Summary: Aplasia Cutis Congenita (ACC) in the scalp is a rare congenital malformation. The treatment for ACC with large defects involving the scalp, bone, and the dura is challenging. Local debridement of necrotic tissue is important to prevent lethal complications such as infection and meningitis. However, debridement has the risk of damaging the sagittal sinus or the dura. Recent developments in ultra-high-frequency ultrasound(US) systems provide frequencies of 70 MHz and capability resolution as fine as 30 μm, which could allow precise imaging of small and thin anatomical structures. The study aimed to describe the methods of precise evaluation of the defect in the scalp and safe debridement using ultra-high-frequency US. This is the first report on direct observation of a newborn’s brain using ultra-high-frequency US. The boy was delivered spontaneously with a large defect of the scalp and bone. After 14 days, due to signs of infection, local debridement was performed carefully under ultra-high-frequency US-based evaluation. The dura, the sagittal sinus, and the small anatomical structures such as arachnoid granulations could be observed. Because the brain herniation gradually aggravated, dural reconstruction using fascia lata and scalp reconstruction using transposition flap was performed. Finally, good skin coverage over the defects was obtained. This method minimizes the risk of damaging the sagittal sinus and the brain parenchyma, which may cause fatal complications. Although further clinical investigations will be required to confirm its efficacy, ultra-high-frequency US has the potential to be a useful device for ACC treatment.

Dermatomyositis (DM) and polymyositis (PM) are autoimmune myopathies and their association with malignancies is well known in adults. However, DM/PM is an uncommon complication of Hodgkin lymphoma (HL), especially in children. An 11-year-old girl presented with deteriorating symmetrical muscle weakness, proximal limb myalgia, and persistent fever, followed by axillary and cervical lymphadenopathy for 2 months. She had no family history of autoimmune disease nor malignancy. Axillary lymph node biopsy revealed mixed cellularity classic HL (Fig. S1); her creatine kinase (CK) level was normal at the time of biopsy. On admission, she could not move due to severe pain with generalized non-pitting edema. She had developed a heliotrope rash but not Gottron papules. She did not complain of dysphasia nor dyspnea. Hematological findings showed elevated levels of CK (5250 IU/L), aldolase (31.9 U/L), lactate dehydrogenase (762 IU/L), C-reactive protein (11.4 mg/dL), and soluble interleukin-2 receptor (2407 U/mL), with leukocytosis (25 9 10 9 /L) and microcytic anemia (hemoglobin 8.9 g/dL). Epstein-Barr virus titer showed the presence of a nuclear antigen (1 : 10) and viral capsid antigen IgG (1 : 40), while the viral DNA load was undetectable. 18 F-fluorodeoxyglucose positron emission tomography (FDG-PET) (Fig.1a) and magnetic resonance imaging (Fig. 1b) showed abnormal diffuse accumulation in the muscles of all extremities, along with a finding of supradiaphragmatic lymphadenopathy, including infiltration of the mediastinal lymph nodes externally (Fig.1c). There was no comorbid interstitial lung disease and no evidence of metastasis to the bone marrow; muscle biopsy was difficult to perform. Clinical findings in addition to myopathic changes on electromyography confirmed a clinical diagnosis of DM, according to Bohan and Peter criteria. Serology was negative for anti-transcriptional intermediary factor 1 c (anti-TIF1c), anti-melanoma differentiation-associated gene 5 (anti-MDA5), anti-histidyl transfer RNA [t-RNA] synthetase (anti-JO-1),

Congenital maxillomandibular synechia is a rare malformation that is characterized by a fusion of the maxilla and mandible. The fusion is fibrous or bony and prevents mouth opening, which causes difficulties in feeding and occasionally in breathing. Although extremely rare, neonatologists must understand the disease because it can be fatal and require emergency treatment after birth. We report the case of a very-low-birth-weight (VLBW) infant with congenital maxillomandibular synechia and other malformations, including cleft palate, syndactyly, and cryptorchidism. The patient presented with extremely limited mouth opening, and endotracheal intubation seemed impossible; fortunately, the patient did not have respiratory distress syndrome. The patient underwent surgical release of the fibrous bands on days 10 and 17, and good mouth opening was achieved. The patient was able to consume breast milk orally and was discharged home at a corrected gestational age of 1 month without recurrence of difficulty in mouth opening or any sequelae. This is the first reported case of a VLBW infant with congenital maxillomandibular synechia who required more complicated management of feeding, surgical intervention, and anesthesia.