3 24
Clinical notes
2.
Crosby WH: Acute granulocytic leukemia, a complication of therapy in ltodgkin's disease, Clin Res 17:463, 1969. 3. Weiden PL, Lerner KG, Gerdes A, iteywood JD, Fefner A, and Thomas ED: Pancytopenia and leukemia in ttodgkin's disease: report of three cases, Blood 42:571, 1973. 4. Sahakian GJ, AI-Mondhiry H, Lacher M J, and Connolly CE: Acute leukemia in Hodgkin's disease, Cancer 33:1369, 1974. 5. Rosner F, and Grtinwald It: ttodgkin's disease and acute leukemia. Report of eight cases and review of the literature, Am J Med 58:339, 1975. 6. Rowley JD, Golomb ttM, and Vardiman J: Non random chromosomal abnormalities in acute nonlymphocytic leukemia in patients treated for ttodgkin disease and nonHodgkin lymphomas, Blood 50:759, 1977.
Ruptured ovarian cyst in a newborn infant Ovarian cysts of clinical significance are uncommon in the newborn infant. Rupture of such an ovarian cyst is exceedingly rare but constitutes one of the acute surgical emergencies of the neonate. The following is the fifth documented occurrence of a ruptured ovarian cyst in the neonate and stresses the importance of prompt diagnosis and immediate surgical intervention. CASE REPORT Patient L.L. was the 2,480 gm product of a complicated pregnancy. The 20-year-old primigravida mother entered a local hospital at 24 weeks" gestation with pyelonephritis requiring treatment with kanamycin. At 31 weeks, hypertension and pedal edema were noted, and treated with phenobarbital and bed rest. Labor was induced at 35 weeks because of persistent hypertension and hydramnios. The Apgar score was 6 at one minute and 8 at five minutes. On initial examination the infant appeared pale and had marked abdominal distension, measuring 30 cm in circumference. Within 30 minutes following delivery, respiratory distress was noted and the infant was referred to the Shands Teaching ttospital of the University of Florida. On arrival at our institution, the blood pressure was 80/60 mm Hg, heart rate 180/ minute, ~,nd respiratory rate 48/minute. There was i ~rked abdominal distension, without palpable masses. The hematocrit was 43 vol%. Abdominal radiograms revealed complete opacification with outline of the lateral border of the liver, providing evidence of neonatal ascites. Both diaphragms were markedly elevated. Two hours following delivery, the hematocrit had fallen to 35 vol% and an abdominal paracentesis was performed, which revealed grossly bloody fluid. After stabilization with intravenous crystalloid therapy, the infant was taken to the operating room with a presumptive diagnosis of hemoperitoneum secondary to neonatal birth trauma to the liver or spleen. At laparotomy, approximately 150 ml of bloody.fluid and clots were encountered. The liver and spleen were found to be entirely normal and, on examining the pelvis, a large ruptured left ovarian cyst was noted to be bleeding from its edge. The cyst measured 10 • 10 cm; no normal ovarian tissue could be identified within the wall. The right ovary and fallopian tube were normal, as were the
0022-3476/78/0293-0324S00.20/0 9 1978 The C. V. Mosby Co.
