Open Gastrostomy by Mini-Laparotomy: Our Method.

When percutaneous endoscopic gastrostomy (PEG) is not feasible owing to anatomical obstacles, laparotomic or laparoscopic gastrostomy (LAG) is an alternative. At our institution, LAG has been the first choice for patients who are unable to undergo PEG; however, we have introduced a small open gastrostomy through a 2-cm-long transverse incision since 2020. By December 2022, 12 patients had undergone this procedure without complications. In one case where the stomach was located cephalad to the rib arch and the patient had a round dorsum, the incision wound was extended, and a lengthy operation was required. We believe that our small-incision gastrostomy is a useful option in addition to LAG for cases in which PEG is difficult to perform. Further studies are required to determine the indications for this procedure.

Association of pediatric inguinal hernia contents with patient age and sex.

BACKGROUND: Although pediatric inguinal hernia (IH) is a very common disease, systematic reviews of herniated organs are scarce. The current study aims to clarify the contents of pediatric IH using preoperative ultrasonography (US) in association with patient age, sex, and risk for developing irreducible/strangulated hernia. METHODS: The medical records of pediatric IH patients who underwent inguinal US examination prior to surgery between 2014 and 2019 were reviewed. Hernia contents were categorized into four groups based on US findings: bowel, omentum, ovary with or without fallopian tube, and ascites. RESULTS: A total of 524 IH lesions found in 220 men and 304 women were analyzed. The most common hernia content in patients under 12 months of age was the bowel (91.0%) in males and ovaries (89.5%) in females. The omentum became the most common herniated organ in both men (78.6%) and women (88.0%) aged 2 years and older. Emergency operations were performed in 3 patients (0.57%) due to irreducible IH, where 2 patients with irreducible ovaries, 5 and 7 months old, developed ovarian torsion and needed to undergo emergent salpingo-oophorectomy. CONCLUSIONS: The contents of pediatric IH depended on patient age and sex. Herniated ovaries in infants can twist in the hernia sac and become strangulated. It is important for clinicians to expect the herniated organ and take appropriate measures in the pediatric primary care setting.

A case of an infant with congenital combined pituitary hormone deficiency and normalized liver histology of infantile cholestasis after hormone replacement therapy.

Congenital combined pituitary hormone deficiency (CPHD) may present with cholestasis in the neonate or during early infancy. However, its precise mechanism is unknown. A 3-mo-old boy presented with cryptorchidism and hypoplastic scrotum after birth. Neonatal jaundice was noted but temporarily improved with phototherapy. Jaundice recurred at 2 mo of age. Elevated direct bilirubin (D-Bil) and liver dysfunction were found but cholangiography showed no signs of biliary atresia (BA). Liver biopsy findings showed giant cell formation of hepatocytes with hypoplastic bile ducts. Subsequent magnetic resonance imaging (MRI) of the head revealed a hypoplastic pituitary gland with an ectopic posterior lobe, and the patient was diagnosed with congenital CPHD based on decreased secretion of cortisol and GH by the pituitary anterior lobe load test. D-Bil levels promptly improved after hydrocortisone (HDC) replacement. We subsequently began replacement with levothyroxine (L-T4) and GH, and liver histology showed normal interlobular bile ducts at 8 mo old. This is the first case report of proven histological improvement after hormone replacement therapy. This suggested that pituitary-mediated hormones, especially cortisol, might be involved in the development of the bile ducts.

Pediatric granulomatous orchitis: Case report and review of the literature.

An 11-year-old boy presented with fever and abdominal pain, and was diagnosed with retroperitoneal lymphadenitis. At the same time, a painless right scrotal mass was observed. On imaging the testis and the epididymal mass both had abundant blood flow, although tumor markers were negative. Although the right testis had shrunk after antibiotic treatment, swelling was persistent and incisional biopsy was therefore performed, resulting in diagnosis of granulomatous orchitis (GO). No recurrence was found. In cases of scrotal swelling in both the testis and the epididymis of an older child, it is necessary to consider the possibility of inflammatory GO, and orchiectomy should not be performed without careful consideration.

Diffuse and multifocal nephrogenic adenoma with Familial Mediterranean Fever: a case report with molecular study.

