Perinatal haemorrhagic necrosis of the adrenal gland A clinical and radiological evaluation of 24 consecutive cases

Up to 1970 only13 fully verified and successfully treated cases of neonatal adrenal haemorrhage were on record. As uniform diagnostic and therapeutic guiding principles are still lacking, our experience of 24 consecutive cases appears worth presentation.--The most frequent clinical signs were a palpable mass in 21 cases, jaundice in 14 patients and urinary infection or sepsis in 10 neonates.--Intravenous urography revealed the diagnosis with a reasonable degree of reliability in all our cases. No additional information of significance was obtained at aortography, employed in 2 of our cases.--Surgical exploration with drainage of the haematoma or adrenalectomy was performed in 18 cases, 6 were treated conservatively.--Recovery was uneventful in all but for one conservatively treated patient. In this case death was caused by a second profuse bleeding, which proved fatal in spite of all life saving efforts.     

Natural history of adrenal haemorrhage in the newborn

5 cases of unilateral and 3 cases of bilateral haemorrhage are described. Only 1 infant died, from venous thromboses elsewhere. Apart from this case, all had an above average birthweight. Probable predisposing causes apart from large size were fetal hypoxia, septicaemia, thrombocytopenia, coagulation defect, and disseminated thromboembolic disease. The condition must be distinguished from renal vein thrombosis. In the acute stage pyelography shows depression of the kidney on the affected side, with flattening of the upper calyces. Calcification develops rapidly round the periphery of the mass, then slowly contracts into an area of the size and shape of the original gland. Treatment is with antibiotics and blood transfusion, with intravenous corticosteroids in severely shocked or bilateral cases. Adrenal insufficiency is rarely found on follow-up even in bilateral cases, but renal hypertension should be looked for. No single cause for the haemorrhage could be discovered, but the preponderance of haemorrhage into the right adrenal gland is probably due to anatomical differences between the venous drainage of the two sides.     

Intermittent testicular torsion

Testicular torsion is the most common cause of acute scrotal pain in prepubertal and adolescent boys and should be foremost in the minds of primary care physicians evaluating these children. Intermittent testicular torsion is a separate entity that should be considered in all young males with a history of scrotal pain and swelling. Acute and intermittent sharp testicular pain and scrotal swelling, interspersed with long intervals without symptoms, are characteristic. Physical findings may include horizontal or very mobile testes, an anteriorly located epididymis, or bulkiness of the spermatic cord from partial twisting. Awareness of this entity and early elective orchiopexy will improve testicular salvage in patients with intermittent testicular torsion.     

Acute idiopathic scrotal oedema: four cases and a short review

We report four cases of acute idiopathic scrotal oedema, an underreported cause of acute painless or moderately painfull swelling and erythema of the scrotum in young boys. It is a self-limiting disease and to prevent unnecessary surgical exploration or antibiotic therapy, it is very important to distinguish acute idiopathic scrotal oedema from more serious diseases.     

Intracerebral hemorrhage at 2 different times in neonatal alloimmune thrombocytopenia

We demonstrate the sonographic and computer-tomographic findings in a newborn with neonatal alloimmune thrombocytopenia (NAITP). Initial examination on the third day of life showed an extensive old and already partially resolved haematoma as well as a new perinatal haematoma in the left cerebral white matter. Follow-up investigations revealed enlargement of the cystic defect, midline-shift to the right and progressive dilatation of the right lateral ventricle caused by compression of the Foramina Monroi. Peritoneal drainage of the cyst resulted in prompt diminution of the enlarged right lateral ventricle. The case report emphasises that in patients with NAITP intracranial haemorrhage may occur in utero and during the perinatal period as well. In pregnant women suspected to produce antibodies against PLA 1 positive thrombocytes, we recommend regular ultrasonic cranial examinations of the fetus. Delivery should be as careful as possible.     

