Journal of Nippon Medical School最新文献

Parastomal hernia of the ileal conduit (PHIC) is a long-term complication of radical cystectomy (RC) for bladder cancer. A systematic review reported an estimated incidence of PHIC after RC of 17%. Several surgical techniques have been reported for treating parastomal hernias. However, few studies have investigated treatment of PHIC, and there are no guidelines for the optimal approach for PHIC repair. Here, we describe a case in which good results were achieved using the laparoscopic Sugarbaker (LS) technique with a tailored mesh for PHIC. An 80-year-old Japanese woman underwent robot-assisted radical cystectomy for bladder cancer. Ten months after surgery, she was diagnosed as having bowel obstruction due to PHIC. The LS technique was performed using a tailored mesh. Severe small-intestinal adhesions from previous surgeries were safely divided using laparoscopic surgery. No hernia recurrence was observed at 2 years postoperatively. We used the LS technique to treat PHIC, and severe small-intestinal adhesions were safely treated using laparoscopic surgery. The LS technique appears to be a viable therapeutic option for such cases.

A 3-year-old boy presented with recurrent eczema that began at 2 months of age. The patient was treated by multiple dermatologists using topical steroids, topical immunosuppressants, and oral antihistamines. The patient was subsequently referred to our clinic for improved symptom control. During the initial visit, he exhibited serous erythema, scaling, and scratch marks on the scalp, face, and limbs. Laboratory findings revealed elevated levels of immunoglobulin E at 5400 IU/mL, thymus and activation-regulated chemokine at 3029 pg/mL, and an Eczema Area and Severity Index score of 16. Due to sleep disturbances caused by nocturnal itching and substantially reduced quality of life, we initiated treatment with dupilumab. A pediatrician conducted pre-treatment assessments, including blood tests and chest radiography. Following the initiation of dupilumab therapy, the pediatrician monitored the patient for potential reactions and side effects, and the dermatologist managed the skin symptoms. This case underscores the importance of interdisciplinary collaboration between pediatrics and dermatology in managing severe eczema in young children treated with dupilumab.

We present a case of permanent bilateral lower limb paralysis after epidural anesthesia. A 71-year-old woman (height 159 cm; weight 48.5 kg; American Society of Anesthesiologists Physical Status 2) with a history of hypertension (treated with nifedipine), benign goiter (under surveillance), surgeries for appendicitis, and a previous left humerus fracture had received general anesthesia with epidural anesthesia during two surgical procedures, namely, laparoscopic-assisted low anterior resection with colostomy and laparoscopic-assisted colostomy closure. She developed left-predominant lower limb paralysis after the first epidural anesthesia (using ropivacaine and levobupivacaine). The symptoms had no identifiable cause, persisted after removal of the epidural catheter, and gradually resolved during rehabilitation. Her lower limb paralysis recurred and progressed, however, after the second epidural anesthesia (using levobupivacaine alone), and she has abnormal spinal reflexes and elevated myelin basic protein in cerebrospinal fluid. Although these findings suggested that bilateral lower limb paralysis was caused by a lesion in the central nervous system (thoracolumbar spinal cord), postoperative MRI scans of the vertebrae/spinal cord and head failed to identify the site of the damage. We concluded that permanent bilateral lower limb paralysis was likely caused by epidural anesthesia, but the mechanism could not be identified.

Psoriasis is a condition characterized by chronic inflammation of the skin, epidermal hyperproliferation, and dysregulated differentiation driven by acceleration of the tumor necrosis factor-alpha/interleukin (IL) -23/IL-17 axis. Herein, we report a case of generalized pustular psoriasis initially managed with etretinate, apremilast, and risankizumab in a Japanese man. Because of side effects, a therapeutic transition was made to brodalumab at 7 months after the initial consultation. His dermatological symptoms improved; however, hemoglobin concentration decreased to 7.6 g/dl after 4 months of treatment. Diagnostic investigation revealed warm autoimmune hemolytic anemia (AIHA). To our knowledge, this is the first report of AIHA during treatment with brodalumab for generalized pustular psoriasis. The etiological association between AIHA and psoriasis is unclear. Future studies should investigate whether AIHA accompanies pustular psoriasis or results from drug-induced AIHA secondary to brodalumab administration. Our findings suggest that the risk of AIHA in patients with psoriasis treated with brodalumab warrants careful consideration.