The Jolwnal of Pediatrics August 1978
uterus and left tube. A left oophorectomy ",,,'as performed uneventfully and the patient was discharged on the sixth postoperative day. Examination of the resected specimen revealed a thin-walled, simple ovarian cyst with focal luteinization and hemorrhage within the wall; no normal ovarian tissue was identified. DISCUSSION Small ovarian cysts of the newborn infant are common. DeSa' reviewed 332 stillbirths and neonatal deaths within 28 days of birth, and found small follicular cysts in 113 (34%) of these infants. Sixty-five of these cysts were single and 48 ",,,'ere multiple, with multiple cysts appearing more commonly in infants of low birth weight. The etiology of these cysts is thought to be excessive stimulation of the fetal ovary from placental gonadotrop!ns; this would explain the more common occurrence of cysts in infants of diabetic mothers, who tend to have larger placentas. Most of these small cysts involute within the first few months of life and are of no clinical consequence. Occasionally, however, large ovarian cysts occur, presenting as abdominal masses in the newborn period. These cysts may be large enough to cause respiratory distress and may undergo torsion on long Fallopian pedicles. The complication of rupture, however, is extraordinarily rare. Beale, 2 in 1891, first described a ruptured ovarian cyst in a newborn infant who died of peritonitis and had a pelvic abscess and a small ruptured right ovarian cyst. Tietz and Davis) in 1957, frst successfully resected a large cyst in an l l-hour-old infant born with progressive abdominal distension and respiratory distress. Mainolfi et al ~first pointed out the association of hydramnios in discussing the resection of a ruptured cyst in a 3-hour-old infant; the contralateral ovary was noted to have several small cysts which were left untreated. Four and a half months later, while repairing a left inguinal hernia in this patient, the authors noted that left ovary to be entirely normal. Progressive ascites in the neonatal period usually suggests urinary extravasation from congenital obstructions; less common causes include.birth trauma to the spleen or liver, or perforated intestine secondary to volvulus. Massive abdominal distension caused by ascites can be the cause of significant respiratory distiess. Differentiation of the causes of neonatal ascites may be expedited by early abdominal paracentesis; the finding of bloody fluid is indication for immediate abdominal exploration. Robert Monson, M.D. General Surger)' Resident Bradle)" M. Rodgers, M.D. Associate Professor of Surger)' and Pediatrics Robert M. Nelson, M.D. Assistant Professor of Pediatrics Thomas K. Young, M.D., P.A. J.II.M. llealth Center Gainesrille, FL 32610 REFERENCES
I.
DeSa DJ: Follicular ovarian cysts in stillbirths and neonates, Arch Dis Child 50:45, 1975.
Vohtme 93 Number 2
2. 3. 4.
Clinical notes
325
I
Beale GB: Fatal rupture o f a n ovarian cyst in an infant, Br Med J 2:1255, 1891. Tietz KG, and Davis JB: Ruptured ovarian cyst in a newborn infant, J PEOIX'rR 51:564, 1957. Mainolfi FG, Standiford JWE, and Hubbard TB: Ruptured ovarian cyst in the newborn, J Pediatr Surg 3:612, 1968.
Meralgia paraesthetica in a c/tiM Meralgia paraesthetica, a mononeuropathy o f the lateral cutaneous nerve of the thigh, is a common entity in middle age. Although it does occur in children, attention is not given to it in pediatric or neuropediatric texts. CASE REPORT A 7-year-old white boy began to notice that his left thigh was "asleep" all the time. tie could recall no recent trauma to the area and his mother found no bruises. There was no history of systemic illness or of other symptoms. On examination there was sensory loss to pinprick on the left anterolateral thigh in an area approximately 6 • 10 cm; hypalgesia was present in a slightly larger area. There was no tenderness to deep palpation in the thigh or pelvis areas. General and neurologie examinations were otherwise normal. Complete blood count was normal. Mononucleosis spot test was negative. Radiographs o f the hip were negative. Three months later, the child had no pain or objective anesthesia; slight sensitivity to touch persisted in the lateral thigh area. Motor activity was entirely normal. DISCUSSION Meralgia paraesthetiea has been reported in two 15-year-old patients,' a 20-year-old adult who had noticed it since age ten," a 10-year-old, 3 a 9-year-old, ~ and an l l-year-old child whose opposite side was involved at age five.