"Stealth surgery": transaxillary subcutaneous endoscopic excision of benign neck lesions

Background

Benign neck lesions are traditionally removed through an overlying incision. The resultant scar can be aesthetically displeasing. We previously reported our experience with a transaxillary subcutaneous endoscopic approach for management of torticollis. We now report a similar technique for removal of benign lesions of the neck.

Methods

The study uses a retrospective review of 5 elective transaxillary endoscopic procedures from March to December 2006. The lesions included an enlarged cervical lymph node, thyroglossal duct cyst, dermoid cyst, ectopic dilated neck vein, and a parathyroid adenoma. Outcome measures included need for conversion, cosmetic outcome, and complications.

Results

All procedures were successfully completed using the endoscopic approach. Postoperative pain was controlled with acetaminophen, and all patients were discharged from the hospital the same day. There were no intraoperative complications. The patient who had a thyroglossal cyst removed developed a postoperative seroma that resolved spontaneously. All families were pleased with the cosmetic results.

Conclusions

A transaxillary subcutaneous endoscopic approach can be applied effectively to a variety of benign lesions of the neck, allowing adequate exposure for dissection, and resulting in a quick recovery. Neck scarring is absent, with small scars well hidden in the axilla.     

Endoscopic excision of benign forehead masses: a novel approach for pediatric general surgeons

Purpose

Benign tumors of the brow and forehead are commonly managed by pediatric general surgeons by excision through an overlying incision. Cosmetic results in children can be suboptimal. Plastic surgeons have used endoscopic brow-lift techniques for the removal of these lesions. We review our experience after adopting this endoscopic technique in a pediatric general surgery practice.

Methods

We conducted a retrospective chart review of 9 consecutive outpatient procedures (5 girls and 4 boys; age range, 5 months to 12 years) between March and October 2005. Seven patients had lesions located on the lateral brow (left, n = 4; right, n = 3), 1 patient had a lesion on the left mid forehead, and 1 patient had a nasoglabellar cyst. All procedures were performed using endoscopic brow-lift equipment through a single small scalp incision 2 cm posterior to the hairline. Outcome measures included need for conversion, operative time, cosmetic outcome, and complications.

Results

All lesions (6 dermoid cysts and 3 pilomatrixomas) were successfully excised endoscopically. The mean operative time was 56 minutes (range, 22-90 minutes). There was no intraoperative or postoperative complication. All families were pleased with the cosmetic outcomes.

Conclusion

This case report shows that endoscopic excision of forehead masses is a safe and efficacious procedure in the hands of pediatric general surgeons.     

Fine needle aspiration biopsy in pediatric space-occupying lesions of liver: a retrospective study evaluating its role and diagnostic efficacy

Background

Liver space-occupying lesions (SOLs) in the pediatric population form a unique and distinct spectrum as compared with those in the adult population. There are no large studies available in the English literature regarding fine needle aspiration cytology (FNAC) in pediatric liver SOLs.

Aim

This study was done to familiarize with the cytomorphological features of these distinctive benign and malignant lesions of liver and to determine the specificity, sensitivity, and diagnostic efficacy of FNAC.

Methods

A total of 41 FNACs from pediatric liver SOLs were reviewed, and the diagnoses were correlated with clinical and radiological findings, histopathological diagnoses, and/or follow-up information. These were categorized into malignant, benign, and indeterminate groups.

Results

There were 20 malignant lesions that included hepatoblastoma (7), hepatocellular carcinoma (4), undifferentiated embryonal sarcoma of liver (3), and metastases (6). The 12 benign lesions included abscess (8), 1 case each of mesenchymal hamartoma, focal nodular hyperplasia, hydatid cyst, and tuberculosis. In the overall FNAC performance calculation, the specificity of FNAC was 100%, sensitivity 95%, positive predictive value 100%, negative predictive value 92.3%, and diagnostic accuracy 96.9%.

Conclusion

The recognition of the distinct cytomorphological features of pediatric focal liver lesions, in conjunction with the clinical data and radiological findings, make FNAC a useful primary diagnostic modality in these unique and distinctive lesions.     

Transaxillary subcutaneous endoscopic release of the sternocleidomastoid muscle for treatment of persistent torticollis

Purpose

Surgical correction of torticollis is occasionally necessary to curtail the facial deformity that can result from this condition. The resultant neck scar can be of suboptimal cosmesis, with consequent psychological distress for the child. We have previously described an endoscopic approach to forehead and brow lesions through scalp incisions. We now describe a transaxillary subcutaneous endoscopic approach to division of the fibrotic sternocleidomastoid muscle.

