Airway management of a pediatric patient with cervical cystic hygroma can be challenging as the large neck mass may extend in the oral cavity, result in tracheal deviation, and cause possible upper airway obstruction. This is a case of a 4-year-old female admitted due to sudden enlargement of neck mass associated with dysphagia and sialorrhea. Patient was intubated under sedation while maintaining spontaneous ventilation with a pediatric flexible fiberoptic scope through a nasopharyngeal airway serving as a passage guide for ease of scoping and a protective device against trauma.
Lymphangioma, Cystic ; Airway Management ; Intubation

A fetal neck mass such as cystic hygroma imposes a challenge in the continuing prenatal care and birth strategy for a 26-year-old gravida 2 para 1 (1001) with a scarred uterus from previous low transverse cesarean section (CS). An intricate delivery for the fetus with a potential airway obstruction necessitates an ex utero intrapartum treatment (EXIT) delivery. The patient was initially scheduled to undergo the procedure at 38 weeks of gestation, but preterm labor ensued. Close antenatal monitoring was done which carried the pregnancy to term. The multidisciplinary approach and planning requires consensus among varied specialties as this maternal–fetal surgery differs immensely from a regular CS. This aims to document and discuss the preoperative, operative and postoperative management done for this patient. The EXIT procedure was performed at 38 weeks' age of gestation with good maternal outcomes. The fetus was delivered safely.
Lymphangioma, Cystic

Adrenal cystic lymphangiomas are extremely rare entities that are often identified incidentally, with less than 60 cases reported to date. We found a protruding ovoid mass consisting of a multiloculated cystic lesion within right adrenal gland in the cadaver of a 75-year-old Korean man. The epithelial cells lining the adrenal cyst were diffusely positive for cluster of differentiation 31 and podoplanin, and negative for pan-cytokeratin. The histopathological diagnosis confirmed a cystic lymphangioma arising from the adrenal gland. Post-mortem findings of the present case are discussed based on the clinicopathological features of adrenal cystic lymphangiomas.
Adrenal Glands ; Aged ; Cadaver ; Diagnosis ; Epithelial Cells ; Humans ; Lymphangioma ; Lymphangioma, Cystic ; Male

Lymphangiomas are rare benign congenital lymphatic malformations. They can be divided into three groups: cutaneous lymphangioma circumscriptum (CLC), cavernous, and cystic. CLC is the most common type, and rarely occurs with cavernous or cystic lymphangioma under the lesion. Here, we describe the case of a 9-year-old girl who presented with an asymptomatic vesicular lesion on her back. She was finally diagnosed with CLC by clinical manifestations, dermoscopic findings, and histologic findings. Seven years ago, there was a history of surgical operation in the department of general surgery, and the surgery was soft tissue cystic lymphangioma removal surgery. There was no skin lesion for seven years after surgery, but one occurred a month ago. Herein, we present a case of CLC that occurred after a long interval after surgery for soft tissue cystic lymphangioma.
Child ; Dermoscopy ; Female ; Humans ; Lymphangioma ; Lymphangioma, Cystic ; Skin

OBJECTIVE: To determine the frequency of chromosomal abnormalities and outcome of pregnancy for fetuses with increased nuchal translucency (NT). METHODS: Between July 2014 and February 2018, 247 fetuses with increased NT (>95th centile)were analyzed by chromosome microarray analysis (CMA). The fetuses were divided into ones with isolated increased NT (168 cases), increased NT with cystic hygroma (20 cases), increased NT with edema (12 cases) or increased NT with other abnormalities (47 cases). All couples were followed up by telephone calls. RESULTS: The rate of chromosomal abnormalities was 31.6% (78/247), which included 66 cases with chromosomal aneuploidies and 12 with copy number variants (CNVs). CNVs accounted for 31.4% (11/35) of total abnormalities among fetuses with isolated increased NT, whilst only 2.3% (1/43) of the total abnormalities among fetuses with non-isolated increased NT. Three fetuses with a normal CMA result had mental and physical retardation. Two of them were diagnosed with single gene disorders by whole exome sequencing. CONCLUSION CMA can detect more chromosomal microdeletion/microduplications among fetuses with isolated increased NT. Furthermore, fetuses with increased NT and anegative CMA result during pregnancy cannot exclude all adverse outcomes.
Aneuploidy ; Chromosome Aberrations ; Chromosomes ; DNA Copy Number Variations ; Edema ; Female ; Fetus ; Humans ; Lymphangioma, Cystic ; Microarray Analysis ; Nuchal Translucency Measurement ; Pregnancy ; Pregnancy Outcome ; Prenatal Diagnosis ; Ultrasonography, Prenatal

