Micropenis is an important sign in neonates, since it may be

the only clue to the diagnosis of panhypopituitarism, a potentially lethal but eminently treatable condition

Dezateux, CA

Leonard, JV

Salisbury, DM

Savage, MO

English

PubMed

UCL Discovery

BRITISH MEDICAL JOURNAL VOLUME 288 25 FEBRUARY 1984Lesson of the WeekMicropenis: an important early sign ofcongenitalhypopituitarismD M SALISBURY, J V LEONARD, C A DEZATEUX, M 0 SAVAGEMicropenis is an important sign in neonates, since it may bethe only clue to the diagnosis ofpanhypopituitarism, apotentiallylethal but eminently treatable condition.Case reportsCase 1-A boy infant was delivered at 37 weeks' gestation afteran uncomplicated pregnancy. Birth weight was 2200 g. He wasasphyxiated and successfully resuscitated but remained hypotonic.Jaundice developed on the third day of life and persisted. At 3 weeksofage hypothyroidism was diagnosed (plasma thyroxine concentration54 nmol/l (4-2 /ig/lOO ml), thyroid stimulating hormone concentration8-5 mU/l). Despite thyroxine replacement he failed to thrive anddeveloped abdominal distension and vomiting. Laparotomy showedmilk curd obstruction of the small intestine, and postoperatively hewas ventilated for three days. Twelve days later he collapsed and had ahypoglycaemic convulsion. For the next three months he was fedintravenously and became hypoglycaemic when attempts weremade to restart oral feeds. At the age of 4 months the diagnosis ofhypopituitarism was suggested because of his micropenis. During ahypoglycaemic episode (blood glucose concentration 10 mmol/l;18 mg/100 ml) the plasma cortisol value was noted to be low at100 nmoll1 (3-6 Ag/lOO ml) and concentrations of both plasmaadrenocorticotrophic hormone (93 ng/l) and growth hormone (7-0mU/l) were inappropriately low, consistent with hypopituitarism.The hypoglycaemia resolved rapidly with hydrocortisone replacementand did not recur. Growth hormone deficiency was confirmed by aglucagon stimulation test and appreciable penile growth occurred afterthree injections of depot testosterone. His jaundice, which had persis-ted despite thyroxine replacement, resolved after initiation of thehydrocortisone.Case 2-A boy infant (birth weight 3650 g) was delivered normallyafter an uncomplicated pregnancy. Shortly after birth he becamehypothermic and hypoglycaemic and micropenis was noted. Threedays after birth he became severely jaundiced with raised plasmatransaminase activities, which persisted. Plasma thyroxine concentra-tion was 16 nmol/1 (12 kg/lOO ml) without the appropriate thyroidstimulating hormone response (5-0 mU/l), indicating hypothyroidismof pituitary origin. Thyroid replacement was started but was in-adequate. At 3 weeks ofage he had several hypoglycaemic convulsions,and a normal blood glucose concentration could be maintained onlyHospital for Sick Children, Great Ormond Street, London WCIN3JHD M SALISBURY, MRCP, senior registrarJ V LEONARD, PHD, FRCP, senior lecturer in child healthQueen Elizabeth Hospital for Children, London E2 8PSC A DEZATEUX, MRCP, registrarM 0 SAVAGE, MD, MRCP, consultant paediatrician and endocrinologistCorrespondence to: Dr M 0 Savage.Prenatal gonadotrophin deficiency may resultin a small but normally formed peniswith 10% dextrose infusion. His condition deteriorated with hypo-thermia, hypoglycaemia, hypotension, and jaundice. A presumptivediagnosis of hypopituitarism was made and after hydrocortisone andfull thyroxine replacement his blood pressure and temperature rose.Hypoglycaemia was satisfactorily controlled only when humangrowth hormone (2 units daily) was also given. Pretreatment 9 amplasma cortisol concentration was 50 nmol/l (1-8 ug/100 ml). Plasmagrowth hormone value was low (0-5 mU/l) during a hypoglycaemicepisode and there was no luteinising hormone and follicle stimulatinghormone response to luteinising hormone releasing hormone. Thesefindings were consistent with multiple anterior pituitary hormonedeficiencies.CommentThe male external genitalia are formed as a result ofandrogenssecreted by the fetal testis under the