Neonatal Onset Diffuse Cutaneous Mastocytosis: A Case Report and Review of the Literature

Abstract: Diffuse cutaneous mastocytosis is a rare variant of mast cell disease with widespread erythroderma, which is normally clinically apparent in early infancy. We report the case of a neonate who presented with diffuse erythrodermic rash and bullous lesions. Biopsy specimens showed a dense dermal infiltrate of mast cells. Serum histamine and tryptase levels were elevated. No somatic mutation of the c‐kit gene was found. Blistering ceased at 5 months of age, but atopic dermatitis appeared at 6 months and allergic workup revealed a high level of food‐specific IgE. Herein, we describe the case and provide the first review of the literature on neonatal onset diffuse cutaneous mastocytosis to clarify the prognosis of this condition.     

Malignant serous cystic neoplasm of the pancreas: report of a case and review of the literature

BACKGROUND: In general, serous cystic neoplasms of the pancreas are thought to be benign. Malignant serous cystic neoplasm of the pancreas is a rare clinical entity. CASE REPORT: We report the case of an 87-year-old woman with a serous microcystic neoplasm in the tail of the pancreas that behaved in a malignant fashion. The neoplasm had also invaded the colonic mesentery and splenic hilum. The pancreatic lesion was diagnosed as a large malignant serous cystic neoplasm, and the patient underwent distal pancreatectomy with splenectomy and segmental colectomy. The resected specimen contained a large tumor, 12 x 9 x 8 cm, which occupied the body and tail of the pancreas. Histologically, the tumor was indistinguishable from serous cystadenoma. However, the tumor had invaded surrounding tissues including the splenic vein, and there were splenic invasion and a regional lymph node metastasis. The postoperative course was uneventful. DISCUSSION: There are few reported cases of malignant serous cystic neoplasm, in which malignancy was histologically confirmed in the resected specimen. There are no reports of a negative outcome with complete resection of the tumor. Surgical treatment should be considered for serous cystic neoplasms, especially large ones, because of the malignant potential.     

Implications of arterial anatomy in patients with cancer of the periampullary region

BACKGROUND/AIMS: Preoperative information on arterial anatomy in the peripancreatic and hepatic areas is valuable to any surgeon performing pancreatoduodenectomy. METHODOLOGY: Between 1994 and 1998, 49 patients with periampullary cancer (31 distal bile duct and 18 ampullary tumors) underwent visceral angiography and radical pancreatoduodenectomy with lymphadenectomy. Surgically "significant" arterial variations and their effects on operative management and results were examined retrospectively. RESULTS: Arterial variations were found in 18 patients (37%); 15 (31%) were "significant" and 3 (6%) were "nonsignificant." All 15 patients with "significant" variants required specific type of various preservations of the hepatic arterial system. Intraoperative blood loss, transfused blood units, and operation time were greater in patients with "significant" variations than in patients without (P<0.05). Histopathologic diagnosis, tumor staging, morbidity, and mortality did not differ between the 2 groups. The 5-year survival was 33% for patients with "significant" variations and 63% for patients without (P<0.05). CONCLUSIONS: Information on arterial anatomy in the peripancreatic and hepatic areas is necessary for preoperative evaluation in patients requiring radical pancreatoduodenectomy. Presence of "significant" arterial variations may be considered as one of the negative prognostic factors in patients with periampullary cancer.     

Successful living domino liver transplantation in a child with protein C deficiency

PC is produced in the liver and inhibits blood coagulation by catalyzing active factors V and VIII. PC deficiency causes abnormal blood clotting that is difficult to regulate by anticoagulative treatments. Four reports of PC deficiency treated with LTx have been published; however, no report of DLT as a therapy for PC deficiency is available. We describe a case of a 23‐month‐old girl who received DLT for compound heterozygous PC deficiency. Her PC activity was below 5%. She developed intracranial lesion and frequent refractory purpura fulminans. Both her parents had heterozygous mutations of PC genes and were excluded as living donors. Furthermore, she was a low priority on the waiting list of deceased‐donor transplantation. We performed living DLT using the liver from a patient with MSUD. Activated PC concentrate safely supported the perioperative period. After DLT, she maintained normal PC activities and BCAA levels. This is the first case of PC deficiency successfully treated by living DLT with MSUD. We propose that DLT using liver from patients with MSUD is a treatment option for PC deficiency.     