OBJECTIVE: To analyze the prognosis of fetuses with cystic hygroma (CH) or nuchal translucency (NT) or nuchal fold (NF) thickening detected by prenatal echography. METHODS: From January 2014 to December 2015, 124 fetuses with CH and NT/NF thickening on prenatal echography were enrolled from Women's Hospital of Zhejiang University School of Medicine. The basic clinical information, ultrasonic results, pregnancy outcomes and newborn follow-ups were analyzed. The cases were grouped by prognosis and the factors affecting prognosis were analyzed with logistic regression. RESULTS: There were 85 cases of labor induction including one stillbirth and 39 cases delivered. Except one infant who died after birth, all live births survived with good prognosis. Univariate analysis showed that the gestational age at diagnosis of poor prognosis group was earlier than that of good prognosis group (<0.01); and the former group also had higher hydrops fetalis rate and additional structural anomalies rate (all <0.01). Multivariate regression analysis showed that hydrops fetalis (=90.105, <0.05) and additional structural anomalies (=61.854, <0.05) were risk factors of poor prognosis in fetuses with CH and NT/NF thickening. CONCLUSIONS Fetuses with diagnosed CH or NT/NF thickening on prenatal ultrasonography are likely to be associated with chromosomal abnormality. Early gestational weeks, hydrops fetalis and additional structural anomalies may indicate poor prognosis.
Female ; Fetus ; Humans ; Hydrops Fetalis ; etiology ; Infant, Newborn ; Lymphangioma, Cystic ; complications ; diagnosis ; Nuchal Translucency Measurement ; Pregnancy ; Pregnancy Outcome ; Prognosis ; Ultrasonography, Prenatal

Dysphasia related to oral anomaly is a common situation in oral and maxillofacial surgery. The etiology of oral anomalies causing dysphasia can be divided into congenital and acquired disease. Congenital diseases include teratoma or benign tumors and congenital defects such as cleft lip and palate. Benign tumors include cystic hygroma in the neck and hemangioma in the tongue. Certain syndromes with macroglossia and micrognathia are also related to difficulty in swallowing. The three common syndromes are Pierre-Robin syndrome, Beckwith-Widermann syndrome and ectodermal dysplasia. Taken together, these congenital diseases require a multi-discipline approach to obtain optimal results. Representative disease of acquired dysphasia is the oral cavity cancer. Cancer ablation results in tissue defect and decreased motor function. Free flap reconstruction is the choice of treatment following oral cavity caner operation; however, dysphasia after cancer operation is inevitable. In this review article, the full scopes of oral anomaly associated with dysphasia were classified and treatment was suggested.
Aphasia ; Cleft Lip ; Congenital Abnormalities ; Deglutition ; Ectodermal Dysplasia ; Free Tissue Flaps ; Hemangioma ; Lymphangioma, Cystic ; Macroglossia ; Micrognathism ; Mouth ; Mouth Neoplasms ; Neck ; Palate ; Pierre Robin Syndrome ; Surgery, Oral ; Teratoma ; Tongue

Fetal cystic lymphangioma, commonly called hygroma, is formed by multiple cysts ranging from few millimeters to several centimeters in size. Fetal cystic lymphangioma is a rare developmental congenital anomaly of the lymphatic system. Most are found in the neck and axilla, rarely in the retroperitoneum, abdominal viscera, limbs, bones, and cervico-mediastinal area. The prenatal diagnosis of cystic hygroma by ultrasound guidance is commonly localized in the nuchal region. We report a case of rare fetal axillary hygroma that was diagnosed at 27 weeks of gestation and delivered at 37 weeks of gestation.
Axilla ; Extremities ; Lymphangioma, Cystic* ; Lymphatic System ; Neck ; Pregnancy ; Prenatal Diagnosis ; Ultrasonography ; Ultrasonography, Prenatal* ; Viscera

Cystic lymphangioma is a true, benign, congenital multicystic tumor arising from sequestrations of embryonic lymphatic tissue. Most often it occurs at birth or during the early years of life, is found most commonly in the neck, and its distribution coincides with that of the primitive lymph sacs. A 23-year-woman presented with a soft palpable mass on her chest wall. Physical and ultrasound examination revealed a well-circumscribed, mobile, nontender, soft subcutaneous mass on the right posterior chest wall beneath the latissimus dorsi muscle. A skin biopsy of the mass showed endothelium-lined lymphatic vessels and a loose, lace-like fibrous tissue stroma. The specimen was stained with D2-40 showing positive staining of lymphatics. These histopathological findings confirmed the diagnosis of a cystic lymphangioma. We report a rare case of a cystic lymphangioma on the chest wall in a 23-year-old woman.
Adult* ; Biopsy ; Diagnosis ; Female ; Humans ; Lymphangioma, Cystic* ; Lymphatic Vessels ; Lymphoid Tissue ; Neck ; Parturition ; Skin ; Superficial Back Muscles ; Thoracic Wall* ; Thorax* ; Ultrasonography ; Young Adult

A 7-year-old boy, diagnosed with an arachnoid cyst and subdural effusion on initial MRI, was admitted with left limb weakness and no history of head trauma. A subsequent follow-up MRI showed different stages of hematoma within multilayered enhancing membranes and in the arachnoid cyst, which was separated by the cerebrospinal fluid cleft. Craniotomy and fenestration of the cyst wall and hematoma removal were performed. The patient was diagnosed as a having a hemorrhagic rupture of an arachnoid cyst into the intradural space, probably via some one-way valve-like defect, based on the MRI and surgical findings. The MRI features and possible mechanism of this rare disease are discussed within the literature review.
Arachnoid* ; Cerebrospinal Fluid ; Child ; Craniocerebral Trauma ; Craniotomy ; Dura Mater ; Extremities ; Follow-Up Studies ; Hematoma ; Hemorrhage ; Humans ; Lymphangioma, Cystic ; Magnetic Resonance Imaging ; Male ; Membranes ; Rare Diseases ; Rupture* ; Subdural Effusion