A Rupture in Silence: A Life-threatening Neonatal Adrenal Bleed

ABSTRACT

Neonatal adrenal hemorrhage (NAH) is a rare condition that commonly presents with retroperitoneal bleeding in neonates. We report a term male neonate born through vaginal delivery who presented with respiratory distress and shock on the first day of his life. Examination revealed pallor, abdominal distension and a tender lumbar mass. Laboratory tests showed severe anemia, and imaging studies confirmed a large perirenal hematoma with absent adrenal shadow. The diagnosis was right adrenal rupture causing hemorrhagic shock. Prompt resuscitation and conservative management led to a favorable outcome. NAH should be considered in neonates presenting with shock and abdominal mass, mimicking renal disorders.

INTRODUCTION

Hypovolemic shock due to retroperitoneal bleed is a rare and life-threatening emergency in neonates, if not recognized and addressed promptly. Neonatal Adrenal hemorrhage (NAH) is recognized as the most common cause of retroperitoneal bleed in the 1^st month of life. It is an uncommon condition in neonates with an incidence of 0.2%–0.55%.[1,2] Unilateral adrenal rupture precipitating massive hemorrhage in the immediate postnatal period is a rare encounter. Sometimes, NAH can masquerade as a renal lump in the absence of risk factors. After taking informed parental consent, we here describe an unusual case of adrenal apoplexy in a newborn.

CASE REPORT

A male infant was delivered at 38-week gestation by vaginal route to a primi-gravida mother and admitted at the age of 20 h with complaints of lethargy and respiratory distress for the last few hours. The antenatal period was uneventful. Maternal medical history was uneventful. A history of bleeding disorders was absent in the family. Newborn cried immediately after birth. There was no history of prolonged labor or instrument application. Baby took the first few feeds well, but after a few hours, suddenly stopped feeding. There was no history of seizures after birth, vomiting, and blood in stool or urine. The baby had passed urine after birth.

On initial assessment, the baby was observed to have gasping type of breathing, hypoxic (70% oxygen saturation at room air), hypotensive (38/25 mmHg) and had a heart rate of 180 beats/min with weak, peripheral pulsations, and cold extremities.

On general physical examination, severe pallor was noted without any skin or mucosal bleed. Immediately, baby was intubated, and ventilation was initiated. The anterior fontanelle was at the level. Gross hematuria was noted on urinary catheterization. Systematic examination of the abdomen showed distension with a palpable, tender mass in the right lumbar region. Margins of the lump were not distinct. The rest of the systemic examination was normal. Given the severity of presentation of shock with severe pallor and a single-sided flank mass on the 1^st day of life, the differential diagnoses considered were hydronephrosis, dysplastic kidney, renal cyst, neuroblastoma or nephroblastoma, renal vein thrombosis (due to blood group incompatibilities or sepsis), and Wunderlich syndrome (WS).

Shock was managed with fast fluid resuscitation, inotropic support, and 4 aliquots of packed red blood cell transfusion. Arterial blood gas analysis showed hypoxemia and severe metabolic acidosis. Initial blood investigations revealed severe anemia (Hemoglobin-6 mg/dL) with normal platelet counts. No blood group incompatibility was noted between the baby and mother, and Coomb’s test was negative. Prothrombin and partial thromboplastin time were within normal limits, ruling out bleeding disorders or hypercoagulable condition. Renal function tests were deranged initially, normalized after 72 h. Urine analysis showed hematuria.

Ultrasonography (USG) abdomen revealed a large perirenal collection of blood on the right side with absent adrenal shadow, later confirmed on tomography [Figure 1]. Thus, imaging studies excluded hydronephrosis, dysplastic kidney, renal cyst, and nephroblastoma. Color Doppler helped to exclude renal vein thrombosis.

Figure 1.

Computed Tomography (CT) abdomen showing right side peri-renal hematoma with non-visualization of the adrenal gland. CT abdomen right peri-renal hypodense hematoma (blood) accumulation measuring 6 cm x 7cm x 5cm (a). The adrenal gland shadow was absent on the right side. Left kidney, adrenal or renal vein were normal with no vascular malformation (b)

A final diagnosis of adrenal rupture leading to hemorrhagic shock was kept. Urine was found red colored upon bladder-catheterization. Twenty-four-hour urinary vanillylmandelic acid levels were normal, ruling out neuroblastoma. The patient gradually improved with medical management. During follow-up visit after 1 month, hematoma reduced in size, and the infant was growing well.