~ Many etiologies have been suggested for this entity, including trauma secondary to mechanical causes (belts, braces, trusses, striking the ilium in accidents); intrapelvic disease; herpes zoster and Epstein-Barr virus infection; a congenitally tight foramen through which the nerve passes the inguinal ligament; obesity; pregnancy. Pediatricians may see cutaneous sensory loss in children in the anterolateral thigh from intramuscular injections. The lateral femoral cuta,neous nerve arises from the dorsal branches o f the second and third lumbar roots. After its intrapelvie course, the nerve passes through an opening medial to the anterior superior iliac spine, under the inguinal ligament. It is in apposition to the bone and the sartorius muscle and continues into the subcutaneous tissues of the thigh, dividing into a posterior branch that pierces the fascia lata and supplies the skin from the level of the greater trochanter to the middle of the thigh, and an anterior branch that passes through the fascia lata to supply the lateral and anterior parts o f the thigh as far :Is the knee. Opinions expressed are those o f the attthors and not the US Na 9"
Anterior view
Lateral thigh
Figure. Stippled area o f the thigh where sensation is supplied by the lateral femoral cutaneous nerve. Sensor)" loss in meralgia paraesthetica may occur in only part of this distribution. Since this is a purely sensory nerve, symptoms are numbness, burning, itching, and sometimes pain over the anterior and lateral aspect o f the thigh. Sensitivity to cutaneous stimuli (clothing and stockings) may be increased. The area o f dysesthesia may be relatively large but is often much smaller than t h e nerve distribution. Bilateral symptoms occur in 20'70 of the patients. In addition to an area o f hypesthesia or hypalgesia that is much smaller than the area o f dysesthesia, approximately 80 to 90% of patients have tenderness over the inguinal ligament. If an). ,her neurologic symptom or deficit is elicited (motor, reflex, or other sensory loss), it should prompt vigorous diagnostic workup. If a history of trauma is not present and symptoms and signs are limited to the distribution o f the lateral cutaneous nerve, observation is best. Treatment o f meralgia has included local lidocaine or steroid injections, neurolysis or section of the nerve, nerve transplani, exercises or weight loss for obese patients, and medical therapy with phenytoin, carbamazepine, amitriptyline hydrochloride, or medication for pain. Since most cases are self-limiting or sporadic, short-term therapy is desired. Complaints may persist for )ears but the course is benign. E. ll'a).ne Masse), M.D. John M. Pellock, M.D. Neurology Department National Naval Medical Center Bethesda, MD 20014
Clinical notes
2.
Crosby WH: Acute granulocytic leukemia, a complication of therapy in ltodgkin's disease, Clin Res 17:463, 1969. 3. Weiden PL, Lerner KG, Gerdes A, iteywood JD, Fefner A, and Thomas ED: Pancytopenia and leukemia in ttodgkin's disease: report of three cases, Blood 42:571, 1973. 4. Sahakian GJ, AI-Mondhiry H, Lacher M J, and Connolly CE: Acute leukemia in Hodgkin's disease, Cancer 33:1369, 1974. 5. Rosner F, and Grtinwald It: ttodgkin's disease and acute leukemia. Report of eight cases and review of the literature, Am J Med 58:339, 1975. 6. Rowley JD, Golomb ttM, and Vardiman J: Non random chromosomal abnormalities in acute nonlymphocytic leukemia in patients treated for ttodgkin disease and nonHodgkin lymphomas, Blood 50:759, 1977.