Methods

This study involved a retrospective chart review of 3 consecutive outpatient procedures (male-to-female ratio, 1:2; age range, 8 months to 7 years) from March to October of 2005. The 2 older patients had established sternocleidomastoid fibrosis, and 1 had complicated torticollis refractory to medical management. All procedures were performed using standard 3-mm-laparoscopic instrumentation through hidden incisions in the ipsilateral axilla. Outcome measures included need for conversion, operative time, cosmetic outcome, and complications.

Results

All patients were successfully treated endoscopically. Mean operative time was 50 minutes (range, 45-55 minutes). There were no intraoperative or postoperative complications. All families were pleased with the cosmetic outcome.

Conclusion

This case series demonstrates the simplicity and effectiveness of a transaxillary endoscopic subcutaneous approach to torticollis.     

Splenic cyst in a wandering spleen: laparoscopic treatment with preservation of splenic function

Background

Wandering spleen and splenic cyst are rare benign congenital conditions that can both cause severe complications related to torsion or trauma.

Case Report

A 14-year-old girl presented a mobile 10-cm-long abdominal mass in the left lower quadrant associated with mild abdominal pain.The diagnosis of an 8-cm-long nonparasitic cyst in a wandering spleen was confirmed by computerized tomography and negative serum indirect hemagglutination titer for hydatid disease.Laparoscopic unroofing of the cyst and splenopexy in a vycril mesh was performed.

Results

No problems were encountered during laparoscopic surgery. Postoperative course was uneventful, and at a 1-year follow-up, the spleen is viable and maintains a normal position in the phrenorenal angle.

Conclusions

In the child, treatment of wandering spleen associated with a cyst should aim at the prevention of vascular accidents and at conservation of the spleen. We achieved these goals with unroofing and splenopexy through laparoscopic surgery.     

Pediatric rib lesions: a 13-year experience

Background

Rib lesions in the pediatric population are rare but significant processes and are often neoplastic.

Methods

All patients with primary rib lesions evaluated by the Department of Surgery at Children's Hospital Boston from 1992 to 2005 were studied. The patient's diagnosis, sex, symptoms and their duration, radiologic evaluation, biopsy status, surgical procedure, and follow-up were assessed.

Results

Thirty-three patients, ages 3 to 23 years (median, 12.7 years), were evaluated. Sixteen patients (48%) had benign and 17 (52%) had malignant lesions. Within the benign cohort of 16 patients, there were 6 osteochondromas, 4 aneurysmal bone cysts, and 2 fibrous dysplasias as well as 1 of each of the following: enchondroma, periosteal chondroma, eosinophilic granuloma, and chondrophyte. Within the malignant cohort of 17 patients, 13 were diagnosed with Ewing's sarcoma, 3 with osteogenic sarcoma, and 1 with chondrosarcoma. The sex distribution for the malignant group was 11 (65%) females and 6 (35%) males.

Conclusions

Rib tumors are rare entities in the pediatric population. However, a significant number of rib lesions are malignant. Therefore, proper diagnosis and expeditious treatment are critical.     

Surgical management of lipoblastoma

Background/Purpose

Lipoblastoma/lipoblastomatosis, a rare benign pediatric tumor composed of embryonic fat, most often presents as a rapidly growing mass in children younger than 3 years. This benign tumor locally recurs up to 25% of the time. This retrospective review was done to emphasize problems with diagnosis, management, and the approach to follow-up care.

Methods

Sixteen cases of pathologically proven lipoblastoma presenting to a single institution over a 16-year period (1988-2004) were reviewed retrospectively for clinical features and outcome.

Results

There were 11 males and 5 females ranging in age from 18 days to 12.75 years. Preoperative diagnoses were accurate in only 3 cases. Two lesions recurred locally over a period of 10 months and 5 years. One lesion presented with local infiltration. Six lesions were right-sided, 5 were left-sided, and 5 were midline.

Conclusions

It is important to consider lipoblastoma in the diagnosis of a rapidly enlarging fatty mass in children. Complete resection is the only definitive treatment and should not be delayed when impingement on surrounding structures is imminent. There is a tendency for these lesions to recur despite presumed complete excision. Therefore, follow-up for a minimum of 5 years is recommended.     

