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Operative management of splenic injury in a patient with proteus syndrome.

Kannan U, Mishra B, Subramanian A, Sagar S, Kumar S, Singhal M - J Emerg Trauma Shock (2014)

Bottom Line: In view of low hemoglobin and possibility of pathologic spleen, splenectomy was done.Microscopic examination of the spleen showed hemangiolymphangioma.The patient was discharged on the 5(th) post-operative day and is doing well at 6 months of follow-up.

View Article: PubMed Central - PubMed

Affiliation: Department of Surgery, Jai Prakash Narayan Apex Trauma Center, All India Institute of Medical Sciences, New Delhi, India.

ABSTRACT
A 20-year-old female with Proteus syndrome sustained splenic injury after fall from a bike. She was initially managed non-operatively at a different hospital for three days and was then referred to our level I trauma center in view of increasing abdominal pain and distention. On admission in the Emergency Department (ED), her pulse rate was 120 per minute and blood pressure was 108/68 mm Hg. Clinical examination showed a distended abdomen with left hypochondrial pain. Ultrasonogram (USG) and Computed Tomography (CT) of the abdomen showed splenomegaly and grade III splenic injury with significant hemoperitoneum. Her hemoglobin was 2.9 g/dl with packed cell volume (PCV) of 12%. In view of low hemoglobin and possibility of pathologic spleen, splenectomy was done. Microscopic examination of the spleen showed hemangiolymphangioma. The patient was discharged on the 5(th) post-operative day and is doing well at 6 months of follow-up.

No MeSH data available.


Related in: MedlinePlus

Disproportionate growth of toes with vascular malformations
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Figure 1: Disproportionate growth of toes with vascular malformations

Mentions: A 20-year-old female, a known case of Proteus syndrome, presented four days after fall from a bike. She was referred to our level I trauma center after being managed non-operatively for three days at a different hospital in view of increasing abdominal distention and pain. The exact details of the treatment given were not available. On admission in the Emergency Department (ED), her pulse rate was 120 per minute and blood pressure was 108/68 mm Hg. Abdominal examination revealed significant distention with tenderness over the left hypochondrium without rebound tenderness or guarding. The general physical examination was remarkable for the manifestations of Proteus syndrome that included arteriovenous malformations and hemihypertrophy of her left leg with multiple lipomas over the abdomen [Figure 1]. Her hemoglobin was 2.9 g/dl with packed cell volume (PCV) of 12%, platelet count of 1,08,000 cells/cubic mm, and PT-INR of 1.29. Ultrasonogram (USG) of the abdomen showed a moderately enlarged spleen with cystic reticulated appearance. Contrast-enhanced tomography (CECT) of the abdomen showed a non-enhancing enlarged spleen of size 17 cm × 15 cm × 10 cm [Figure 2], massive hemoperitoneum with normal liver and kidney with no leakage of the contrast. In view of low hemoglobin and possibility of pathologic spleen, splenectomy was done. Intra-operative findings included an enlarged spleen with 3 cm laceration along the lower pole and 2 liter of hemoperitoneum. Her Injury Severity Score (ISS) was 9 (3 for splenic injury). Pathological examination showed an enlarged spleen of size 17 cm × 15 cm × 10 cm with spongy cut surface, weighing 230 gm. Histological examination showed multiple vascular spaces lined by single flattened layer of endothelium filled with fibrin and red blood cells, suggesting a hemangiolymphangioma [Figure 3]. She required 5 units of blood transfusion in the peri-operative period, and her hemoglobin increased to 8.7 gm% with a hematocrit of 30%, platelet count of 1,30,000 cells/cubic mm, and PT-INR of 1.20. She was discharged on the 5th post-operative day and is doing well at 6 months of follow-up.


Operative management of splenic injury in a patient with proteus syndrome.

Kannan U, Mishra B, Subramanian A, Sagar S, Kumar S, Singhal M - J Emerg Trauma Shock (2014)

Disproportionate growth of toes with vascular malformations
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4126126&req=5

Figure 1: Disproportionate growth of toes with vascular malformations
Mentions: A 20-year-old female, a known case of Proteus syndrome, presented four days after fall from a bike. She was referred to our level I trauma center after being managed non-operatively for three days at a different hospital in view of increasing abdominal distention and pain. The exact details of the treatment given were not available. On admission in the Emergency Department (ED), her pulse rate was 120 per minute and blood pressure was 108/68 mm Hg. Abdominal examination revealed significant distention with tenderness over the left hypochondrium without rebound tenderness or guarding. The general physical examination was remarkable for the manifestations of Proteus syndrome that included arteriovenous malformations and hemihypertrophy of her left leg with multiple lipomas over the abdomen [Figure 1]. Her hemoglobin was 2.9 g/dl with packed cell volume (PCV) of 12%, platelet count of 1,08,000 cells/cubic mm, and PT-INR of 1.29. Ultrasonogram (USG) of the abdomen showed a moderately enlarged spleen with cystic reticulated appearance. Contrast-enhanced tomography (CECT) of the abdomen showed a non-enhancing enlarged spleen of size 17 cm × 15 cm × 10 cm [Figure 2], massive hemoperitoneum with normal liver and kidney with no leakage of the contrast. In view of low hemoglobin and possibility of pathologic spleen, splenectomy was done. Intra-operative findings included an enlarged spleen with 3 cm laceration along the lower pole and 2 liter of hemoperitoneum. Her Injury Severity Score (ISS) was 9 (3 for splenic injury). Pathological examination showed an enlarged spleen of size 17 cm × 15 cm × 10 cm with spongy cut surface, weighing 230 gm. Histological examination showed multiple vascular spaces lined by single flattened layer of endothelium filled with fibrin and red blood cells, suggesting a hemangiolymphangioma [Figure 3]. She required 5 units of blood transfusion in the peri-operative period, and her hemoglobin increased to 8.7 gm% with a hematocrit of 30%, platelet count of 1,30,000 cells/cubic mm, and PT-INR of 1.20. She was discharged on the 5th post-operative day and is doing well at 6 months of follow-up.

Bottom Line: In view of low hemoglobin and possibility of pathologic spleen, splenectomy was done.Microscopic examination of the spleen showed hemangiolymphangioma.The patient was discharged on the 5(th) post-operative day and is doing well at 6 months of follow-up.

View Article: PubMed Central - PubMed

Affiliation: Department of Surgery, Jai Prakash Narayan Apex Trauma Center, All India Institute of Medical Sciences, New Delhi, India.

ABSTRACT
A 20-year-old female with Proteus syndrome sustained splenic injury after fall from a bike. She was initially managed non-operatively at a different hospital for three days and was then referred to our level I trauma center in view of increasing abdominal pain and distention. On admission in the Emergency Department (ED), her pulse rate was 120 per minute and blood pressure was 108/68 mm Hg. Clinical examination showed a distended abdomen with left hypochondrial pain. Ultrasonogram (USG) and Computed Tomography (CT) of the abdomen showed splenomegaly and grade III splenic injury with significant hemoperitoneum. Her hemoglobin was 2.9 g/dl with packed cell volume (PCV) of 12%. In view of low hemoglobin and possibility of pathologic spleen, splenectomy was done. Microscopic examination of the spleen showed hemangiolymphangioma. The patient was discharged on the 5(th) post-operative day and is doing well at 6 months of follow-up.

No MeSH data available.


Related in: MedlinePlus