A rare gallbladder ciliated foregut cyst in chronic cholecystitis

IntroductionCiliated foregut cysts (CFC) are rare anomalies due to aberrant embryological development. It is thought to arise from a remnant of the embryologic foregut. The solitary cysts are characterised by ciliated pseudostratified columnar epithelium. They are usually located above the diaphragm but they can also arise in relation to the liver, gallbladder and pancreas.Presentation of caseWe present the first ciliated foregut cyst of the gallbladder case reported in Australia, and the ninth known case to be reported worldwide. A 61-year-old male with chronic cholecystitis and cholelithiasis underwent an elective laparoscopic cholecystectomy and intraoperative cholangiogram. Intraoperatively, ‘out-pouching’ was noted on the lateral border of the gallbladder. Microscopically the histopathology showed that the cyst was lined by ciliated columnar epithelium the characteristic feature of a ciliated foregut cyst.DiscussionTo date only 8 cases of these ciliated foregut cysts in the gallbladder have been reported in literature. Our case is the first reported in Australia. It is unique in that the patient was an older male as opposed to most other previous cases, which were younger females. These cysts can be difficult to distinguish from neoplasms clinically and radiographically. Reports have shown that these cysts may become dysplastic and is best excised when discovered.ConclusionDespite the rarity of CFCs and their potential to mimic malignancy, we propose awareness and understanding of the management for them—being excision and hopefully not cause any confusion or devastatingly allow it to become malignant.     

Ciliated hepatic foregut cyst in a young child

Ciliated hepatic foregut cysts are a rare entity usually found in adults. We present a case of a 3-year-old boy incidentally noted to have a radiographically complex liver cyst on computed tomographic scan. Given the complex appearance, the cyst was excised. Pathology revealed a ciliated hepatic foregut cyst. This is the second child and youngest patient affected with this lesion reported in the literature. The etiology of the lesion and an argument for surgical removal in pediatric patients are presented.     

Prenatal diagnosis and postnatal resection of intraabdominal enteric duplications

Purpose

The high definition provided by modern imaging techniques allows the delineation of fetal anatomy with unprecedented accuracy. The early prenatal detection of enteric duplications is possible and facilitates a prompt postnatal treatment strategy, thereby, decreasing the risk of potential complications.

Materials and Methods

We retrospectively reviewed the medical records of all patients treated at our institution with a prenatally diagnosed intraabdominal enteric duplication between 2001 and 2009.

Results

Eighteen patients were included in the series. The diagnosis was made as early as 20 weeks of gestational age and included gastric duplications (3 cases), jejunoileal duplications (11 cases), and duodenal duplications (4 cases). None of the patients had prenatal complications. Two patients had associated intestinal malrotation, whereas the remaining 16 had no other malformations. Postnatally, 14 patients had an uneventful clinical course until the time of surgery, 3 patients had complications within the first 2 weeks of life that required emergency surgery, and 1 patient who had been lost to follow-up presented with duodenal obstruction at 3 years of age. Four patients underwent laparoscopic resection.

Conclusion

Because intraabdominal enteric duplications can cause complications, we believe that prenatally diagnosed enteric duplications should be resected after birth.     

Ciliated hepatic foregut cyst

A male infant was found to have a large congenital hepatic cyst, first noted in late gestation by prenatal ultrasound scan. The cyst communicated with the biliary tree and was eventually removed completely by an extended right hepatectomy. Histopathologic examination showed a thick-walled, unilocular cyst lined predominantly by ciliated, stratified squamous epithelium with an outer wall composed of smooth muscle cells and fibrous tissue. These features are diagnostic of a ciliated hepatic foregut cyst, a rare congenital malformation with histologic similarities to bronchogenic cysts. The young age of our patient, prenatal detection, large size of the cyst, and a clear communication with the biliary tree have not been previously described with ciliated hepatic foregut cysts.     

Solitary liver cysts in children: not always so simple

Aim

Liver cysts in children are uncommon. Many are simple and solitary and do not require intervention. However, this series demonstrates a broad range of potential pathologies, some of which are life-threatening.

Methods

All children referred to our unit during an 8-year period (1998-2005) and found to have a solitary liver cyst were prospectively recorded. Clinical, radiologic, and pathologic features were analyzed. Children with an isolated extrahepatic choledochal cyst and polycystic disease were excluded.