Predictors of malignancy in screen-detected breast masses with indeterminate/equivocal (grade 3) imaging features

Background

The mammographic grading system used by BreastScreen Australia accredited programs requires needle biopsy of lesions with indeterminate features (grade 3). We wished to determine the predictors of malignancy for these common lesions, so as to reduce surgery for benign lesions.

Design

Grade 3 masses assessed between Jan 1996-Dec 2005 are included. New or changing lesions were identified through film review. Imaging, demographic and final outcome data were tabulated. Statistical analysis was performed to determine the predictors of malignancy.

Results

1181 lesions, including 623 new or changing masses are assessed. Of these 98 (8.3%) were malignant. Malignancy was found in 3% first round masses versus 13% of new or changing lesions. Withholding needle biopsy results, interval change (OR 2.85, p = 0.0001), increasing age (p = 0.0001) and diameter were independent predictors of malignancy, lesion diameter having an inverse relationship with malignancy.Once needle biopsy results were included, this parameter became the sole predictor of malignancy as other factors lost significance in a multivariate model.

Conclusion

Surgical biopsy is not indicated in well-sampled grade 3 masses with benign needle biopsy results. Surgery is valuable for diagnosis or treatment of the remaining such masses without benign needle biopsy results.     

Early diagnostic and endoscopic dilatation for the treatment of acquired upper airway stenosis after intubation in children

Background

Upper airway stenosis (UAS) after endotracheal intubation is a common problem in children. Most literature refers to a surgical treatment for these lesions. Laryngotracheal reconstruction and cricotracheal resection are used for low- and high-grade stenosis, but decannulation is not always possible immediately after surgery.

Purpose

The aim of this study was to verify the feasibility and results of endoscopic dilatations for treatment of subglottic stenosis.

Method

The study encompassed a 12-year retrospective analysis of patients treated for UAS in a tertiary center. All children were symptomatic at the time of the endoscopic diagnosis. The stenosis was graded according to the Myer-Cotton criteria. Endoscopic dilatation was initiated immediately after the diagnosis. Children with grade IV stenosis underwent surgery.

Results

Children with tracheal stenosis and no involvement of the subglottic area did not respond to endoscopic dilatations and underwent surgery. There were 45 children with grade I stenosis, 12 with grade II, 7 with grade III, and 4 with grade IV lesions. Patients with grade I, II, and III stenosis were all treated by endoscopic dilatations alone and were decannulated when asymptomatic. The average time for decannulation was 18.90 ± 26.07 months for grade I, 32.5 ± 27.08 months for grade II, and 27.57 ± 20.60 months for grade III stenosis (P < .01, for grade II vs grade III).

Conclusion

Grade I, II, and III subglottic stenoses can be safely managed by early endoscopic dilatations with a high rate of success and low rate of morbidity but require a significantly long period of treatment.     

The eyelid crease approach to angular dermoid cysts in pediatric general surgery

Purpose

We recently adopted an approach described by eyelid surgeons for angular dermoid cyst excision. The aim of this study was to compare the outcome of this technique with that accomplished through a conventional trans-eyebrow incision.

Methods

Prospective nonrandomized study of 34 consecutive children treated between January 2000 and December 2006. Twenty-six patients (group I) were operated on through a trans-eyebrow incision, whereas 8 (group II) received an upper eyelid crease incision. Performance measures included cosmesis, operative time, and complications. Cosmesis was assessed blindly with comparative photographs, using a 100-mm visual analog scale.

Results

No significant differences were found between groups I and II with regard to age at surgery (22 ± 17 vs 24 ± 4 months; P = .07) and operative time (42 ± 21 vs 40 ± 16 minutes; P = .9). In group II, the scar resulted invisible when the affected side was assessed with the eye open and still significantly better than group I, when evaluated with the eye closed (96 ± 7 vs 81 ± 18 mm; P = .0001). All patients and their families reported great satisfaction and an excellent scar. There were neither major postoperative complications nor recurrence of the lesion.

Conclusions

Angular dermoid cyst excision using an eyelid crease approach yields excellent cosmesis and represents a safe, simple, and easily reproducible procedure in pediatric general surgical practice.     

Can biliary-cyst communication be predicted before surgery for hepatic hydatid disease: does size matter?

Background

The aim of this study was to determine if there is any predictive factor indicating the risk of bile leakage before surgery for hepatic hydatid disease in clinically asymptomatic patients.

