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Poland syndrome accompanied by internal iliac artery supply disruption sequence: a case report.

ABSTRACT: BACKGROUND:Poland syndrome is a congenital malformation characterized by ipsilateral hand and chest wall depression, including an absence or hypoplasia of the breast and pectoral muscles. These hypoplastic defects are reportedly caused by a subclavian artery supply disruption sequence. CASE PRESENTATION:A 45-year-old Japanese woman, an out-patient, underwent an emergency examination for intense left lower abdominal pain. Computed tomography images revealed a hydronephrotic left kidney and dilatation of the left ureter. No ureteral calculus or neoplasm was found. In addition, no abnormalities connected to her left abdominal pain were found. Nephritis was diagnosed based on the results of urine analysis, and a course of antibiotics was administered. Computed tomography images also revealed a history of breast reconstruction with a custom-made silicone implant in her right breast. The present case showed symptoms of Poland syndrome, which were absence of the sternal head of the right pectoralis major and asymmetrical malformation of the chest wall due to hypoplasia of the right rib cage. In addition to typical Poland syndrome symptoms, she had hypoplasia of her right kidney, hypoplasia of the right gluteus minimus muscle, right-sided pelvic hypoplasia, spinal curvature to the right, and a cystic mass in her right ovary. CONCLUSIONS:In the present case of Poland syndrome, computed tomography images revealed malformation of the chest wall, absence of the pectoral muscle, and hypoplasia of a left kidney. Unilateral visceral hypoplasia is reported to be caused by a subclavian artery supply disruption sequence that occurs around 7 to 8 weeks of gestation. The present case can be considered a rare atypical phenotype of Poland syndrome with possible subclavian artery supply disruption sequence with internal iliac artery supply disruption.

SUBMITTER: Gonda K

PROVIDER: S-EPMC6203193 | biostudies-literature | 2018 Oct

REPOSITORIES: biostudies-literature

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Poland syndrome accompanied by internal iliac artery supply disruption sequence: a case report.

Gonda Kenji K Tachiya Yosuke Y Hatakeyama Yuichi Y Momma Tomoyuki T Tamaoki Tomoko T Maejima Yuko Y Rokkaku Yuichi Y Saji Shigehira S Shimomura Kenju K Kono Koji K

Journal of medical case reports 20181026 1

<h4>Background</h4>Poland syndrome is a congenital malformation characterized by ipsilateral hand and chest wall depression, including an absence or hypoplasia of the breast and pectoral muscles. These hypoplastic defects are reportedly caused by a subclavian artery supply disruption sequence.<h4>Case presentation</h4>A 45-year-old Japanese woman, an out-patient, underwent an emergency examination for intense left lower abdominal pain. Computed tomography images revealed a hydronephrotic left ki ...[more]

PMID: 30360754

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Project description:ObjectiveInternal iliac artery (IIA) preservation is associated with improved outcomes after both open and endovascular aortoiliac aneurysm repair. Total robotic laparoscopic repair of aortoiliac aneurysms has been reported in the past, but not in combination with sutureless anastomosis applied to the IIAs. The objective of this study was to demonstrate the feasibility of the total robotic laparoscopic technique including a method of deploying the Gore Hybrid Vascular Graft (GHVG; W. L. Gore & Associates, Flagstaff, Ariz) using robotic instruments.MethodsBetween June 2015 and December 2016, four patients underwent total robotic laparoscopic repair of isolated common iliac artery (CIA) aneurysms. Two patients had unilateral aneurysms and two had bilateral aneurysms. Unilateral CIA aneurysms were treated with a graft from the proximal CIA to the proximal external iliac artery, and bilateral CIA aneurysms were treated with a bifurcated graft between the distal aorta and both proximal external iliac arteries. The nitinol reinforced section of the GHVG was then inserted and deployed into the corresponding IIA, and the nonreinforced segment was sewn in an end-to-side fashion to the iliac graft.ResultsThe median age of patients was 55.5 years (range, 48-64 years); median body mass index was 24.9 kg/m2 (range, 23-26.4 kg/m2). All four cases were technically successful. Operative times were 325 and 332 minutes for unilateral cases and 491 and 385 minutes for bilateral cases. For the entire series, median proximal clamping time was 143 minutes (range, 110-163 minutes), and the median time to deploy the GHVG was 15 minutes (range, 8-27 minutes). The median estimated blood loss was 1800 mL (range, 800-2100 mL). Intraoperative cell salvage was used in all cases. No intraoperative or postoperative complications occurred. No patient required blood transfusion. All patients tolerated a regular diet on postoperative day 2 and were discharged on postoperative day 4. Patients returned to work and full physical activity within 6 weeks (range, 2-6 weeks). At 6-month follow-up, computed tomography angiography demonstrated 100% patency of iliac artery grafts as well as of the GHVGs.ConclusionsTotal robotic laparoscopic CIA aneurysm repair is feasible in both unilateral and bilateral cases in carefully selected patients. The GHVG can be successfully deployed using robotic technique for IIA preservation during total robotic CIA aneurysm repair.

Dataset Information

Poland syndrome accompanied by internal iliac artery supply disruption sequence: a case report.

Publications

Poland syndrome accompanied by internal iliac artery supply disruption sequence: a case report.

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