Unilateral renal agenesis with or without ipsilateral adrenal agenesis

We analyzed 7 patients with unilateral renal agenesis. The coexistence of unilateral renal agenesis with ipsilateral adrenal agenesis was confirmed surgically in 2 patients, 1 of whom had clinical features of Cushing's syndrome owing to adrenocortical adenoma of a solitary adrenal gland. It is imperative to suspect abnormalities of the ipsilateral adrenal gland when unilateral renal agenesis is encountered. Unilateral renal agenesis usually is asymptomatic. Recognition of this anomaly is essential for early correct diagnosis.     

Unilateral renal agenesis associated with bicornuate uterus

A case of bicornuate uterus associated with unilateral renal agenesis in a 45-year-old woman is reported. The embryologic problems of müllerian or wolffian ducts in this anomaly are discussed. By using radiological, nuclear or ultrasound methods unnecessary surgery could be evaded.     

Unilateral renal agenesis associated with various metabolic disorders in three siblings

Unilateral renal agenesis with impaired renal function was found in all 3 siblings of a single family, in association with various metabolic disorders such as diabetes mellitus, hyperuricemia and hyperbilirubinemia with gallstones. The present report suggests that unilateral renal agenesis could occur as a manifestation of a genetic disorder.     

Unilateral renal agenesis and urethral atresia associated with ergotamine intake during pregnancy

Ergotamine, an ergot alkaloid, used for the treatment and prevention of migraine attacks, is considered as a teratogenic drug and, therefore, should be avoided in pregnancy. Here, we report a newborn infant with unilateral renal agenesis, urethral atresia, and pulmonary hypoplasia associated with the use of ergotamine for the treatment of migraine attacks at early pregnancy. Genitourinary anomalies in association with ergotamine usage were rarely reported and this was the third case of renal agenesia in association with ergotamine usage in literature. We suggest that ergotamine teratogenicity may be dose dependent and should be avoided in pregnancy for the possibility of genitourinary anomalies.     

Unilateral agenesis of the diaphragm

During a period of 4 1/2 years, 37 infants with congenital diaphragmatic hernia were treated. The overall survival rate was 68%. Survival depended more on cardiopulmonary function than the size of the diaphragmatic defect. There was little evidence that infants with agenesis of the diaphragm formed a special group with a poor prognosis, and four of the ten patients with unilateral agenesis survived. A Dacron prosthesis is recommended as a substitute for the missing diaphragm.     

Penile agenesis associated with urethral and bilateral renal agenesis

Penile agenesis is a rare anomaly, with an estimated incidence of 1 in 30 million. Most cases are compatible with life but require early surgical intervention. This case is unusual in that there was an associated agenesis of the kidneys and urethra leading to fatal oligohydramnios sequence with pulmonary hypoplasia.     

Poland syndrome associated with renal agenesis

Poland syndrome is characterized by unilateral aplasia or hypoplasia of the sternocostal portion of the pectoralis major muscle and ipsilateral syndactyly. In some cases other associated anomalies, including renal malformations, dextrocardia, and vertebral abnormalities, have been reported. We report a 7-month-old girl with Poland syndrome who also presented with ipsilateral renal agenesis. This report suggests that renal structural anomaly may be an integral part of this syndrome. We recommend renal imaging studies be performed on all children with Poland syndrome. Received: 23 July 2001 / Revised: 23 October 2001 / Accepted: 23 October 2001     

Unilateral diaphragmatic agenesis precluding laparoscopic cholecystectomy.

BACKGROUND: Complete absence of a hemidiaphragm or diaphragmatic agenesis in adulthood is rare with only one previous report in the literature. Its significance in relation to performing laparoscopic procedures has not been documented previously. METHODS: We report a case of previously undiagnosed diaphragmatic agenesis in adulthood precluding laparoscopic cholecystectomy and comprehensively review the literature for papers relevant to diaphragmatic agenesis in adulthood. RESULTS: Diaphragmatic agenesis in adulthood may complicate and preclude laparoscopic cholecystectomy. The principles of investigation and management of diaphragmatic agenesis complicating laparoscopic surgery are discussed. CONCLUSIONS: In adults with diaphragmatic agenesis and intrathoracic abdominal viscera precluding laparoscopic cholecystectomy, conservative management is recommended.