Results

Twenty-one children with a liver cyst were identified. Two had undergone unsuccessful surgical intervention before referral. There were 11 prenatally detected cysts. Median gestational age at detection was 22 weeks (19-35 weeks); only 1 was specifically characterized as a liver cyst prenatally. Six of these required surgery: 2 large simple cysts, 2 intrahepatic choledochal cysts, 1 giant complex biliary cyst causing respiratory distress, and 1 ciliated hepatic foregut cyst. Of the 5 cysts remaining under ultrasound surveillance, 4 decreased in size or resolved. In 10 children presenting between birth and 15.8 years, a liver cyst was diagnosed postnatally: 3 huge cystic mesenchymal hamartomas, 1 type V choledochal cyst, 1 hydatid cyst, and 5 simple cysts. Four of these required surgical resection. Simple cysts tended to be small and could be distinguished from other pathologies using a combination of imaging techniques (ultrasound, magnetic resonance imaging/magnetic resonance cholangiopancreatography [MRCP], radionuclide scan). Only 2 of 12 children with “simple” cysts required surgery for symptoms. However, a wide range of other cyst pathologies were found in 9 children, and although none was malignant, some were life-threatening and 7 required resection.

Conclusions

Simple solitary nonparasitic liver cysts rarely cause symptoms or require surgery, but the pediatric surgeon should be aware of the wide range of other types of liver cyst in children to ensure appropriate treatment.     

Comparing characteristics and outcomes in infants with prenatal and postnatal diagnosis of esophageal atresia

Background

Previous studies of infants with esophageal atresia (EA) suggest those diagnosed prenatally have worse outcomes because of a higher incidence of associated anomalies. The purpose of this study was to compare characteristics and outcomes of infants with EA diagnosed after fetal center evaluation to those diagnosed postnatally.

Methods

The records of all neonates treated for EA at our institution from 2002–2012 were reviewed. Infants with a prenatal diagnosis of EA were compared with those postnatally diagnosed using chi-square and Student t-test as appropriate.

Results

Of 91 patients treated with EA during the study period, 15 (16%) were diagnosed prenatally at our fetal center. Although those prenatally diagnosed had a higher incidence of pure EA and polyhydramnios, the gestational age and birth weight in that group were similar to those diagnosed postnatally. There were no differences in outcomes between groups with regard to the incidence of major cardiac anomalies, surgical complications, hospital length of stay, and survival.

Conclusions

Treatment at a tertiary care center provides excellent outcomes for all infants with EA, despite an 80% frequency of concurrent anomalies. Prenatal diagnosis of EA and attentive obstetric management of polyhydramnios decrease the risk for prematurity and prematurity-associated morbidity.     

Laparoscopic excision of ciliated hepatic foregut cyst: a first report in Korea

Ciliated hepatic foregut cyst (CHFC) is very rare and apparently originated from embryologic foregut. It is generally benign, solitary cyst and microscopically consists of inner ciliated pseudostratified columnar epithelium, subepithelial connective tissue, a smooth muscle layer, and outer fibrous capsule. Although ciliated cysts commonly arise from the tracheal bronchial tree and the esophagus, they could also be found in the liver with rare incidence. There are several reports indicating this lesion can cause clinical problems, such as biliary obstruction and malignant transformation. Herein, we present a case of 56-year-old male patient who recently underwent laparoscopic excision of CHFC, focusing our discussion on review of literatures and rationale of laparoscopic surgery on CHFC.     

外科治疗非寄生虫性肝囊肿的选择策略

目的 探讨非寄生虫性肝囊肿外科治疗方法的选择策略及疗效.方法 回顾性分析上海市第六人民医院1995-2005年应用三种不同方法治疗284例非寄生虫性肝囊肿的临床资料.结果 囊肿穿刺抽液+注射无水乙醇161例:出现并发症9例(5.59%),复发53例(32.92%),病死率为0.开腹手术71例:出现并发症16例(22.54%),复发8例(11.27%),病死率2.82%.腹腔镜手术52例:出现并发症7例(13.46%),复发6例(11.54%),病死率为1.92%.结论 非寄生虫性肝囊肿外科治疗方式的选择,目前无统一标准.腹腔镜手术具有一定优势,但临床上应该根据病人的具体情况选择"个体化"的治疗方式.     

Prenatal pulmonary hypertension index: novel prenatal predictor of severe postnatal pulmonary artery hypertension in antenatally diagnosed congenital diaphragmatic hernia

Purpose

This study aim to assess the potential of prenatal predictors of postnatal severe pulmonary artery hypertension (PAH) in isolated left congenital diaphragmatic hernia (CDH) and to define a new prenatal pulmonary hypertension index (PPHI).