influence of placentalhuman chorionic gonadotrophin. This process is completed bythe end of the first trimester of pregnancy.' After the 14th weekof gestation human chorionic gonadotrophin concentrationsfall and pituitary luteinising hormone is necessary to maintainfetal testosterone so that growth of the penis may continueuntil birth.2 Consequently prenatal gonadotrophin deficiencyof any cause may result in a small but normally formed penis-micropenis.Deficiencies of other anterior pituitary hormones may notbe obvious at birth but symptoms, notably collapse, cyanosis,and fits, may develop during the first 24 hours. These are notspecific and may be mistaken for other, more common disorders.Untreated congenital hypopituitarism, however, has a highearly mortality3 and micropenis may be the only clinicalindication for immediate investigation and treatment.4Whereas paediatricians recognise thesignificance ofvirilisationof female genitalia as occurs in congenital adrenal hyperplasia,the importance of underdevelopment of male external genitaliamay not be appreciated.5 In both cases reported here earlierinvestigation of pituitary function would have disclosed theneed for hormone replacement. Persistent neonatal jaundiceis a well recognised feature of thyroid deficiency, but it is notwidely known that cortisol deficiency may also be responsible.6In both patients the jaundice disappeared only once fullreplacement was given.Standards for stretched penile length in the newborn periodhave been established7 but in true micropenis examinationshows an obvious lack of erectile tissue, which may be a morevaluable sign than measurement of length. Micropenis is a621622 BRITISH MEDICAL JOURNAL VOLUME 288 25 FEBRUARY 1984clear pointer to possible pituitary deficiency and is an importantphysical sign in newborn boys.We thank Dr D Stone and Dr A J Franklin for permission toreport their patients. Plasma adrenocorticotrophic hormone waskindly measured by Professor Lesley H Rees in the department ofchemical endocrinology at St Bartholomew's Hospital, London.Other hormone assays were performed in the department of chemicalpathology at the Hospital for Sick Children, London.References'Wilson JD, Griffin JE, George FN, Leshin M. The role of gonadalsteroids in sexual differentiation. Recent Prog Horm Res 1981;37:1-39.' Clements JA, Reyes Fl, Winter JSD, Fairman C. Studies on humansexual development III. Fetal pituitary and serum, and amnioticfluid concentrations of LH CG and FSH. J7 Cln Endocrinol Mctab1976;42:9-19.Cornblatt M, Schwartz R. Hypoglycemia in the neonate. In: Disorder-s ofcarbohydrate metabolism in infanicy. 2nd ed. Philadelphia: W B SaundersCo Ltd, 1976:178.Lovinger RD, Kaplan SL, Grumbach MM. Congenital hypopituitarismassociated with neonatal hypoglycemia and microphallus: four casessecondary to hypothalamic hormone deficiencies. J Pediatr 1975;87:1171-81.Moncrief MW, Hill DS, Archer J, Arthur LJH. Congenital absence ofpituitary gland and adrenal hypoplasia. Arch Dis Child 1972;47:136-7.6 Leblanc A, Odievre M, Hadchouel M, Gendrel D, Chaussain JL,Rappaport R. Neonatal cholestasis and hypoglycemia: possible role ofcortisol deficiency. J Pediatr 1981 ;99:577-80.Flatau E, Josefsberg S, Reisner SH, Bialik 0, Laron Z. Penile size in thenewborn infant. 7 Pediatr 1975;87:663-4.(Accepted 11 August 1983)For Debate . .Paramedics in the United Kingdom?A D REDMONDThere is no doubt that until recently the United Kingdomcould boast the best ambulance service in the world. This isno longer the case. Standards have not fallen-indeed theyhave risen. The rest of the world, however, has caught up andin some places has overtaken us. These ambulancemen are nobetter trained than our own but are better equipped andallowed to do more. The obvious and best example to quote isthe ambulance system now developing in the United States ofAmerica.Twenty years ago there was no effective ambulance servicein the United States. In many places any vehicle of suitabledimension was used to transport a sick or injured patient tohospital. Ironically, the most suitable