Nephrogenic adenoma, also referred to nephrogenic metaplasia, is a benign proliferative lesion of urothelium, usually associated with chronic physical stimuli or inflammation. Familial Mediterranean fever is an inherited autosomal recessive disease characterized by recurrent short episodes of fever. The site of mutation is found in MEFV gene which controls inflammatory responses. We have experienced a case of nephrogenic adenoma in a 16-year-old girl with Familial Mediterranean Fever, showing proliferative lesions diffusely in the urinary bladder and multifocally in the other parts of urinary tract. These lesions disappeared after colchicine treatment. We searched for MEFV gene mutation using the specimen from the resected urinary bladder and detected heterozygous mutation of E148Q. There is a possibility that control of inflammation caused by the surgery for vesicoureteral reflux in the local site didn't work well on the background of heterozygous mutation of MEFV gene, and as a result, nephrogenic adenoma appeared. This is the first report of a combination of two rare diseases. We have to be aware that nephrogenic adenoma can occur in association with Familial Mediterranean Fever, and the former condition should be taken into consideration when rendering a correct pathological diagnosis.

Ectopic neuroblastoma in monozygotic twins with different ages of onset: possible twin-to-twin metastasis in utero with distinct genetic alterations after birth.

We describe neuroblastoma (NB) in monozygotic twins whose ages at the onset of the disease had a 3-year interval. The primary tumor site of twin 1 was the adrenal gland, whereas that of twin 2 was the jejunum/mesentery. MYCN amplification, DNA index, ALK mutation, and copy number alterations of DNA were different between each primary tumor. NB in ectopic sites may have resulted from twin-to-twin metastasis through vascular anastamoses in the placenta. The pathogenesis of this NB involved a premalignant stage of NB during the fetal development and subsequent molecular alterations after birth, resulting in NBs that were phenotypically similar but genetically different.

A case with sacrococcygeal primitive myxoid mesenchymal tumor of infancy: a case report and review of the literature.

A girl, aged 19 months, presented with a sacrococcygeal tumor that developed at 5 months after birth and gradually enlarged. Serum tumor marker levels were negative. Ultrasound imaging showed abundant blood flow in the tumor. However, neither computed tomography (CT) nor magnetic resonance imaging (MRI) showed contrast agent incorporation. The surgically resected tumor consisted of immature cells with myxoid stroma and proliferating small blood vessels. Immunostaining showed extensive vimentin expression. However, smooth muscle actin, muscle-specific actin, and S-100 protein expression was negative. Neither the ETV6-NTRK3 fusion gene nor the FUS gene rearrangement was detected. Thus, the patient was diagnosed with a primitive myxoid mesenchymal tumor of infancy. This tumor primarily consisted of a mucosal stroma with a low absorption on CT, a low signal on T1-weighted MRI, and a high signal on T2-weighted MRI. A diagnosis of primitive myxoid mesenchymal tumor of infancy should be considered in cases of soft tissue tumors in infants that show prominent vascularity but little contrast enhancement on MRI or CT.

Idiopathic testicular infarction in a boy initially suspected to have acute epididymo-orchitis associated with mycoplasma infection and Henoch-Schönlein purpura.

Idiopathic testicular infarction without torsion of spermatic cord is a rare condition. We present a 12-year-old boy originally suspected of acute epididymo-orchitis, but subsequently an orchiectomy was necessary owing to unpredicted testicular infarction not associated with torsion. Elevation of immunoglobulin M against mycoplasma, reduction in serum factor XIII and the presence of sufficient blood flow detected by color Doppler ultrasonography upon initial manifestation suggested that the boy was affected by epididymo-orchitis associated with Henoch-Schönlein purpura or mycoplasma infection. However, progressive testicular enlargement was observed and subsequently testicular blood flow became barely detectable. Our case indicates that the presence of sufficient blood flow upon initial diagnosis in the affected testis does not necessarily exclude infarction and continuous monitoring of blood flow may be required to avoid radical orchiectomy.

Mesoblastic nephroma: a case report of prenatal detection by MR imaging.

We report a case of mesoblastic nephroma detected prenatally by magnetic resonance (MR) imaging. MR imaging could provide valuable information about the origin and nature of a fetal abdominal mass and help define the relationship of the mass to adjacent structures.