caucasian patient presented with a rare diagnosis of complete penile duplication, without any associated systemic malformation. At 5 years of age, he was successfully submitted to surgical treatment. The procedure included the following steps: penile dissection up to the point of ischial-pubic insertion; dissection of the hypoplastic urethra; amputation of the less developed penis; termino-lateral urethral anastomosis and phalloplasty.ResultsThree years after surgery, penile morphology was normal, with a normal urinary flow according to age.ConclusionsTreatment should always be individualized. The malformations that are potentially life-threatening should be solved first. Fortunately, this patient had only penile duplication, successfully corrected after surgical treatment.