Ruptured ovarian cyst in a newborn infant Ovarian cysts of clinical significance are uncommon in the newborn infant. Rupture of such an ovarian cyst is exceedingly rare but constitutes one of the acute surgical emergencies of the neonate. The following is the fifth documented occurrence of a ruptured ovarian cyst in the neonate and stresses the importance of prompt diagnosis and immediate surgical intervention. CASE REPORT Patient L.L. was the 2,480 gm product of a complicated pregnancy. The 20-year-old primigravida mother entered a local hospital at 24 weeks" gestation with pyelonephritis requiring treatment with kanamycin. At 31 weeks, hypertension and pedal edema were noted, and treated with phenobarbital and bed rest. Labor was induced at 35 weeks because of persistent hypertension and hydramnios. The Apgar score was 6 at one minute and 8 at five minutes. On initial examination the infant appeared pale and had marked abdominal distension, measuring 30 cm in circumference. Within 30 minutes following delivery, respiratory distress was noted and the infant was referred to the Shands Teaching ttospital of the University of Florida. On arrival at our institution, the blood pressure was 80/60 mm Hg, heart rate 180/ minute, ~,nd respiratory rate 48/minute. There was i ~rked abdominal distension, without palpable masses. The hematocrit was 43 vol%. Abdominal radiograms revealed complete opacification with outline of the lateral border of the liver, providing evidence of neonatal ascites. Both diaphragms were markedly elevated. Two hours following delivery, the hematocrit had fallen to 35 vol% and an abdominal paracentesis was performed, which revealed grossly bloody fluid. After stabilization with intravenous crystalloid therapy, the infant was taken to the operating room with a presumptive diagnosis of hemoperitoneum secondary to neonatal birth trauma to the liver or spleen. At laparotomy, approximately 150 ml of bloody.fluid and clots were encountered. The liver and spleen were found to be entirely normal and, on examining the pelvis, a large ruptured left ovarian cyst was noted to be bleeding from its edge. The cyst measured 10 • 10 cm; no normal ovarian tissue could be identified within the wall. The right ovary and fallopian tube were normal, as were the
0022-3476/78/0293-0324S00.20/0 9 1978 The C. V. Mosby Co.
The Jolwnal of Pediatrics August 1978
uterus and left tube. A left oophorectomy ",,,'as performed uneventfully and the patient was discharged on the sixth postoperative day. Examination of the resected specimen revealed a thin-walled, simple ovarian cyst with focal luteinization and hemorrhage within the wall; no normal ovarian tissue was identified. DISCUSSION Small ovarian cysts of the newborn infant are common. DeSa' reviewed 332 stillbirths and neonatal deaths within 28 days of birth, and found small follicular cysts in 113 (34%) of these infants. Sixty-five of these cysts were single and 48 ",,,'ere multiple, with multiple cysts appearing more commonly in infants of low birth weight. The etiology of these cysts is thought to be excessive stimulation of the fetal ovary from placental gonadotrop!ns; this would explain the more common occurrence of cysts in infants of diabetic mothers, who tend to have larger placentas. Most of these small cysts involute within the first few months of life and are of no clinical consequence. Occasionally, however, large ovarian cysts occur, presenting as abdominal masses in the newborn period. These cysts may be large enough to cause respiratory distress and may undergo torsion on long Fallopian pedicles. The complication of rupture, however, is extraordinarily rare. Beale, 2 in 1891, first described a ruptured ovarian cyst in a newborn infant who died of peritonitis and had a pelvic abscess and a small ruptured right ovarian cyst. Tietz and Davis) in 1957, frst successfully resected a large cyst in an l l-hour-old infant born with progressive abdominal distension and respiratory distress. Mainolfi et al ~first pointed out the association of hydramnios in discussing the resection of a ruptured cyst in a 3-hour-old infant; the contralateral ovary was noted to have several small cysts which were left untreated. Four and a half months later, while repairing a left inguinal hernia in this patient, the authors noted that left ovary to be entirely normal. Progressive ascites in the neonatal period usually suggests urinary extravasation from congenital obstructions; less common causes include.birth trauma to the spleen or liver, or perforated intestine secondary to volvulus. Massive abdominal distension caused by ascites can be the cause of significant respiratory distiess. Differentiation of the causes of neonatal ascites may be expedited by early abdominal paracentesis; the finding of bloody fluid is indication for immediate abdominal exploration. Robert Monson, M.D. General Surger)' Resident Bradle)" M. Rodgers, M.D. Associate Professor of Surger)' and Pediatrics Robert M. Nelson, M.D. Assistant Professor of Pediatrics Thomas K. Young, M.D., P.A. J.II.M. llealth Center Gainesrille, FL 32610 REFERENCES
I.
DeSa DJ: Follicular ovarian cysts in stillbirths and neonates, Arch Dis Child 50:45, 1975.
Vohtme 93 Number 2
2. 3. 4.
