Humans ; Encephalocele ; Meningocele/complications* ; Temporal Bone

Encephalocele ; Facial Bones ; Humans ; Meningocele ; Temporal Bone/diagnostic imaging*

STUDY DESIGN: A retrospective cohort study. PURPOSE: To examine the validity of prophylactic surgery for children with tethered cord syndrome (TCS). OVERVIEW OF LITERATURE: Prophylactic surgery for pediatric patients with TCS remains controversial. METHODS: We retrospectively analyzed the surgical outcomes of 14 children (nine boys and five girls) with asymptomatic TCS who were surgically treated at Hokkaido University Hospital between 1989 and 2015. RESULTS: The median age at the time of initial surgery for asymptomatic TCS was 28.6 months (range, 0–66 months). The median final follow-up period was 142 months (range, 7–232 months). Of the 14 children with asymptomatic TCS, 12 had lumbosacral lipoma and two had meningocele. According to the classification of spinal lipoma, two children had dorsal type, four had caudal type, two had transitional type, and four had filar type. There were no children with lipomyelomeningocele. All children were free of neurological symptoms until 94 months after the initial surgery. Subsequently, one child exhibited delayed neurological deficits and underwent a second surgery because of motor and sensory disturbances; slight sensory disturbance was noted at the final follow-up examination. Another child later showed bowel and bladder dysfunction. However, a second surgery was not performed for this child because his motor and sensory functions were normal; hence, we chose to avoid nerve injury in the case of dissecting adhesion. CONCLUSIONS: All 14 children with asymptomatic TCS were free of neurological symptoms until 94 months after the initial surgery. However, two children exhibited delayed neurological deficits at 94 months and 177 months. We believe that prophylactic surgery for asymptomatic TCS is effective for a certain period. However, because the natural history of TCS is poorly understood, strict follow-up after surgery is necessary.
Child ; Classification ; Cohort Studies ; Follow-Up Studies ; Humans ; Lipoma ; Meningocele ; Natural History ; Neural Tube Defects ; Retrospective Studies ; Sensation ; Spinal Dysraphism ; Urinary Bladder

Local flaps exhibit excellent color matching that no other type of flap can compete with. Moreover, surgery using a local flap is easier and faster than surgery using a distant or free flap. However, local flaps can be much more difficult to design. We designed 2 templates to plan a V-Y rotation advancement flap. The template for a unilateral V-Y rotation advancement flap was used on the face (n=5), anterior tibia (n=1), posterior axilla (n=1), ischium (n=1), and trochanter (n=2). The template for a bilateral flap was used on the sacrum (n=8), arm (n=1), and anterior tibia (n=1). The causes of the defects were meningocele (n=3), a decubitus ulcer (n=5), pilonidal sinus (n=3), and skin tumor excision (n=10). The meningocele patients were younger than 8 days. The mean age of the adult patients was 50.4 years (range, 19–80 years). All the donor areas of the flaps were closed primarily. None of the patients experienced wound dehiscence or partial/total flap necrosis. The templates guided surgeons regarding the length and the placement of the incision for a V-Y rotation advancement flap according to the size of the wound. In addition, they could be used for the training of residents.
Adult ; Arm ; Axilla ; Femur ; Free Tissue Flaps ; Humans ; Ischium ; Meningocele ; Necrosis ; Pilonidal Sinus ; Pressure Ulcer ; Reconstructive Surgical Procedures ; Sacrum ; Skin ; Skin Neoplasms ; Surgeons ; Surgical Flaps ; Tibia ; Tissue Donors ; Wounds and Injuries

BACKGROUND: Median cleft lip is a rare anomaly consisting of a midline vertical cleft through the upper lip. It can also involve the premaxillary bone, the nasal septum, and the central nervous system. In our current report, we present the clinical features of 6 patients with a median cleft lip and their surgical management according to the accompanying anomalies. METHODS: From December 2010 to January 2014, 6 patients with a median cleft lip were reviewed. Five of these cases underwent surgical correction; alveolar bone grafting was performed in a patient with a median alveolar cleft. The surgical technique included inverted-U excision of the upper lip and repair of the orbicularis oris muscle. The mean follow-up period was 20.4 months (range, 7.4-44.0 months). RESULTS: The study patients presented various anomalous features. Five patients received surgical correction, 4 with repair of the median cleft lip, and one with iliac bone grafting for median alveolar cleft. A patient with basal sphenoethmoidal meningocele was managed with transoral endoscopic surgery for repair of the meningocele. Successful surgical repair was achieved in all cases with no postoperative complications. CONCLUSIONS: Relatively mild forms of median cleft lip can be corrected with inverted-U excision with good aesthetic outcomes. In addition, there is a broad spectrum of clinical features and various anomalies, such as nasal deformity, alveolar cleft, and short upper frenulum, which require close evaluation. The timing of the operation should be decided considering the presence of other anomalies that can threaten patient survival.
Alveolar Bone Grafting ; Bone Transplantation ; Central Nervous System ; Cleft Lip* ; Congenital Abnormalities ; Follow-Up Studies ; Humans ; Lip ; Meningocele ; Nasal Septum ; Postoperative Complications

Intrathoracic meningoceles are relatively rare entities found in patients with neurofibromatosis type I (NF1). Given that both the BRCA1 and NF-1 genes are located on the same long arm of chromosome 17, one would expect concurrence of neurofibromatosis and breast cancer. However, incidence of such co-disorders is very rare in the literature. Here, the authors report a case of a 50-year-old female patient with NF-1 and concurrent cancer of the left breast, who had a huge bilobulated intrathoracic meningocele with thoracic dystrophic scoliosis, treated surgically via a posterior-only approach for the meningocele and spinal deformity in the same setting.
Arm ; Breast Neoplasms* ; Breast* ; Chromosomes, Human, Pair 17 ; Congenital Abnormalities ; Female ; Humans ; Incidence ; Meningocele* ; Middle Aged ; Neurofibromatoses* ; Neurofibromatosis 1* ; Scoliosis