Differentialdiagnosis of neonatal adrenal haemorrhage and congenital neuroblastoma by colour coded Doppler sonography and power Doppler sonography

The differentiation between neonatal neuroblastoma and adrenal haemorrhage may be very difficult in the individual case. We investigated eight neonates with adrenal haemorrhage and one patient with congenital neuroblastoma by colour coded Doppler sonography and power Doppler sonography. Six haemorrhages were cystic, whereas the neuroblastoma and four adrenal haemorrhages had a solid appearence. Colour coded Doppler sonography and power Doppler demonstrated vessels within the neuroblastoma. In none of the ten adrenal haemorrhages could flow be shown either with conventional colour coded Doppler sonography or power Doppler sonography. Conclusion Conventional colour coded Doppler sonography and power Doppler sonography are useful to differentiate between neuroblastoma and adrenal haemorrhage. Received: 28 January 1997 and in revised form: 30 April 1997 / Accepted: 22 August 1997     

The rare association of right adrenal haemorrhage and renal vein thrombosis diagnosed with duplex ultrasound

Neonatal adrenal haemorrhage with renal vein thrombosis, an almost exclusively left-sided phenomenon, may occasionally be bilateral in the presence of inferior vena cava thrombus but has only twice been reported as confined to the right side. These cases required a combination of ultrasound (USS), excretion urography, cystoscopy with retrograde pyelography, radioisotopes, and CT to diagnose this rare right-sided combination. We report a case of right adrenal haemorrhage causing right renal vein thrombosis accurately diagnosed using only duplex USS and radioisotopes. The potential complications of renal vein thrombosis and hypertension associated with adrenal haemorrhage requires accurate diagnosis and this paper emphasises the value of duplex USS.     

Aortic thrombosis diagnosed by ultrasound

A neonate developed aortic thrombosis following catheterization of the umbilical artery. The thrombus was identified and followed up by ultrasonography. Fibrinolytic therapy led to the almost complete disappearance of the aortic thrombus but the child died of cerebral haemorrhage. Physicians should be alerted to the possibility of aortic thrombosis in neonates following umbilical arterial catheterization. Ultrasonography is an appropriate method for diagnosis and follow-up in these cases.     

Adrenal hemorrhage versus testicular torsion — a diagnostic dilemma in the neonate

Three boys aged 2–3 days were admitted to the Pediatric Surgical Clinic on suspicion of testicular torsion. One of the boys had a scrotal mass that on surgical exploration was shown to be a hematoma within the processus vaginalis beside a normal testis. An immediate laparotomy revealed an adrenal hemorrhage with rupture into the peritoneal cavity. Two further patients in whom a bluish discoloration of one hemiscrotum and groin had appeared postnatally were examined by ultrasound, which revealed a right-sided suprarenal mass. It is concluded that a scrotal mass or hematoma in the neonate may be the only symptom of an adrenal hemorrhage. When a scrotal mass is found an intra-abdominal hemorrhage into an inguinal hernia should be considered and if possible ultrasonographically examined before surgical exploration is performed on the suspicion of testicular torsion or tumor. Furthermore, a scrotal or inguinal hematoma should lead to an ultrasound examination of the retroperitoneal organs, especially of the adrenal glands. Offprint requests to: B. Karpe     

A rare retroperitoneal tumor: abdomino-scrotal hydrocele. Two case reports]

Abdominoscrotal hydrocele is a very rare condition, different from the majority of cases of hydroceles related to the persistence of the processus vaginalis. The anomaly consists of a large scrotal hydrocele which communicates in an hour-glass fashion with a large abdominal component through the inguinal canal. We report two cases observed in infants. CASE REPORTS: 1. A nine-month-old infant was referred because of a large bilateral hydrocele. On clinical examination on the right side in the lower quadrant of the abdomen was found a mass which communicated with the right scrotal pouch. Diagnosis of abdominoscrotal hydrocele was established by ultrasonography. During the surgical exploration the right testis was found to be dysmorphic, fusiform and ectopic in the inguinal canal. Surgical treatment comprised hydrocelectomy and right orchidectomy. 2. A six-month-old infant presented with a large right hydrocele in communication with an abdominal pouch located in the right lower quadrant, in association with a lymphoedema of the right limb. Diagnosis of abdominoscrotal hydrocele was confirmed by ultrasonography. During the surgical procedure the pouch was opened and everted. The testis was dysmorphic as in the first case, in normal scrotal position and was preserved. COMMENTS: Abdominoscrotal hydrocele is a rare condition reported in adult population and also in the infant. The exact mechanism by which it develops is unknown. The diagnosis can be suspected on clinical examination if an abdominal mass in a lower quadrant is palpable just above the inguinoscrotal pouch. The diagnosis relies on ultrasonography. Complications sometimes occur due to the pressure on adjacent structures (ureters, iliac vessels). Testicular dysmorphism has been reported in some patients. In any case, as spontaneous resolution of abdominoscrotal hydrocele has never been reported, surgical treatment is indicated.     