Methods

The data of 116 patients who underwent surgery for hepatic hydatid disease were reviewed retrospectively. There were 43 men (37%) and 73 women (63%) with a mean age of 45 ± 15 years. Because of high preoperative serum bilirubin and liver function test levels, 12 patients were excluded from the study. These patients underwent preoperative endoscopic retrograde cholangiopancreatography. In addition, 2 medically treated patients were excluded from the study. The following variables were analyzed as potential predictors of biliary-cyst communication: age, sex, physical examination findings, leukocyte count, liver function test results, and ultrasonographic cyst features (type, diameter, number, and localization).

Results

Bile leakage was detected in 24 out of 102 patients. There were no differences in age, sex, cyst type, alkaline phosphatase level, γ-glutamyl transpeptidase level, alanine aminotransferase level, aspartate aminotransferase level, bilirubin level, and number of cysts and cyst locations between the patients with and without bile leakage. The mean cyst size in patients with biliary leakage was 10.2 cm as compared with 6.1 cm in patients with no biliary leakage (P < .05). When the cut-off value of cyst diameter was accepted as 7.5 cm, the specificity and sensitivity for biliary-cyst communication were 73% and 79%, respectively.

Conclusions

These data suggest that cyst diameter is an independent factor that is associated with a high risk of biliary-cyst communication in clinically asymptomatic patients. Preoperative endoscopic retrograde cholangiopancreatography should be performed in these asymptomatic patients to reduce the incidence of postoperative complications.     

Hepaticojejunostomy and intrahepatic cystojejunostomy for type IV-A choledochal cyst

Background

The incidence of choledochal cyst with intrahepatic involvement (Todani's type IV-A cyst) is considerably high. Dilatation of the intrahepatic bile duct is frequently observed around the hepatic hilum, occasionally in the umbilical portion, and rarely in the more upstream intrahepatic bile duct, associated with or without downstream stricture. We recently encountered 2 children with type IV-A cyst associated with upstream intrahepatic ductal dilatation; one with a cystic dilatation of the medial branch arising from the left hepatic duct and another with a cyst of the medial branch arising from the anterior hepatic duct.

Methods

After excision of the extrahepatic bile duct cyst at the hilum and making a large fenestration of the intrahepatic duct cyst, hepaticojejunostomy and intrahepatic cystojejunostomy were performed using a Roux-en-Y jejunal loop in both children.

Results

Postoperatively, both intrahepatic cysts were remarkably reduced in size, and recurrent bouts of abdominal pain did not occur for up to 4 or 5 years.

Conclusion

Hepaticojejunostomy at the hepatic hilum, combined with intrahepatic cystojejunostomy, appears to be a recommendable procedure for an upstream intrahepatic ductal cyst of type IV-A, preventing postoperative cholangitis owing to bile stone formation.     

Clinical value of office-based endoscopic incisional biopsy in diagnosis of nasal cavity masses

Objective

To evaluate clinical features and the diagnostic accuracy of office-based endoscopic incisional biopsy in patients with nasal cavity masses.

Study Design

Diagnostic test assessment with chart review.

Setting

Tertiary referral center.

Subjects and Methods

From January 1997 to August 2006, preoperative diagnosis was achieved using endoscopic incisional biopsy in 521 patients. Cytopathologic and histologic findings were categorized as malignancy, benign neoplasm, or non-neoplastic lesion. Preoperative imaging was done in 462 patients (computed tomography: 438 cases; magnetic resonance imaging: 24 cases). We investigated the accuracy of endoscopic incisional biopsy and preoperative imaging by comparing it with pathologic results from tumor resection as the “gold standard.”

Results

Most of the patients had unilateral nasal symptoms (e.g., nasal obstruction, unilateral epistaxis, unilateral facial pain), and the clinical symptoms were of little diagnostic value in the differentiation of tumor and inflammatory lesion. The sensitivity and specificity of endoscopic incisional biopsy were 43.7 and 98.9 percent, respectively, for the diagnosis of nasal cavity malignancies, and 78.2 and 96.2 percent, respectively, for the diagnosis of benign neoplasms. The sensitivity and specificity of preoperative imaging were 78.3 and 97.5 percent, respectively, for the diagnosis of nasal cavity malignancies and 66.4 and 86.3 percent, respectively, for the diagnosis of benign neoplasms. Combining the two modalities increased diagnostic accuracy in nasal cavity masses.

Conclusion

Endoscopic incisional biopsy alone did not ensure accurate diagnosis of nasal cavity tumors, but in combination with preoperative imaging it was helpful for the diagnosis of nasal cavity malignancies.     