Neurofibromatosis 1 (NF1) is a common autosomal dominant disorder that causes several systemic diseases. Many studies have reported that NF1 is associated with intrathoracic meningoceles and scoliosis. The incidence of vertebral artery dissection is estimated to be 1-1.5 per 100,000 population. We experienced an autopsy case of massive intrathoracic hemorrhage due to spontaneous vertebral artery dissection in a patient with NF1, who had intrathoracic spinal meningoceles and scoliosis. A 47-year-old man was found dead at his home in the morning. He had a history of NF1 including numerous cutaneous neurofibromas and hyperpigmented macules, scoliosis, and deformity of the leg. The autopsy revealed the dissection and rupture of the left vertebral artery, and a pseudocyst that had formed due to arterial leakage on the wall of the meningocele on the left side. The pseudocyst had eventually ruptured and leaked blood, resulting in a massive hemothorax on the left side. Thus, it was revealed that the patient had suffered from NF1-associated intrathoracic meningoceles and scoliosis, and we concluded that the cause of his death was a massive hemothorax on the left side, caused by the dissection and rupture of the left vertebral artery.
Autopsy* ; Congenital Abnormalities ; Hemorrhage ; Hemothorax ; Humans ; Incidence ; Leg ; Meningocele* ; Middle Aged ; Neurofibroma ; Neurofibromatoses* ; Neurofibromatosis 1* ; Rupture ; Scoliosis* ; Vertebral Artery Dissection* ; Vertebral Artery*

Meningocele is a protrusion of meninges through a defect in the skull base. It is a rare but potentially life-threatening condition requiring surgery. We present a case of 59-year-old woman complaining of persistent otorrhea of the right ear and a pulsating cystic mass occupying the external auditory canal in her right ear. The patient had undergone right canal wall up mastoidectomy and tympanoplasty about 30 years ago. Radiological evaluations revealed a bony defect at the tegmen tympani and tegmen mastoideum, with a cystic mass filling this defect and the external auditory canal, suggesting temporal bone meningocele. The meningocele was resected and the bony defect of tegmen was successfully repaired via combined transmastoid and middle cranial fossa mini-craniotomy approach.
Cranial Fossa, Middle ; Ear ; Ear Canal* ; Female ; Humans ; Meninges ; Meningocele* ; Middle Aged ; Skull Base ; Temporal Bone* ; Tympanoplasty

Mucoceles are expansile, encapsulated, benign cystic lesions with the potential for adjacent bony remodeling and resorption. Paranasal mucoceles predominantly develop in the frontal sinuses (60%), followed in frequency by the ethmoidal (20-30%), maxillary (10%), and sphenoid (2-3%) sinuses. Nasal septal mucoceles develop in only very seldom cases. Septal mucoceles might develop from infected nasal septum or trauma. In the differential diagnosis of a midline septal mass, dermoid cyst, encephalocele, meningocele or intraseptal abscess should be considered. We present a case of nasal septal mucocele, which was managed by endoscopic excision and marsupialization.
Abscess ; Dermoid Cyst ; Diagnosis, Differential ; Encephalocele ; Frontal Sinus ; Meningocele ; Mucocele* ; Nasal Obstruction ; Nasal Septum*

PURPOSE: Sacral cutaneous lesions, such as dimples and hairy patches, may be associated with occult spinal dysraphism and urogenital abnormalities. This study aim to delineate high risk infants who need early screening for intraspinal and urogenital abnormalities by identifying the association between cutaneous lesions and combined abnormalities. METHODS: Sacral ultrasonography was performed in 777 infants with sacral cutaneous lesions from January 2010 to July 2014. Of these, 317 infants underwent abdominal ultrasonography for urogenital abnormalities. We reviewed the patient's medical records and radiographic findings retrospectively. RESULTS: Of the 777 infants, abnormal intraspinal findings such as tethered cord or meningocele were reported in 26 (3.4%). Sixteen of these 26 patients with abnormal findings underwent follow-up ultrasonography or MRI; 4 infants were diagnosed with lipomeningomyelocele through MRI, and 1 infant underwent a neurosurgical procedure. Among the 317 infants who underwent abdominal ultrasonography, 78 infants (24.6%) had congenital hydronephrosis and 8 infants (2.5%) had other urogenital abnormalities including duplication of kidney, vesicoureteral reflux, horseshoe kidney, renal cyst, or multicystic dysplastic kidney. Urogenital abnormalities were more common in patients with sacral dimples associated with hair or deviated gluteal folds than in those with simple dimples (OR 3.24 and 8.88; P=0.007 and P=0.001, respectively). CONCLUSION: Midline sacral cutaneous lesions may be associated with occult spinal dysraphism and urogenital abnormalities. To detect intraspinal lesions, ultrasonography is recommended for infants with sacral cutaneous lesions. Likewise, infants with sacral dimples associated with either hair or deviated gluteal folds, should be evaluated via abdominal ultrasonography to assess for combined urogenital abnormalities.
Follow-Up Studies ; Hair ; Humans ; Hydronephrosis ; Infant* ; Kidney ; Magnetic Resonance Imaging ; Mass Screening ; Medical Records ; Meningocele ; Multicystic Dysplastic Kidney ; Neural Tube Defects ; Neurosurgical Procedures ; Retrospective Studies ; Ultrasonography ; Urogenital Abnormalities* ; Vesico-Ureteral Reflux