The pediatric acute scrotum

The pediatric acute scrotum can be a diagnostic dilemma. The history, physical examination, and appropriate tests are important. The correct diagnosis will be arrived at if the systematic approach is remembered (Figure 14). In cases where testicular torsion cannot be ruled out (such as an epididymitis or torsion of the appendix testis where torsion of the testis is mimicked), the patient will need scrotal exploration. It is hoped that with further availability of scanning and expertise by pediatric surgeons the false negative exploration will decrease in number. Remember, occasionally a patient will require scrotal exploration with biopsy of the epididymis to delineate the underlying cause. In most cases of epididymitis, effort should be made to identify the cause by culture of urine, urethral discharge, or appropriate fluid from the epididymis. Urological evaluation will be necessary in the younger boys, particularly those with recurrent episodes, to rule out congenital anomalies. Only by following this systematic approach can clinicians hope to preserve gonadal function and achieve best care of their patients.     

Adrenal bleeding in neonates: report of 37 cases

Adrenal hemorrhage is more common in neonates than in children or adults. The incidence of detected cases ranges from 1.7 to 2.1 per 1000 births. Because adrenal bleeding may remain asymptomatic, the real occurrence is probably higher. In this retrospective study, we evaluated epidemiologic properties, risk factors and clinical presentations of adrenal hemorrhage in 37 term newborn babies diagnosed as adrenal hemorrhage with abdominal ultrasonography between January 2003 and July 2007 in Dr. Sami Ulus Children's Hospital Neonatal Intensive Care Unit (NICU). We also evaluated the role of adrenal hemorrhage among the etiologic factors of unexplained jaundice. Abdominal ultrasonography was applied to 2280 newborns, and 37 newborns (25 male, 12 female) were diagnosed as adrenal hemorrhage (1.6%). The male/female ratio was 2.08. The average age and birth weight at admission were 4.9 +/- 0.3 days and 3333 +/- 939 g, respectively. Adrenal hemorrhage was right-sided in 24, left-sided in 9 and bilateral in 4 newborns. Resolution time of adrenal hemorrhage was a minimum of 3 months, maximum of 9 months in ultrasonographic follow-up. The most common clinical feature in infants with adrenal hemorrhage was jaundice, which was observed in 67.6% of cases (n = 25). We advise that, in cases of hyperbilirubinemia of unknown etiology, adrenal hemorrhage must be kept in mind. We recommend abdominal ultrasonography for further evaluation.     

Pediatric granulomatous orchitis: Case report and review of the literature

An 11‐year‐old boy presented with fever and abdominal pain, and was diagnosed with retroperitoneal lymphadenitis. At the same time, a painless right scrotal mass was observed. On imaging the testis and the epididymal mass both had abundant blood flow, although tumor markers were negative. Although the right testis had shrunk after antibiotic treatment, swelling was persistent and incisional biopsy was therefore performed, resulting in diagnosis of granulomatous orchitis (GO). No recurrence was found. In cases of scrotal swelling in both the testis and the epididymis of an older child, it is necessary to consider the possibility of inflammatory GO, and orchiectomy should not be performed without careful consideration.     

