Congenital chylothorax: report of unique case associated with 'H' -type tracheo-oesophageal fistula.

A case of congenital chylothorax associated with a congenital 'H'-type tracheo-oesophageal fistula is reported. The development of the thoracic duct is described and the possible embryological association with tracheo-oesophageal fistula is discussed. The management of the case is reviewed.     

Congenital atresia of the portal vein and extrahepatic portocaval shunt associated with benign neonatal hemangiomatosis,congenital adrenal hyperplasia,and atrial septal defect

A rare case of congenital atresia of the portal vein and ductus venosus, extrahepatic portocaval shunt, benign neonatal hemangiomatosis, congenital adrenal hyperplasia, and an atrial septal defect is reported. Twenty-two cases of congenital extrahepatic end-to-side shunts have been described before. Although additional anomalies are common in this type of shunt, hemangiomatosis has been described only once. Adrenal hyperplasia has never been reported in this anomaly.     

Congenital pelvic arteriovenous malformation: uncommon symptoms of lower limb venous hypertension.

Congenital pelvic arteriovenous malformations (AVMs) are rare and their clinical behavior is quite variable. A case of congenital pelvic AVM manifesting with unusual extrapelvic symptoms of ipsilateral leg pain is described. The causes of symptoms associated with congenital pelvic AVMs are discussed.     

Unexpected difficult intubation in the patient with Morning Glory syndrome

Morning Glory syndrome is an uncommon congenital optic disc anomaly with occasional systemic associations. A case of unsuspected difficult intubation in a three-year old patient is described in this case report.     

Congenital hypoplastic thumb in the Silver syndrome--a case report and review of upper extremity anomalies in the world literature

A case of classical Silver syndrome presented with a congenital hypoplastic thumb, absent thenar musculature, and partial index ankylosis. No previous association of congenital hypoplastic thumb and the Silver syndrome has been noted by Dr. Silver or described in the world literature. Restoration of thumb function was achieved in this particular case by the abductor digiti minimi transfer described by Huber. The upper extremity anomalies thus far reported with Silver syndrome in the world literature are summarized.     

The palmar nail syndrome. Case report

The congenital ectopic nail is very rare and usually present as an ectopic abnormal nail alongside the normal fingernail. Ridder described in 1992 the "congenital palmar nail syndrome" sharing a palmar ectopic nail, a loss of flexion and a small distal phalanx on x-ray. We report a case of a congenital palmar nail syndrome of both little fingers on a boy 13 years old.     

The ski jump deformity of the distal phalanx: a new entity?

A case of a congenital hand anomaly is presented in which the distal phalanx of the little finger was curving dorsally in the sagittal plane. This may be the first reported case. A surgical approach to correct it is described.     

Polyhydramnios associated with congenital pancreatic cysts and asphyxiating thoracic dysplasia. A case report.

The antenatal ultrasonic detection of congenital pancreatic cysts is described in a case of polyhydramnios associated with fetal asphyxiating thoracic dysplasia. The formation of polyhydramnios is discussed.     

Congenital complete simple anonychia

Anonychia (absence of nails) is a very rare congenital or acquired anomaly. Congenital anonychia has been described in association with a wide variety of other congenital anomalies. Of these abnormalities, the most frequent are some hypoplastic nails, this is incomplete anonychia. Simple anonychia, meaning congenital absence of the nails without any other coexisting major congenital anomaly, is an extremely rare variety of this condition. This is mostly due to autosomal recessive inheritance. A newborn case with congenital but non-inherited absence of all fingernails and toenails together with the absence of both fifth distal phalanges without any other major structural anomaly is reported.     

Bilateral congenital pseudarthrosis of the olecranon

A case of bilateral congenital pseudarthrosis of the olecranon is described. The clinical features, radiological appearance and prognosis are distinct from the type of congenital pseudarthrosis of the forearm reported previously. The lesion appeared to be a localised failure of ossification within the proximal ulnar metaphysis. Excision, stabilisation and bone grafting led to rapid fusion.     

Non tumoral aqueduct stenosis. Multiple microscopic subependymal glial protuberances demonstrated at hystological examination

A case of hydrocephalus associated to aqueduct stenosis is described. The microscopic examination showed aqueduct gliosis which was not possible to distinguish congenital or acquired.     

Congenital ureteric cyst—rare anomaly

A rare example of a congenital cyst of the ureter is described and the embyologic development of the abnormally discussed. The world literature relevant to the case is reviewed.     

Primary repair of tetralogy of Fallot combined with unilateral left lung agenesis with crossed-ectopic right lower lobe

Unilateral lung agenesis is a rare congenital anomaly often combined with congenital malformations of different systems. A case of tetralogy of Fallot repair with unilateral left lung agenesis via median sternotomy is described for the first time. In this case, the choice of surgical approach to the heart could be affected by the fears of not getting optimal surgical exposition via median sternotomy due to severe displacement of the chest organs. The case demonstrates the median sternotomy as an optimal surgical approach even in high degrees of organ displacement caused by a combination of left pulmonary agenesis and cardiac lesions.     

Distraction osteogenesis of a congenital amputation at the elbow

A case is described demonstrating the effectiveness of distraction osteogenesis for the lengthening of a short below elbow congenital amputation. To our knowledge this technique has not been previously used in a patient with a congenital amputation. The surgical technique of corticotomy and postoperative lengthening is detailed. The result converted the level of patient performance from elbow disarticulation to functional below elbow status.     

Congenital cervical neuroblastoma associated with Horner syndrome

A rare case of congenital neuroblastoma of the neck associated with complete Horner syndrome and respiratory distress is described. The case was successfully treated by total excision secondary to chemotherapy. Horner syndrome was of value for early diagnosis of this lesion.     

A 30 year functional follow-up of a neglected congenital clubfoot in an adult: a case report

Neglected congenital clubfoot in adults has been described in literature, but is not common to see adult patients with this orthopaedic disorder in developed countries with 30 years of follow-up. We report an asymptomatic case of neglected congenital clubfoot in an adult, who is incidentally seeking treatment for her 18-month-old son with congenital clubfoot. Although the cosmetic appearance is unacceptable, this mother remarkably has no functional limitations and for this reason she refuses any surgical treatment for her foot. To our knowledge, this is the first report of an untreated congenital clubfoot with 30 years follow-up.     

Congenital bilateral pseudarthrosis of the index metacarpal bones

Congenital pseudarthrosis is very rare in upper extremity, and has only previously been reported in the forearm. A case of bilateral congenital pseudarthrosis of the index metacarpal bones is described.     

Congenital flexion deformity of the wrist owing to an anomalous insertion of the flexor carpi ulnaris: a case report

A case of congenital flexion deformity of the wrist is described. The cause was an anomalous insertion of the flexor carpi ulnaris into skin and palmar aponeurosis.     

Congenital absence of superficial posterior compartment calf muscles

Although various congenital abnormalities have been described, congenital absence of calf musculature is extremely rare, with only one report on its complete absence. We are the first to describe a case of congenital absence of muscles of the superficial posterior compartment of the calf presenting in a toddler. The child presented with a history of a painless limp, however no significant difference was found in functional gait analysis. We suggest that such cases should be monitored and parents can be reassured that no immediate treatment is required.     

Congenital accessory ectopic eyelid: case report

An extremely rare case of congenital accessory ectopic eyelid associated with an oblique facial cleft is reported. The clinical features, histological findings and morphogenesis are briefly described.