Mucinous cystic neoplasms of the liver (MCNs-L) are rare cystic tumors characterized by multilocular cysts lined with a mucin-producing epithelium and supported by ovarian-like stroma. These tumors account for <5% of all cystic liver lesions and carry a potential risk of malignant transformation, necessitating early diagnosis and surgical resection. This report describes the successful laparoscopic partial liver resection of segments 4 and 5 for a large MCN-L in a woman in her 40s who presented with upper abdominal discomfort. Imaging studies revealed a multilocular cystic lesion measuring 11.9 × 8.5 × 13.0 cm in segments 4 and 5 of the liver. The primary challenges were the size of the lesion and the need to prevent cystic content spillage during surgery. Using meticulous techniques, including the use of a retrieval bag and controlled aspiration, we successfully excised the tumor laparoscopically and without contamination. Pathological analysis confirmed a diagnosis of mucinous cystic adenoma with no evidence of malignancy. The patient recovered uneventfully and was discharged on postoperative day 7. This case highlights the feasibility and safety of a laparoscopic approach for large MCNs-L. Furthermore, it underscores the importance of preoperative planning, precise surgical techniques, and measures to prevent cystic content dissemination. Minimally invasive surgery offers significant benefits, including reduced recovery time and improved cosmetic outcomes, and can be effectively applied to large hepatic cystic lesions by experienced surgical teams.

High blood flow and intravariceal pressure in pipeline esophageal varices (EVs) can cause refractory variceal bleeding that is difficult to control with endoscopic procedures and interventional radiology. We used combination therapy with Hassab's procedure (HP) and subsequent endoscopic variceal ligation (EVL) to successfully treat two patients with pipeline EVs.Case 1A woman in her 30s with recurrent hemorrhagic pipeline EVs caused by idiopathic portal hypertension was referred for surgery. Magnetic resonance imaging revealed a dilated left gastric vein (LGV) connected to EVs with no palisade zone. Esophagogastroduodenoscopy (EGD) identified high-risk EVs (Ls, F3, Cw, RC1) and gastric varices (GVs) (Lg-c, F2, Cw, RC0). Eradication of the esophagogastric varices was maintained 5 years after laparoscopic HP followed by EVL at 1, 6 and 16 months postoperatively.Case 2A woman in her 50s with cirrhosis (type B) and enlarged pipeline EVs was referred for prophylactic surgery. Computed tomography (CT) imaging revealed that the EVs were supplied by a dilated LGV without a palisade zone. EGD identified high-risk EVs (Lm, F3, Cb, RC2) and GVs (Lg-c, F3, Cw, RC1). Combination therapy with laparoscopic HP and EVL at 2, 4, and 6 months showed the eradication of esophagogastric varices 8 months after surgery. CT scanning performed 10 days postoperatively in case 2 revealed residual EVs supplied by gastric intramural blood flow, strongly supporting the need for additional EVL after HP. HP involves gastroesophageal devascularization and splenectomy, which reduce blood supply to EVs, facilitating subsequent EVL to eradicate pipeline EVs. This combination therapy appears to enhance outcomes and should be considered a viable option for treating refractory pipeline EVs.

Painless thyroiditis, which is rare in children, exhibits the characteristic sequence of hyperthyroidism, including aggressive and disruptive behaviors. Unlike subacute thyroiditis or Graves' disease, painless thyroiditis is challenging to diagnose because of its mild symptoms and minimal or absent physical findings. Moreover, aggressive and disruptive behaviors in children with psychiatric disorders may be misconstrued as exacerbation of underlying symptoms. The present patient was a 16-year-old male with adjustment disorder who presented to a pediatric psychiatric clinic for assessment of irritability. After 4 months, he developed aggressive and disruptive behaviors that prompted initiation of risperidone but without improvement. After 1 month, he reported palpitations and dyspnea. His neck was supple and non-tender without thyroid enlargement. Thyroid studies revealed elevated free T4 and T3 levels and suppressed thyroid-stimulating hormone level, suggesting hyperthyroidism. A radioactive iodine uptake test revealed a barely visible thyroid gland, consistent with thyroiditis. Painless thyroiditis, without thyroid tenderness, was diagnosed. We describe a case of painless thyroiditis in an adolescent patient with aggressive and disruptive behaviors that were initially attributed to worsening of an underlying adjustment disorder. Even when minimal or no signs of hyperthyroidism are present, painless thyroiditis should be considered in the differential diagnosis of children with aggressive and disruptive behaviors. Awareness of potential anchoring bias is also recommended to prevent its delayed diagnosis of such behaviors.