Clinical notes
325
I
Beale GB: Fatal rupture o f a n ovarian cyst in an infant, Br Med J 2:1255, 1891. Tietz KG, and Davis JB: Ruptured ovarian cyst in a newborn infant, J PEOIX'rR 51:564, 1957. Mainolfi FG, Standiford JWE, and Hubbard TB: Ruptured ovarian cyst in the newborn, J Pediatr Surg 3:612, 1968.
Meralgia paraesthetica in a c/tiM Meralgia paraesthetica, a mononeuropathy o f the lateral cutaneous nerve of the thigh, is a common entity in middle age. Although it does occur in children, attention is not given to it in pediatric or neuropediatric texts. CASE REPORT A 7-year-old white boy began to notice that his left thigh was "asleep" all the time. tie could recall no recent trauma to the area and his mother found no bruises. There was no history of systemic illness or of other symptoms. On examination there was sensory loss to pinprick on the left anterolateral thigh in an area approximately 6 • 10 cm; hypalgesia was present in a slightly larger area. There was no tenderness to deep palpation in the thigh or pelvis areas. General and neurologie examinations were otherwise normal. Complete blood count was normal. Mononucleosis spot test was negative. Radiographs o f the hip were negative. Three months later, the child had no pain or objective anesthesia; slight sensitivity to touch persisted in the lateral thigh area. Motor activity was entirely normal. DISCUSSION Meralgia paraesthetiea has been reported in two 15-year-old patients,' a 20-year-old adult who had noticed it since age ten," a 10-year-old, 3 a 9-year-old, ~ and an l l-year-old child whose opposite side was involved at age five.~ Many etiologies have been suggested for this entity, including trauma secondary to mechanical causes (belts, braces, trusses, striking the ilium in accidents); intrapelvic disease; herpes zoster and Epstein-Barr virus infection; a congenitally tight foramen through which the nerve passes the inguinal ligament; obesity; pregnancy. Pediatricians may see cutaneous sensory loss in children in the anterolateral thigh from intramuscular injections. The lateral femoral cuta,neous nerve arises from the dorsal branches o f the second and third lumbar roots. After its intrapelvie course, the nerve passes through an opening medial to the anterior superior iliac spine, under the inguinal ligament. It is in apposition to the bone and the sartorius muscle and continues into the subcutaneous tissues of the thigh, dividing into a posterior branch that pierces the fascia lata and supplies the skin from the level of the greater trochanter to the middle of the thigh, and an anterior branch that passes through the fascia lata to supply the lateral and anterior parts o f the thigh as far :Is the knee. Opinions expressed are those o f the attthors and not the US Na 9"
Anterior view
Lateral thigh
Figure. Stippled area o f the thigh where sensation is supplied by the lateral femoral cutaneous nerve. Sensor)" loss in meralgia paraesthetica may occur in only part of this distribution. Since this is a purely sensory nerve, symptoms are numbness, burning, itching, and sometimes pain over the anterior and lateral aspect o f the thigh. Sensitivity to cutaneous stimuli (clothing and stockings) may be increased. The area o f dysesthesia may be relatively large but is often much smaller than t h e nerve distribution. Bilateral symptoms occur in 20'70 of the patients. In addition to an area o f hypesthesia or hypalgesia that is much smaller than the area o f dysesthesia, approximately 80 to 90% of patients have tenderness over the inguinal ligament. If an). ,her neurologic symptom or deficit is elicited (motor, reflex, or other sensory loss), it should prompt vigorous diagnostic workup. If a history of trauma is not present and symptoms and signs are limited to the distribution o f the lateral cutaneous nerve, observation is best. Treatment o f meralgia has included local lidocaine or steroid injections, neurolysis or section of the nerve, nerve transplani, exercises or weight loss for obese patients, and medical therapy with phenytoin, carbamazepine, amitriptyline hydrochloride, or medication for pain. Since most cases are self-limiting or sporadic, short-term therapy is desired. Complaints may persist for )ears but the course is benign. E. ll'a).ne Masse), M.D. John M. Pellock, M.D. Neurology Department National Naval Medical Center Bethesda, MD 20014