Sentinel lymph node biopsy in the pediatric population

Background

Sentinel lymph node biopsy (SLNB) has only been recently used for childhood neoplasms.

Methods

We reviewed all patients younger than 19 years who underwent SLNB for 5 years.

Results

Twenty patients were identified (11 male, 9 female). Sentinel lymph node biopsy was performed for 10 sarcomas (5 synovial, 3 rhabdomyosarcoma, 1 epitheliod, 1 other); 9 skin neoplasms (4 melanomas, 3 Spitz nevi, 2 melanocytomas); and 1 acinic cell carcinoma. All patients underwent Technetium 99m sulfur microcolloid injection and 4-quadrant subdermal injection with Lymphazurin 1% (Autosuture, Norwalk, Conn). Six patients required either sedation for lymphoscintigraphy. Intraoperative gamma probe was used. Primary lesions were found in lower extremity (n = 8), upper extremity (n = 6), trunk (n = 3), and head and neck (n = 3). The lymphatic basins were inguinal (n = 8), axilla (n = 8), neck (n = 3), and both inguinal and axilla (n = 1). At least one lymph node was identified in each procedure. Of 20 patients, 5 (25%) had metastatic disease (4 skin neoplasms and 1 sarcoma). There were no complications in our series, and all patients are alive with no recurrence at an average follow-up of 2.2 years.

Conclusions

Sentinel lymph node biopsy allows for an accurate biopsy in children. However, some younger patients may require sedation, and it may be more challenging to isolate the sentinel node.     

Endoscopic mucosal resection as a staging technique to determine the depth of invasion of esophageal adenocarcinoma

Background

Endoscopic ablation and vagal-sparing esophagectomy offer the potential for reduced morbidity in patients with high-grade dysplasia or early esophageal adenocarcinoma, but neither includes a lymphadenectomy. Although adequate for intramucosal tumors, both are potentially inadequate for patients with submucosal tumor invasion given the high prevalence of nodal metastases with these lesions. Currently there is no test including endoscopic ultrasound that can accurately determine whether a small tumor is confined to the mucosa or has penetrated into the submucosa. The aim of this study was to compare the pathologic depth of invasion by endoscopic mucosal resection with findings and outcome after surgical resection to assess the accuracy and reliability of endoscopic mucosal resection for staging early esophageal adenocarcinoma.

Methods

From 2001 to 2003, 7 patients presented with small, endoscopically visible adenocarcinomas. All underwent endoscopic mucosal resection followed by surgical resection.

Results

Analysis of the resected specimens confirmed that the endoscopic mucosal resection had accurately determined the depth of tumor invasion in all patients, and had completely excised the lesion in all but 1 patient (86%). Lymph node dissection was included as part of the resection in 2 patients with submucosal invasion by endoscopic mucosal resection, and a vagal-sparing esophagectomy was used in the 5 patients with only intramucosal tumors. All patients are alive and disease-free at a median follow-up of 7 months.

Conclusions

Endoscopic mucosal resection accurately determines the depth of tumor invasion, and should be used as a staging procedure in patients with early esophageal cancer when therapies that do not include a lymphadenectomy are considered.     

Ex utero intrapartum treatment with placement on extracorporeal membrane oxygenation for fetal thoracic masses

Purpose

We describe our experience with fetuses diagnosed with life-threatening chest masses who were delivered by ex utero intrapartum treatment with placement on extracorporeal membrane oxygenation (EXIT-to-ECMO).

Methods

The first fetus presented with a cystic mediastinal mass and enlarging echogenic lungs. Bronchoscopic evaluation during ex utero intrapartum treatment (EXIT) revealed complete airway obstruction secondary to a carinal bronchogenic cyst. The second fetus presented with a massive left congenital cystic adenomatoid malformation. The EXIT procedure was performed because of significant mediastinal shift, severe compression of the normal lung parenchyma, and signs of fetal distress.

Results

In both cases, extracorporeal membrane oxygenation (ECMO) was initiated while on placental support. The fetuses were then delivered, and a definitive resection of their thoracic lesions was successfully performed. There were no major perioperative complications. Both children made expedient recoveries without significant cardiopulmonary sequelae.

Conclusion

To our knowledge, this is the first report describing the successful use of EXIT-to-ECMO as a bridge to definitive resection of large chest masses diagnosed in utero. EXIT-to-ECMO is a novel and effective management strategy for stabilizing patients with profound respiratory compromise secondary to congenital thoracic lesions.     