Schizophrenia develops during adolescence. Maternal infections during the fetal period increase the incidence of schizophrenia in children, which suggests that the pathogenesis involves neuroinflammation. Here, we report a case of new-onset schizophrenia in a 16-year-old boy after COVID-19. After developing COVID-19, he entered a catatonic state 4 days later and was hospitalized. Benzodiazepines alleviated his catatonia, but hallucinations and delusions persisted. Encephalitis and epilepsy were excluded by magnetic resonance imaging (MRI), encephalography, and cerebrospinal fluid examination. Psychosis persisted after the virus titer declined and the inflammatory response subsided. Moreover, the patient exhibited delusions of control-a Schneider's first-rank symptom. Schizophrenia was diagnosed, and olanzapine improved his symptoms. He had a brief history of insomnia before COVID-19 but his symptoms did not satisfy the ultra-high-risk criteria. However, COVID-19 may have facilitated development of schizophrenia through neuroinflammation and volume reduction in the gray matter of the right medial temporal lobe. This case demonstrates that infectious diseases in adolescents should be carefully managed, to prevent schizophrenia.

Mixed connective tissue disease (MCTD) is characterized by mixed features of systemic lupus erythematosus, systemic sclerosis, and polymyositis/dermatomyositis and is rare in children. Here, we report a case of MCTD in a 10-year-old girl who presented at our hospital with arthralgia, Raynaud's phenomenon, and fatigue. Blood tests were positive for anti-U1-ribonucleoprotein (RNP) antibodies and for rheumatoid factors (RFs) IgG-RF and anti-galactose-deficient IgG. Levels of myogenic enzymes and hypergammaglobulinemia were elevated. Macrophages were prominent in bone marrow, with scattered phagocytic macrophages. MCTD was diagnosed based on the patient's symptoms and laboratory findings. Methylprednisolone pulse therapy combined with oral tacrolimus was administered, which led to resolution of symptoms. Three months after pulse therapy, arthralgia worsened and methotrexate was administered. Arthralgia improved but did not resolve. Magnetic resonance imaging performed to investigate the hip pain revealed a mature ovarian teratoma, which was surgically removed. Because the pain persisted and interfered with her daily life, she was treated with tocilizumab for joint pain relief, which decreased the pain level. Tocilizumab is a candidate for additional treatment of juvenile idiopathic arthritis-like arthritis associated with childhood-onset MCTD.

Surgical repair of thoracoabdominal aortic aneurysms (TAAA) is a complex procedure that is associated with marked morbidity and mortality. Endovascular TAAA repair, initially reserved for patients at high risk, has yielded promising results and is performed on an increasing range of patients. This case report describes a hybrid approach-a combination of endovascular and open repair-for a patient with high-risk TAAA after aortic dissection. A 79-year-old man had undergone aortic replacement for chronic aortic dissection 20 years previously. His saccular TAAA, extending from the Th12 level to the renal artery origin, demonstrated progressive enlargement (56-60 mm) on computed tomography (CT) scans and compressed the celiac artery. In light of the patient' s age and thoracotomy history, a hybrid repair strategy involving total debranching thoracic endovascular aortic repair (TEVAR) with four-branch abdominal reconstruction was selected to minimize surgical risk. The surgery was performed via a midline abdominal incision. Bypass surgery was first performed using a quadrant artificial graft to restore left renal, right renal, superior mesenteric, and common hepatic artery blood flow. The celiac artery was coiled, and TEVAR was completed. Intraoperative contrast and postoperative CT showed no endoleak and a patent debranching graft. The patient recovered without complications and was discharged 10 days postoperatively. The findings for the present hybrid procedure indicate that debranching TEVAR is less invasive and yields better outcomes than open aortic replacement, particularly for patients at high risk.