vehicle to hand wasusually the local hearse. It was no doubt difficult for themortician to separate the demands of his patient and thedemands of his profession. To overcome these problems manylocal communities developed a volunteer rescue squad. Theyfought fires, rescued trapped victims, and transported peopleto hospital. In an attempt to coordinate the many differentsquads, a programme of training was introduced in the early1970s. This is now widespread and very well organised. Theambulancemen are referred to as emergency medical technicians,or EMTs. The basic emergency medical technician has between80 and 140 hours of training, which covers basic anatomy andphysiology and simple first aid. This "simple first aid" does,however, include a thorough training in cardiopulmonaryresuscitation. This emphasis on control of the airway andbelief in the positive effects of early cardiopulmonary resuscita-tion is a major contribution to the saving of life. A basic emer-Stockport Infirmary, Stockport, CheshireA D REDMOND, MD, FRCSED, consultant in accident and emergencymedicineCorrespondence and requests for reprints to: Mr A D Redmond.gency medical technician can then do further training to becomean intermediate emergency medical technician. He has a further60 to 200 hours of training and will be able to insert intravenouslines, obturator airways, and apply a medical antishock trousersuit. The obturator airway seals off the oesophagus and willallow air to go down only the trachea. There are many problemswith this device including misplacement in the trachea andinducement of vomiting. It is rapidly losing favour in theUnited States. The medical antishock trouser suit is an in-flatable suit put over the legs of the patient in an effort toincrease peripheral resistance and thereby raise the blood pres-sure. It will also stabilise pelvic fractures. Having completedthis training the emergency medical technician can then electfor even further training. A cardiac emergency medical tech-nician can do all the above, but in addition can give drugs anddefibrillate patients. A paramedic emergency medical techniciandoes an extra 400 to 800 hours of training, and is then very welltrained, and can give help equivalent to that ofa trained physicianin emergencies. The paramedics can pass endotracheal tubes,insert central venous lines, and interpret electrocardiograms tothe envy of many a medical registrar.There are problems, however, with training technicians tosuch a high level. As their knowledge and expertise is so wideand their capabilities are so great the medical profession feel aneed to keep a close eye on their activities. Strict and lengthyrules are laid down for what they can do at the scene of anemergency, and usually any invasive action has to be precededby permission from the local hospital. In addition, therefore,to the colossal expense of training these people and equippingthem, there is the added expense of providing sophisticatedradiocommunications with the hospital. Furthermore, whensuch a highly trained paramedic arrives at the scene, his wholetraining and raison d'etre is to apply those skills that havebeen so hard won. Hence the main drawback with such a systemis delay. When the paramedic arrives at the scene he has firstofall to work through a check list. This includes taking a historyand taking the patient's blood pressure, pulse, and an electro-

Congenital absence of pituitary gland and adrenal hypoplasia.

Congenital hypopituitarism associated with neonatal hypoglycemia and microphallus: four cases secondary to hypothalamic hormone deficiencies.

Hypoglycemia in the neonate. In:

Neonatal cholestasis and hypoglycemia: possible role of cortisol deficiency.

Studies on human sexual development III. Fetal pituitary and serum, and amniotic fluid concentrations

The role of gonadal steroids in sexual differentiation. Recent Prog Horm Res

Micropenis: an important early sign of congenital hypopituitarism

Micropenis: an important early sign of congenital hypopituitarism

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