Methods

A retrospective chart review of CDH patients between May 2005 and October 2008 was conducted. Ten patients with systemic/suprasystemic and 9 patients with subsystemic pulmonary hypertension at 3 weeks of age were identified. Diameters of the right pulmonary artery, left pulmonary artery (LPA_d), aorta, and the length of vermis of the cerebellum were obtained from prenatal magnetic resonance imaging to calculate the PPHI [=(LPA_d/length of vermis of the cerebellum) × 10] and the modified McGoon index (MGI) [=(diameter of the right pulmonary artery + LPA_d)/diameter of aorta]. Prenatal pulmonary hypertension index and MGI were compared with lung-to-head ratio, percent predicted lung volume, and total lung volume for pulmonary hypertension and survival.

Results

The PPHI and MGI had a significant, negative correlation with pulmonary hypertension (r = −0.61, P = .005, and r = −0.72, P < .005, respectively). The PPHI and MGI are significantly lower in the systemic/suprasystemic PAH group compared with the subsystemic PAH group (1.11 ± 0.32 versus 1.63 ± 0.28, P = .004, and 0.71 ± 0.15 versus 1.05 ± 0.11, P < .001, respectively). There were no significant differences between the groups comparing the lung-to-head ratio, percent predicted lung volume, and total lung volume.

Conclusion

Both PPHI and MGI accurately predict the severity of postnatal PAH in isolated left CDH.     

Prenatal diagnosis and management of abdominal diseases in pediatric surgery

Purpose

The aim of this study was to investigate the prenatal courses and management of abdominal surgical diseases.

Methods

Of the 327 patients registered with our fetal treatment board since March 2002, 83 fetuses referred to the surgical department were enrolled for the current study. The prenatal diagnosis, sequential fetal images, and perinatal courses of these cases were reviewed retrospectively.

Results

Of the 83 cases, abdominal diseases were suspected in 34, lung and thoracic diseases in 25, genitourinary diseases in 12, and other anomalies in 12. Meconium peritonitis (MP), intestinal obstruction, and abdominal wall defects accounted for approximately 65% of the abdominal diseases. Five patients with prenatally diagnosed lung diseases underwent fetal surgical intervention, and 17 of the 22 liveborn patients survived. In contrast, none of the patients with prenatally diagnosed abdominal anomalies underwent fetal surgical intervention, yet, 23 of the 24 liveborn patients survived. However, preterm labor and hydrops were seen frequently in the patients with giant cystic MP, suggesting a fetal critical condition.

Conclusions

Although the clinical outcome of abdominal diseases seemed favorable with postnatal treatment, the current results suggested the occurrence of hidden mortality in utero and the potential need for fetal intervention for some abdominal conditions, such as MP.     

Urologic and anorectal complications of sacrococcygeal teratomas: Prenatal and postnatal predictors

Purpose

Anorectal and urologic sequelae are observed in long-term survivors of sacrococcygeal teratoma (SCT). In this study we evaluate the incidence and predictors of anorectal and urologic complications in SCT.

Methods

A retrospective review was performed for all SCT patients who underwent resection at a single institution between 2000 and 2012. Enrollment criteria included a minimum of 12 months follow-up. Categorical variables were analyzed by Fisher’s exact test and continuous variables by Mann Whitney test (p < 0.05).

Results

Forty-five patients were studied. Anorectal complications occurred in 29%, including severe chronic constipation (n = 13) and fecal incontinence (n = 4). Urologic complications occurred in 33%, including neurogenic bladder (n = 12), vesicoureteral reflux (n = 5), and urinary incontinence (n = 7). Prenatal imaging by fetal MRI demonstrated mass effect with obstruction of the bowel (n = 4) or bladder and collecting system (n = 7) in a subset of patients with postnatal complications (anorectal 4/4, PPV 100%; urologic 6/7, PPV 86%). Postnatal complications were associated with obstructive findings on prenatal imaging, prenatal therapeutic interventions, Altman classification, perineal reconstruction, and tumor recurrence. No anorectal or urologic complications occurred in patients with Altman type I tumors.

Conclusions

Urologic and anorectal complications are common in patients with SCT. Higher Altman classification and prenatal imaging suggestive of intestinal or urologic obstruction should prompt focused prenatal counseling and postnatal screening for anorectal and urologic dysfunction.     