Intermittent bundle branch blocks in a patient with uncommon-type atrioventricular nodal reentrant tachycardia and enhanced atrioventricular nodal conduction

We report on a patient with uncommon-type atrioventricular (AV) nodal reentrant tachycardia with a short tachycardia cycle length (235-270 ms), in whom transient wide QRS tachycardia with both left bundle branch block and right bundle branch block aberrancy were followed by narrow QRS complexes. In addition, His-ventricular (H-V) block and a sudden prolongation of the H-V interval occurred during the tachycardia. As the determinant of these unusual findings, the possibility that the anterograde limb of the reentry circuit has an enhanced AV nodal conduction property is discussed, as is the clinical significance of this type of tachycardia.     

An extramural gastrointestinal stromal tumor of the duodenum mimicking a pancreatic head tumor

We report the case of a 53‐year‐old woman with a gastrointestinal stromal tumor (GIST) of the duodenum that showed only extramural growth, mimicking a pancreatic tumor. Preoperatively, computed tomography (CT) and angiography revealed a hypervascular mass, 3.0 cm in diameter, in the pancreatic head. Hypotonic duodenography showed compression of the second and third portions of the duodenum by the pancreatic lesion. Endoscopic examination showed no specific mucosal abnormalities in the duodenal lumen. The pancreatic head tumor was diagnosed preoperatively as a nonfunctioning islet cell tumor of the pancreas, and the patient underwent pylorus‐preserving pancreaticoduodenectomy. A hard mass was palpated intraoperatively in the pancreatic head region, and neither peritoneal dissemination nor metastasis was detected. Histologically, the tumor was composed of spindle‐shaped cells with a fascicular growth pattern, and only a few mitotic features were seen. Immunohistochemically, most of the tumor cells were positive for c‐kit oncoprotein and CD34, but negative for alpha‐smooth muscle actin and S‐100 protein. Therefore, this neoplasm was finally diagnosed as a duodenal GIST of the uncommitted type. This is a rare case of a duodenal GIST with exclusively extramural growth mimicking a pancreatic head tumor.     

Robotic mitral valve plasty for mitral regurgitation after blunt chest trauma in Barlow's disease

We successfully treated a case of mitral regurgitation due to chest trauma in Barlow's disease. A 71‐year‐old man was admitted with severe mitral regurgitation after blunt compression of the chest by a heavy object 5 months earlier. Preoperative examination revealed wide chordae tendineae rupture and myxomatous changes to the bileaflets. Neo‐chordae reconstruction of the anterior mitral leaflet using loop technique, triangular resection of the posterior mitral leaflet, and ring annuloplasty was performed via surgical robot. Robotic mitral valve plasty for severe mitral regurgitation due to chest trauma in Barlow's disease was achieved safely with good clinical and excellent cosmetic results.     

Two cases of early recurrence after transabdominal preperitoneal inguinal hernia repair

We performed transabdominal preperitoneal inguinal hernia repair in 46 patients (58 diseases), two of whom experienced early recurrence after mesh repair. Case 1 was a 76‐year‐old man with a bilateral inguinal hernia (recurrence site, left indirect hernia) after appendectomy. The recurrence occurred 1 month after transabdominal preperitoneal inguinal hernia repair. The mesh was dislocated to the lateral side, and we repaired it using the direct Kugel ® patch with an anterior technique. Case 2 was a 79‐year‐old man with a bilateral inguinal hernia (recurrence site, right direct hernia with an orifice >3 cm) after appendectomy. The recurrence occurred 3 months after transabdominal preperitoneal inguinal hernia repair. The mesh was dislocated to the lateral side, and we repaired it using an ULTRAPRO ® Plug with an anterior technique under laparoscopic observation. We believe the recurrences resulted from insufficient internal exfoliation and fixation affected by complicated exfoliation of the preperitoneal space with omental adhesion after intraperitoneal surgery.