DISCUSSION

Spontaneous NAH is a relatively less common entity with an incidence ranging from 0.2% to 0.55% following trauma, birth asphyxia, or sepsis.[1,2,3] Peak incidence occurs within 1^st weeks after birth and is most commonly seen in term male infants.[3,4] It requires a high index of diagnostic suspicion and prompt management, else may lead to morbidity and mortality in a newborn.

The increased vulnerability of the adrenal glands to rupture is due to their unique anatomy and physiology at birth. As compared to adults, adrenals in neonates are approximately 10–20 times larger. At birth, the adrenals are one-third the size of the kidney, and later on in adults, it shrinks further. The adrenal cortex comprises dense network of arteries, which are sensitive and may rupture to pressure changes and trauma. Physiologic stress response at birth releases adrenocorticotropic hormone and catecholamines, leading to an increase in blood flow and may worsen the adrenal hemorrhage. The right adrenal is more susceptible to compression during difficult birth due to its anatomical position between the liver and spine and drains directly into the inferior vena cava. The common predisposing factors that may increase the risk of bleed include traumatic delivery, macrosomia, hypoxia, hypotension, coagulopathy, septicemia, shock, an underlying tumor, and thrombotic state.[3,4,5]

The clinical presentation can vary widely. Some common presentations include an asymptomatic newborn with an incidental finding detected on ultrasound, or lethargy, vomiting, jaundice, flank or abdominal mass, inguinal hematoma, acute scrotum, hematuria, or severe anemia requiring blood transfusion.[6,7,8,9,10,11,12] Involvement of bilateral adrenals is seen in 5%–10% cases.[13] When both adrenal glands bleed following rupture, it can lead to adrenal insufficiency, and dyselectrolytemia in addition to hypovolemic shock.[4,14] The index case presented with spontaneous ruptured right adrenal gland. It was severe enough to cause a massive hematoma, presenting as a renal lump, severe anemia, hemorrhagic shock, and hematuria.

Any newborn presents with a one-sided lumbar mass, it is crucial to consider other differential diagnoses such as congenital kidney anomalies and tumors.[15] These conditions are common and should be investigated accordingly. Imaging studies helped to exclude any renal disease. Imaging studies help to exclude any renal malformations. while normal urinary Vanillylmandelic acid levels rule out neuroblastoma.

Adrenal hemorrhage, on the other hand, involves bleeding into the adrenal gland, which may mimic WS; it is a surgical emergency and requires urgent intervention. The classic WS refers to spontaneous renal hemorrhage presenting as acute flank pain, mass, and hypovolemic shock, collectively known as Lenk’s triad, described by German physician Carl Reinhold August Wunderlich, caused by renal tumors and vascular causes.[16] In infants, WS can be caused by birth trauma, perinatal asphyxia, infection, or disseminated intravascular coagulation, renal vascular thrombosis, and bleeding disorders.

Ultrasonography with color Doppler is useful as the first imaging modality to distinguish hematoma from renal masses, and abdominal computed tomography may confirm the origin of the bleed. Doppler study aids in assessing blood flow and thus helps rule out renal vein thrombosis.[17]

NAH is mostly a self-limiting condition with a good prognosis.[3] Surgical indication is reserved for adrenal hemorrhage undifferentiated from cystic neuroblastoma with or without hemorrhage.[18] In patients where the conservative approach is failing, or not responding to transfusion due to catastrophic bleeding, vascular interventions such as transarterial embolization are recommended over exploratory laparotomy.[19] Sometimes, adrenal hemorrhage can complicate and lead to adrenal abscess formation, and delay in intervention may lead to death.[13,20]

Despite the turbulent clinical course, our patient responded well to prompt and aggressive fluid resuscitation, inotrope support, and blood product transfusion, ultimately leading to a favorable outcome.

CONCLUSION

This case expands the limited literature on severe NAH, shedding light on its clinical presentation and striking similarity to WS and more.

This case demonstrates that a ruptured adrenal gland on one side can precipitate torrential hemoabdomen, perirenal hematoma, and severe cardiorespiratory collapse. Timely intervention and aggressive support care are crucial in the management of adrenal hemorrhage in infants and can lead to a favorable outcome.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal patient identity, but anonymity cannot be guaranteed.

Conflicts of interest

There are no conflicts of interest.

Funding Statement

Nil.