先天性肾上腺皮质增生的产前诊断及治疗

先天性肾上腺皮质增生是肾上腺皮质激素合成途径中酶缺陷所致,是导致新生儿两性畸形的常见原因之一。随着分子遗传学技术的发展,达到了对本病的早期产前诊断。早期官内干预治疗可以有效改善新生儿外生殖器畸形症状。本综述以21-羟化酶缺乏致先天性肾上腺皮质增生为例介绍近年来其产前诊断及早期宫内治疗的进展。     

Prenatal diagnosis of urinary tract malformation

Malformation of the urinary tract (UT) is among the most common of all congenital malformations. Prior to the common usage of prenatal ultrasound, these anomalies were undetected until pediatric complications prompted investigation. When diagnosed and treated in early infancy, children with urinary tract malformations have a much better prognosis than when diagnosis is delayed beyond 1–2 years. Since the first report of the prenatal diagnosis of polycystic kidney disease by Garrett et al. in 1970, most forms of congenital urinary tract malformation have been diagnosed antenatally with the use of sonography. A review of the normal and abnormal development of the urinary system, some genetic aspects of UT malformations, and an overiew of the major UT anomalies and their prenatal diagnosis is presented.     

Cervico-Mediastinal Bronchogenic Cyst Occurring in the Prenatal Period: Report of a Case

We experienced a case of cervico-mediastinal bronchogenic cyst in which a cervical cystic mass was detected by prenatal ultrasonography. On prenatal ultrasound, a unilocular, well-defined and hypoechoic mass was detected in the fetal neck. The baby was born by a normal vaginal delivery at 40 weeks of gestation, and had no respiratory distress. Radiological investigations demonstrated a cyst in the cervico-mediastinal region, which displaced the trachea to the left. At the age of 32 days, an elective resection was easily performed through a right inferior collar incision after first aspirating the contents of the cyst. Prenatal sonography showing abnormal findings is effective for identifying cysts in the perinatal period and allows for the timely resection of such cysts before respiratory distress occurs. Received: October 22, 1999 / Accepted: May 30, 2000     

超声产前诊断胎儿畸形

目的探讨超声在产前诊断胎儿畸形的临床价值。方法采用彩色多普勒超声诊断仪对2230例孕11～40周的孕妇进行系统超声检查并随访。结果2230例孕妇中，超声诊断胎儿畸形28例，漏诊8例。发生畸形位居前三的是心血管畸形，神经管畸形。泌尿系统畸形。漏诊病例多为心血管畸形及较小畸形。结论超声检查可以对胎儿形态结卞句方面的明显畸形作出明确的产前诊断，明显减少出生缺陷，提高人口素质。对于心血管畸形及较小畸形还有待于改进方法，加强规范操作。     

Thoracoabdominal foregut duplication cyst with respiratory epithelium and alimentary epithelium

Thoracoabdominal foregut duplication is a rare congenital abnormality. The authors report a case of thoracoabdominal foregut duplication cyst in a 13-year-old male patient. The pathologic report revealed that a thoracic mass with a pseudostratified, ciliated, columnar epithelial lining (respiratory tract epithelium), an abdominal mass with gastric mucosa (alimentary tract epithelium), and the cyst originated from the foregut.     

Prenatal ultrasonographic gastrointestinal abnormalities in fetuses with gastroschisis do not correlate with postnatal outcomes

Purpose

In the setting of gastroschisis, the clinical significance of prenatal ultrasound findings of secondary changes in bowel appearance remains unknown. The purpose of this study was to correlate prenatal identification of additional gastrointestinal sonographic abnormalities with postnatal clinical outcome.

Methods

A retrospective review was conducted on 64 fetuses with a prenatal diagnosis of gastroschisis treated at the Children's Hospital of Philadelphia from 2000 to 2007. Postnatal outcomes were compared between newborns with additional sonographic gastrointestinal abnormalities and those without secondary changes to the bowel appearance.

Results

Thirty (47%) patients had at least one gastrointestinal abnormality (eg, bowel dilatation, echogenic bowel, thickened bowel, matted bowel, herniation of the stomach through the abdominal wall defect, or segmental loss of bowel peristalsis) on prenatal ultrasound. There were no significant differences between groups with respect to the time to initial and full enteral nutrition, total hospital stay, requirement for ventilator support, central line infection rates, reoperation rates, or mortality.

Conclusions

In the setting of gastroschisis, isolated findings of gastrointestinal abnormalities on prenatal ultrasound do not correlate with adverse postnatal outcome.     

Congenital lingual cysts

Congenital lingual cysts are rare congenital tumors of the oropharynx, which should be suspected when the tongue appears enlarged by a mass. Prenatal diagnosis by ultrasound allows for an optimal management at the time of delivery because the mass can obstruct the airways. Unless the cyst enlarges and hinders feeding or breathing, surgical treatment is indicated only a few months after birth because spontaneous regression can sometimes be observed.