Torsion of an indirect hernia sac within a hydrocele causing acute scrotum: case report and review of the literature

We report a case of acute scrotum in a 2-year-old child caused by torsion of an indirect hernia sac within a hydrocele. This is an extremely rare cause of acute scrotal swelling and pain in the pediatric population, with this case representing only the eighth instance reported in the literature. Despite the scarcity with which this diagnosis has been encountered, clinicians should include torsion of an indirect hernia sac on the differential diagnosis for acute scrotum in a child.     

Acute pulmonary hypertension complicating the arterial switch procedure

Two neonates undergoing arterial switch procedure developed life-threatening pulmonary hypertension intraoperatively. In one patient, bradycardia, hypotension, and electrocardiographic (ECG) evidence of myocardial ischemia suddenly occurred 20 minutes after uneventful weaning from cardiopulmonary bypass. Lifting a palpably hypertensive main pulmonary artery (MPA) resulted in reproducible hemodynamic improvement. Because the patient was already on full ventilatory support and a nitroglycerin infusion, the MPA was suspended onto the anterior chest wall. In the other patient, after removal of intraoperative drapes, severe generalized swelling and cyanosis were noted. The central venous pressure had risen to 25 mmHg, and the PO₂ had dropped to 52 mmHg on 100% FIO₂. The systolic arterial pressure and ECG remained normal. Immediate reexploration revealed a palpably hypertensive MPA. The coronary arteries implanted more laterally on the neoaorta were uncompromised. Amrinone loading and infusion produced immediate improvement. We believe that surgeons should be aware that pulmonary hypertension can cause coronary artery compression and right heart failure in neonates undergoing the arterial switch procedure. Lateral placement of the coronary artery and aggressive use of pulmonary vasodilators can minimize the problem.     

Diagnosis and management of neonatal adrenal haemorrhage

Three patients with neonatal adrenal haemorrhage (NAH), presenting with various signs and symptoms, are reported. The role of ultrasonography in diagnosis and management of this entity is emphasized. Conservative management is advocated, once the diagnosis is confirmed. Surgery is indicated only for cases of uncontrollable haemorrhage.     

Scrotal hematoma due to neonatal adrenal hemorrhage: the value of ultrasonography in avoiding unnecessary surgery

Scrotal hematoma is an uncommon presentation of neonatal adrenal hemorrhage. Nine previous cases of such an association have been reported in the literature, and unnecessary surgery was carried out in five of these cases. The authors report three new cases observed during a 3-year period and stress the critical role of scrotal and abdominal ultrasonography in order to avoid unnecessary surgical exploration. Received: 13 June 1996 Accepted: 28 January 1997     

ULTRASONOGRAPHY IN THE DIAGNOSIS OF NEONATAL ADRENAL HAEMORRHAGE

Abstract. Nordshus, T. and Monn, E. (Department of Radiology, Section of Paediatric Radiology and Department of Paediatrics, Rikshospitalet, The National Hospital, University of Oslo, Oslo, Norway). Ultrasonography in the diagnosis of neonatal adrenal haemorrhage. Acta Paediatr Scand 69: 695, 1980.—Two cases of neonates with adrenal haemorrhage are presented. The clinical, radiological and ultrasonographical signs are described. The future role of echography as compared to other radiological procedures, including computerized tomography, is discussed. Ultrasonography is recommended as a screening method, and is well suited for assessing the size and internal structure of pathological processes.     

Frequency of adrenal hemorrhage in fatal forms of purpura fulminans in children. Etiopathogenic and therapeutic considerations

Between 1971 and 1985, 43 children died of purpura fulminans in our intensive care unit: 11 had autopsy and adrenal haemorrhage was observed in 8 (73%). All these patients had an extensive purpura and a severe disseminated intravascular coagulation. Our series confirms the findings of previous studies: 69 autopsies showed 51 cases (74%) of macroscopic adrenal haemorrhage. Adrenal haemorrhage may be only one manifestation of multiple system organ failure consecutive to septic shock; however, its association with low plasma cortisol levels (as previously reported) suggests that glucocorticoid replacement therapy should be reconsidered in purpura fulminans.