Role of intraoperative enteroscopy in the management of obscure gastointestinal bleeding at the time of video-capsule endoscopy

Background

This study aimed at evaluating the role of intraoperative enteroscopy (IOE) for the management of obscure gastrointestinal (GI) bleeding in patients who had been preoperatively explored by video-capsule endoscopy (VCE).

Methods

Eighteen patients who underwent IOE for obscure GI bleeding were prospectively recorded between November 2000 and January 2007. The bleeding site was preoperatively localized by VCE in the small bowel in 15 patients, but the origin of bleeding remained unknown in 3 patients.

Results

In the 3 patients with negative VCE, IOE was normal, but intraoperative conventional endoscopy identified gastric (n = 1) and colonic (n = 2) lesions. Among the 15 patients with VCE positive for small-bowel lesions, laparotomy and IOE yielded localization and treatment (surgical n = 11 and endoscopic n = 2) guidance for 13 of 15 (87%) lesions. At median 19-month follow-up, 3 bleeding recurrences (3 of 15 [20%]) were recorded, resulting in a 73% therapeutic efficacy of IOE.

Conclusions

IOE remains useful for the management of obscure GI bleeding when preoperative VCE is positive for small-bowel lesions that are not reachable by nonoperative enteroscopy. When VCE is negative, new conventional endoscopy should be proposed instead of IOE.     

Endoscopic versus open component separation in complex abdominal wall reconstruction

Background

Open component separation has a high wound complication rate. Newer endoscopic approaches are described with no comparative trials.

Methods

A retrospective review (2005-2009) of patients undergoing open or endoscopic component separation was performed.

Results

Forty-four cases were identified (22 endoscopic; 22 open). All perioperative variables were the same except age (65 open vs 55 endoscopic; P < .05). Hospital length of stay was 11 days in the open group versus 8 days in the endoscopic group (P = .09). Wound complications were 52% in the open group versus 27% in the endoscopic group (P = .09). Wound-related interventions occurred in 45% of the open group and 33% of the endoscopic group. Hernia recurrences rates were similar (open, 32%; endoscopic, 27%; P = .99).

Conclusions

Open and endoscopic components separation have similar rates of recurrence. The endoscopic group had shorter lengths of stay and less major wound complications. The endoscopic approach may be the ideal technique for complex abdominal wall reconstruction.     

Laparoscopic distal pancreatectomy using radiofrequency energy

Introduction

The pancreatic remnant remains a significant source of morbidity during laparoscopic pancreatectomy. Previous series have relied heavily on the endoscopic stapler for transection. Our study is the first to report use of a laparoscopic radiofrequency device for pancreatic transection.

Methods

The laparoscopic Habib 4x delivers high-energy radio waves through a hand-held device consisting of 4 electrodes and allows for bloodless tissue transection. We retrospectively evaluated prospectively collected data. Fourteen patients were identified and used in our analysis.

Results

There were no conversions, blood transfusions, reoperations, or mortalities. Average length of stay was 4.6 days. There was 1 readmission. Clinically significant fistula occurred in 2 patients (14%), only one of which required an intervention.

Conclusion

Radiofrequency energy is safe and feasible for use during laparoscopic pancreatic transection. Moreover, it is technically simple to use.     

Portal vascular anomalies in Down syndrome: spectrum of clinical presentation and management approach

Purpose

The occurrence of portal vascular anomalies in Down syndrome has been sporadically reported in the literature. These rare disorders have a wide spectrum of anatomical and clinical presentations. The aim of this communication was to describe the clinical course, imaging features, and management approaches in patients with this association.

Methods

We conducted a comprehensive search of the databases of the Vascular Anomalies Center and the Department of Radiology at Children's Hospital Boston for patients with Down syndrome and portal vascular anomalies. Medical records and imaging studies of varying modalities were reviewed.

Results

Three children with Down syndrome and portal anomalies (portosystemic shunt, simple arterioportal shunt, complex arterioportal shunt) were managed at our institution. The portosystemic shunt was clinically insignificant and resolved without any intervention. The simple arterioportal shunt was successfully treated with embolization. The complex arterioportal shunt was associated with major congenital cardiac defects and the child ultimately expired despite a decrease in the arterioportal shunting after embolization.

Conclusions

Three is a wide spectrum of clinical and anatomical features of portal vascular shunts in Down syndrome. The management approach should be tailored based on the severity of symptoms. Percutaneous embolization can offer a safe, effective, and minimally invasive alternative to the surgical approach in selective cases.