Prevalence of Rare Craniofacial Clefts.

BACKGROUND: Craniofacial clefts are extremely rare congenital malformations that have adverse functional, psychosocial, and aesthetic effects on patients' life. Although the exact incidence is unclear, it is estimated between 1.4 and 4.9 per 100,000 live births. Prevalence of the rare craniofacial clefts is imprecise due to the paucity of literature as well as their etiologies. METHODS: All the patients with rare craniofacial clefts during 10 years in a plastic surgery tertiary referral hospital were included, and Tessier craniofacial clefting classification was used for classifying the clefts. RESULTS: Of 964 patients with craniofacial clefts, 80 (8.29%) patients were identified with rare craniofacial clefts. There were 39 (48.7%) males and 41 (51.3%) females. Family history was determined positive in 30 (37.5%) patients. Tessier number 0 (58.7%) was the most common cleft in the authors' study. Tessier numbers 8, 13, and 30 were the rarest clefts. There was no patient with Clefts numbers 5, 6, or 9. Maternal smoking during pregnancy was observed in 1 (1.3%) of the women and 3 of the women had used drugs, 1 of them used the dexamethasone tablets and 2 of them could not remember name of the used drug. CONCLUSIONS: Tessier number 0 was the most common cleft and Tessier numbers 8, 13, and 30 were the rarest types. The precise etiology of rare craniofacial clefts remained undetermined in this study. Women should be educated about the risk factors and subsequent ways of preventing from these risk factors.

Primary Hydatid Cyst of Umbilicus, Mimicking an Umbilical Hernia.

Hydatid cyst caused by Echinococcus granulosus demonstrates an endemic infection in several countries such as Middle Eastern countries. Liver is the most frequently involved organ, followed by the lung. The case we present is solitary primary localization of cyst in abdominal wall which is extremely rare. A 57-year-old woman presented with an abdominal wall lesion in umbilical area that had been evolving for about 2 years with recent complaint of pain and discomfort. We detected a midline abdominal mass 12⁎13 centimeters in diameter which was bulged out in umbilicus. Preoperative clinical diagnosis of incarcerated umbilical hernia was made due to its physical examination while surgical exploration disproved the primary diagnosis and we found cystic mass adherent to superficial fascia without any communication to peritoneal space. The cyst was excised completely without any injury or perforation of containing capsule. The diagnosis of hydatid cyst was confirmed by histopathological examination of specimen. The retrograde evaluation showed no involvement of other organs. The patient was followed for two years and no recurrence of hydatid disease has been observed. Hydatid cyst should be considered as a differential diagnosis of abdominal wall and umbilical lesions especially in endemic regions.

Extrahepatic bile duct neurilemmoma mimicking Klatskin tumor.

Neurilemmoma rarely develops in the biliary tree. Here, we report a 39-year-old Iranian woman with neurilemmoma in the extrahepatic bile duct presenting with progressively deepening jaundice. On the basis of clinical and radiological features, this tumor was initially suspected as Klatskin tumor. Histologically, the tumor was a typical neurilemmoma. Immunostaining showed that tumor cells were strongly and diffusely positive for S-100 protein, which supported the diagnosis of neurilemmoma. Neurilemmoma should be considered in the differential diagnosis of obstructive jaundice.